Archive

10/09/2004 T+366

Happy One Year BMT anniversary 

A year ago today Sophie was re-born. The kindness of a stranger enabled her to have chance of a full life. While we watched this simple, amazing procedure we held our breaths and prayed. What followed was a rollorcoaster of a ride, she survived Grade 3 GVHD, a cardiac and respitory arrest, adenovirus and a fungal infection in her lungs . In April 2004 she was back in PICU following a febrile convulsion. In August 2004 she was "signed off" by the BMT team.  

As I watched her today I marvelled at how much progress she has made. Her smiles fills our life with sunshine and her joy for life is a constant reminder of how lucky we are. Sophie you are our miracle baby and we love you.

Love Tina

08/09/2004 T+364

Sophie continues to do well and is doing more and more physically each day. She can now pull herself up to standing from sitting and is able to climb the first few steps on the stairs (it's time to get the stair gate back out). She is still a bit wobbly when standing up but she only needs to hold on with one hand. She is very busy about the house opening drawers and banging the doors. She is delighted that she can now "chase" Amy about the house.

We got a call from Great Ormond Street last week and Sophie is going in for her back surgery next Tuesday (14th September). She will be admitted on Tuesday and have the surgery on Wednesday. She may need to spend a few days in PICU after the operation. She should be in hospital for about 7-10 days which should be a walk in the park compared to her last 5 month stay. While we are very pleased that the surgery is taking place so soon we are getting very nervous about it. If all goes well she may even be out of her back brace by Christmas.

It was Amy's birthday on Saturday and she had a wonderful day. Sophie's and Amy's friends Zoe and Joy came up from Devon for Amy's party and they all had great fun. Sophie particularly enjoyed the ball pit. We went to Legoland on Monday and had a great day out. There is just so much to do there, I think next year we will have to buy an annual family ticket. This year Legoland's chosen charity is Great Ormond Street hospital so there were collections being made throughout the park to support this wonderful hospital.

This Friday Sophie will be one post transplant. We will post another update on Friday for Sophie's first "re-birthday".

Love

Padraic

30/08/2004 T+355

We all had a wonderful time in Dublin. It was lovely to see Padraic's parents and they saw great improvements in Sophie, or to give her full name "Sophie what do you think you are doing?" We had some lovely daytrips and even managed to get the beach. Sophie loves the seaside and spent the whole time laughing. 

The day before we travelled Sophie had her pre-op assessment. As usual she was a complete star and underwent all the tests with great humour. They are happy to proceed with the operation.  She is scheduled to come in late October at Great Ormond Street. She is having two rods put in her back to straighten her spine. As the curvature is a small part of her lower spine it should not affect her growth. The operation usually takes between 3-4 hours and there is a 60% chance she will need on be on the vent for a little while to help with her breathing. Should she only be in hospital for 7 to 10 days.  

On Friday a year ago today we were admitted to Fox ward and began our long BMT journey. Many memories have been flooding back some awful and some wonderful.  The friends we have made, the children and their stories. We are coming to the end of our BMT journey but we will never forget this year, it has changed us completely.

I have added some new pictures from Dublin.

Love Tina 

18/08/2004 T+343

Today was a landmark in Sophie's life. We went to the BMT clinic and as before they are very happy with her progress. It is almost a year since Sophie was transplanted and she is 100% engrafted and off all BMT medication (with the exception of Penicillin which all the children take following transplant). The consultant started to tell us that Sophie's needs were now no longer BMT related and I could feel the tears starting to flow. We have been signed off the BMT team back to the metabolic team who will deal Sophie's needs from her Hurler's syndrome. We met lots of Sophie's friends on Fox ward and it was really great to see them all again and to share the good news. They are all amazed at Sophie's hair and how well she looks. 

We can never say enough good things about the BMT team at Great Ormond Street Hospital. We owe Sophie's life to them, she has on-going medical problems but as long as we wake up to her smile then life is great. We so grateful to them all, thank you really is never enough.

On Friday Sophie has her pre-op assessment for the back surgery. She is having a several X-rays, lung functions tests, blood tests and an ECG. She will also be assessed by the anaesthetist, respiratory and cardiac specialists. She will also be taken for medical photographs, I can not get the mental picture of her sitting with all the Doc' s having her photos taken out of my head. 

We are all off to Dublin on Saturday to spend some time with Padraic's Parents. You might hear the singing where you are!!!

Love Padraic + Tina

PS I have added some new pictures for August 

11/08/2004 T+336

Today has been a good day we went to the Cardiac Day Care today and Sophie began with a repeat echo. After an agonising wait the Cardiac consultant told as that Sophie heart function has improved. She still has Dilated Cardiomyopathy but the degree is in keeping with her mild mitral regurgitation, some blood is pumping back into her heart. He believes that the problems last week was caused by a virus. Sophie has had a temperature for the last few days and started antibiotics for a throat infection, he agreed that this was a probable cause. We still started a new drug Captopril which should help with her heart function and we will be seen in Cardiac clinic in three months.  Padraic and I are so relieved, ok she has a heart problem but if there is no more decline she should be able to lead a active life. There is no reason why it should decline in fact with this drug it could even improve. 

So once again we hold Sophie in our arms looking forward to a wonderful future. 

Love Tina

05/08/2004 T+330

We had a BMT clinic appointment yesterday at which we hoped Sophie could be signed off. How wrong we were, what should have been a joyous day turned out to be a roller-coaster of emotions. Sophie was weighed and her blood pressure taken both of which were fine, (she now weighs 12.2kg up from 9.9kg when she was discharged in January). Sophie then had an echo on heart and we were told to wait to see the doctor afterwards, which was unusual. The cardiologist told us that Sophie had a condition called Dilated Cardiomyopathy which is a disorder affecting the muscle of the heart. Basically, Sophie's heart is enlarged and is not working as efficiently as it should do. We were told that if it gets worse the outlook would be very poor. We were devastated, we thought that this was condition that Sophie would only get if she didn't have a transplant and we said this to the cardiologist. He said that it could also be caused by a virus which would be the best case scenario as it would then not get any worse, however he had spoken to the cardiology consultant and the consultant thought that the Hurlers was the most likely cause. We arranged to go back to Great Ormond Street next Wednesday so that Sophie can start a drug treatment for the condition. 

We then went back to the clinic and saw the BMT consultant. We discussed Sophie's progress from a BMT point of view and he was extremely pleased with her progress. He then talked about the Cardiomyopathy and he was as bemused as we were that it could be caused by the Hurlers. Sophie has been 100% engrafted throughout her transplant so we would not expect her to have heart problems related to her Hurlers. Also, Sophie had an echo when she was in Intensive care in April and there was no sign of any damage. While the Hurler's cannot be ruled out as a cause he said that he thought that a virus was the most likely cause. He will be discussing the echo results with the cardiologist this Friday and the echo will be repeated next Wednesday to look for any changes in the condition. If the condition has been caused by a virus the outlook is a lot better as the heart will stay as it is and Sophie should not be too badly affected. Please pray for a virus. Sophie was then a very brave little girl at the end of a very long day while the doctor took a large number of blood samples for virus checking. The doctor said she was a little star and I agreed.

Apart from this Sophie is doing wonderfully well and is causing havoc around the house. This morning Amy came into the kitchen sat down at the table and asked if I would sit down. I did and we had the following conversation...

AMY:  "Mummy when I was two I could walk but I still didn't know what was naughty. Well, Sophie is two and she doesn't know what is naughty"  

ME:  "Has Sophie done something naughty?"

AMY: "Well.. yes..but she is only two"

I went into the playroom and Sophie had pulled the digital TV box off the cabinet onto the floor ! Padraic and I are secretly happy by her escapades and it seems Amy is as well.

Love Tina

02/08/2004 T+327

Firstly I have to apologise for not updating sooner, we have a problem with our internet connection so I have had to dust down the old dial-up modem to get back on line. Sophie had her MRI scan on Wednesday and it all went well. She was a little out of sorts for an hour or so afterwards but she was quickly back to her old self. I will be chasing up the results this week to find out when her next appointment will be. The next stage should be a pre-operation assessment prior to surgery. Sophie had a cold a few days before the MRI scan, so we were worried we would have to cancel again. Luckily her immune system is obviously getting better as it has almost totally gone by the time we went in.  

Last Sunday week we went to some friends for a barbeque (thanks Alison Peter, Ellie and Stacey for a lovely day). Just before we left Sophie crawled for the first time...hooray for Sophie...She isn't very interested in her toys at the moment she just wants to practice getting around either by sliding on her bottom on the laminate floor in the playroom or shuffling along on the carpet. I don't know how she does it but she has a sixth sense for finding baby wipes, every time I leave the room she manages to get hold of a packet which I never knew we had and has usually emptied it by the time I get back. Sophie is getting very good at standing and now lets go with her hands as soon as she is upright. Unfortunately she hasn't learnt how to sit down gracefully so it normally ends with a bump !!

When we went for the MRI scan we bumped into a few old friends who were in for BMT clinic appointments. It was nice to catch up with everyone. Some are doing well, some not so well so we thank our lucky stars for Sophie's continued good health. Please say a little prayer for our friend Tashan who is having a rough time, he is over a year post transplant but had to be readmitted as his blood counts were low and he needed transfusions. Fran, Tashan's Mum has been a wonderful support to us through the transplant and we hope Tashan can get over this latest hurdle.  Prayers please for another friend of Sophie's, Oscar, he is back in hospital with a bad reflux problem. This is totally unrelated to both his condition and his transplant and is so unfair. We pray that Alex who recently had his second transplant gets a little GVHD to kick out all the nasty old cells and that another friend Matthew continues to do well. Matthew's brother had a transplant a few years ago and had a very rough time although he is now doing well. I was chatting to Matthew's Dad and we were commenting on how nice it was to be able to do the normal things in life again, like taking the whole family going shopping or going out for a meal together. All the things we previously took for granted. On Wednesday we have a clinic appointment which will be Sophie's 1 year post transplant. It is a little early but we want to "clear the decks" for the back surgery. If it all goes well we may be signed off the BMT team back to the metabolic team...fingers crossed.

Sophie's newest friend Caterina will be having her transplant on Friday 6th August. Her website is on Sophie's friends page, please check in on her and sign her guestbook, your support really helps.

Padraic 

18/07/2004 T+313

Well, after a few phone calls we have a new date of Wednesday 28th July for Sophie's MRI scan. Hopefully there will be no more complications. Sophie had another good week and her feeding is going so well that we have stopped Sophie's overnight feed. She is eating more and more each day and trying lots of different types of food. We are still working on her drinking but still have a little way to go before we can think about removing the NG tube. I had a lovely trip to the supermarket with Sophie this week, she sat up in the shopping trolley and was so pleased with herself. She spent the whole time bouncing in her seat and looking around for people to "chat" to. It was such a normal thing to do but it was the first time I had taken Sophie shopping since the transplant so it made my day. Sophie had her blood tests on Wednesday and the nurses are amazed at Sophie's hair and how much it has grown, I think it is developing a life of it's own. While we are on the subject of hair, the hair on Sophie's body has almost all gone.  

Physically Sophie is doing more and more each day. She is soooo.... determined to get moving, she is now able to bear weight on her legs and pulls herself to standing up each time her hands are held. It has been a long road but we are seeing so much progress that hopefully it is only a matter of time before she is fully mobile. 

I have added some new pictures for July.

Padraic

12/07/2004 T+306

Sophie's MRI scan on Saturday was cancelled. I got a phone call on Friday afternoon, the anaesthetist would not give Sophie a GA on a Saturday. After looking at her notes he says it to risky at the weekend. So we will have to wait for another cancellation. We want this MRI to happen as soon as possible but if the anaesthetist it not happy to do a GA at that time we have to accept that this was the best decision. We are a little concerned because we were told that the anaesthetist would also not be happy to do a GA on a Friday, but our fall back appointment is on a Friday. We are trying to find the right person to talk to today with no luck, we will continue tomorrow. 

On Wednesday Sophie and I went to our first therapy group. Sophie did really well and had a lovely time. She was a little quiet but she participated for the whole time and interacted with the therapists and other children. There was a lovely moment where she held a little girl's hand and stroked her hair.  

Sophie is really doing well everyday eating a little more, doing a little more. I talked to the dietician today and we are going to wean her off her overnight feed.  She can now get from laying down to sitting up , making bedtime interesting. We put her to bed and then Padraic and I sit outside her room on the floor  watching her working hard sitting up, attempting to pull herself up on cots sides and generally moving around. We just sit and smile at each other there no need for words.

Love Tina

06/07/2004 T+300

Wow, it's 300 days since Sophie's transplant, it all seems a world away now. Sophie is doing well, her feeding is getting better and judging by the amount of weight she seems to be gaining we will have to discuss reducing her feed soon. We had a lovely weekend and managed to get out for a meal together on Saturday. It is the first time we have all been to a restaurant since last August and we had a wonderful time. 

Sophie was in top form and she really enjoyed herself. We got a call this morning and Sophie is going to have a MRI scan on her back on Saturday as there has been a cancellation. We are really pleased about this as we are very worried about Sophie's back and want her to have the operation as soon as possible. Physically, Sophie is getting much stronger and is now able to push herself up using her arms. She tries to stand up whenever she is lifted up and never sits or lies still for a moment. So overall things are going very well although it would be nice if Sophie didn't start her day at 5:00am; still it gives me an hour to play with her before I go to work so I am not complaining. 

In all of this we try to remain positive, to accept Sophie's problems and to enjoy all the things she can do but sometimes something hits you like a ton of bricks. Yesterday, Amy was singing "I can sing a rainbow" and we suddenly realised that Sophie will never be able to see a rainbow, sometimes it can be so hard but we will pick ourselves up and carry on and remember all the love Sophie has be shown from so many people.

Following last week's good news about enzyme replacement crossing the blood-brain barrier I was angered to read in the latest copy of the MPS society magazine that Enzyme Replacement Therapy for children with Hurlers syndrome has been rejected in Scotland and Wales. It also transpires that not one child in the UK has been able to receive this treatment without first taking their local health trust to court. We should not have to fight for this treatment, it has been approved in so many countries around the world including America, Norway and just recently The Netherlands. I firmly believe that it is been rejected on the basis of cost. There are other enzyme treatments which have been improved in the UK for other conditions so why not for Hurlers. In fact why did the UK bother to endorse the treatment at European level if there was never any intention of providing it. How can a treatment be rejected if it can help a child to walk and have a little independence in their lives ? Sophie has been through so much in her short life and we will fight tooth and nail for her to have this treatment if it can be of benefit to her.  

I will get off my high horse now and finish by saying that Sophie's newest friend Caterina will be undergoing a transplant soon. I have added her to Sophie's friends page, please check in on her and say hello, she is adorable.

Padraic 

 29/06/2004 T+293 

Our big news is Sophie is mobile. I am not quite sure how she is doing it, sheer will power and determination I think. She is also getting onto her tummy and rolling back onto her back. I have been putting toys around the room for her to explore, of course she is ignoring them and playing with all the wires and getting stuck under chairs. It is so wonderful to see her exploring her world and having the independence to go where she wants to go.

We have had some lovely weekends. Our friends Jan and Pat with their girls Zoe and Joy came to stay. It was lovely to see Sophie interacting with other children and Zoe and Sophie started a mutual fan club. For Sunday lunch more friends, Elaine, Tony, Anne and Ben come and Sophie loved all the extra cuddles. She is so sociable, she loves being with people. The next weekend Padraic's  parents come over to stay at Padraic's brother's family for our niece Elizabeth first Holy Communion and we went over for a visit. Everyone was thrilled with how well Sophie was doing and we had a lovely time. It is so nice just to do normal things.  

Padraic learnt how to insert the NG tube last week. It means no more late night visits to the local hospital. I am so proud of him and I certainly could not do it. 

We had some wonderful news the about the fight against the MPS we wanted to share. It was posted on the MPS Forum. The website it refers to is Ryan Foundation.  

THE RYAN FOUNDATION CELEBRATES ANOTHER SCIENCE BREAKTHROUGH ON MPS RESEARCH! Breakthrough Will Be Celebrated at the 2004 Ryan Foundation Gala and Will Mark the End of Major Fundraisers by the Foundation. June 2004
Huge news in the world of MPS research has just recently been broken thanks once again to the incredible work at the Kakkis Lab at UCLA and its’ dedicated employees. The Ryan Foundation has been helping fund three major projects at the Kakkis Lab since the mid 1990’s. The first project was to develop a treatment that can be given to children suffering through the pain of MPS I to treat the disease in the body of the patients. The second project has been to develop a method for immune tolerance to keep the body from rejecting the new treatment drug Aldurazyme, which received FDA approval last year. The third project has been to develop a way to treat brain disease in MPS I. Project number one was completed in 1998 with the beginning of the FDA trial to approve Aldurazyme. The treatment works quite well on treating the body, as the Ryan Foundation web site attests (see Ryan Update page.) Now approved in the US and Europe, thousands of MPS I patients are now or will be soon finding out first hand just how well the new drug works. With regards to project number two, data developed at the Kakkis Lab was released in the fourth quarter of 2003 regarding their recent success in developing a method of preventing rejection. The data was in fact so overwhelming that it has already been published in the January issue of the Proceedings of the National Academy of Sciences of the USA. Two projects down, one to go! Treating the brain for children with MPS I to date has not been possible because of what scientists refer to as the Blood Brain Barrier (BBB), or the bodies mechanism for keeping foreign substances from the brain. The treatment drug called Aldurazyme, is administered to Ryan and many other children around the world in weekly IV infusions and works astonishingly well on the bodies of MPS I patients. However, when administered through the blood in the standard IV method, the drug is not able to cross the Blood Brain Barrier (BBB) and therefore does not treat the brain. Over time, an untreated brain in children with MPS I will most likely result in mental regression. Children also experience severe spinal cord compression, which usually results in paralysis. Recent laboratory data provided by the Kakkis Lab shows that tiny amounts of Aldurazyme when delivered in intrathecal injections (IT Injections are similar to a spinal tap) not only crosses the Blood Brain Barrier, but shows that it normalizes glycosaminoglycan storage in the brain, the material which causes brain damage in MPS I patients. The goal is that the IT injections will be needed only twice annually. In addition, the IT treatment has been shown to dramatically reduce spinal cord compression. Look for the data from this work to be published soon in the scientific journal, Molecular Genetics and Metabolism.

Can we begin to hope for a cure.

Finally please spare a thought for one of Sophie friends who is back in hospital almost a year following transplant. Yasmine (Yasmine's Website)  is a wonderful little girl who had been making such great progress and we all hope will soon making us laugh again.

Tina

16/06/2004 T+280

It has been a little while since our last update. We were going to post on Sunday but one of Sophie’s friends, Christopher, passed away on Saturday. We were very upset by the news and coming so soon after Niki’s passing it has been a difficult week.

Someone once said that when a child goes through transplant they are taken to the brink of death and then brought back. Far too many children never make it back. It just reiterates how lucky we are that Sophie has made through her transplant and how dangerous a procedure it is. We always felt that we had to take the risks involved in transplant to give Sophie a better chance in life but it is a decision that no parent should ever have to make. Reese, Isobel, Max, Niki and Christopher we will never forget you and the beauty you all brought into the world.

Last Monday we got the results of Sophie's immune system tests. Her CD4 count is now 560 and we are officially off restrictions. The engraftment is thankfully still 100%. Although we were expecting the results it proved to be a very emotional moment as it marks the beginning of the end of the transplant process. We are so proud of our wonderful girl.

Sophie turned two on Tuesday and we had a wonderful day at the beach. In celebration of Sophie's restrictions removal we all went into our local Marks and Spencer, a normal event for most families but a big landmark for us. When we set out from Pinner it was 31 degrees but thankfully by the time we got to the beach it had cooled to a more bearable 21 degrees. Sophie had a great time, I have never heard her laughing so much. She really enjoyed playing in the sand although she was determined to taste the sand. Amy was in seventh heaven and was making sandcastles, running into the sea, playing with the waves and burying her feet in the sand all at the same time. 

Everyday Sophie is doing more and more physically, she doesn't want to sit still for a moment. She goes onto her stand for 45 minutes at a time and it has been move up a notch towards vertical in the past week. She is doing all the things that children do before crawling, in Sophie's case she is just doing them a little later than most children. Physio and speech therapy came on Friday and as Sophie's restrictions have been stopped her name will be put down to join a therapy group for physio, speech and occupational therapy. 

Sophie had an audiology test this Monday and she did much better than last time. The hearing in her left ear seems fine, we are not so sure about her right ear but as always it is difficult to know how bad it is as Sophie is so young. At this stage as long as Sophie can hear that is fine with us. We will have another test in 8 weeks time to determine if Sophie may benefit from hearing aids. We had a meeting with our local paediatric consultant today and we will have one more meeting with him before being assigned to a paediatric developmental consultant, another sign we are moving away from transplant support. Sophie's feeding continues to go well and she is trying more and more different foods. We are trying to get to drink some milk and while she does quite well she finds it very funny to blow raspberry down her cup and spray Daddy and Mammy with milk.  

I have added some new pictures so you can see that Sophie's hair continues to grow aplenty.

Padraic

06/06/2004 T+270

Sophie continues to do well and is more and more eager to get moving. She now "launches" herself from your lap to get to the floor whenever she can and she is spending longer and longer in her standing frame. Hopefully in time Sophie will have the strength in her legs to support her weight. 

We had a BMT clinic on Wednesday and it was all good news. We discussed Sophie febrile convulsion with the consultant and the view is that it was caused by a virus for which there is no test. Interestingly, he said that children who are prone to convulsions are more likely to have them while immune suppressed. It's not that the immune-suppressants can cause convulsions rather that they hinder the bodies naturally ability to regulate temperature. So we are very pleased that Sophie is off cyclosporin; in fact the only drug Sophie now receives is Penicillin which she will continue to take for the next year or so. Our next appointment is at the end of July when Sophie will have her "one year post transplant" tests. They have been brought forward as Sophie is due to have back surgery soon. Provided the tests are ok there is every possibility that we will be signed off by the BMT team. We are awaiting the results on Sophie's latest immunity tests and all going well we may be able to reduce Sophie's restrictions. I am looking forward to us all having a nice meal out in a restaurant as a family again, the last time we managed that was last August. 

The BMT Team are quite happy for Sophie to have her back surgery so we have been in contact with the orthopaedic to let them know and to move things along. We are never happy when consultants use the word "urgent" and we want this operation to be carried out as soon as possible.

It is Sophie's 2nd birthday on Tuesday so we are going to the beach for the day, the temperature will be about 28 C so it should be a good day out. I'm sure it will also be a very emotional day, there were so many times in the past year that we feared Sophie would never make it this far. We have so much to be grateful for.

Today Sophie discovered ice-cream MMMMmmmmmm.........

Padraic

01/06/2004 T+265

We are still getting used to fact that Sophie has been registered blind although if wotsits were used in the eye tests I'm sure Sophie would have done a lot better. She seems to have a radar system for finding them. Thank you everyone for all you messages of support, they help us to look past the diagnosis and concentrate on the positive. As Anne Himes said 'We came out of that room with the same Sophie we walked in with.'

The big news is that Sophie has started to eat again. She started with a half pot of fromage frais last Wednesday which is the most she has eaten in 8 months and she now has two full pots a day. Sophie is also improving physically, she is much more active and today she managed to get down onto the floor from Tina's lap. She is spending more and more time in her "stand" and is trying very hard to get mobile. The physio and speech therapist came on Friday and said that Sophie has definitely moved up a stage in the past two weeks, we noticed it as well but it is always nice when someone else sees the changes.

We had a bank holiday yesterday and given Sophie's history of hospitalisation on bank holidays we held our breath !!! Sophie didn't let us down but thankfully this time it was only a trip to the local hospital to have her NG tube replaced. Sophie has pulled her tube out 3 times in the past few days, I think it time that we bit the bullet and learnt how to change her tube.

After weeks of hard labour and toil we finally managed to get a new swing set up in the garden. It didn't help that I found a beehive when I was clearing the space. Amy and Sophie had a wonderful day yesterday in the garden, there are two swings so they can play together which is great. We are off to clinic tomorrow and we will hopefully get the green light to relax the restrictions. We are starting to have friends around again which Amy loves as does Sophie. As it is now June I have added some new pictures. 

Please keep Christopher (Christopher's Website) in your prayers, he had a respiratory distress yesterday and is on the oscillating vent. 

Padraic

19/05/2004 T+252

Sophie is much happier this week, I can see more of a molar and it seems to have come through!! The GVHD of the skin is a little worse but it does not seem to bother her and is being controlled with the cream. We do seem to get flare ups when she is teething but now she is off all her immune suppressants drugs I do not think we are doing too badly.

Sophie had an outpatients appointment with Ophthalmology clinic on Tuesday to check her eyesight. We were aware she has a  problem but it had got better since the transplant. The corneal clouding was certainly better and were hoping for more improvement. Unfortunately the results are not good, she is severely visually impaired. She is not quite blind but she cannot see detail and anything at a distance. She has suffered brain damage either from the Hurlers but more likely from the Hydrocephalus. The damage is irreversible and as the the corneal clouding is now minimal we can not expect any more improvement.

We are feeling very low at the moment I know we will survive. I know we will adapt and learn ways in which to help Sophie and become the parents she needs. I know we will soon remember how lucky we are and this will soon become another part of Sophie. I know this because Sophie will show us how, she is our star and she will lead the way as she has always done.

Love Tina

16/05/2004 T+249

Sophie has been very clingy this week and hasn't been her usual happy self. She seem to be teething and wants to be held and walked around the whole time. She is not getting any lighter (thank goodness) and after 10 minutes of carrying her your back starts to ache so we sometimes have to resort to "wotsits bribery" to get Sophie to sit in her highchair while we recover. Sophie arm has recovered well from the PICC line removal, it is so nice that the line is out but I am not looking forward to next Tuesday when Sophie has a blood test which will have to be done "the old fashioned way". 

Sophie has a new "standing chair", it allows Sophie to also stand up at a table. She is quite happy in it for about ten minutes at a time, the idea is that Sophie will get used to being in a standing position while her body is totally supported and it will hopefully be one step in getting Sophie to stand up for herself. We are waiting for a special chair which will also Sophie to sit comfortably and give her adequate support. 

We had a meeting with the orthopaedic consultant on Friday and the curvature in Sophie's spine is getting much worse. The consultant is very concerned about this because if the front to back curvature (kyphosis) worsens Sophie is at risk of becoming paraplegic. The consultant will be liasing with the BMT team to determine when Sophie will be ready to undergo surgery. This will involve the insertion of rods either side of Sophie's spine to stop the curvature progressing. Sophie will then be in a back brace for about 3 months. The curvature is now so severe that we will not be able to use "growth rods" which would have helped Sophie's spine to grow. We were aware that Sophie was at risk of becoming paraplegic but it was a real hammer-blow when we were told how urgent it had become.  Sophie has been through so much in the past year and just when we were starting to relax we have another major worry. I watched the consultant's face as Tina told him about everything Sophie had overcome in her transplant, the GVHD of skin and gut Grade 3, the cardiac and respiratory arrest, the adenovirus, the fungal infection and not forgetting the fibril convulsion which landed Sophie back in PICU and it was as if he couldn't believe one little girl could survive all these things. We will have a pre-op assessment arrange in the next few weeks, this will involve a number of medical test so that the surgeon are totally prepared prior to the operation.

Please keep Christopher (Christopher's Website) in your prayers, he is making good progress but he had trouble breathing and is back on the vent. 

Padraic

09/05/2004 T+242

Sophie is doing well but we had some very sad news today. One of our friends Niki has passed away; in the early hours of this morning she suffered a cardiac arrest and could not be resuscitated. She was a brave little girl who suffered complications following her second transplant. She faced every trial with a smile and her parents love for her was obvious in every word of every update.

So tonight we will remember Niki, Max, Isobel, Reese and all the children who passed before them and we will hold Sophie and Amy a bit tighter.

Love Tina.

02/05/2004 T+235

Another busy week and Sophie continues to do well. Sophie had her last immunoglobulin (antibodies) transfusion on Thursday as her immune system is maturing. Cyclosporin, Sophie's immune system suppressant will be stopped on Wednesday. There have been a few GVHD flare ups in the past week but they have all responded well to steroid cream. From Wednesday Sophie will only have a twice daily dose of penicillin and on overnight anti-sickness drug. A great improvement on the 20 odd separate drug does we had when we came home from hospital. Next Wednesday Sophie will have her PICC line removed, this is good as it eliminates another source of infection, however it means that Sophie will have to give blood in the "normal" fashion which has in the past proved very difficult. Hopefully now that she is older it will be easier to find her veins. Sophie got a bath seat and she really enjoyed her bath tonight (she normally cries from start to finish) as I think the seat makes her feel more secure.

The physiotherapist and speech therapist came on Friday. Sophie was very happy and put on a great "show". The physio, Nicky, who hadn't seen Sophie since before the transplant said that Sophie was moving more although her joints are very stiff. She will be coming back on Tuesday with a piece of equipment which will help Sophie to stand. The speech therapist hadn't met Sophie before but they hit it off straight away and Sophie gave an impromptu game of peek-a-boo using what came to hand which in this case was a sock. The speech therapist suggested that Sophie attend the weekly therapy group. These are small groups of children with similar abilities who take part in various activities under the watchful eyes of physiotherapists, occupational-therapists and speech-therapists. It will be a great opportunity for Sophie to mix with children with similar needs although we won't be able to attend these classes until her immune system is ready. 

On Thursday Tina had the opportunity to pass on our thanks to the "crash team" at Northwick Park for doing such a wonderful job when Sophie had her febrile convulsion. 

Finally, there are few people who need your prayers. Sophie's friend Niki is still in PICU, she is making small improvements please check in and say hello...Niki's Website.  Christopher is also having a rough time and needs all your support..Christopher's Website. 

Padraic

25/04/2004 T+228

Firstly please say some extra prayers for our friend Niki. She is currently undergoing her second transplant and is in the PICU having a difficult time, Niki's Website.

As I have often said this is a roller-coaster of a ride and our Sophie likes to keep us on our toes. After Easter, which I have to say scared me to death, I have been completely stressed. To ensure that she does not have another fit we have to give her paracetemol as soon as she goes above 37.5 degrees C.  The plan was then to take her to the hospital to have bloods taken, just in case. As you can imagine I had visions of us being at our local every other day. Also, we were not sure what to do about the PICC line, the plan was to remove it as soon as her CD4 count reached 200+.  This would reduce the risk of infection and the need to go the hospital with fevers. However, she would have to have had blood tests every two weeks until her CD4 counts were over 300.  Although any reduction in the chance of infections is always welcome the thought of fortnightly blood tests, it was always a nightmare before the BMT, for an indefinite period was worrying.

So off to clinic on Wednesday to find out the results for the immunity test and make some decisions. Sophie's CD4 count has jumped from 129 to 290 in a month!!!!  So decision made, thank you Sophie for making this easy yet again. She is now off most of her medications, her PICC line will be removed next Thursday and we can give her paracetemol as soon as she hits 37c without taking her to the hospital. She will have fortnightly blood tests but it should only be for a short time.

On Friday we had a visit from the Occupational therapist from Social Services, she was very nice and assessed Sophie needs. The main thing is a chair that will give her more support and a bit more independence. We have been problems with Sophie's car seat, it rubs the bump on her back, unfortunately that is not covered by Social Services but she did recommend someone who help adapt Sophie car seat. We also found some advice on the MPS forum. We will have to investigate further.

We have had a lovely weekend, the weather was wonderful and we spent most of the time in the garden. We had a picnic in the garden on Saturday. Sophie spent the whole time laughing and she had Wotsits again. It does not get much better than this.

Tina

18/04/2004 T+221

Sophie was discharged from Great Ormond Street on Wednesday. We were meant to go back to our local hospital but as Sophie's temperature was no longer peaking (and as our local hospital had no beds) we were able to take Sophie home. All the tests came back negative which was reassuring, there are so many virus's that Sophie is at risk from. Sophie has been doing well since she has come home although she has been staring into space a little and has been quite sleepy. This is probably due to the drugs she had when she was in PICU, the last time she left PICU it took her at least a week to recover. We have an appointment at Great Ormond Street on Wednesday so we raise any concerns then if she isn't back to her usual self. As Sophie's PICC can be a source of infection it will probably be removed in the next few weeks perhaps after her next flabbagamma infusion (which should be the last one).

Saturday finally gave me an opportunity to get the garden in shape for summer. Sophie had a great time playing on her rocking horse and pushcar. It is so much easier if we can get out into the garden, it can be so claustrophobic being stuck in the house all day. I'm glad we got out yesterday, it hasn't stopped raining all day today. 

We are both still quite shell shocked from all this, we take Sophie's temperature if she feels even the slightest bit warm. It is just one more thing that we have to take on board but hopefully Sophie will grow out of it.

I have added some new pictures so you can check out Sophie's new curly hair.

Padraic

13/04/2004 T+216

I spent Saturday night with Sophie and I didn't sleep too well. Sophie was fine but every time I woke up I had to check on her to make sure she was ok. I guess it will take me a little while to relax again. We were both awake at 5:00am and we had a nice "chat". Sophie's temperature was still high overnight on Sunday and Monday but overall it is on a downward trend. The source of the fever has yet to be found, the more likely cause is some kind of viral infection. The neurosurgeon said that Sophie is likely to have another seizure in the future if her temperature "spikes" rapidly and so we will have to keep a closer eye on her temperature and give her paracetemol as soon as she goes above 37 degrees C.

The BMT consultant checked in on Sunday and was amazed at how much Sophie has grown since January. We have started to reduce the cyclosporin as planned and it will be stopped totally in about 4 weeks time. This will help Sophie's immune system to recover from the transplant. Sophie has developed a rash in the last few days which is most likely to be GVHD of the skin, we will get some cream to treat it if necessary. The BMT team will also discuss whether Sophie PICC line can be removed as it can be a source of infection. While it would be great to lose the line it would mean that Sophie would have to give blood "the old fashioned way" which has been a problem. Hopefully now she is little older it will be easier to find her veins. We will probably stay a Great Ormond Street for another day before transferring back to our local hospital. We have had lots of comments on Sophie's new curly hair so I will try to get some new pictures loaded at the weekend.

Thank you all checking in on Sophie and for all the messages of support.  

Padraic

10/04/2004 T+213

Monday morning Sophie started getting warm, I took her temperature and she was 38.2. She was over 38 for four hours so I know we had to take her to our local hospital. They admitted her, took blood cultures and started antibiotics. Her temperature went away following paracetamol and over night she was fine. Clinically she was very well and the next day  did not have a temperature. We would have to stay at Northwick Park until she was below 38 for forty-eight hours. We waited the results from the tests. On Wednesday the fever came back and continued all night and into Thursday, it was controlled well with the paracetmol. I swapped with Padraic to see Amy and went home. I got a call at 9.30pm, Sophie was having seizures which were not stopping. A friend came to my rescue and drove myself and Amy to the hospital and looked after Amy until my Mum got there and drove them home, thank you Alison. 

Sophie's room was full of Doctors, Nurses and, I was later to find out, the anaesthetist team. Sophie was still fitting and they had run out of options they had to intubate and give her medication to shut everything down. Intubating a Hurlers child is not a easy task and they had problems but they were prepared and in the end succeeded. At 3am we were then transferred to Great Ormond Street PICU were she was put on the vent. The next day a barrage of tests started to determine the cause of the fevers. Currently they believe the fit was caused by a rapid increase in her temperature. At the end on Friday she had a Chest X-Ray , CT scan of her head and Heart echo and nothing was found and the fever was getting worse. However we still ended the day positively as at 8pm she was taken off the vent. At 11pm we admitted defeat and went and got some sleep. A wonderful sight greeted upon our return the next morning, she was playing and said Mama when she saw me. She had been very hot over night but today her temperature has been fine and we had some smiles and even a couple of laughs. She was very happy to have visits from the wonderful friends from Fox ward.  

Today we left PICU and was transferred to Parrot ward as there was no beds on Fox. We spent a week on Parrot last year when Sophie had her shunt fitted. Soon after she got there she had her shunt tapped and the initial results shown no virus. 

The temperature has came back and we are waiting for results from blood test etc. We need the temperature to stop and to find out what is causing them in the first place. We will update in a couple of days. 

Love Tina

04/04/2004 T+207

Not much to report in the O'Connor household things are steadily improving. She is not so upset when her spoon is put front of her, she still does not eat, but it is a step forward. I think she gets frustrated with her inability to communicate and lack of movement. We have a meeting with the physio-therapist and speech therapist soon and I hope we can set up more therapy sessions as I feel she needs a more structure approach than what I am offering her.  I try to pick up on their suggestions but I feel somehow she needs more. In the hospital I could give all my time but now at home I feel torn between Sophie and Amy and just end up I feeling like I am failing them both. I know this is a common feeling, I think during birth the midwives inject you with a guilt potion while you are not looking and that's it, you have entered the world of motherhood.

On the April 7th it will be a year since Sophie diagnosis, a year since we sat in a room had our world ripped apart. What a roller-coaster of a year, the things we have faced, Sophie's unbelievable inner strength, Amy's fortitude, the incredible friends we have made and the children whose journeys have touched us. I wonder what the next year will bring ? 

Love 

Tina 

28/03/2004 T+200

It has been another good week for Sophie. Last week we stopped her anti-fungal medication and she has been much happier as a result, we checked the possible side-effects of this drug and are not surprised she feels better off it. She is much more interested in playing and doesn't want to sit on anyone's lap for too long. She trying more and more to get mobile, her arms are quite strong but her legs can't support her weight. Sophie's feeding is going well she has actually started to show an interest in eating. The NG feed is almost at the stage that we can give Sophie all her feed during the day and stop the overnight feed. This will give her stomach a much needed rest at night and will hopefully make her a little more interested in oral food during the day. Our health visitor came on Wednesday and she will be responsible for coordinating Sophie's educational needs. We have not been to hospital this week and we don't have any appointments next week. This will be the longest period we have had without a hospital appointment since Sophie's diagnosis last April and I must admit it feels a little strange. We came across a wonderful website last week called the mpsforum.com, it is a great place for families of children with MPS conditions to get in touch with each other and discuss the many aspects of MPS conditions. 

Yesterday Amy decided we had to have a "celebration" for Sophie coming home from hospital. We never really celebrated this when we first came home as I suppose we were a little shell shocked. Amy was very enthusiastic about the "celebration" and chose everything from the cakes to the paper napkins and plates. We really enjoyed ourselves although I was quite hoping to see some jelly and ice-cream on the menu. 

Padraic

21/03/2004 T+193

Clinic went well this week, Sophie has put on more weight, they taken her off the medication for her fungal infection and they are happy enough for us not to come back for three weeks. On our the next visit she is going to have a chest x-ray and a ultra-sound on her heart in honour of being six months post BMT. Even better we are going to start tailing off her cyclosporin over a six week period. When it is finished her immune system should really pick up and we will be able to stop many other medications. Although this is really exciting I am a little scared as this is her only protection against GVHD. It is just another step but sometimes going forward can be scary if you keep looking back.

Padraic's mum came over from Dublin yesterday for two weeks to see Sophie and Amy and give my Mum a break. It is Mother's Day today in the UK and Ireland and I have being thinking how hard this would be without our Mothers. Thanks Mum for being there. 

There was some bad news for one of our friends Niki this week, her BMT did not work and she will have to go through it again. It is a hard time for her family but from reading her site regularly I have a feeling her family have the strength to cope. 

Also a message for Angela, we are thinking of you all and pray that this mountain will be finally conquered.

Finally Padraic was reading a Winnie the Pooh story which contained the following conversation between Winnie the Pooh and Christopher Robin, it touched us both.

"If there's a tomorrow when we're not together, there's is something you must remember."

"Oh, and what might that be, Christopher Robin?"

"You are braver than you believe, and stronger than you seem, and smarter than you think." 

Tina

14/03/2004 T+187

Another week over and things are still going well. On Wednesday Sophie was 6 months post transplant which is another milestone passed. We didn't have a clinic appointment this week although we had a meeting with the paediatric consultant at our local hospital to check that everything was ok with our local support, which it is. Sophie will be starting speech therapy soon which we hope will help with her feeding. Sophie is only interested in chip-sticks, she won't even try anything else. The NG feeding is still going well and we have reached the volume and rate limits we aimed for when we left the hospital. Provided everything continues well the next thing we are waiting for is Sophie's CD4 count to reach 300. The CD4 count is an indication of how good Sophie's immune system is, once it reaches 300 we will be able to relax some of the restrictions and we will be able to take Sophie out in public a little more. It is tested monthly and last month it was 110. Sophie has been teething for the past few days and her temperature has been high a few times but thankfully not so high that we needed to take her to hospital. I have added some new photos. 

I will leave the last word to Amy who said the following...

Mummy.....when Sophie is all better...can all my friends come around...and tell Sophie how much they love her.

Padraic

07/03/2004 T+179

Things are getting a bit quiet in the O'Connor household. Sophie is hardly ever sick now, and even the dirty nappies are down to about 5-7 a day. She is tolerating more feed in fact as of next week she will have meet her overall target and we can stop increasing for a while and try to push oral feeding. Sophie is soooo uninterested in any solids, after our initial successes I thought we would be ok. Whenever I get near her with the spoon she is tight-lipped and turns away. The only thing she will eat are her crunchy sticks. When I tried to dip the end into some mashed food she turned it around and ate the other side. I am planning to try some more finger foods but I am so terrified of making her sick as she gags so easily. Well we will keep trying and hope it happens one day.

Sophie seems to be getting really frustrated with her lack of movement, when she is sitting on your lap she tries to climb down onto the floor once her feet are touching the floor and she stands with our support she gets really upset. She likes to stand up on your legs but she only stands on her toes. It quite difficult as I feel out of my depth and do not know how to help her. I suppose it's the frustration that will spur her on but I wish I could make it easy for her. 

Overall she is still doing great, this is a long journey and we are only beginning.

Tina

P.S As there is no clinic on Wednesday so the next update will be on Sunday and then weekly from then on.

03/03/2004 T+175

This week has thankfully been much quieter than last week. Sophie's GVHD has settled down and Amy is over the worst of her chicken pox. We went to the BMT clinic today and had to stay in an isolation room as Sophie (and Daddy) are at risk of picking up chicken pox. This meant that Sophie couldn't say hello to any of her friends although Mummy passed on her regards to everyone. Sophie's weight is up to 9.75kg from 9.5kg last week which everyone is very pleased about. We spoke to the dietician and once Sophie's weight gets to 10kg we can try to encourage her to eat a little more by reducing one of her feeds. Sophie's chicken pox cover medication has been increased to four times a day. It had been reduced to three times a day when we left the hospital as Sophie's kidney function was down, her kidneys are now working normally so we can increase the dosage back to normal levels. We were told that if Sophie does get chicken pox it should be very mild and she should only get a few spots. As Sophie is doing so well our next BMT clinic will be in two weeks time.

Sophie has now perfected the art of removing her nappies. When her nappy is being changed by the time you get back to her after washing your hands she has normally taken the nappy off and is waving it in the air with gusto.

Padraic

29/02/2004 T+172

Sophie's GVHD has settled since Wednesday and although she has been teething her temperature has not been high. To add to our interesting week Amy came down with chicken pox on Thursday. We spoke to the hospital and we just need to keep a close eye on Sophie. She already takes a medication to protect her against chicken pox so hopefully there will be nothing to worry about. The physiotherapist came on Friday and gave us some stretching exercises to do with Sophie. The speech therapist will hopefully be coming next month so we can start teaching Sophie some more sign language. Sophie already waves hello and goodbye so it will be great if we can add to her repertoire. The NG tube feeding is going well and Sophie is not being as sick as she used to, we are still increasing the amount of feed she receives. She hasn't been very interested in oral feeding this week but as she has been teething it is not surprising. Her weight hasn't gone up in the past week but more importantly it hasn't gone down. 

Sophie has been in good form and Amy has been very good considering she is covered in spots which are itching.  Tina has already had chicken pox but I haven't so I will spend the rest of the week looking out for spots and getting worried everytime I get an itch.

Padraic 

25/02/2004 T+168

A bit of a strange week, first, Monday the day of the MRI that did not happen. Everything was going well then the time of Sophie's MRI was delayed. Sophie had a nap and when she woke up she felt warm, in fact she was 38.6, and her feet and hands were bright red. I spoke to the nurse and the anaesthetist came straight up and we all agreed it was too much of a risk, so the MRI was cancelled. I phoned the BMT team and as I turned around Sophie's favourite Doctor appeared. She was checked out and bloods taken for tests and she was allowed paracetamol. After an hour her temperature went down and off home we went.

Yesterday she was fine and very happy but still a little warm however, not over 38. Her rash on her arms and legs was still quite red. I was beginning to think that teething was not the reason and maybe GVHD was the culprit.

Today it was clinic and everything was going fine and it had happened again. She went up to 38.5 and her hands and feet was bright red. I suppose we were in the right place but still I was beginning to get worried.  The results from the blood tests so far show no infections or virus so it is all pointing to GVHD. As she has actually had a decrease in dirty nappies and sickness the flare up seems only to be in the skin. We were told that this kind of flare up can been caused by infection, virus or even teething. It is still mild and we were assured it should not get as bad as before. We were given some different cream which should help and have to go back next week for a follow up. 

Everything is still ok, she is doing great happy, playful and getting cheekier everyday but Mummy and Daddy are a little more anxious.

Tina  

22/02/2004 T+165

Sophie's teething continues to irritate her and we think it has been pushing her temperature up a little. On Thursday it hit the 38 mark so we had to prepare ourselves for a trip to hospital, thankfully it eased off by the afternoon and it has stayed below 38 for rest of the week. Tomorrow we are at Great Ormond Street for an MRI scan on Sophie's back. We have an appointment with the orthopaedic surgeon in May to discuss an operation to fuse her spine and the MRI scan will provide information about the extent of the Sophie's kyposcoliosis (spinal curvature). This will be the next procedure Sophie will have to undergo but it will only be carried out when she has been "released" from the BMT consultants which could be around September. The scan has to be carried out under General Anaesthetic which is always a concern but Sophie will need to be still for about an hour in the MRI machine so we can't do it under sedation. It is never nice leaving your child when they are given an anaesthetic and despite the fact I have lost count of the number of times we have had to do this it never gets any easier. Hopefully tomorrow we may be able to stay with Sophie while she has the scan which will make it a little easier.

Sophie has also invented a new night time game called "kick my pyjama bottoms off" in which she kicks her legs so hard her pyjama bottoms come off. On Thursday night she was playing a particularly enthusiastic game and she managed to get both her bottoms and nappy off. She woke me up with her babbling and I have never seen her look so pleased with herself !!

Padraic

18/02/2004 T+161

Another clinic day and another chance to meet up with old and new friends. It was quite busy today so the nurses and doctors had to work at getting the children away from chatting parents. Sophie has lost a little weight but she is still up on her discharge weight. It could be that last week was too high but as we have had a couple days of extra dirty nappies there is a concern,  so we are back next week. GVHD is always at the back of everyone minds however she has cut another tooth so lets hope that is the cause. Anyway clinic is becoming more of a social engagement. Sophie loves the attention and waves and blows kisses to everyone.

Some good news the dietician suggested some acceptable snacks, almost like wotsits, which Sophie loves. When she is eating them she has that look that some men have smoking a cigar, pure pleasure.

Tina

15/02/2004 T+158

Sophie's blood results came back from clinic and they are all fine. The adjustment to the Cyclosporin dosage has brought her counts back to just under 100 which is in the correct range. The occupational therapist came to assess Sophie on Friday and although Sophie is delayed for a 20 month old she was very impressed by how much Sophie could do considering she has been in hospital for 5 months. We were given some new things to try and we will be encouraging Sophie to move more while she is sitting up. Ironically, the therapist also suffered with scoliosis and she said that even now rolling out of bed is not easy and she has to use her legs a great deal. Sophie's legs are quite weak so it is little wonder that she has trouble in rolling. We have has a lovely weekend and as we were the only people in the children's playground on Sunday Sophie got to play on the swings. She absolutely adored it and laughed the whole time, Amy was so happy to have her baby sister next to her on the swings. 

Sometimes memories come flooding back, I remember playing with Amy and Sophie on these swings last August a few days before Sophie went into hospital for her transplant. Knowing the risks involved in a Bone Marrow Transplant I wondered whether they would ever play together on these swings again. It was wonderful to see the joy on their faces today and it make us realise how far we have come in such a short space of time. When Sophie was diagnosed last April we had to face losing Sophie before she was 10, since then we have found a wonderful donor, Sophie is 100% engrafted and we have made it home after transplant. We treasure every moment we spend with our two girls, we know how lucky we are.

We have added a new child to Sophie's "Friends with Hurlers" section. Niki is a 16 month old girl who will be starting the countdown to her transplant next week. Please check in on her and lend her your support she is a wonderful little girl.

Padraic 

11/02/2004 T+154

The eye appointment went well and although it was a quick check up the doctors were pleased with the improvements in the corneal clouding. We will have to wait until Sophie's immune system is stronger before she has more in depth tests. We felt a bit like royalty, we were in an isolation room and the doctors came to us, no waiting rooms for our Sophie.

Clinic went really well today, we saw more friends. I really like this aspect of clinic it is so lovely to catch up with all the other families and see how they are doing. Some good news, some not but as always there is hope. 

Again everyone is really happy with Sophie and the best news she has put on weight (yippee!!!). We are back next week and then hopefully we will be going every fortnight. 

The last couple of weeks have been strange, we have been so happy but I have had moments of feeling really low. I am not sure why, I keep thinking back over the last five months and feel overwhelmed by the future. Recently  I committed the ultimate sin of asking "Why us?". However, after watching Amy dancing to Sophie playing the piano; seeing Sophie's sheer joy in just sitting next to Amy; listening to Sophie babbling in the morning and Amy coming in to say Good Morning Sophie; hearing Sophie say Mama and giving me a kiss. "Why us?", because we are the luckiest parents in the world. If you want proof check out the new photos.

Tina

08/02/2004 T+151

On Thursday Sophie had her 3 weekly infusion of "flabbagamma" at the local hospital. This is blood product which helps to boost Sophie's immune system. I got to meet the community nurse who will be visiting us on a weekly basis. She is very nice and was very keen to ensure that we were getting all the local support we need. 

On Tuesday Sophie has an appointment with the eye specialist at Great Ormond Street. We know that Sophie eyesight is poor although it has improved since the transplant. We are keen to find out how good her eyesight is and what treatment options are available to us. When I spoke to Toby's mum last week she said that it took about 8 months for Toby's corneal clouding to clear, we hope that Sophie's eyesight will continue to improve. 

Sophie's first session with the occupational therapist is next Friday and the following week physical therapy will start. Speech therapy should start early next month. 

On the home front Sophie continues to do well with her feeding and we are slowly increasing the amount she receives on a daily basis. She is less sick than she has been so she is not losing very much fluid. We increased Sophie's Cyclosporin dosage on Friday as Wednesday's blood test showed her level was down. We will have it rechecked next Wednesday and the dose will be adjusted as necessary.  

We are often tired, sometimes stressed but constantly amazed how wonderful our children are. 

Padraic

04/02/2004 T+147

Clinic day today and we were a bit nervous but there were so many familiar faces making a fuss over Sophie it was really nice. Everyone was happy with her and although she has not put on any weight, she has not lost any either. We have been increasing her feed very slowly over the last week and she does seem to be tolerating more. She is still sick once or twice a day and is on average having six to eight dirty nappies a day but it is not increasing as we are increasing her intake. Hopefully soon she will start to put on weight. We will get the results of the blood tests tomorrow afternoon. 

We were very happy to meet another child with Hurlers called Toby who is one year post BMT. He had a really rough time through transplant and seemed to stolen everyone's heart on the ward. It took him many months to recover but now he is doing really well and in the last few weeks has started to stand. It was the first time we have actually met another child with Hurlers, it was like a glimpse into the future and the future looks good. 

Tina

01/02/2004 T+144

As we are now home there is thankfully not much to report on on a daily basis. We will therefore update the site twice a week, on Wednesday following the BMT clinic at Great Ormond Street and also on Sunday evening. On Thursday evening Sophie decided to take her NG tube out and as a result we had to go to our local hospital to have the tube replaced. Normally an NG tube replacement takes about 5 minutes to complete but for some reason the tube wasn't "playing ball" and it took 2 hours before we were happy it was ok. The biggest piece of excitement this week was the arrival of what Amy described as "the biggest box in the world", inside this box were 10 helium balloons a lovely present from Jan, Pat, Zoe and Joy. Sophie and Amy are having so much fun with the balloons, you can image the chaos caused by 10 helium balloons attached to a 3 year old as she charges through the house. Sophie continues to do well and we are slowly getting used to the daily routine. The nights are getting easier as Sophie is not producing as many dirty nappies and so she is sleeping better. We have increased the feed rate and from tomorrow we will start reintroducing solids into Sophie's diet. Next Wednesday will be our first appointment at the BMT clinic and on Thursday Sophie will have her 3 weekly infusion of "flabbagamma" at Northwick Park Hospital. Sophie is so happy to be home, she spends most of the day giggling and chatting, we can really see an improvement in such a short space of time. It is also really nice to be together as a family again and to enjoy such simple things as a walk in the park and sitting down together at home for a meal.

Padraic

28/01/2004 T+140

We woke up to snow this morning which was lovely until we had to get to our appointment at the local hospital. The roads were jammed because the trains had been suspended, it took only 1mm of snow to bring the Metropolitan line to it's knees. We met the community nurse and went through all the details of the local support we will be receiving. We also met Dr Hyer the paediatric doctor who discussed the arrangements for Sophie's speech, physical and occupational therapy. We will start all these in March so we have a few weeks to settle into a routine, we also have a number of appointments in the next few weeks so we are quite happy to keep Sophie's "social diary" to a minimum. Sophie was weighed today and her weigh has increased a little which is great. She loves being home and she spends the entire day playing. She is doing more and more physically  and is quite happy to lie on her stomach pushing her head up. 

Thank you for all the welcome home messages, it was wonderful to read them all. Please pray for another of Sophie's friends, Isabella is back in hospital and is having a difficult time, Isabella's Website.

Padraic

27/01/2004 T+139

We have had a great day, it has been hard work and very emotional but with moments of sheer joy. When Sophie went back to the hospital on Monday she was really quiet but once we were discharged and back in the car she was giggling and chatting, so I think she was really happy to go back home. She had all her tests and although she lost a little weight they were happy to let us home.   

Today she has been playing and even rolled from her back to her tummy and started pushing herself around on the floor. This was something she was never did before and it was so wonderful to see her so happy with herself. Her favourite game at the moment making music with her various toys and watching Amy dance. I think she lost weight because of the extra moving around.

I have raised her Neocate feed by 5ml and it seems to be going well, a few extra dirty nappies but only sick once. When we tried this a fortnight ago she was really sick and had to go back on IV cyclosporin so it's encouraging that she seems to be tolerating the extra feed.  

I spent this morning organising delivery of feeds and medications etc. Everyone has been very helpful and after a couple of phone calls the chemist is going to be delivering a month supply of Neocate (non-milk feed) and Maxijul (extra calories in the water) this week.  We were discharged with a months supply of medication so I will have to chase that up in a few weeks, but based on today I do not think there will be any problems.

Tina

26/01/2004 T+138

Sophie is home....More details tomorrow.

Tina

25/01/2004 T+137

We had a wonderful weekend, it was great to have Sophie back home again after all this time. The nights have been difficult as there are no night nurses to change the feed and give Sophie her 06:00am drugs but we will soon get used to it. We don't know how much Sophie remembers about her home but she settled down very quickly and had a great time playing with Amy. She is still being a little sick so we haven't cracked the NG feeding just yet but she hopefully isn't losing so much fluid that her drugs levels are affected. We will be back to hospital first thing tomorrow morning for blood tests, if the results all come back ok, we will be discharged !!!

Padraic

23/01/2004 T+135

Sophie is at home in her cot fast asleep, we are going back on Monday for blood tests and if everything is ok we will be discharged.  On the way out of the hospital she was waving at everyone going past. She slept most of the way home but it was all a bit much by the end and she was a little sick. Once home she did not take her eyes off Amy and just kept laughing at her running around the room. 

Today was ward round and the consultant again was so pleased with Sophie and said that they were scared at several times but she is a fighter. Sophie has got a little rash at the moment which is probably GVHD, it does not seem to bother her so we are just using cream. 

It is all a bit overwhelming at the moment and I just hope we have the strength to meet this new challenge.  I have to go now I have not checked on her for at least ten minutes.

Tina

21/01/2004 T+133

Sophie continues to do well with her feed and although she is still being sick she is managing to take the fluid volume required. Apart from the IV cyclosporin there is nothing keeping Sophie here. If she carries on as she is for the next few days we will come home on Friday and go back on Monday for a check up. If she is ok on Monday then we will take her home for good. This is the best solution for us as it gives us a little "safety net" should Sophie have trouble with her feed or if her Cyclosporin level drops. When Sophie comes home we hope that her oral feeding (i.e. non-NG tube) will improve as we will be able to feed her when she is ready rather than at the times set by the hospital kitchen. If all is quiet tomorrow I won't do an update so hopefully the next update will be when we have Sophie home. 

Padraic 

20/01/2004 T+132

Another nice quiet day today. Sophie is tolerating her feed well and her weight was up a little this morning. Her Cyclosporin will remain on IV until Sophie is tolerating her feed. It was ward round this morning and they are still very pleased with Sophie's progress. They do not think that Sophie's GVHD has returned, although she is producing lots of dirty nappies she does not have diarrhoea. The thinking is that Sophie is just getting used to her NG feed and that is will improve. There is no talk of a specific going home date but it will hopefully be days rather than weeks. We need Sophie to tolerate her feed for a few days in a row and for her to maintain her cyclosporin levels while on oral cyclosporin before we will be happy to go home.

Padraic

19/01/2004 T+131

Sorry for the delay in writing an update, it has been very busy getting everything ready in the house. Sophie didn't come home today, she is not quite ready. She didn't tolerate her feed over the past few days and her weight dropped again this morning. Sophie's cyclosporin level was very low and she had a dose through her IV line. It would appear that she isn't absorbing the drug into her body. Cyclosporin is a very important drug as it keeps the GVHD at bay, it has got to be kept at a consistent level for it to be effective. She has been sick quite a few times and although we are not doctors it looks to us as if her stomach is unable to process 800ml of fluid per day. She is normally sick at the end of an NG feed when it appears that her tummy is simply too full. Our concerns are that Sophie will have to go back on TPN in order to maintain her weight, more worrying is that the GVHD in her gut has reappeared, this would need to be treated with either steroids which leaves Sophie vulnerable to virus' or infections. 
It is ward round tomorrow so hopefully we will have some answers but I think we may be a little further away from going home. While we are quite happy to stay in hospital until everything is sorted out it is difficult having been so close to taking Sophie home. Despite all this Sophie is in very good form she does not want to stay in her cot, she wants to play all day which is wonderful.  She is doing more physically and is really starting to build up her leg muscles. Her play is much more involved and she spends long periods working on her problem solving skills. 

I have added some new pictures to the website so you can check out Sophie's new hairstyle !! 

Padraic

16/01/2004 T+128

Another good day, Sophie coped with a slight increase in the amount of feed and we have nearly reached the magic 800 ml a day. 

It was ward round and the consultant is delighted with Sophie progress and if this weekend continues in the same way we could go home on Monday. There were three Doctors in the room and Sophie was laughing and attempting to grab their aprons. I think they were more interested in playing with her then talking to me.  

I was taught how the make her special non-milk feed, it is very like formula milk. I been given so much information this week I hope I remember it all. I am feeling many emotions about going home but I am feeling less frightened then I thought I would be. I suppose that is because she is so well and I know there will always be someone I can call and get help night and day.   

 So I am going to spend this weekend cleaning, so unless there is a real news I will not do a update tonight but let you know how the weekend went on Sunday night.

 Tina

15/01/2004 T+127

WBC 3.9 (T-cells 0.8)

Sophie had a better day today and her feeding is going better. She will have lots of smaller feeds rather than trying to feed her at 80ml/hour. She had some additional IV fluids last night and her weight was up a little this morning. We will increase the feed rates very slowly when we think Sophie is ready rather than at a set amount each day. Her nappies have been less frequent which is a good sign. In other news the bone marrow sample taken when Sophie had her Hickman line removed shows that Sophie is still 100% engrafted which is wonderful news. The adenovirus counts are zero, they were 1100 last week which is technically a negative result but we like zero better.  In a lot of ways it has come as a bit of a relief that we are not going home tomorrow, it will be stressful enough without having to worry about Sophie's feeding. Thank you all again for the wonderful guestbook entries, it is so nice that you all continue to write and support us after all this time.

Padraic

14/01/2004 T+126

Well I suppose we have seen the finish line a little too early again. Sophie won't be coming home on Friday, she has been having trouble with her feeding and her weight has dropped to 9.7kg (it should be about 10.2kg). Sophie has been sick quite a lot in the past 24 hours so she will have some additional IV fluid tonight. The lack of fluids is showing in her blood counts so we will have to get them back to normal before we leave. While it is disappointing that we are not going home we are not too surprised. Sophie hasn't done very well on her feeding since the weekend and we would rather go home when Sophie is ready than leave early and have to come back into hospital again a few days later. We had a meeting with the community nurse so we are all set if we are given the green light again. Thank you for all the guestbook entries I'm sorry to disappoint you all, now you will all have to add the guestbook entries again when we do make it home !!! 

(Anne, home is our house, fortunately we are only about 45 minutes drive from the hospital.) 

Padraic

13/01/2004 T+125

I will start with the best news, Sophie will be discharged on Friday. We knew this was very likely and it was confirmed today. Tina had a discharge meeting this afternoon and was given details of all the "rules and regulations" we must follow. It has been a long journey and it feels so good that Sophie has come so far. Sophie was in very good form today and it is now very tiring taking care of her as she doesn't want to be in her cot. She likes to be carried around her room and look out the window at the nurses. This afternoon Tina put her in her chair and faced her towards the window. Sophie had a great time rocking in her chair and waving at her reflection in the window. She loves to clap along to "If you're happy and you know it clap your hands" and has started nodding today. Her feeding is going well, the calorie content of her milk feed has been increased and some sugar will be added to her water to up the calories. The consultant said that it would be difficult to get 1000ml of fluid into Sophie every day so we should aim for 800ml instead. This should be achievable. I will start cleaning the house tomorrow in preparation for Sophie's homecoming, it will be wonderful to have her home again.

Padraic

12/01/2004 T+124

Sophie was a little sick this morning just after she had her medications which isn't good timing. In an effort to increase Sophie's daytime milk feed we tried to give her 120ml at 70ml/hour. Perhaps, it was a little too much too soon and Sophie was so sick her NG tube came out...yuk. The first NG tube developed a kink and Sophie had to have it removed and another tube put in. Needless to say she was less than impressed !! We need Sophie to have over 1 litre of fluid a day so that she can process her medication, it doesn't all need to be milk so we will be giving Sophie additional water through her NG tube. The doctors will liase with the dietician and work out the milk/water balance required.  

Padraic

11/01/2004 T+123

Sophie had a little extra fluid (not TPN) overnight as her fluids were a little short of her target. Sophie was in very good form today and her teething seems to have subsided for the moment. Her feeding is going very well, she had four 100ml feeds during the day and she will have 8 hours at 40ml/hour overnight. This will give her more breaks during the day and hopefully we can continue to increase the daytime feeds until no night feeds are required. She is processing all the fluids properly as her weight has stayed static for the past few days. Amy came up to the hospital and she was very impressed with Sophie's new hair and with the fact that it matched hers. There were lots of cuddles and kisses between the two of them. Amy really misses Sophie and she is looking forward to having her baby sister home again. We are now both up to speed with the administration of all Sophie's NG drugs and her milk feed pump. We are still waiting for a definite going home date but at the moment next Thursday or Friday is looking promising.

Padraic 

10/01/2004 T+122

Today is the first day Sophie is off TPN and all the IV drugs as the CT scans results were good enough to stop the anti-fungal medications.

We seemed to got the NG feed sorted, she is fed at 40 ml per hour all night with a couple of hours off and during the day she has equal periods on and off the feed at a higher rate. Hopefully we will build up the daily rates and decrease the feed over night and thus create a more natural feeding pattern. This is all going to take a while, she really has been fed by TPN for the last couple of months. She seems to really enjoy her soup, she had a few teaspoons but she seemed to like the whole experience so I think this is a really good sign.

She has had a good day, not been sick or any major problems. She is a little tearful and does not want to been put down on the mat to play. She could be teething, still sore from surgery, recovering from the sedation for the CT scan or generally in need of cuddles. We must not forget it was only two months ago she was in intensive care on a vent, a month ago she was fighting for every breath. Although it is very easy to forget when you see her smiling, laughing and generally being the cheeky little girl she has always been.  A couple of days she was sick and my heart sunk when she brought up a really hard mass. What could it be? On closer inspection she had eaten a steri-strip (sterile medical tape)  which had been used to keep her NG tube stuck to her face!  

Tina

09/01/2004 T+121

WBC 4.9 (T-cells 0.8)

Sophie had a good night and she has been in good form all day. She was still a little sick this morning so we will increase the daytime milkfeed rate and decrease the overnight rate to see if that helps. Sophie had some soup for lunch and she really enjoyed it. The TPN will be stopped tomorrow and we will continue to build up the normal feeding. She has been a little warm and sneezy today, I hope she is not coming down with a cold. It was ward round this morning and the CT scan results show Sophie's lungs to be significantly better. I asked about when Sophie would be ready for her back operation and it will be at least 6 months before Sophie would be ready. She needs to be off the CycloSporin and eating and drinking normally before the operation can be carried out. The physiotherapist came in this afternoon to assess Sophie so that she can write a report to be sent to our local physio. Sophie was very tired and wasn't up to doing much. We were given some additional legs exercises to do. The physio will order a special chair for Sophie so that she can sit comfortable at home.

By the way, we are still on course to go home at the end of next week.

Tina

08/01/2004 T+120

Sophie had a very unsettled night. Her PICC line kept blocking because of the dressing position and it set the TPN alarm off regularly. Sophie's arm is hurting her, she is still getting used to the PICC line and she was tired and unhappy for most of the day. In preparation for the CT scan today Sophie needed to have a new line inserted into her arm, apparently the Hickman line is normally used to aid the contrast settings in the scan. As it is very difficult to insert a line into Sophie the doctor decided he would only try once. He was unable to insert the line so he phoned the CT scan dept to tell them. They said they didn't need a new line and that they could use the PICC instead, so Sophie went through an attempted line insertion for nothing...Grrrrrr.. We had the same confusing information yesterday when we were first told that Sophie had to be sedated and we were then told later that it wasn't necessary....Grrrrrr again... The CT scan didn't go very smoothly, Sophie was kept waiting in a room and then in the corridor.. do these people realise that Sophie has a suppressed immune system...more Grrrrs....Sophie's nurse (and Tina) were very irate...when the scan was eventually attempted it took several attempts to get a successful reading as there was a "problem" with the software. Throughout all this Sophie's arms had to be held above her head which hurts her and she was very upset. Sophie got back to her room at about 4:00pm and fell fast asleep for a few hours. This is the first bad experience we have had at the hospital and Tina and Sophie just didn't need any additional stress today as they were both very tired after a broken nights sleep.  We should have the results of the CT scan tomorrow and we hope that the fungal infection is continuing to clear. It is also ward round tomorrow so all going well we may get a discharge date.

Padraic

07/01/2004 T+119

WBC 6.0 (T-cells 0.8)

Sophie started the day early today by having an hours play at 3:00am. Nicki, the music lady came in this morning and Sophie had a good session. She played very well on the drums but once the keyboard was in range she almost threw herself at it. I think Nicky was lucky to get it out of the room !! The milk feed is now at 40ml/hour so hopefully we can reduce the amount required overnight and stop her being sick in the morning. At teatime I popped in to see Sophie and she was in very good form and very active. We gave her a little soup and fruit puree which she enjoyed. It is the first food she has eaten in over three months. I checked Sophie's file today and the top page was Sophie's discharge sheet which was a nice reminder. Sophie will have a CT Scan tomorrow, as Sophie normally lies very still for her scan she probably won't need any sedation which is good as it take her a day to recover. 

Padraic

06/01/2004 T+118

It was a busy day today and there is lots of good news. Last night Sophie's SATS machine was taken away as she no longer needs her oxygen levels to be checked. It is always a good sign when medical equipment is leaving the room rather than coming in. The results of the bone marrow tests from Friday have shown that everything is normal. As Sophie’s counts are now starting to rise it seems that it was the Septrin that was suppressing the neutrophils. The Septrin replacement is a monthly medication which is given using a nebulizer. In a months time Sophie will be reassessed and we will either restart the Septrin or discontinue it if it is not required. There are no WBC counts today as blood tests will now be only taken every two days. Sophie is now quite stable and the less blood she loses the better. Sophie will have a CT Scan under sedation on Thursday to check the status of the fungal infection in her lungs. On the feeding front we hope to start Sophie on solids in the next day or two. The TPN is now on a minimal setting and will be stopped in the next few days. It was ward round today and the BMT team are delighted with Sophie’s progress.…they said that we can go home next week probably around the 17/18^th  (Saturday/Sunday) ….hooray !!!! It will take a day or two to sink in but it is now a reality. Our local community nurse will be coming in next Wednesday (14th) so that we can meet up before we go home.

We have been through so much in the last few months and we often wondered whether we would ever take our Sophie home. She has overcome a cardiac arrest, GVHD, adenovirus and a fungal infection to get home and she has shown us an inner strength, courage and a will to live that is incredible for one so young. Whatever the future holds for Sophie we can handle it, with love and caring and tears of joy. Sophie is never going to be like other children but she has so many wonderful qualities that she stands out from the crowd. Last week one of the nurses told me that when one of her colleagues found out she was taking care of Sophie she told her it would take only 10 minutes for her to fall in love with Sophie, she said it only took less than five. We are so proud of our Sophie, she has taught me more about life in the past four months than I have learnt my entire life. 

Padraic

05/01/2004 T+117

WBC 4.38 (T-cells 1.33)

Sophie had a good night and although she was a little sick this morning she has been in very good form all day. We will adjust her milk feed so that it stops at 5:00am and gives her tummy a chance to process the milk before she gets up. The occupational therapist came in and has given us a few things to do. We have some new exercises for Sophie which will help her to sit up properly. We will be teaching Sophie some sign language so she can indicate when she wants to lie down and do other things. Sophie's pointing skills will be fine tuned by showing her 2 toys and getting her to point to the toy she requires. The Septrin (anti-fungal medication) has been changed to another drug to see if it is keeping Sophie's neutrophil count down. The new medication will be given via a nebulizer but as it is only a monthly occurrence it shouldn't be a problem. Sophie has been very happy today, there were lots of smiles and giggles and lots of chatting. She only slept for an hour during the day so when I saw her at 5:30pm she was exhausted and ready for an early bedtime. 

Padraic

04/01/2003 T+116

WBC 4.3 (T-cells 0.55)

Although Sophie was a little sick this morning she is continuing to tolerate her feed well at 35ml/hour. We shall be meeting the dietician early to discuss which foods to try as Sophie starts back on solids. She has had nothing by mouth for several months now so we will have to treat her stomach as if she was a young baby starting on solids. The more solids she can handle the less TPN and milk feed she will need and the quicker we can go home. Sophie was a little tired today but as her Hb count is low that is understandable. 

She was delighted to see Amy in the afternoon and she was giggling and laughing a lot. It is wonderful to see our two girls playing together, I think Amy is a more relaxed with Sophie now that her hair has started growing back. She is particularly happy that Sophie's new hair is the same colour as hers. Sophie showed her empathy again this afternoon, when Amy was tired and started to cry Sophie got very upset and started crying herself. Sophie always gets upset when she hears other children crying it is heartbreaking but so moving that she feels so much for other children. I gave Sophie most of her medication through her NG tube today and so far I have not made her sick, Tina started this evening and she was fine too. It doesn't take as long to do as I anticipated and it is another step in the right direction. We know that it doesn't take much to keep Sophie from coming home but we are so happy that as we speak, getting Sophie home in the next few weeks is a real possibility.

Padraic    

03/01/2003 T+115

WBC 3.7 (T-cells 0.78)

After yesterday today was really only ever going to be a recovery day and Sophie has done really well. She is a little sleepy and a bit sore but she is still playful and chatty. Padraic started to learn how to give Sophie her meds. He learnt how to give them via the ng tube, he was worried that he would make her sick but so far it has gone fine. She will need to have 16 doses a day, some of those are the same medicine but she has it three times a day. 

As well as having her hickman line removed and a picc inserted into her arm yesterday, she also has a sample of her Bone Marrow taken.  Her counts have been getting lower recently, this is probably due to her medication but as she was under GA they took a sample to run some tests. We should get the results early next week.  

I still find it hard to believe we might be home soon, I am actually quite scared. It does seem to be a normal reaction, everyone who has gone home seems to feel the same way.  Without the security of all the Doctors and Nurses we are going to feel a bit on the edge, but this is another challenge and we are ready to face it.  

Tina

02/01/2003 T+114

WBC 2.5 (T-cells 0.57)

Sophie was very happy as Mummy and Daddy was both with her to spoil her all day.  I talked to another Doctor about our concerns with the Picc line. He explained that a Picc line could stay in for several months not the 2-4 weeks I was originally told. So we opted for this as although it has one less line of access, there is less chance of infection and when it is removed she will not need a GA. 

It was ward round today and the Consultant was very happy with Sophie's progress. There was even talk of maybe going home in two weeks and in preparation we are to start to learn how to administrate Sophie's drugs. A lot can happen in two weeks but the fact Sophie is well enough for us to think about this is wonderful news.

Sophie went to theatre at 3.30pm and we went to the recovery room at 6pm. She was very sore and upset but after some painkillers, she was awake and happy enough for a quick game of peek-a-boo, she even has a little dance and sing-a-long to her new music tape. 

Tina

01/01/2004 T+113

WBC 2.8 (T-cells 0.9)

Sophie had a good day today and is tolerating her feed at 25ml/hour. It will be increased to 30ml/hour tonight. Sophie is scheduled to have an operation tomorrow to rectify the Hickman line problem. We are still awaiting confirmation of whether a new Hickman line is to be inserted or if a "pickline" is to be used. Amy and I came up to the hospital today and Sophie and Amy had lots of cuddles. Sophie adores Amy and was "patting" her arm and head at every opportunity. Amy, Tina and I managed to get out to a restaurant for lunch which Amy really enjoyed. Sophie was in good form today and continued to play even when her eyes were drooping and she was ready for sleep. She was giggling and laughing, something I haven't heard her do for months.

Padraic

31/12/2003 T+112

WBC 2.5 (T-cells 0.7)

Sophie has a good night and although she was a little sick this morning she has been in good form all day. She has been smiling and waving and doing "hooray" with her arms in the air. The milk feed is now at 25ml/hour and Sophie is continuing to tolerate it well. Sophie had an X-ray today and as suspected her Hickman line has moved and will need to be replaced. While the procedure is a simple one it will mean that Sophie will need to have a general anaesthetic which is obviously a concern. It has been suggested that the Hickman line be removed and replaced with a "pickline" which goes into the arm. The main problem with this solution is that the "pickline" would need to be removed in 2-4 weeks. Sophie will need to give blood samples regularly for the next 6-9 months, as it took 3 different doctors before it was possible to get a blood sample from her arm we don't believe that this is really a viable option. Sophie's cyclosporin has been converted from IV to oral which means that the only regular IV drug she receives is the anti-fungal medication (caspofungin). We are heading in the right direction and if Sophie continues to tolerate her milk feed the exit sign may possibly be in sight. We are very aware that it doesn't take much to set us back but we will enjoy every moment of Sophie's improvements. Sophie's next door neighbour (Kajeeban) came back from ICU today. He is still on oxygen but is much better than he was last week, it is so nice to have him back.

I have added some new pictures, as you can see the cyclosporin has had it's usual "increased hair growth" side effect.

30/12/2003 T+111

WBC 3.9 (T-cells 1.1)

I am pleased to report that today was a very good day. Sophie woke up in very good form and was very eager to play all day. She has been moving about in her cot and kicking her legs at every available opportunity. The milk feed rate was increased to 20ml/hour which Sophie tolerated well, the dietician checked in and is very pleased with her progress. 

The BMT team round took place this morning and they are very pleased with Sophie, they reiterated that the TPN is only thing keeping us here. The "mystery" infection has not been found and as Sophie was well today the antibiotics were stopped. Tests will be carried out in the next few days to check on Sophie's engraftment and also to determine the level of the enzyme which Sophie is missing. It will take a few weeks for the results to come back. I saved the best news until last, the latest viral counts came back and Sophie is now negative for both the adenovirus and enterovirus...hooray!!! Today was a good day. 

Padraic

29/12/2003 T+110

WBC 2.6 (T-cells 0.8)

Sophie had a good night but was a little sick this morning. Her feed was stopped to let her recover and restarted later at a lower rate. Sophie is still a little "shaky" and she seems to be a little stiff, she no longer rolls from side to side in her sleep and is not moving her legs quite so much. The doctors still think that Sophie has an infection although the exact source has yet to be found, it can take up to 5 days to find the infection from the blood cultures. Sophie's temperature has been fine all day and her diarrhoea of the past few days appears to have stopped. The TPN feed has been increased from 70% to 100%, hopefully this increase will give Sophie a little more energy although we shall have to monitor her fluid balance closely to ensure she can deal with the additional amount. The IV specialist checked Sophie's Hickman line today as it has been difficult taking a blood sample from one of her lines, some "clearing fluid" was put down the line which will hopefully resolve the problem. A chest x-ray has also been ordered to check that the Hickman line is still positioned correctly, it is over 4 months since the line was inserted and Sophie has grown quite a bit since then.

Padraic

28/12/2003 T+109

WBC 4.2 ( T-cells 0.78)

Sophie had a slow start to the day. She woke at about 7:00am and then drifted back to sleep until about 10:00am. She was still quite agitated but was better than yesterday. Sophie's weight is up again today (possibly as a result of the additional fluids she had last night) so she was given some additional frusamide to shift the excess fluid. She continued to improve throughout the day and when I left her this evening she was playing with her new toys. 

Padraic

27/12/2003 T+108

WBC 4.4 ( T-cells 0.84)

Sophie was quite agitated today. She is definitely teething and her temperature was high. There is a possibly she has an infection so she was started on some antibiotics.  She was a little "shaky" in the afternoon although the shakiness subsided once she settled down to sleep. In the evening Sophie blood gases weren't very good so she was given some Sodium Bicarb which corrected it. Her heart rate was high and her Sats dropped below 90% for short periods. As a result she was given a little oxygen to help with her breathing. A battery of tests was completed to find the origin of the temperature. From the results so far Sophie's kidney and liver functions are fine and her chest X-ray is fine. We are waiting on results from her blood cultures. Despite all this Sophie had a reasonable nights sleep and continues to tolerate her milk feed.

Padraic

26/12/2003 T +107

WBC 4.4 ( T-cells 1.09)

Sophie didn't have a particularly good day. She was sick a few times so her milk feed has been reduced. We know that getting Sophie off the TPN is the final hurdle that we must overcome before we go home but we have got to tread very slowly with the increases. Sophie has been up to 25ml/hour but we have had to drop back to 20ml/hour while her tummy recovers. If we can get to 30ml/hour and Sophie tolerates it then the TPN can be stopped. We have been told that children with Hurlers normally have great difficulty coming off TPN and that Sophie is doing very well.

Amy and I spent the day at my brother's house with his family and my parents. Amy had a wonderful time playing with her cousins who absolutely adore her (and Amy idolises them). We played "Charades for Kids" and Amy's rendition of "Balloon" was an exercise in simplicity she clenched her hand and held in front of her. It was a lovely day although throughout the day I felt that Sophie and Tina should have been there. Hopefully next year will be different.

Padraic

25/12/2003 T+106

WBC 3.8 ( T-cells 0.62)

Christmas day felt a little strange. Amy and I spent the morning at home opening all the presents Santa brought for her. We got to the hospital at about lunchtime and Amy then helped Sophie to open all her presents.  Sophie wasn't feeling very well for most of the day (still teething) although we did get a few smiles which was all we wanted for Christmas. There was a lovely buffet laid on for everyone and Amy enjoyed "sampling" all the different foods. 

It was a day of mixed emotions, we are so thankful that Sophie has made it this far but we are so sorry that so many of our Hurler's families are spending Christmas without their children. Yesterday the baby next door to Sophie went down to ICU as he was having trouble breathing, thankfully he is now recovering but it just reinforced how fragile these childrens' lives are. 

Padraic

24/12/2003 T+105

WBC 4.5 (T-cells 0.6)

Another good day for Sophie. She was full of energy this morning and is now complaining every time she is put back in her cot. She just wants to play on the mat or in her high chair as much as she can, she will only go to bed willingly when she has exhausted herself. The increase in the milk feed is progressing nicely and if we carry on at the current rate of increase we will be off the TPN by early next week. We can then start trying Sophie back on solids. Amy had a lovely day at Tina's Mum's house playing with her cousins Jessica and Johnathon. 

We would like to thank everyone who checks in on Sophie wish you all a peaceful Christmas. The updates will be sparse for the next few days as we will be spending a lot of time visiting our families. 

Padraic

23/12/2003 T+104

WBC  3.2 (T-cells 0.67)

Adenovirus count - 347 

Sophie was a bit tired today but as she needed a blood transfusion that was understandable. Nana and Granddad from Ireland visited so that must have been a joy to see.

The BMT consultant told us to today that Sophie's recovery will be a slow journey, we have to tread very carefully with regards to milk feeds and solids. I am not sure how long we have to stay in hospital but I do not mind, I would rather take tiny baby steps instead of leaping forwards and ending up going backwards.

We got the official report concerning the CT scan today, although there is still evidence of the fungal infection is certainly shows an improvement. There is still concerns regarding bleeding in the lung, but this was never going to be a quick recovery, so as long as she going in the right direction we are happy. 

Tina

22/12/2003 T+103

WBC  4.5 (T-cells 0.65)

Another good day, I can hardly hear Padraic on the phone with all the noise in the background. She now complains when you put her back in the cot,  she wants to be out on the mat playing.  Its is the little things that fill your heart. She has started moving about her cot at night, you lie her down lengthways and in the morning she is widthways with her feet hanging through the bars.  This is something she used to do before she got ill and the first morning I found her like this I nearly cried. 

We are hoping by the end of the week she will be tolerating 30ml an hour of her special milk feed, if there are no problems we can start her on solids. Sorry no Wotsits or chocolate cake for a while, back to first principals of a little apple mixed with baby rice.

Tina

21/12/2003 T+102

WBC  4.6 (T-cells 1.0 )

Another good night, heart rate a bit high but that could be the teething. She was full of energy this morning practicing her kicking and singing. Daddy has not really seen her like this, as last weekend she had a bit of a relapse, I not sure who is having most fun.

She is still teething quite badly, the dentist said she could see four molars coming through! Her gums were bleeding a bit. I hope they break through soon. Although it does not seem to stop her having fun.

Today Amy and I went to a Pantomine version of Peter Pan, a British Christmas tradition. JM Barrie donated the rights of Peter Pan  to Great Ormond Street in 1929 and due to its never ending  popularity it has raised a great deal of money for the hospital. So thank you JM Barrie and thank you Peter Pan.

"When the first baby laughed for the first time, its laugh broke into a thousand  pieces...and that was the beginning of fairies".         JM Barrie (Peter Pan - 1904)

Tina

20/12/2003 T+101

WBC 4.12 (T-cells 0.9)

Great news Sophie adenovirus count is down to 919, if it carries on like this it will be gone before Christmas. It also looks as the infection in her line is gone and with her CT scan results showing improvements in her lungs we look set for a wonderful new year. Every day she is getting stronger and happier, it is so wonderful to see her play and interact with everyone. 

Teething was her only problem today, how we have dreamt of having just to worry about teething. 

Unfortunately I have come down with a cold so Daddy had to come up early and miss his extra time with Amy. I was very upset this morning, I was so concerned that I had passed the cold to Sophie. I was told not to worry as Sophie was out of the danger now and although a cold would not be good, she could cope. As upset as I was it was lovely to hear those words! I will stay away from Sophie until I am over the worse. 

Tina  

19/12/2003 T+100

WBC 3.2 (T-cells 0.6)

Sophie had a good night, her temperature, blood pressure and heart rate were all fine. This morning her weight was a little up so she was given some frusamide to shift some of the excess fluid. The dietician is not sure whether Sophie is ready to try solids yet, she will speak to the doctors and get back to us.  Sophie had a CT scan this afternoon to check on the fungal infection and it is better than last time. We may have more detailed information on Monday but for now we will settle for it being better. I popped in to see Sophie this afternoon and she was still very sleepy from the sedative, it normally takes her a day to recover. She is also still teething but I still managed to have a "chat" before I left. The BMT team came round this morning and in their own words they are delighted with Sophie. Last week they were "worried" and the previous week they were "very worried" so it is amazing to think of the progress Sophie has made in just two weeks. The adenovirus is under control along with the fungal infection, she is off oxygen and her liver and kidney functions are returning to normal. At the moment everything is heading in the right direction and we are delighted that Sophie will be well enough to enjoy Christmas and play with all the presents Santa will be bringing her. 

Padraic

18/12/2003 T+99

WBC 4.5 (T-cells 0.91)

Sophie had a good night, her temperature, blood pressure and heart rate were all fine. The dentist came in to look at Sophie's mouth as it had been bleeding. One of Sophie's molars is pushing her gum out. Although this is not normally a problem we are obviously concerned as it may be a risk of infection. One of the cranofacial consultants will examine Sophie to determine the best course of action. The infection in Sophie's Hickman line has not been found again in the daily tests since the 13th. If the tests for the 18th December come back clear (on the 23rd) then we will be sure that the infection is gone. In other news an infection has been found in Sophie's stool, it is not a major concern provided it stays in her stools and it will be monitored closely to ensure that it doesn't cross over into her blood. As a result of this infection we have moved to "Red" status,  so it is back to wearing aprons in the room and it marks the end of Sophie's "playroom escapes" for the time being. We have to be even more careful when changing Sophie's nappies to ensure there is no chance of her Hickman line being contaminated. 

Sophie had a session with the physiotherapist this afternoon, although she was quite tired she did some rolling from side to side. We will be meeting the nutritionist to discuss the best foods to try in order to get Sophie back on solids. Getting Sophie off the TPN is the main hurdle we have got to overcome before we can think about going home. 

Padraic

17/12/2003 T+98

WBC 5.0 (T-cells 0.89)

Sophie had a difficult night last night. Her Sats dropped at about 11:00pm which set off the alarm and woke her up, she then accidentally pulled her NG tube out at 1:00am and woke a few more times with dirty nappies and teething pain. Despite all this her heart rate and temperature were fine. The doctors believe that Sophie's Sats may be dropping as she falls into a deep sleep, this is a known scenario and as long as her Sats recover (which they do) it is not a major cause for concern. Another anti-biotic has been started to deal with the infection in her Hickman line. Cultures were taken to ensure that the infection is not a false reading. Sophie's liver and kidneys are now functioning normally, the new antibiotic can affect the liver and kidneys, if there is any noticeable deterioration we may look at stopping the second antibiotic. A CT scan under sedation has been scheduled for Friday. This will give us a better picture of how Sophie is recovering from the fungal infection. The dentist will examine Sophie tomorrow as there has been a little blood on her gums probably due to the new teeth which are cutting through. 

Sophie was in good form today and had a good session with Nicky the music lady. In the afternoon she had a little walk to the playroom. Amy came up to the hospital this morning and went to the hospital's children's party. She had a lovely time and came away with a Christmas "snow scene" which she loved. On her way home Amy was wearing a pair of "reindeer antlers" and a red flashing nose. She got on the train, sat down and then said to the man sitting opposite her, "Sing Rudolph the Red Nosed Reindeer". The poor man had to sing "Rudolph the Red Nosed Reindeer" all the way from Finchley Road to Harrow-on-the-Hill, what a good sport !!

Padraic  

16/12/2003 T+97

WBC 5.1 (T cells 0.67)

Sophie had a reasonable night, her heart rate was up and although her Sats dropped periodically she managed to stay off the oxygen. She was given some codeine for her teething and settled down around midnight. Sophie was a little sick this morning, her weight was up so she was given some frusamide. The physiotherapist came in but Sophie wasn't up to doing very much. She did however sit up for a while on her mat. The BMT team were on their rounds this morning. The adenovirus count is now 2,800 which is down from 7,000 last week so it is just about gone. An infection has been found in Sophie's Hickman line, this is not too serious and as she has already started a course of antibiotics we hope it will clear up quickly. Sophie will also have another CT scan possibly later this week to check on the current state of the fungal infection. Everything else will carry on as normal. The main things to work on are increasing the NG feed and starting Sophie back on solids. We are going to try some fromage frais later in the week. Once Sophie is off the TPN, is taking more food orally and her medications are being tolerated through her NG tube we can start to thing about the possibility of going home. We are not going to get too excited about going home just yet but it is nice that it is being talked about. I spoke to Tina tonight and Sophie has shifted the excess fluid she had this morning. She was very brave this evening as her NG tube had to be replaced and Mummy got lots of cuddles before bedtime.

Padraic

15/12/2003 T+96

WBC 5.7 (T-cells 0.64)

Sophie had a good night and slept until 9:00am, her weight was up a little but as she is not too bloated and is weeing regularly no action needs to be taken. Sophie's kidney and liver functions continue to improve along with her blood pressure for which Sophie no longer requires regular medication. Her temperature was back to normal this morning and was good all day. Sophie was taken off the oxygen this afternoon and her Sats have been fine ever since. 

Sophie has been very happy and chatty today, she played well on her piano and even tried some water from a cup in the afternoon. Once she was free from her TPN she played on her mat and spent some time on her tummy trying to push herself up. She then went for a walk down the hallway to the playroom.

Padraic

14/12/2003 T+95

WBC 5.4 (T-cells 0.7)

Sophie went back on the oxygen last night, her Sats kept dropping while she was asleep so she was given a very low dose to help keep her Sats at 100%. She is retaining a little fluid so she was prescribed albumen and frusamide to help her "process" the excess fluid. Sophie has been teething quite badly for the past few days and her temperature has been hovering around 38 degrees C. Blood samples were taken to be cultured. Nothing has been found but Sophie has started a course of antibiotics just in case there is a virus lurking about. 

Sophie slept a lot today, whenever she was awake she was in good form and very interested in playing and was very chatty. Amy came up this afternoon but as Sophie was asleep she just said a quick hello. Amy was very impressed by Sophie's "bedlights", she said she thought they were beautiful.  Unfortunately there was no "escape" outside today, by the time she was free of all her lines at 5:30pm she was asleep. Sophie's TPN times have been altered so that she is free from 2pm to 6pm each day, this will give her the opportunity to go for walks to the playroom and it makes bath time much easier.

Padraic 

13/12/2003 T+94

WBC 5.8 (T-cells 1.4)

Another quiet day, Sophie plays energetically then tires herself out and has another a sleep. She is still teething quite badly and is not happy about being moved. She had another wander up and down the ward which she enjoyed but it takes a lot of effort holding her head and body up straight in Daddies arms so she fell straight to sleep once she got back to the room. 

Her milk feed has been turned up to 10ml an hour which she seems to be tolerating. Next week I would like to try to see if she will take some water from her beaker, I have to check it out with the doctors.  

Weekends are a quiet time on the ward, it is strange the same things seem to happen but without the outpatients the hospital seems sleepy. Padraic makes the most of his time with Sophie and hopefully Amy will come with me tomorrow for a visit, which creates its own excitement.

Tina 

12/12/2003 T+93

WBC 12.1 (T-cells 1.9)

Sophie had a good night, however as she is teething her heart rate was up a little and I was woken up regularly by the heart monitor alarm. The BMT team came in today and they are very pleased that Sophie has managed to do well following some challenging infections. The oxygen levels in Sophie's blood are very good so hopefully we have seen the end of the oxygen tube. Sophie will have a CT scan next week without sedation to check on the status of the fungal infection. The next step is to get Sophie feeding through her NG tube and then start her on oral feeds. 

Sophie played really well today, she gets so excited it is such a pleasure to watch. I captured her on video today so hopefully we can share this with you soon.

The adenovirus counts stayed the same which is really good. 

Tina

11/12/2003 T+92

WBC 7.0 (T-cells 0.9)

Sophie had a good night and has been off her oxygen since yesterday afternoon. Her blood pressure and temperature have been good all day. The occupational therapist came in this morning and Sophie had a nice play on the mat. She was sitting up well but she required all her strength to stay upright. A new firmer playmat has been ordered to help with Sophie's movements. She has been teething all day and wasn't as happy as she was yesterday. This afternoon all Sophie's lines were disconnected so we took her on a trip to the playroom which she enjoyed very much. It was another good day it's just a pity that Sophie's teething is spoiling her enjoyment, then again it is wonderful that teething was the only thing bothering Sophie today. 

Padraic 

10/12/2003 T+91

WBC 7.0 (T-cells 0.55)

Sophie had a good night, her liver and kidney functions continue to improve so hopefully the reduction in medications has done the trick. Sophie had session with Jess the play specialist this morning. She was so excited playing in her chair she kicked her monitor off her toe she was kicking so much which set the alarm off. The nurses rushed in to find Sophie giggling and rocking in her chair. Nikki, the music lady also came in this morning and Sophie had a great time, she practically threw herself at the keyboard when it was put on her bed (I think Sophie may be asking Santa for a keyboard for Christmas). In the afternoon Sophie was unattached from all her lines so she "escaped" into the hallway for a walk with Mummy. She really enjoyed the decorations and her little bit of "freedom". Sophie has been off oxygen all afternoon and her Sats have been good even while she has been asleep. Sophie had a wonderful day.

Padraic

9/12/2003 T+90

WBC 6.9 (T-cells 0.7)

Sophie had a good night although she was a little sick this morning. Her legs and arms were a little red but they responded well to the steroid cream. Sophie is teething which is pushing her temperature up a little, she is gaining a little weight so she was given some Frusamide to help her wee. The blood pressure medication will now be given on demand rather than every 4 hours so last night Sophie had no overnight IV drugs for the first time in months. 

The BMT team came around this morning and it is all good news. The adenovirus count is now 7,000 and as anything below 10,000 is not usually registered Sophie's adenovirus is technically negative. Her liver function has improved over the past few days so hopefully the reduction in medications has done the trick. They are very happy with Sophie's progress in relation to the Fungal infection and are pleased that she is not totally dependant on the oxygen. Sophie will have a CT scan in a few weeks time to check on her lungs. As there are no symptoms we will hopefully see the Enterovirus disappear from our list of medical concerns. Sophie's counts continue to be good which seems to be the key to her ability to fight off all these virus' and infections.

In preparation for Christmas Sophie's bed has been decorated with some flashing lights. She really likes the lights and likes to play with them whenever our backs are turned. 

Padraic

8/12/2003 T+89

WBC 5.7 (T-cells 0.7)

Sophie had a good night. She decided not to play her "Call Dada or Mama and then pretend to be asleep game". She had some Frusamide last night as her weight was up to 10.0 kg. The play specialist and occupational therapist came in this morning and Sophie had a good play in her chair. She was trying to rock the chair so the safety bar was taken off and she had a good "rock". Sophie's right side is a little weak so we will work on it over the next few days.

Sophie's liver function is worse again today, there are three possible reasons for this. Firstly, it could be GVHD in her liver, however this is unlikely as Sophie's skin and stomach have been ok. Secondly, it could be the adenovirus; again this is unlikely as the virus counts have been down for the past week. The most likely reason is that Sophie's medications are affecting her liver. As a result Sophie's anti-sickness medication, her regular paracetemol and a drug she receives to regulate her gastric gases have been stopped as they all affect the liver. Sophie's liver function will be closely monitored over the next few days. If there is no improvement we may have to consider carrying out an biopsy on Sophie's liver. This is something we really don't want to have to undertake as Sophie is still on regular oxygen and is not strong enough to have a general anaesthetic.

I popped in to see Sophie this evening and she looked very well. She sat on my knee and she did something she hasn't done for months, she smiled. It was the most wonderful thing to see her beautiful smile again. After I left, Sophie took another trip out into the hallway. Tina said she was very excited and she had difficulty holding Sophie in her arms as she reached out to touch the Christmas decorations.  

Padraic

7/12/2003 T+88

WBC 4.6 (T-cells 0.4)

Sophie had another good night. She has however started to say "Dada...Dada" in her sleep which meant I jumped (ok crawled) out of bed several times to find Sophie sleeping peacefully looking very comfortable. Sophie was sleepy again today, her temperature and blood pressure were fine and all in all she was in good form and played well.  Her nappies have been fine and there has been no blood, thank goodness, for the past few days. Amy came up to the hospital today but could only wave to Sophie through the window as she has a cold which was a disappointment for her. Amy really misses her baby sister, we have already explained to her that we have told Santa to bring Sophie's presents to the hospital so Amy doesn't need to worry. Amy is really looking forward to Christmas and we hope that Sophie's steady progress continues so that we can give Amy the Christmas she deserves.

Padraic 

6/12/2003 T+87

WBC 5.7 (T-cells 0.7)

Forgot to say yesterday the virus load for the Adenovirus stayed the same which was great news.

Another good night and a quiet sleepy day. One exciting thing Sophie was completely unattached apart from her oxygen for a couple hours. So this was seized upon and Sophie went for a walk up and down the ward, this entailed Debbie following closely behind with the oxygen canister. 

Debbie is one of the fantastic nurses at Great Ormond Street, words cannot express the gratitude we feel towards them. There have been times I have sat holding Sophie's hand; singing to her; watching her trying to breath and worrying that she was going to have another attack. The only thing that kept from cracking up was knowing they were there, in the room giving her the care she needed, ready to act if necessary.  Thank you never seems enough.

Tina

5/12/2003 T+86

WBC 6.0 (T-cells 0.42)

Sophie had the best night this week, she wasn't sick and her temperature and blood pressure were fine. There has been no more blood in her nappies and less in the stomach.

The BMT team came around this morning. The bleeding is still a worry as it can be very dangerous if she has a big bleed. They are happy that clinically she is getting better but still monitoring her very closely. Sophie will not have a CT scan on Monday as she is improving and we won't learn anything useful from a new scan. We will carry on with the same treatment. 

They are not too concerned about the enterovirus found in her CSF (brain) and blood. It is difficult to determine the significance of its presence and the effect, if any, it it is having on Sophie.  

Sophie's kidney function is a little better today but as her weight was up this morning to 9.9kg from 9.5kg last night, she was given some frusamide. It is a balancing act now between not letting her get do overloaded with fluids but not letting her get to dry because of her kidneys. 

The milk feed will be started again and we will try to build it up slowly. 

Sophie was a little grizzly this morning but she perked up in the afternoon. She really enjoys kicking on her new air matress as she bounces up in the air every time she kicks.

Tina

4/12/2003 T+85

WBC 7.1 (T cells 0.24)

Sophie had a reasonable night, she was sick a few times and had a temperature but she eventually settled at about 1:00am. There has been some blood in her sick and in her nappy the source of which has yet to be found. Sophie's skin has been fine so hopefully it is not the GVHD coming back. One of the surgeons came in to discuss the bleeding. He said that although they can't be certain but the blood is probably coming from her stomach rather than her lungs. If it gets worse there is a procedure that can stop the bleeding which can be performed without an anaesthetic. Although it is unlikely that Sophie will need surgery, the surgical team will be prepared in case the bleeding gets worse. 

Sophie had a new NG tube put and she was very brave while it was being done. She was taken off the oxygen and her Sats remained good even when her temperature went up and when she was sleeping. She went back on the oxygen in the afternoon as her heart rate was up and she was shaking. The physiotherapist came in and was very impressed with Sophie's progress especially her leg movements. She said that once Sophie starts to feel better she should improve in leaps and bounds as she is very keen to play. I checked in on Sophie this evening. She woke up when I sat down beside her and when she saw me she reached out, squeezed my hand and said "Dada".......which melted my heart. 

Padraic

3/12/2003 T+84

WBC 6.4 (0.4)

Sophie had a rough night, she was being sick and there were small amounts of blood in her sick. The origin of the blood has yet to be found, it could be from her throat as she has been coughing quite a lot. Sophie has not lost any quantifiable amount of blood so hopefully it is not too serious. Sophie's blood gases indicate that her kidney function is a little worse. The renal consultant examined her and has made some recommendations. The blood pressure drugs will only be given when necessary and a new type of diuretic prescribed. She also said that Sophie was "a little dry" and that she would benefit from more fluids, however we don't want Sophie to start retaining too much fluid again. I suppose it is the same as the treatment for water retention being to drink more water to flush the "excess" fluid from your body. Sophie was been taken off the oxygen this afternoon, which she was very happy about as it was irritating her greatly. Sophie's nappies have been better today and her skin has been fine. She was put back on the oxygen tonight as her Sats drop when she sleeps.

Sophie has been playing very well and kicking her legs. She has been very vocal saying "Mama", "Dada" and "Ama" which I think is Amy. I am sure she is getting louder everyday so she can be heard over the ceiling fan. 

Padraic

2/12/2003 T+83

WBC 4.5 (T-cells 0.3)

There is some good news to start with. We got the latest adenovirus results today and the count is now 20,000. This is a big drop from the 490,000 on Friday. It looks Sophie has been kicking that virus out of her system over the weekend. Sophie's weekly antibiotic to treat the adenovirus has been stopped, so there is one less medication for her kidneys to deal with.

The BMT team had their round today and the CT scan was discussed. Although the scan is worse than last week it is not that much worse. They are very happy that Sophie is breathing well and will carry on with her current medication. The renal consultant will examine Sophie and advise on how best to maintain her fluid balance. Sophie has been coughing a lot today and when she has been sick there have been bits of old blood. We need her to cough up some mucus so it can been examined in order to determine if there is any blood in her lungs. To this end the physiotherapist came in and helped Sophie to "clear her throat". Sophie was taken off her oxygen for 5 minutes this morning and her Sats stayed above 97%. The oxygen flow rate was halved and we aim to stop it once Sophie has stopped coughing. We are still keeping a close watch on Sophie's diarrhoea as we don't want her GVHD to start coming back. The virus which was found in Sophie's CSF sample has also been found in her blood. It is called an Enterovirus and it can cause infections. Hopefully Sophie's "super T-cells" will give it the same treatment that her adenovirus got. Sophie was given an extra dose of a blood product known as "flabbagamma" which will help her to fight the virus.

Sophie has been playing very well today. I phoned this afternoon and all I could hear in the background was "Dada...Dada". She has been practicing on her piano in preparation for her next music lesson. We treasure every good day that Sophie has, we save them in our memory banks and we return to them on the bad days. Today has been a day of mixed emotions, we are delighted that the adenovirus counts are down, we are so happy to see Sophie is making progress in getting rid of the fungal infection but we are concerned about this enterovirus. 

I looked at the counter on Sophie's website today and saw that it has been accessed over 5,000 times since October. The support we have received throughout Sophie's transplant has been overwhelming and we have made so many new friends. Thank you all for watching over our darling Sophie, your support helps us more than you could ever imagine. 

Padraic

1/12/2003 T+82

WBC 8.2   (T-cells 0.4)

Sophie was sick again last night so her milk feed was stopped for most of the day while her stomach recovered.  Sophie had a CT scan this morning (without sedative) to check the state of the fungal infection in her lungs. She was very brave and stayed quite still through the whole procedure although it was clear she wasn't very happy. Not having the sedative means that she doesn't spend a day recovering from the scan. We got the results of the scan this afternoon and despite the fact that Sophie has improved in the last few days the infection in her lungs is worse than last week. The BMT consultant will discuss the scan with one of the infectious diseases consultants at the hospital and report back to us tomorrow. Although we are not doctors we asked if we could be looking at the increase in infection which peaked on Thursday and has since improved. The doctor said that this is a possibility as it takes a long time to see an improvement on the scan. There is no rush to alter Sophie's treatment as a result of the scan, she is doing really well and while she continues to improve her treatment will be determined by Sophie rather than the CT scan.

Sophie has been sleeping quite a lot today, she is probably catching up on the sleep she lost last night. I popped in to see her tonight and she was in very good form. She was showing off for the nurses, kicking her legs and even rolling from side to side, something she hasn't done for weeks. 

Padraic 

30/11/2003 T+81

WBC 6.0 (T cells 0.3)

Another lovely quiet day today. Sophie's breathing is getting better and she is using her stomach muscles less and less. She slept a lot today which understandable following the past week's trials. Looking at her sleeping I think about how much she has been through in the last few months and how much she continues to amaze me. Only a few days ago it looked like we would have to take a trip to intensive care and now she is sleeping peacefully without a temperature and with normal blood pressure. As soon as she wakes up she is chatting, wants to play and show off in front of the nurses. I am so proud of her. She has been sleeping very well over the last few nights (she didn't wake up until 8:00am this morning) so hopefully Mum will be able to catch up on some much needed sleep tonight.

Padraic  

29/11/2003 T+80

WBC 8.4 (T-cells 0.2 )

Sophie continues to improve, she is still working at her breathing  and coughing but no high temperatures or sickness. We were very close to her being ventilated again but hopefully she has turned a corner and will continue to improve. Her skin seems very fragile at the moment all her wires are cutting into her, even the blood pressure cuff is causing problems. So today she was given an air mattress which seems to worked as Padraic said she looks very cosy.  

She is tolerating her milk feed, she is getting 3ml an hour which will slowly be built on. We have to be very careful she is still retaining fluid so this will be a slow process.

Padraic had a lovely day today she was quite sleepy but in between she was playful and he even got a few da-da. 

We are both filled with a very mixed bag of emotions. At one end we are both scared, there is so many things to worry about. At the other extreme we are so incredibly grateful that Sophie it is still here, still fighting and still playing peek-a-boo. 

28/11/2003 T+79

WBC 8.1 (T-cells 0.1)

Sophie had a good night and was quite playful today when she was not sleeping. Her temperature only went up to 38 for a short time but most of the day stayed down. She was breathing much better then the last few days. Her weight was up to 9.9kg and she is looking a bit puffy and so she was given additional frusamide. This weekend she is going to start having a small milk feed again. We hope this will help her tolerate her medication and aid her fluid balance. 

The BMT team visited today and they are worried about Sophie's condition although it is good that she hasn't deteriorated in the past few days. Sophie's T-cells have dropped recently which they said is hopefully a sign that a large number of her T-cells are in her lungs fighting the infection. The fungal infection is not "holey" which is good as that is when it starts to cause permanent damage. The EEG (brain scan) was fine. 

The latest adenovirus count has gone up to 440 thousand again this is not a big jump but it is going in the wrong direction.

Tina

27/11/2003 T+78

WBC 12.8 (T-cells 0.01)

Sophie had a good night. She had difficulty breathing this morning but once her temperature had come down she was breathing more easily. She had a busy afternoon with an EEG (brain scan) and another CSF sample taken from her shunt. The previous CSF sample showed the "footprint" of a virus from the polio family. It is not something that is normally seen in the brain so another sample was taken to determine if there is actually a virus or just it's "marker". The EEG was to check if Sophie is having any seizures, she has been staring into space a little recently and has had a few "body shakes". We were talking to one of Sophie's doctors today and she said we are in a "day to day" situation. Each day that Sophie is able to breathe for herself is another day that her body and her medication has time to fight the infection. 

After I left the hospital I met up with Amy and Tina's Mum at the local Pantomime evening. We had a great time, Amy was showing off her Irish Dancing steps and she went on a steam train with her Mormor (Tina's Mum). We went on the "spinning cups" together and it was so wonderful to look into her face and to see her so happy as she waved to on the onlookers shouting "hello everybody". Our time with Amy at the moment is very limited so it was wonderful to have to fun with her. 

Padraic

26/11/2003 T+77

WBC 13.0 (T-cells 0.3)

Sophie had a rough night. She was being sick and had difficulty breathing. She seems to be using her stomach muscles to help her breathe which is stopping her sleeping. Sophie now has an oxygen tube under her nose as her Sats were dropping. She has been taken off the Milk feed and will receive TPN only. Her chest sounds clear but she is not very settled. We were shown the CT scan results from Monday and the infection in Sophie's lungs was pointed out. Sophie's legs were a little red but thankfully they responded to the steroid cream. We don't know at what stage we will have to look at putting Sophie on the vent but it is a distinct possibility if she has any more difficulty breathing. Sophie had an ultrasound to check her tummy and kidneys. It is all fine although her kidneys are a little read. It seems that all the drugs Sophie has for the virus and infection affect the kidneys so they have to monitored closely. The adenovirus counts came back today and although they hasn't dropped it is still in the same range of counts. Sophie will be given a weekly antibiotic to keep it under control.  I checked in on Sophie this evening and she was quite settled, her breathing is quite good if a little forced. The eye specialist checked Sophie's eyes for fungal infections and they were all clear. The ICU consultant dropped in to see Sophie and let us know that they will be ready for Sophie if she need to go on the vent.

Please say a prayer for Mighty Max's family tonight. Max passed away today after a long and courageous battle.  I can't put down in words how we feel about Max and his family. Max has been a inspiration to us all and his family have been an incredible source of support. Even though Max was nearing the end of his life they took the time to check in on Sophie and offer words of encouragement. Mike and Margaret always seem to know exactly what to say to lift us. One of the first things I read when Sophie was diagnosed is that children with Hurlers are born to incredible parents. I must admit I didn't believe it until I came into contact with Mike and Margaret. They truly are very special people. To Max and your wonderful family I wish you a peaceful day.

Padraic

25/11/2003 T+76

WBC 8.5 (T-cells 0.2)

Sophie was quite sleepy today as a result of the sedative yesterday. The BMT visited and the brain CT scan is all clear (one less thing to worry again, thank goodness). The primary concern is the fungal infection. It needs to be kept under control so that no permanent damage is done to Sophie's lungs. The rapid response team at Great Ormond Street have been informed of Sophie's condition and will ensure that all the necessary equipment is ready should Sophie need to be intubated at short notice. Sophie is being given a little oxygen to keep her Sats up. We are still waiting on the adenovirus counts which due today, hopefully we will get them tomorrow. Sophie's fluid balance has been a little positive today so she was given some frusamide to make her wee. A lot of the drugs Sophie is receiving can affect the kidneys and so monitoring and maintaining her fluid balance is very important. We are both very scared about this infection as we are very aware how it can progress. We hope that Sophie can fight this and that she continues to show her incredible inner-strength.

Padraic

24/11/2003 T+75

WBC 8.6 (T-cells 0.4)

Sophie had a reasonable night although she was a little warm all night. At 7:30am her SATS (blood oxygen saturation levels) dropped again and she was put under the oxygen mask and given additional IV fluids. The mask was kept on all morning as Sophie had difficulty breathing.  By mid-morning she only needed to have the mask next to her and she eventually went to sleep. She had a temperature all morning which did not respond to paracetemol so Sophie was given Nurofen and another drug to "settle" her stomach. 

Sophie had a CT Scan this afternoon and the Fungal Infection is worse. It is now in both lungs and although it is not too bad at the moment, it could become very serious. The fungal infection is causing Sophie's SATS to drop and her breathing is sometimes "forced". Sophie will be given oxygen whenever her SATS drop too much. An additional anti-fungal medication has been prescribed and Sophie has been taken off her steroids. Sophie needed steroids to keep the GVHD under control but the steroids "help" the adenovirus and fungal infection to grow. As Sophie's GVHD is under control she will be taken off the steroids so she can fight the infection. There are a few factors in Sophie's favour as she fights the fungal infection. Sophie's WBC is very good and she is producing the large number of neutrophils needed to fight the virus. The steroids have been stopped so we are not trying to balance two conflicting treatments and the infection has been spotted very early. 

Despite all this Sophie has been quite happy and babbling away. As I was not there she has been saying "Dada" all day, all she said yesterday was "Mama". I will take that to mean that she wants us both with her all the time. Unfortunately, we can't manage that very often but it is nice to be needed. When Sophie went for her CT Scan she was sedated but not asleep. She really enjoys the trip to the scanning room and was blowing kisses to the doctor and chatting the whole way there. Sophie doesn't get out of her room anymore so the change of scenery was very exciting. When Sophie was placed on the scanner (sedated but still very much awake) two foam pillows were placed either side of her head to keep her still. Sophie was less than impressed by this so she grabbed one of the pillows and threw it at the nurse. That's my girl !!

Padraic

23/11/2003 T+74

WBC 7.8 (T-cells 0.3)

Sophie had a reasonable night although she was sick a few times. She had a high temperature most of today and was very grizzly. In the afternoon Sophie's SATS (Oxygen levels in her blood) dropped and she was given additional oxygen and IV fluids which settled her down. As she was being sick she wasn't keeping her medication down. She was given IV paracetemol and by teatime she had settled down although she was very tired. Hopefully she will have a good sleep tonight and be back to usual self tomorrow.

Padraic

22/11/2003 T+73

WBC 10.8 (T-cells 0.6)

A quiet day for Sophie which she deserved after all her checks and tests of last week.  She slept quite a lot and was a bit grizzly.   

We are playing a waiting game at the moment every day she does not have any problems with the GVHD is another day she is can concentrate on fighting the virus. We are playing another waiting game too every day waiting for her counts, twice a week waiting for her virus load and constantly waiting for the results from various tests and examinations.

What we try to do is to concentrate on the moment the time we spend with our children: playing, caring and loving.  

Tina 

21/11/2003 T+72

WBC 8.36 (T-cells 0.84)

Sophie had a good night but was quite sick this morning. She spiked a temperature and was given Nurofen and Paracetemol to reduce it along with some Codine as she seemed to be in pain. She is teething which seems to make her feel quite bad at the moment. As Sophie has been putting her hand to her ear a lot lately when she has her CT scan on Monday she will have her head, ear and stomach checked. The BMT team came around this morning. One of Sophie's anti-biotics has been stopped as she does nothing has been found which would require this drug. This antibiotic can cause fevers and sickness so maybe Sophie will feel a little better once it has left her system. (An antibiotic drug which can cause fevers ???). The BMT team are very happy with Sophie's counts. Her WBC and T-cells are good and the increase in her platlet count means than she can be given Nurofen. This is good as Nurofen seems to work much better on Sophie. As the counts are good the BMT team believe than we should see some improvements soon. Sophie's diarrhoea has been very good and her skin has not had any major rashes. As a result it was decided that the weekly antibody will not be given unless the GVHD returns. Hopefully with the changes in her medications Sophie will stop being sick soon. Sophie had a small rash this afternoon but it cleared quite quickly once some Hydrocortisone cream was applied. The Ear, Nose and Throat specialist came in the afternoon and gave Sophie's ears the all clear. The virus tests on the fluid from Sophie's shunt have come back all clear. Sophie has not been shaking as much today, she was examined by a neurologist who couldn't find any problems. She said that if Sophie's sickness was related to her shunt she would be a lot sicker by now. The CT scan on Monday will be checked to ensure that her shunt is working properly. I have saved the best news until last...Sophie's virus load count is down from 390,000 to 189,000, so she is kicking that nasty virus out of her system.

Although Sophie has a bad morning she was in very good form in the afternoon. When I spoke to Tina I could barely hear her above Sophie's babbling. Tina told me that Sophie said "Mama, Dada" this afternoon. Today was a good day.

Padraic

20/11/2003 T+71

WBC 8.4 (T-cells 0.8)

Sophie had a reasonable night following her "funny turn". She was given some IV fluid and medication to deal with the temperature. She still had a high temperature early this morning but it went back to normal by 9:00am and stayed down all day. The BMT doctor came in and believes that the "turn" that Sophie had last night may be related to the antibody she received last night. Sophie has cut down on her steroids and only gets them every other day. She didn't get any steroids yesterday which may have been the problem. Next week when she has her weekly antibody she will be given a steroid dose along with piriton and paracetemol. Sophie will have a CT scan of her head on Monday when she has her follow up fungal infection scan in order to check that her shunt is working correctly. Sophie will also be examined by a nuerosurgeon to see if her trembling could be related to her hydrocephalus. All of Sophie's medication will be looked at by the BMT team to see if there are any drugs or combinations of drugs which could be causing her trembling. Sophie weight is down a little (9.1kg) so an additional element (called Limpids sp?) will be added to her TPN feed.

Sophie slept a lot today but when she was awake she was very happy and played very well. She played "push Mummy away with my feet" in the afternoon to build up her leg muscles for piano playing. When she was sitting on my knee she kept trying to slide off so I think she wants to get mobile again. She really loves her new mirror and is still working out how it works. Each time she sees her toy in the mirror she throws the toy away and tries to pick up the "toy in the mirror" which has now "mysteriously disappeared". It's lots of fun. 

Thank you all for the guestbook entries and support. It helps us more than you can believe to know that Sophie has so many friends praying for her.

Padraic

19/11/2003 T+70

WBC 5.5 (T-cells 0.5)

Sophie had an unsettled night. Her blood gases were low so she was given some additional fluid through her IV line. Sophie's heart rate was up and she spiked a temperature at about 5:00am. She was given some nurofen and settled down to sleep until 8:30. The physiotherapist visited today and is happy with Sophie's progress. She explained that she may take a few steps back if she is not feeling to well and not to be too concerned. Sophie is using her legs much more and she likes to swing them when she is sitting on your knee. I popped in to the hospital at lunchtime today and Sophie was is quite good form considering how grotty she is feeling. She is still trembling periodically and her hands/feet are very cold. Sophie is now rolling onto her side in her cot which will hopefully help her to sleep better. Nikki (the music lady) came in today and Sophie had a great time playing along on the piano.

We bumped into another BMT family today and it was wonderful to hear how well their son is doing. Oscar had a condition called SCIDS (Severe Combined Immune Deficiency Syndrome) and had a bit of a rough time through his BMT. He looked so peaceful sleeping in his pushchair and it was very thoughtful of his Mum and Dad to drop in to the ward to say hello.

Sophie wasn't very well this evening. She had trouble breathing and her temperature and heart rate were up. She had a chest X-ray and we are waiting for the results. The doctor examined Sophie and believes that her high temperature was the cause. Sophie was given some nurofen and paracetemol to bring down the temperature. She settled back down after an hour but it was a very scary hour as her heart rate was over 200 bpm at times.

18/11/2003 T+69

WBC 5.8 (T-cells 0.65)

Sophie's virus counts came back to day and they are up from 50,000 to 390,000. As a result, Sophie has restarted a daily antibiotic. As Sophie's GVHD is under control her steroids have been reduced again. This should help stop the virus counts increasing as in medical terms the adenovirus "likes" steroids. Sophie will continue to get an antibody (Infliximab) on a weekly basis until we are sure that the GVHD has gone. A "liquid sample" was taken from Sophie's shunt and analysed, the results came back tonight and it is all clear. In other good news the MPA (nasal fluid sample) has come back negative so the virus is not in Sophie's lungs. Sophie had a blood transfusion this afternoon as her Hb level was down. Sophie will have a CT scan every 10 days to monitor the fungal infection.

Sophie had a good morning and played well with Jess her play specialist. In the afternoon however Sophie was a little "shaky" and had a temperature which didn't respond well to paracetemol. The shaking may be the virus or possibly related to a drop in her Cyclosporine levels. Sophie was sick quite a lot which meant that her paracetemol wasn't being absorbed. I popped in on my way home and although I got a nice cuddle Sophie was feeling very nauseous and was sick a few times. She was given some Nurofen at teatime and settled down for a sleep. I spoke to Tina at 9:00pm and Sophie has been settled ever since. 

Sophie's friend Max is having a rough time at the moment, please keep him in your prayers.

            Max's Website             

Padraic

17/11/2003 T+68

WBC 4.1 (T-cells 0.4)

Sophie had a good night last night. She was ill at 6:00am and then slept until 9:30am giving Tina some much needed rest. Sophie's weight is up again today to 9.8kg (from 9.6kg yesterday), her fluid balance will be checked later today and Sophie will be given some frusamide (diuretic) if needed. I believe that the anti-fungal medication can affect the kidneys so perhaps this is why Sophie is retaining a little fluid. 

I checked in on Sophie on my way home tonight and she was in the best form I have seen her in weeks. She sat on my knee and we played for ages with her new toy mirror. The mirror is about 2 foot square so Sophie can play peek-a-boo by herself. She was smiling and trying to kiss "the baby in the mirror" but unfortunately she only succeeded in bumping heads. Sophie was kicking her legs and bouncing along to the music from her piano. It is so nice to see Sophie so happy, flirting with the nurses and grabbing my glasses. We are still a long way from the finish line but it lifts our spirits so much when she is in such good form.

Padraic

16/11/2002 T+67

WBC 2.7 

Sophie has a restless night. I think she was feeling queasy and found it difficult to settle. She was sick a few times and eventually went to sleep at about 5:00am. She was a lot happier during the day and once she had a good midday nap was ready for some playtime. Sophie has been given an additional anti-sickness drug in the hope that it may stop her feeling so sick. Another quiet day and hopefully Sophie will have a better night tonight. I just spoke to Tina (8:45pm) who told me that Sophie was much happier after I left at teatime. Was it something I said ?

Padraic

15/11/2003 T+66

WBC 3.4

T-lymphocytes (T-cells) 0.4 

A short update today not much to report.

Sophie still feeling the effects of yesterdays sedation and has being sleepy for most of today. 

She still had a low grade fever but on the plus side she has not been sick since early yesterday morning. I can tell she still feels queasy a lot of the time and stomach rubs were the order of the day today.  

Amy visited today but was a little anxious as Sophie was not feeling great and looked a bit puffy, so we did not stay for long.  

Tina

14/11/2003 T+65

WBC 4.2 

Sophie had a restless night, she spiked a temperature and was sick at about 5:00am.  The chest x-ray from yesterday came back all clear. As Sophie has had a lot of temperature spikes over the past few days she had a full body CT scan this afternoon. The purpose of the scan was to determine whether there is any other infection in Sophie's body which could be causing the temperature spikes. We got the results late this afternoon and Sophie has a mild fungal infection in her right lung. The BMT team are not worried about this as it is very mild. It will be treated with a course anti-fungal medication. The BMT team are very happy with Sophie's progress, the details are as follows.

GVHD: 

Sophie has completed the course of antibodies. Her sick is rash free and her diarrhoea has cleared. As a result of this Sophie's steroids were halved again today. As the steroids can affect Sophie's ability to fight virus's (and increase her blood pressure) we are happy that the dose is now very small (only one dose per day).

Adenovirus:

Sophie is fighting this virus with her own T-cells and antibiotics. As the virus counts are very low we have been able to reduce the antibiotics she is receiving. Sophie's T-cell count (a subset of the WBC count) has been averaging 0.3 and above which we are told is the minimum level required to clear the adenovirus. If her T-cell level was below this, say 0.1, Sophie would have more trouble fighting the virus. It will take a few more weeks before we can expect the virus to be gone.

Feeding:

Sophie is receiving a combination of TPN and milk feed through her NG tube. The milk feed is slowly being increased with a view to eliminating the TPN. We may try Sophie on some solid food soon.

Sophie's hair continues to grow and the jury is out as to what colour it will be. Some parts are dark while others are blonde. I will load some pictures soon. Clinically Sophie is doing really well, she has regained nearly all the skills she lost when she went to the ICU. She is playing really well and doing new things every day. She has also started babbling again. I gave Tina a kiss goodbye today and Sophie immediately started blowing kisses.......I think she was saying "Parents can so embarrassing sometimes".

Sophie's friend Max has come through his surgery today like a champ. Please keep him in your prayers.

            Max's Website             

Padraic

13/11/2003 T+64

WBC 3.2

Sophie has a restless night and was sick a few times. As a result the flow rate on her milk feed has not been increased. It will be kept at it's current level until Sophie stops being sick. The last dose of antibodies was given last night. Sophie's GVHD will be monitored and if it gets worse she will be given antibodies to treat it rather than increasing the steroids. As the steroids have caused Sophie's blood pressure to go up we are happy that we will hopefully be able to eliminate them soon. Sophie had a chest x-ray this afternoon to check her lungs for infections. She will a chest x-ray a weekly basis until we are sure she is free of the virus.

Sophie has been playing her "piano" with her feet today. I think she wants to get a bit of practice in before Nikki (the music lady) returns next week !! She has been quite happy sitting up and playing in her cot, her head control is now very good and she is moving her legs much more. She still lets herself fall backwards when she has had enough so we have to be ready to catch her !!

Please keep Sophie's friend Max in your prayers. He is having a rough time at the moment and will undergo an operation later today to have his Hickman line replaced by a dialysis catheter as his kidneys need a little help.

            Max's Website             

Padraic

12/11/2003 T+63

WBC 3.3 

Sophie had a good night. She was a little sick overnight but as she immediately started banging an empty foil bowl like a drum I don't think she was too upset. The doctor came in and told us how happy they are with Sophie's progress. Her GVHD is under control, the last dose of the current antibody course was given today. Sophie's steroids have been reduced to almost nothing so we will see if the GVHD is gone in the next week or so. The reduction of the virus counts can be attributed to Sophie's T-cells fighting the virus as the drug treatment she was given is only capable of maintaining the virus counts not reducing them. This is a very good sign that Sophie's immune system is coming back. Although the WBC count for the last few days has been low it is not a cause for concern as the T-cell element is still good.  

Sophie was sitting up by herself today and playing very well in her cot. The "Music Lady" (Nikki) came in today and Sophie had great fun playing along with the music on the keyboard and tambourine. Nikki said that she could see a big improvement in Sophie's hearing since the last time she saw her 6 weeks ago. We hope the good news keeps coming.

Padraic 

11/11/2003 T+62

WBC 3.3

Sophie had a good night. She was sick at 5:00am but settled down afterwards and slept until 08:00am. The good news today is that the virus counts have come back and they are down from 60,000 to 30,000. The BMT team checked in on Sophie and they are very happy with her progress. As a result of the virus count reduction the daily antibiotic is no longer required. Sophie will still have the weekly antibiotic until the virus has cleared. 

Sophie's diarrhoea is a little worse and will be monitored closely. The physio came in today and saw an improvement in her head control and is going to suggest some more exercises.

Sophie played well all day and has invented a new game known as "where your tummy". This is based on her favourite game "peek-a-boo". Mummy says to Sophie "Where's your tummy" and Sophie lifts her top to show off her tummy.

Padraic 

10/11/2003 T+61

WBC 2.9

Sophie had a good night, she was sick twice and slept until 08:00am. She was a little snuffily when she woke, the doctor checked her lungs and they were fine. A blood sample was taken which will be used to analyse Sophie's T-cells in more detail. They are particularly interested in the count of CD4 cells which are important in dealing with viruses. The doctors are very pleased that Sophie's GVHD is under control and that the steroids have been reduced. Sophie's blood pressure is being maintained at normal levels with her current anti-hypertensive medication. Although Sophie is being sick on regular basis there is no cause for concern as it is probably just a reaction to some of the oral supplements she is receiving. 

Sophie has been on top form today and has been very chatty with the doctors and nurses. The occupational therapist said that each time she visits Sophie she sees big improvements. She is more and more mobile and we think that her "leg sensitivity" may be related to her previous skin GVHD and that Sophie thinks it will hurt if you touch her legs. If her legs are touched while she is distracted she doesn't mind. All in all another good day.

Padraic

09/11/2003 T+60

WBC 3.5

Sophie had a good night, she slept until 6:30am so Daddy had a good night too. She played really well all day and is definitely returning to her former self. She is now clapping and reaching for hands and toys. Her legs are still quite stiff and Sophie complains whenever they are touched. Her head control is getting better and she even did a few "push ups" when sitting on Mummies lap. Sophie has been in a very good mood all day. She had two goods naps which suggests that she may be getting back into her daily routine. She spiked a temperature in the afternoon and has had cold hands all day. This is probably the virus but some blood samples were sent for analysis just in case. Sophie is tolerating her milk feed very well. The flow rate has been increased every day for the last week and part of her Sophie's TPN feed (the protein element) is no longer required. The TPN will be reduced as the milk feed is increased so hopefully the TPN can be stopped in a few weeks. 

All in all it was a very good day with lots of smiles and fun and cuddles.

Padraic

08/11/2003 T+59

WBC 4.5

Not a good night for Sophie apart from being sick she pulled out her NG tube at twelve. Georgina was able to put it back in really quickly and Sophie was really brave so it was not too traumatic. Her temperature did not go over 38 all night but her heart rate kept going quite high. 

She had her first dose on this new antibiotics today which required a hour of fluids before and after. She also she needed a platlets transfusion so we were concerned about her fluid intake following her attacks.  She was given it all during the day and the everything was careful monitored. 

She was quite tired today after her eventful night but Padraic saw a great improvement in her from last week. He was particularly happy that she has started holding his hand again. 

I had a lovely day with Amy, she should be on the NHS, those queues would be reduced overnight. 

07/11/2003 T+58

WBC 4.7

Sophie was sick again in the night and her temperature was quite high but overall she slept more and didn't wake up until 10 O' Clock. The results from the MPA was negative so we can stop the nebulizer, this means the Adenovirus is not in her lungs. Unfortunately the counts in her blood have gone up from 32 thousand to 60 thousand, it is not really a huge jump as it seems and is still relatively low but it is going in the wrong direction. She been prescribed a new strong antibiotic which she will have once a week. 

In the afternoon she had a ultra-sound on her stomach; of course she throw up and soon as the probe pressed on the tummy. They didn't find any problems. 

She is still very playful and taken to holding all the Nurses and Doctors hands and playing with their fingers. As for peek-a-boo she will be entering into the records books soon.

Tina

06/11/2003 T+57

WBC 12.3

Sophie was sick quite a few times overnight. It could be the virus or she may being having trouble handling the increased NG milk feed.  The milk feed is being increased on a daily as we want Sophie to increase her protein intake. Low protein intake can cause fluid retention which was a factor in Sophie's recent cardiac arrest. Blood samples were taken to check the virus counts and Sophie will have an MPA (nasal fluid sample) tomorrow. The doctors are concerned that Sophie's temperature is up and may add another drug to the virus treatment. Sophie's lymphocyte count (T-cells) is up today so hopefully Sophie's T-cells are "assembling the troops" needed to kick the adenovirus out of her body. Sophie's blood pressure has been fine for the last few days which is good, the combination of the new anti-hypertensive drugs and the reduction in her steroids dosage have probably done the trick. Her heart rate has been high which the doctor believes is likely to be a combination of her temperature spikes and her body fighting the virus. She had a blood transfusion today as her Hb count was low. Sophie was sick this afternoon, she was given some anti-sickness medication and has not been sick since.

Despite being sick Sophie continues to play well. She is also becoming more mobile, rolls from side to side in her cot and is moving her legs much more. She is quite happy sitting in her chair for short periods and is enjoying her new toys. 

There will not be an update tomorrow night as Mum will be going straight to bed once Amy is asleep as she needs to catch up on some much needed sleep.

Padraic

05/11/2003 T+56

WBC 4.4

Sophie had a good night, she woke a few times for a play and then settled herself back to sleep. Her temperature and blood pressure have been fine all day although her heart rate is still a little high. Sophie had another course of antibodies this afternoon and she will have a blood transfusion tomorrow as her counts are down. Sophie is only having one blood product a day at the moment as a result of her recent "scare". She has been very happy all day, playing peek-a-boo, flirting with the doctors, holding the nurses hands and "chatting" with Amy on the phone. The "music lady" tried to get to play with Sophie today but she was under the nebulizer and then when she returned Sophie had fallen asleep. It was a pity as Sophie does enjoy making a racket !!

Sophie is slowly returning to her usual "cheeky" self and it is a joy to see.

Padraic

04/11/2003 T+55

WBC 7.0

The BMT team came round today and they are very happy with Sophie's progress. The GVHD is under control, the rash has gone along with her diarrhoea. There are still a few days to go on the current antibody course and the treatment will be reviewed once this has been completed. Sophie's virus is still a concern and will continue to be treated with antibiotics and the nebulizer. Sophie's steroid dosage will be halved again which will hopefully give her more "fighting" T cells to help clear the virus. She had a chest X-ray today which came back all clear. She has had a few temperature spikes and was sick a few times today when she was moved. It may be that she is feeling very nauseous from all the medication, if she continues to be sick she will be prescribed some "anti-sickness" medication. 

Sophie is still playing with her toys and peek-a-boo with the nurses but becomes very distressed whenever she is moved. Maybe she is still a little stiff or maybe she feels nauseous, it is so difficult to know how she is feeling. She is such a good patient we know that she must be in quite a bit of discomfort if she is complaining about it. We were given a "play program" today which has been designed to get Sophie more mobile and get her back to where she was before her "trip" to the ICU.  

We are playing a waiting game at the moment with the virus and GVHD but as long as we are making steady progress we're happy. We are in no rush to get out of here, we will go home when Sophie is ready, whenever that may be.

Please keep Sophie's friend Max in your prayers. He is continuing to make progress against all the odds and is an inspiration to all of us.

Padraic

03/11/2003 T+54

WBC 4.5

Sophie slept well last night, I think she is still catching up on lost sleep from her visit to the ICU. She spiked a temperature last night while her Vancomycin (antibiotic) was being administered. As Sophie has had a reaction to this drug (wonderfully known as "red face syndrome" ) she was given some piriton and her temperature returned to normal. She has been quite happy sitting and playing in her chair. The physiotherapist and occupational therapist came in this morning, they are happy that Sophie is making progress. They will pop in on a weekly basis to check on Sophie. In other Sophie news I have noticed some new hair on her head. Alas, her blonde hair is not returning, I think her new hair will be brown. It's probably just as well as Sophie's eyebrows are jet black and would clash with blonde hair. I have added some new pictures so you can see Sophie's new eyebrows.

Padraic

02/11/2003 T+53

WBC 3.1

Sophie was given a new blood pressure drug last night. Her blood pressure was checked every 15 minutes following the first dose and the new medication had no effect whatsoever. She was given a second new drug later which brought her blood pressure down nicely. Hopefully the correct combination of old and new drugs will keep her blood pressure under control. Sophie spiked a temperature of 38.7 last night and a set of blood samples were sent away for tests. Her temperature responded well to paracetemol so hopefully it was just Sophie's teething which pushed her temperature up. Sophie had a good night's sleep, her diarrhoea is much better so she doesn't need to be changed overnight quite so often. Her WBC count is down quite a bit today; we will discuss this with the consultant if it continues to drop but the nurse said it is quite normal for the counts to go up and down. 

Amy came to the hospital today and Sophie's face lit up the moment Amy walked into the room. It was so nice for us all to be together again for the first time in almost a month. Amy is very impressed with Sophie's new room particularly the "magic taps". She spent ages saying "abracadabra" and waving her arms about to get them to work. We spend much of our time waving our hands about, perhaps we need to say "abracadabra" too.

Padraic  

01/11/2003 T+52

WBC 7.8 

It is quite common after a visit to ICU for your body clock to be out of synch and Sophie was seems to lost the track of day and night but she was still settled over night.  

Her blood pressure is becoming more of a concern as it is not responding enough to the changes in her medication. The BMT consultant came to see her today and has prescribed a new medication. This medication can have an effect on her kidneys which is why they were checked and will continued to be monitored. 

He is happy that the virus was caught early and is not too concerned about it. He can also see some real improvements with the GVHD symptoms so the course of monoclonal antibodies (daclizimob and inflizimob) will continue. We will then be able to see if Sophie's new and old cells have learnt to be nice. 

Tina 

31/10/2003 T+51

WBC 5.1

Sophie had another good night. She woke a few times but settled herself back to sleep. The physiotherapist examined Sophie today and wants us to continue with the ankle physio and to put Sophie back on her tummy so she can regain her head control. She is going to liase with the occupational therapist and put together a program that we can follow along with the Jess, Sophie's play specialist.

Sophie's blood pressure has been better over the last few days. It is still high but not as high as it was before. Her diarrhoea has improved and her skin is getting better so hopefully the antibodies are helping the GVHD.  Sophie is being given a milk feed through her NG tube at a very slow rate to keep her stomach "ticking over" and she is tolerating it well. She is still quite weak and has been prescribed potassium as her level is too low. Her weakness may also be attributed to the fact that she is being kept very dry.  She had an Hb transfusion this afternoon and will have another course of antibodies tomorrow. Following last weeks "scare" all Sophie's blood products will be given during the day and she will only receive one blood product per day whenever possible just to be on the safe side. The virus levels in her blood are up from the last test, this is not an immediate concern as the tests were conducted before the treatment was started.

Sophie has been much chattier today and even said "Dada"; maybe she is getting ready for me to take over for the weekend. She got a new toy drum and is having great fun playing with it. Tina is off home tonight for the first time in 2 weeks. She has to catch up on cuddles from Amy and some much needed sleep.

Padraic

30/10/2003 T+50

WBC 9.6

Sophie had a good night. She had her first "go" under the nebulizer at about 10:30pm. The cover is quite big and leaves enough room for her to play with her blanket, she was a little bemused but was very brave and didn't cry. Her skin continues to improve and it is only red in places. Sophie's weight is slowly increasing, she lost a kilo while in ICU and already she has regained 300g, we hope she can build her weight up slowly and not start retaining fluids again. She has been staring into space quite a bit, the doctor said that this is common and is probably due to the large amount of drugs she received in ICU which are still in her system. She had an MPA (“lung liquid sample” ) this afternoon as the MPA from yesterday didn’t produce enough liquid for all the tests required. We will have the results in next few days. Sophie had an ultrasound scan of her heart which showed that her heart has recovered the loss of functionality she had following her cardiac arrest on Friday. Sophie continues to play well and seems to have been weaned off sucking her thumb. Perhaps this is the result of a having a tube stuck in her mouth for 4 days. I hope she starts again as she looks so cute with her thumb in her mouth. 

Sophie is still very weak and has a little trouble supporting her head although this is slowly improving. The play specialist will consult with the physiotherapist and will put together a plan to slowly get Sophie back to sitting by herself.

Sophie, however, is not just lying there passively. Today she needed to have a heck-prick done, this is painful even for adults. Sophie was being consoled by Mummy and she kept reaching for the aluminium foil bowl in her cot. Mummy gave it to Sophie thinking she wanted to play with it but once Sophie had it she threw it at the nurse !! Good girl Sophie, you let them all know when you have had enough. I hope Kate (Sophie’s nurse) wasn't too upset, it was nothing personal. 

Padraic

29/10/2003 T+49

WBC 9.4

We went back to our old room yesterday and it felt good to be back. Sophie came off the vent on Monday at 4:00pm and stayed in the ICU overnight so she could be kept under close observation. We eventually got back to our room at about 3:00pm. This has been the most frighten few days of our lives and we were continually asked how we felt. Well, when your child has almost died and you spend the next few days watching her get better, kicking off the oscillating vent and then the regular vent and taking the glasses from you face five minutes after she has returned to her old room you feel like you are on cloud nine. We were so close to losing Sophie and now I only have to watch her lying and playing for my eyes to fill up. We have been truly blessed. 

Words cannot express how much we owe to the ICU staff here at Great Ormond Street. Once the emergency button was hit Tina said it was like an episode of E.R. Doctors rushed in from all directions within 30 seconds and it was this speed of response that probably saved Sophie's life. If we had been at home I don't think she would have made it. We spent 4 days on the ICU and I have never seen anything like the care Sophie received. There were two nurses permanently assigned to Sophie on the first day and they spent the entire day managing her condition. I lost count of the number of specialist doctors who examined Sophie and added input to her recovery plan. We also had members of the ICU response team who were on duty  when she had the cardiac arrest popping in to see how she was recovering along with our Fox ward nurses and all the BMT team. In a big hospital you sometimes think that Sophie is just one of many sick children but we saw how much the staff here love and care for these children. We will never forget this.

No sooner had we got back to our room that we were told that a virus had been found in the liquid sample taken from Sophie's lungs on Friday. Sophie will be put under a nebulizer for two hours three times a day to help clear the virus. The current counts for the virus are very low in her blood and would not have been detected if the virus had not been found in her lungs. As Sophie is on a nebulizer we have moved rooms as her room needs to have an airlock. I feel like I am entering a space ship each time I come into the room. Sophie's blood pressure continues to be a concern, Sophie's kidneys were examined using ultrasound and are fine. She will hopefully be able to start a new course of treatment which has been recommended by the renal consultant. 

Finally, thank you to everyone who signed the guest book over the last few days. We really appreciate your kind words and support. As we have moved rooms we now have a new extension number. If you phone us at home Tina's Mum will be able to give it to you.

Please continue to pray for Sophie's friend Max, he is very very sick. Max's website 

25/10/2003 T+45

13:00pm Update

Firstly Sophie is ok but we have had a major scare. At 3:00am on Friday morning Tina woke because she heard Sophie having trouble breathing. The nurse hit the emergency button and doctors raced in from all directions. Sophie stopped breathing and had a cardiac arrest that lasted three minutes. She was given CPR and her heart started beating again. She had trouble breathing for about 30 minutes until she could be intubated. We got down to the ICU at about 5:00am, Sophie was on a oscillating vent, we were told that she was very sick (when a doctor tells you she is very sick you know it is very serious).  The first 24 hours are critical under these conditions and Sophie came through like the hero she is. Her gas count improved steadily through the day and by 6:00pm she had recovered enough to be switched to the conventional ventilator. We left her in the capable hands of the ICU nurse and got some badly needed sleep. We returned this morning to find that Sophie had a good night and was continuing to recover. Her ventilator was reset at 8:30am to allow Sophie to breathe whenever she can and to only help when she doesn't breathe or her breath is shallow. Today we will be concentrating in getting Sophie to breathe on her own with a view to taking her off the vent tomorrow (26th). Sophie is sedated as she is on the vent and her muscle relaxant was stopped this morning. Tina spoke to me at 12:30 and said that Sophie had just squeezed her finger. Sophie has been incredible throughout this scare and our hearts are filled with joy and pride as we watch her fight her way back from this.

Yesterday Sophie had a barrage of tests. She had an EEG (brain scan) which showed some damage which thankfully the neurosurgeon believes to be reversible, this damage could be attributed to the trauma. There is still a risk of some brain damage as Sophie had trouble breathing for about 30 minutes and we don't know if her brain was starved of oxygen in that time. We will only be able to determine if there is any damage once she is awake. Her heart is not working as well as it should and her leaky heart valve is a little worse but thankfully not an area for concern. 

While we are both up at the hospital it will be difficult to keep the website up to date. We will use the guest book to post the latest news so please check there if there is no daily update.

Padraic 

23/10/2003 T+43

WBC 8.6

Sophie had a very good night. She woke a few times but settled herself back to sleep. She was eventually ready to start the day at about 8:30am. She played well in the morning and decided that she had earned a nap by about 11:30am. Sophie's rash is a little better, she was quite tolerant when her cream was done both last night and this morning. Her face is not quite as swollen as it was yesterday but she still looks like a little Buddha.

The doctors checked in and told us that Sophie's antibiotics are to be stopped as she has not had any infection since the Hickman line infection over a month ago. The temperature spike she had on Monday was most likely to have been due to the antibody treatment. Her fluid balance and weight will remain closely monitored along with her blood pressure which is still high. The steroids will be reduced slowly and will hopefully be stopped in a few weeks. Sophie had her second course of antibodies this afternoon, she will finish the  course at the end of next week, Sophie's GVHD will then be reassessed and the next course of action will be decided. Sophie will have a blood transfusion tonight as her Hb level is a little low.  

Please pray for our friend Max he is back on the vent again and is very sick. Max's website 

Padraic

22/10/2003 T+42

WBC 10.8

Sophie had another restless night. She settled down at 10:00pm but woke up at 11:00pm and was very agitated. The skin on her face was white and peeling off as if she had been sunburnt. We put some Diprobase on Sophie's face to help the peeling. She was given strong painkillers at 2:00am which helped her to sleep until 06:00am. Sophie spiked a temperature at about 6:00am and given paracetemol, the doctor said that it is likely to be related to the antibody treatment. 

She had a heart ultrasound to check for bugs which was all clear. Her leaky heart valve has not changed since it was last tested. The leakage is now classified as mild to moderate and will not need any treatment in it's current state. 

Sophie's diarrhoea has all but disappeared which means that her stomach is resting. Her rash might be getting better as I was able to hold her and give her a cuddle but putting the cream on was still painful. She slept most of the day which is good as it means she is comfortable and as her last dose of morphine was 7am in the morning most of the afternoon she was painkiller free, another good sign.

She is retaining a little fluid and is looking puffy, imagine a little Buddha and you will get the picture, nurses was on the case when I left so I guess Padraic will be changing a few wet nappies tonight.   

Tina

21/10/2003 T+41

WBC 9.5

Sophie didn't sleep very well again last night. Her skin is very tender and this combined with her teething kept her up most of the night. She was given some strong painkillers this afternoon and spent the rest of the day catching up on lost sleep. The BMT consultant's round was this morning. He raised some concerns over the fact that the steroids had been unable to clear the GVHD. He believes that the GVHD is acute and probably moderate (not mild). While this is not really news to us it was upsetting to be formally told that the GVHD is no longer considered mild. We also found out that the antibodies will "attack" only those T-cells which are as the consultant put it "geed up to fight Sophie's cells" so Sophie will less susceptible to infection. 

Sophie was very upset when I popped in this evening and she was trying desperately to go to sleep when I left her. What is most upsetting is that she wants to be held  and raises her arms but her skin is so tender that she cries when we touch her. We have to "lie" next to her on her cot. She is due some more painkillers at 10:00pm so we hope she will have a better nights sleep tonight. The last few days have been tough as it is the first time that Sophie has been sick all day. It is heartbreaking to see her suffer and we pray that the antibodies start to work real soon.

Please check out Max's website  and read the update for Monday from Max's granddad, it is a wonderful letter and it sums up so beautifully how we feel.

Padraic

20/10/2003 T+40

WBC 7.35

Sophie didn't have a very good night last night. She was was sick at about 11:00pm. It's probably a combination of teething and the rash on her arms and legs making her feel very grotty. She didn't sleep for more than a few hours and was very upset when her nappy needed to be changed. On the positive side the two blood pressure medications appear to be keeping her blood pressure under control. I forgot to mention yesterday that Sophie had to have her NG tube replaced as it had become blocked. She was so brave, it must be horrible having a tube put through your nose. Thankfully, Jin-Hua, Sophie's nurse for the day is somewhat expert at the procedure and was able to insert the tube very quickly. In order to soothe her legs Sophie is wearing what can only be described as "wet stockings" on her legs; this is a treatment which is normally used for children with eczema and it should give her some relief.

Sophie started the antibody treatment this afternoon. From my limited understanding of these drugs they are a very powerful medication which suppress the effects of the GVHD (rash etc) in order to give the body sometime to get used to the new T-cells. After a few weeks the donor T-cells should be happier in their new environment and stop attacking what they see as foreign bodies. The danger with these drugs is that it will be more difficult to spot infection as the normal signs will be suppressed; this is why we had to be sure that Sophie is free of infection before the treatment starts. Sophie started on TPN (all nutrition through her Hickman line) this evening.

Padraic

19/10/2003 T+39

WBC 6.3

Today was a quiet day for Sophie. Her blood pressure was good all day. She has been prescribed another medication to control her blood pressure, this means she will get one of the two medications every 3 hours which should keep her blood pressure down. One of the senior BMT doctors was on call today. We discussed Sophie's GVHD which thankfully is mild. However as the steroids are not clearing it Sophie will start on the antibodies from Monday. Her steroid dosage will be slowly reduced as it will no longer be required and we hope this will reduce the blood pressure problems that Sophie is having. While Sophie is receiving the antibodies she will be put on TPN (Total Parental Nutrition i.e. all nutrition through her Hickman line) in order to keep her stomach rested. We hope that these antibodies can resolve the GVHD and ensure that Sophie is heading in the right direction. It feels as it thing have been standing still for the past week or so.

Sophie's main irritation today was the breaking through of a new molar. She wasn't in a playful mood and wanted to lie down and rest most of the day. She hasn't got much energy but then again she is getting all her nutrition through her Hickman line so it's understandable. 

Padraic

18/10/2003 T+38

WBC 7.85

The doctor visited Sophie early in the evening and put her on a additional IV drip and gave her some medication to help absorb the IV fluids, Sophie then slept all night.  

She has not wanted to play today, just cuddles and rest. Blood pressure was a issue all day and her normal medication was not bringing it down. There is other medication that could help but she cannot take it if she dehydrated otherwise it will cause other side effects, this is of course something we are having a problem with. 

Poor Sophie is also cutting another tooth, one of the molars. It seems she has already got another molar which I completely missed.  Daddy felt them today when gave her some teething gel because she was chewing her fingers. 

Thank you to all the people checking in on Sophie, your emails, guestbook entries and phone calls really make a difference. I know it can be hard to know what to say and people often do not want to bother us, but these messages of support give us a lift. 

Tina

17/10/2003 T+37

WBC 8.2

Sophie woke up at 11:00pm last night and was very agitated, her hands and feet were very cold and her she looked terrified. Her pulse and blood pressure were low and she would not drank any dirolyte. The doctor was called and put Sophie on an IV drip and gave her some medication to help absorb the IV fluids. Sophie settled a little but then started to shake her head, she was given some paracetemol as it looked like she had a headache. Sophie settled back at about 12:30am and slept until 6:00am. Sophie was a little "shaky" today but still managed to play well. Her mood this morning is best described as grumpy (I think she has every reason to be grumpy considering what she has been through this week). Sophie's blood pressure is now swinging between low and high whereas it has been high or very high all week. Her heart rate is down so her blood pressure will be checked every hour. 

The BMT team visited today. The gut biopsy from Monday does not show any GVHD in Sophie's gut, the results from the skin biopsy are not back yet. However it is still thought that Sophie has GVHD of the gut as she is showing all the clinical signs of GVHD.

Later in the day we got the results from the skin biopsy and they found mild GVHD of the skin and although they did not find GVHD of the gut this is enough to confirm their suspicions. Sophie is going to continue to rest her stomach and if there is no improvement the 'magic' antibodies will be called in. I have to say I not sure I understand how they work but I was told after two weeks they should be able to reduce the steroids, after a month stop them completely. This will mean all the side effects she is experiencing should disappear too.   

I am off for the weekend, and Daddy get some much needed cuddles from his angel.

Tina

16/10/2003 T+36

WBC 7.2

Sophie woke at 1:00am and was extremely agitated. Her heart rate was extremely high. She drank more than 240ml of diorlyte and was given IV fluids. She settled down at about 3:00am and had a little play with Mummy's glasses before going back to sleep. Her rash still comes and goes on her arms and legs. She has been put on an additional blood pressure medication as her blood pressure "normal level" has been too high too high. The doctor came in at about 10:00am on her daily round. Although all the results from the blood and urine tests are not back yet, no infection has been found in the tests completed so far. The BMT team suspect that Sophie's current problems are more likely to be due to GVHD rather than an infection. As Sophie's diarrhoea has been quite bad for the past week she will be put on an IV drip and given only diorlyte for a few days to rest her stomach and to see if the diarrhoea improves. If this works then Sophie may be put on what is called TPN (Total Parental Nutrition), this means she will receive all her nutritional requirements through her Hickman line (i.e. she would have no food or drink orally). This would be done in order to clear the diarrhoea and to give her stomach a much needed rest but is not a step which will be taken lightly. 

We should have the results of the biopsy tomorrow and hopefully will be in a position where we know exactly was is happening with Sophie's stomach. The BMT team will then be able to formulate a strategy for detailing with the GVHD (and/or the infection) rather than just reacting to the resulting effects. Despite all her ailments Sophie has been playing well and has been very smiley and happy all day.

Best wishes to Anthony (Tony the Tigre) on his transplant today. He is another Hurler's child and a good friend of Mighty Max. His website is Tony the Tigre, please drop in and wish him good luck.

Padraic

15/10/2003 T+35

WBC 16.2

Sophie had quite a restless night. Her diarrhoea has been quite bad and her nappy needed to be changed every hour. Sophie has been a little "out of sorts" all day, nothing specific just not her usual self. The rash on her arms and legs has returned and her hands and feet are very cold. Sophie was put on an IV drip to ensure she didn't become dehydrated. Her temperature spiked at 38 degrees C this afternoon so she was given some paracetemol to control it. Sophie's WBC is way up today which we are told may be a sign of something brewing (e.g. cold,virus,infection etc). It may also be the GVHD. Blood and urine tests have been taken and we will have the results tomorrow.  Sophie's blood pressure was very high late this afternoon, her 6:00 Nifedipine had been cancelled as her blood pressure was expected to drop as a result of her temperature spike. It was reinstated to keep it under control. Sophie is being very closely monitored at the moment and we will hopefully have a better idea tomorrow what the cause of her temperature is. We are feeling quite apprehensive at the moment, we don't which is the lesser of two evils an infection or GVHD.

Padraic

14/10/2003 T+34

WBC 7.2

Sophie had a good nights sleep and was quite settled overnight. She played really well in the morning but by the afternoon she was quite sleepy. This was probably the result of her busy day yesterday. She is back on full strength diorlyte and is quite happily drinking away. We think that Sophie's tastebuds have changed; she loves to drink diorlyte which I am reliably informed tastes horrible and she has also started to suck the anti-septic mouthwash from the swabs. Sophie's rash is getting better but the diarrhoea is still the same. 

We discussed the findings from yesterday's procedures with the BMT consultant, we will sit tight until all the results come through which should be by Friday. The BMT consultant said everyone was very impressed by how well Sophie coped with all that was thrown at her yesterday. 

Padraic

13/10/2003 T+33

WBC 6.5

Although Sophie was scheduled to have her biopsy this morning it was delayed until the afternoon as there were a few emergency cases in the morning. Sophie was put on the emergency list for the afternoon as the doctors didn't want to postpone the biopsy and have to start the whole "pre-operation" process again. She eventually went to theatre at 2:00pm. Leaving Sophie to be put under anaesthetic was an very emotional experience. This is the fifth time that she has had a general anaesthetic and it doesn't get any easier for us. Although you know that she in expert hands you just feel so helpless. Once Sophie was "under" skin and gut samples were taken for biopsy. Sophie also had an endoscopy which showed that there is no inflammation in her stomach (good news). She may still have GVHD in the gut and the lack of inflammation could be because the steroids are doing their work. We will have the results of the biopsies in the next few days. No sooner had Sophie got back to her room that she had to have an ultrasound scan. This was all fine and there is no sign of infection. 

Sophie was so good today. She was "nil by mouth" from 5:30am and had not eaten since 11:00am on Sunday and yet didn't complain. She was a little confused when she regained consciousness but had a good look around when Mummy was carrying her back to her room. Sophie is a wonderful patient and only gets upset when something really causes her discomfort which makes life so much easier for us and the nurses.

Padraic

12/10/2003 T+32

WBC 10.0

Sophie was very quiet today, she was also quite irritable and didn't want to play very much. She perked up when Mummy and Amy arrived and wanted lots of cuddles from Amy. Her blood pressure has been high and she was given additional Nifedipine to reduce it. Sophie's enthusiastic drinking of diorlyte will have to be curtailed as her sodium levels are too high. We have been giving her diluted diorlyte today but she is not very keen on it.  To add to her wonderful day she had a "heel prick" blood test (which Sophie hates because it hurts) to check her Amicaycine (sp?) levels. The countdown to the biopsies started this morning. She has been on "clear fluids only" since 11:00am and will be "nil by mouth" from about 2am. She will be put on an IV drip to ensure she doesn't become dehydrated. 

The current thinking is that Sophie has GVHD of the stomach and intestines and that this is the reason that she has had diarrhoea. If this is confirmed then Sophie will be given steroids to help fight the condition and her cyclosporine dosage will be increased so that her cyclosporine levels are kept at about 200 (they are currently kept at 150).

Here is a good GVHD Definition. 

To finish on some positive news, Sophie's hearing has definitely improved over the past few weeks (she had grommets inserted at the end of July). She now reacts to loud and sudden noises and turns when she hears her door opening. She will have another hearing test soon which will hopefully confirm this improvement.  

Padraic

11/10/2003  T+31

WBC 8.5

Sophie had a quiet day today she was not any better but more importantly she was not any worse. I think she was catching up on cuddles from her Daddy. 

It is mainly dads in at the weekend so I believe the the telly room will be taken over tonight for the big game (England vs Turkey Football). I have been told not to mention the score when I phone, like I know the score! 

Tina

10/10/2003 T+30

WBC 9.6

Sophie's temperature and blood pressure was constant last night and today, a little high but no great swings. She is being so good with the Dirolyte and maintaining her fluid balance without the need of a IV drip. The diarrhoea is still here but not getting any worse.  

The BMT consultant came to have a look at the rash and he is convinced it is GVHD. So on Monday morning she is going to have a skin  and intestines biopsy under general anaesthetic to confirm one way or another. As things are under control they are not going to increase the steroids because of her blood pressure. The rash is not bothering her as much this time, which is good and she is still very playful and happy. 

I thought I would share a Amy moment with you all.

In our window we have a Genes for Jeans day poster which we have not got round to taking down. After a walk with MorMor (my mum) Amy spotted the poster and had the following conversation. 

Amy :  "MorMor it is not Jeans for Genes day anymore is it?"

MorMor : "No we will have to take it down won't we"

Amy : " I suppose its Trousers for Trousers day today"

Tina

09/10/2003 T+29

WBC 9.45

Sophie was sick again last night at about 2:30am but despite this she had quite a good nights sleep. Her blood pressure and temperature were high, she was given some Nifedipine to reduce her blood pressure and anti-biotics to bring down her temperature.

Sophie played well today and enjoyed her session with the play specialist. She has been much more alert and didn't even cry when she had her bath. Her fluid balance has been very good and she seems to have acquired a taste for the diorlyte which she is given to help keep her fluids "flowing". The BMT team met to discuss Sophie's blood pressure and have increased the frequency that Sophie receives  Nifedipine in order to keep it from becoming too high. Sophie still has diarrhoea but it is better than it has been in the last few days. This afternoon Sophie's broke out in a rash, the doctors checked her and confirmed that although the rash was not the same as before it was probably GVHD. A steroid cream will be used to keep it under control. Sophie's weight is being closely monitored, if she gains more than 300g she will be given Frusemide (a diuretic), if she loses more than 300g she will be put on an IV drip. 

We think that Sophie's eyesight is improving, she is now looking and waving at the people as they pass outside her room. One of the doctors who saw Sophie at the start of her transplant has said that Sophie's eyes look less cloudy and that she is definitely looking around much more and focusing on peoples faces. I think it is time to put on the PINGU videos. I am looking forward to spending some time with Sophie over the weekend as I haven't seen enough of her (or Amy or Tina) this week. 

Padraic

08/10/2003 T+28

WBC 7.7 

Sophie had quite a broken nights sleep last night. She was disturbed by the frequent (and necessary) examinations and was sick at about 1:00am. She recovered quickly from her vomiting and decided that now was a good time for a game of "peek a boo" with Mummy. Mummy told Sophie that it was 1:30am and not the right time for games. I am not sure if Sophie understood but she went back to sleep anyway, the little angel. She has been in good form all day despite all her examinations. She was given a blood transfusion tonight as her Hb count was a little low (I think Sophie is determined to retrieve all the blood she has given away to date for blood tests.)

Sophie's blood pressure has been a lot better today. It went up quite a bit when she had her steroids but once Sophie had the blood pressure reducing medication it dropped back to normal levels. Her blood pressure is still being checked hourly which unfortunately stops Sophie from having long naps. The overnight examinations although less frequent still wake her up so she is getting a lot of broken sleep. Sophie had mild shakes today and was given some piriton which brought it under control quite quickly. 

The gut specialist (I'm sorry but I don't know what the correct name is for this area of expertise, the nurses call them this as well!!) examined Sophie and asked Tina lots of questions about Sophie's medical history. The BMT team met afterwards and have decided to perform an internal examination of Sophie stomach and a biopsy under general anaesthetic early next week. This will determine whether or not Sophie has GVHD in her gut. We are quite happy that this is being carried out as we will know where we stand. We have had a lot of uncertainty about the GVHD in the last few weeks. If Sophie has GVHD and the current steroids are not clearing it then another type of antibody medication will need to be used. 

Tina and I managed to pop out for dinner tonight at a local Italian restaurant. It was nice to spend a little time together and to eat a meal in relaxed surroundings. It is a very long week for Tina as she stays at the hospital from Sunday to Friday so we will try to make Wednesday night a regular "night out", if Sophie lets us.

Padraic 

07/10/2003 T+27

WBC 10.5

Sophie had a good night and was happy and played well all day. However, she started shaking at about 9:30am as if she was cold. Her pulse was very high and her blood pressure very low, she was put on an IV drip as she had become dehydrated. She recovered quickly and both her blood pressure and pulse went back to normal. Her diarrhoea has come back which may explain why she had become dehydrated. 

The BMT consultant checked in today. As Sophie's diarrhoea has returned her steroid dosage has been increased. The gut specialist will examine Sophie to determine if anything other than GVHD could be causing the diarrhoea. If the diarrhoea does not clear Sophie will have an internal gut examination to determine whether she has GVHD. The diarrhoea and GVHD rash are our primary concerns at the moment. We also know that the increase in the steroid dosage may increase blood pressure/dehydration problem. Sophie's blood pressure will continue to be monitored hourly during the day and every 2-4 hours overnight. Her fluid balance will continue to be closely monitored and she will be given IV drips whenever necessary.

We are both a little down today. These current issues are not too serious but I think that as Sophie has been doing so well we both started to think about the possibility of going home in a few weeks. We will have to wait until the GVHD and diarrhoea are under control  before we think about going home.

Padraic 

06/10/2003 T+26

WBC 9.4

Sophie was closely monitored last night as her blood pressure was high throughout the day. The doctor came at 11:00pm and checked her and was quite happy with that problem was not related to her shunt. When Sophie was checked again this morning the doctor said that her high blood pressure was probably due to the steroids, as a result her blood pressure lowering medication dosage has been increased. 

Sophie had a good day today, she played well and was very chatty. I noticed quite an improvement from yesterday when I popped in on my way home from work this evening. The GVHD skin rash comes and goes but it not as bad as it was when it first started. Her cyclosporin level is at a "perfect" level of 200 which will help to keep GVHD under control. 

I have added some new pictures following yesterdays family reunion.

Padraic

05/10/2003 T+25

WBC 8.0

Today was a special day as we were all together in the same room as a family for the first time in over a month. Amy had a cold earlier in the week but was all clear today so we were able to take her into Sophie's room. Amy took a little time to get used to Sophie's new haircut but before long they were playing happily together. It was a wonderful feeling being back together, I got to hold my two girls in my arms at the same time, a simple thing which I have longed to do for what seems an eternity.

Sophie was a little up and down today her blood pressure was high most of the day. Her medication dosage has been increased to reduce it. It is thought that Sophie's high blood pressure today was probably caused by her retaining too much fluid. We are currently trying to perform a balancing act with Sophie's fluid intake/output, some days Sophie doesn't drink very much and becomes dehydrated, her overnight fluid intake is then increased to correct this but sometimes she starts to retain fluid which increases her blood pressure and she has to be given a diuretic to help her lose the excess fluid. She was given a diuretic at teatime and Mum reports that she was "weeing for England" and in much better form. Her diarrhoea is getting better and the rash on her legs has almost disappeared so hopefully the drugs are keeping the skin GVHD is under control. 

I have just read Sophie's friend Max's update for today and he has been put back on the ventilator again. Please drop by and lend him your support. He is one amazing child with a wonderful family around him. His website is www.mighty-max.com 

Padraic

04/10/2003 T+24

WBC 8.7

Sophie was sick in the middle of the night but apart from that she had a ok day. She is still very tired, she still has diarrhoea, we are still worried about GVHD but trying to keep everything in perspective she is doing great.

I have not seen her today and although I try give all my attention to Amy as I will not be seeing her to next weekend, as the day goes on my mind keeps wondering back to Sophie.  I can not imagine how hard it is for Padraic who does not see Sophie much in the week now and all our family and friends who have not seen her for weeks. Lets pray our good fortune continues and we all home together soon.

Tina

03/10/2003 T+23

WBC 6.58

After a good night Sophie woke up at nine and was much more alert and responsive, which was a great relief. She still has diarrhoea but it was getting better. The BMT consultant believes the diarrhoea is caused by GVHD of the Gut as all the tests for infection came back negative. He said it was mild and will be controlled by the steroids she is already on for GVHD of the skin.  It is always scares you when they mention the dreaded GVHD, seeing what Max and his family are having to cope with .... well I have no words.

There are two BMT consultants at GOSH and every six weeks they alternate between the ward and the clinic. Today it was the consultant we saw prior to transplant who recommended the new protocol.  I think he was as pleased as us with how well Sophie has done. 

Tina  

02/10/2003 T+22

WBC 9.2

Sophie had a very restless night, she still has diarrhoea and had to have her bed sheets changed which disturbed her. Her weight has dropped again as a result. Although she is drinking lots of water she is now on an IV drip to replenish her fluids. The diarrhoea might be caused by GVHD, an infection or a reaction to her being on antibiotics for so long. They are running more tests and we should have the results tomorrow. She slept most of the day but Mummy had a nice little play before bedtime. A rash appeared on Sophie's legs in the afternoon which is being closely monitored as it could be skin GVHD. Her cyclosporin (a drug which fights GVHD) has been put back to IV this evening as she was not absorbing the drug correctly through her NG tube as a result of her diarrhoea. 

Padraic

01/10/2003 T+21

WBC 11.2

Tina had to come home last night as she wasn't feeling too well. The nurses took care of Sophie until Tina was well enough to return at lunchtime. Thanks Debbie and Laura for minding her for us. Laura had to give Sophie her bath today and apparently although she didn't cry she glared at Laura the entire time she was in the bath. Poor Laura, she was only trying to help. Sophie still has diarrhoea and as a result her weight has dropped a little. Her fluid balance is being closely monitored although she has been drinking lots of water in the last few days so hopefully she won't need additional fluid through the NG tube. She remains in good form and her appetite is improving. The "music lady" came in today and Sophie had a great time playing the piano and banging the drums, she loves to make a racket !!

I returned to work today for the first time in almost a month which took a little getting used to. Everyone was asking about Sophie which was nice and they were all so impressed by her progress (as are we and the nurses and doctors). I even got a hug from Phil !! We have had some wonderful support from everyone and it was nice to let them all know how much those guest book entries mean to us. I am told that Sophie's website may be appearing in the most accessed website audit report this month.  

Tony is making a big sacrifice on Friday.....he is having his legs waxed to raise money for "Jeans for Genes" day. What a brave (crazy?) man.

Padraic

30/09/2003 T+20

WBC 11.5

Sophie had a touch of diarrhoea last night and although Daddy somehow managed to sleep through all the nappy and sheet changes I think Sophie had a bad nights sleep as she had a 2 hour nap in the morning. She is getting back to her old self again and her latest trick is to pretend to be tired and lie down until her favourite nurse Mari comes in. She then gets a new boost of energy and starts showing off and playing "peek a boo".  It helps that Mari makes a bit fuss of her every time she comes into the room. The BMT consultant was here today and they are extremely happy with Sophie's progress. All her blood tests have come back clear of infection. The steroids may be reduced on Friday which will hopefully bring down her blood pressure. All going well we may be having discussions about when Sophie will be going home in a few weeks time which is something we haven't been thinking about. All Sophie's medication is now given orally so we are going in the right direction as regards the "H" word (home).

Padraic 

29/09/2003 T+19

WBC 14.1 

Sophie woke up this morning and was sick so I was a bit apprehensive about today, but she really perked up and was her old self again. Her blood pressure was consistently high so she been put on regular medication to keep it low. She been taken off more of her antibiotics as they are happy the temperatures are not caused by an infection.

She still teething, what is it with teeth!  

Tina

28/09/2003 T+18

WBC 11.8

Another quiet day today. Sophie was a little out of sorts, this may be because she's teething or what the doctor described as "post-BMT blues" which can often happen 2-3 weeks after transplant. Her steroids have been reduced and her blood pressure is a little high, this may be related to the steroids or to the engraftment. 

Sophie's cousins were her yesterday with their Nana. Katie who is 5 asked me why was Sophie in the hospital. I thought, "How can I explain Hurlers and a bone marrow transplant to a 5 year old". I told Katie that Sophie was missing a part from her blood and that she was at the hospital to get the missing part. Katie thought about this and then said "like a puzzle ?". "Exactly like a puzzle" I said. Sometimes it takes a 5 year old to explain things properly.

Padraic

27/09/2003 T+17

WBC 14.7

A quiet day compared to yesterday, not many days like yesterday. Although Sophie ventured out of the room today for a walk along the ward. Sophie was fine but Daddy needed medical care. Sophie was a little sleepy and wanted lots of cuddles, nothing wrong with that though.  The doctors are still pleased but monitoring her very closely.

Nana is going home to Ireland tomorrow so it was wonderful that Sophie was well enough for her to go into the room and give Sophie a cuddle. I was not there but I am sure it was very emotional. I was planning to take Amy tomorrow but alas she has come down with a cold so it has to be postponed. Soon my family will be together in the same room again, I find it hard to think about without crying.

Thank you for all your wonderful messages following yesterday. I know for many of you the battle has not been so uncomplicated I thank you for your generosity. 

Tina  

26/09/2003 T+16

WBC 10.9

100% Engraftment 100% Engraftment 100% Engraftment 100% Engraftment 100% Engraftment 100% Engraftment 

We got the news at lunchtime and even with the BMT consultant trying the keep our feet on the ground we were floating sky high. I find it hard to explain how we feel. We know it is still a long road ahead but a man, a stranger to us, gave us back our daughter.   

Sophie has been so brave and good natured. Simple things like mouth care she has made it easy for us to for her even though it tasted awful. 

I also hope the new protocol, the new chemo drug, played its part in all of this because that means other families will also share our joy. From the limited experience of the drug in Europe, mainly Poland, they thought it would be better for Hurlers children.

Lets just celebrate this joy for a moment, tomorrow is another day.

Tina

p.s. Sophie's WBC fell today as the G-CSF was stopped, this reaction is normal.

Tina 

25/09/2003 T+15

WBC 17.1

Sophie had another good day today, although she has been quite sleepy. She is probably catching up on some lost daytime naps. The doctors are very pleased with her progress as are we. Her fluid intake/output is being monitored along with her rash and her stools (sorry to be so graphic). She still has a low-grade fever which seems to come and go every few days. Sophie was very brave today as she had to have two heel prick blood tests to check her liver. She didn't cry and at one point laughed at her nurse Rosie as she tried to squeeze the blood from her feet. This was a great relief to Rosie as she hates doing "heel-pricks" on children.

We got a card from Sophie's donor today which contained the following poem which made us feel so grateful and made us cry:

Just one thing from me to you

It's the very least I could do

As if the child was my own

I'll give you something from the bone

I hope that things will go ok

I hope you now see past today

There's only one thing you must do

Tell people to give blood and marrow too

24/09/2003 T+14

WBC 14.0

Another big jump in Sophie's counts today, we still don't know if this is good. The G-CSF (growth factor) has been reduced so her counts should stabilise soon. The blood for the engraftment test which taken on Monday has gone missing, another sample was taken today and hopefully the results will available by the end of the week. Sophie is retaining a little water and has been given some medication to relieve her !! 

Her rash is much better and is not irritating her as much. She is feeding well and is starting to drink water again. She is quite happy in herself except for a bout of teething.

Sophie's Uncle Alan's 10k run raised £614 for Great Ormond Street Hospital. Well done Uncle Alan and thank you all for your donations. Please remember that it is "Jeans for Genes" day in the UK next Friday 3th October. This appeal raises money for 4 charities, 2 of which are directly related to Sophie's condition, Great Ormond Street Hospital and the MPS society. More information is available at the jeans for genes website www.jeansforgenes.co.uk , if you are not allowed to wear jeans at work there are denim ribbons you can buy.

Padraic

23/09/2003 T+13

WBC 7.9 

Sophie's counts have gone into overdrive. We will know whether these are the donor cells or Sophie's cells by the end of the week. What troubles us is how quickly her counts have gone up. We have read a lot of other Hurlers children's websites and have never seen another child's WBC count increase as quickly as this. When Sophie was admitted her WBC was 8.5 and it has now gone to 7.9 in a matter of days. The BMT consultant is also not convinced that the Sophie rash is GVHD ( Graft Versus Host Disease ) although it is being treated as if it is to cover all bases. If Sophie has got GVHD then the donor cells are engrafting incredibly quickly and she is probably already 100% engrafted with the donor cells. The other factor in all this is that Sophie is using a new chemo drug which has not been used at this hospital before. The consultants have no experience of how the engraftment works with this new drug so this may all be ok. We will have a nervous few days until the results come through. 

Sophie sneezed out her NG tube today and had a few hours of "nasal freedom" before a new tube was inserted. I thought it was quite funny when it came out (think spaghetti through the nose) although I don't think Sophie agreed. She has been a little grizzly today and still scratches as soon as she has access to her skin (pray it is skin GVHD). Nappy changes are a real battle, I'm sure that "inserting an unhappy octopus into a string bag" is easier that changing Sophie at the moment.  

Padraic

22/09/2003 T+12

WBC 3.6

Sophie cells are shooting up at a incredible rate which of course is good, but we are resisting the urge to think to far ahead and remain cautious. We will not know until the end of the week how much she has engrafted, how many of these cells are the new donor cells.  The BMT Consultant came into see Sophie and said that although the WBC is good because it means she can begin to fight infections it not a real indication of how well everything is going until we get the full picture. He also told us we cannot be 100% certain the rash is GVHD. 

Apart from the itching which gave her moments of distress Sophie had a good day and played really well. 

I just wanted to thank you for all the e-mails and guestbook entries, I cannot explain how important it to us, it gives us a lift to know you are out there thinking and praying for Sophie. Thank You.

21/09/2003 T+11

WBC 0.9

What a day! After I spoke to Padraic yesterday the doctor prescribed something to help and she went straight to sleep. The next morning it was the same again and her skin was covered by a rash that was worse then it has ever been. The doctor came in straight away and first told us the good news of Sophie's white blood count, it was 0.3 yesterday and 0.9 today. This is fantastic much quicker then we  could have hoped for however the rash is Growth Versus Host Disease (GVHD) of the skin. She was very positive and said with steriods and cream this will not be a problem and is quite common.  Tomorrow they are going to do more tests the see whether the new cells are Sophie's or the donors. Keep your fingers crossed.

Apart from that Sophie had a great day once the anti-itching medicine kicked in and played and ate really well.

----   The latest update for Max is here  Max's news ----

20/09/2003 T+10

Sophie had another good night last night she woke at about 7:00am. She decided that she didn't need a nap in the morning and kept Tina busy until about 1:00pm. Sophie spent most of the morning playing "peep a boo" with every nurse that came into the room. She now turns whenever she hears someone at the sink washing their hands as it means there is a new face to play with. I think she is at her happiest when the BMT consultant comes round as there are normally a few doctors and nurse in tow to admire her !! 

Amy and her Nana came up to the hospital in the afternoon. Amy thought that Sophie looked beautiful with her new "haircut" as did her Nana. I spoke to Tina tonight and Sophie is itching her head and tummy which is stopping her sleeping. The night nurse will speak to the doctor on call to see if there are any meds which she can be given to alleviate the problem. 

Padraic  

P.S. 

For those of you who are following our friend Max's progress his website won't be updated until Sunday as his webmaster is on vacation. I have added the latest update here Max's news

19/09/2003 T+9

Sophie had a good night last night and so did Daddy as Sophie filled her nappy at 1:00am while the night nurse was checking on her which meant Daddy didn't have to change her. Thanks Nurse Debbie.  Sophie's chest X-ray came back clear and the infection in her Hickman line is now gone. The BMT consultant had a look at Sophie's spots, he thinks it may a reaction to one of the antibiotics and so he has changed it to another antibiotic which performs the same function. Sophie's anti-fungal medication will be increased as she is still running a mild temperature and we need to cover all bases. We discussed her progress which has been very good so far. Sophie had her first dose of G-CSF (growth factor) yesterday her WBC counts should start to increase in about a weeks time. We are now entering the critical phase of the transplant process. Tina brought up some chocolate cake which Sophie enjoyed very much for her tea.

I am still getting used to her new hairstyle, I have added some new pictures so you can let me know what you think but I think she looks adorable.

Padraic 

18/09/2003 T+8

A better night was had by all except Sophie woke up at 2am and wanted to play but she soon settled.  

Her spots on her body are turning into blisters so the doctors think it might be another infection. They said not to be too concerned as she already on so many different antibiotics she should be covered, but they called in another doctor to have a look and last I heard he was on his way.   

The clippers were brought in and now our baby has very short hair. Padraic says she looks wonderful so I am looking forward to seeing her tomorrow. Pictures have been taken so you should all see her soon.

Tina

17/09/2003 T+7

Sophie was up most of the night again and was sick at 5 o'clock. I think she might have had too much milk from the overnight feed. 

She was not quite her self today and was quite tired but this might be because her Platlets were 10 so she had a transfusion at teatime.

Although her temperature is going in the right direction the doctors decided to double check everything so she had a chest X-Ray today and will be having a echo in the next few days.

She is still eating well and had over half a jar of pork and pasta for lunch and another Fromage Frais for tea. Of course Wotsits were well received for her afternoon snack.

Her hair is really falling out and my efforts at hairdressing will not be enough, so tomorrow the big guns will be called in. 

Padraic brought in a picture of Amy that he took today and Sophie got really excited and kissed it.

Tina  

16/09/2003 T+6 

Sophie didn't have a great nights sleep last night as she had diarrhoea and needed to be changed regularly. Her temperature has been  below 38 degrees all day so hopefully the line infection is clearing. Sophie played really well today and her appetite has started to come back. For tea she had a whole fromage frais and drank some water. She was given some new drugs today and while Tina was playing with her her entire face went red except for her cheeks. This is known as "red face syndrome" and is quite a common reaction although that doesn't make it any less startling. Her drugs have been diluted more to alleviate this problem. Sophie started to lose her hair today so Mum gave her a quick short back and sides but apparently the results will not lead to a new career in hairdressing. The BMT doctors checked in today are very pleased with Sophie's progress, everything is as expected so far.

Sophie's Nana has arrived from Dublin to give her Mormor (Tina's Mum) a break from taking care of Amy. 

Padraic

15/09/2003 T+5

Sophie had another good day today. An infection has been identified in her Hickman line which we hope is the source of her temperature. She is already on a course of antibiotics which should clear the infection. She is still quite sleepy and not feeding at all so she now has two daily feeds through her NG tube. She has a rash in her nappy area which is not clearing, it could be a reaction to one of the chemo drugs as it started prior to the transplant. We are trying various creams to clear it but so far without success. We will have it checked out by the BMT consultant tomorrow. When she is awake Sophie plays well but she gets tired quite easily and quickly lets you know that she wants to play lying down in her cot by flinging herself backwards. 

These first five days have been quite easy but we are preparing ourselves for a more difficult time from about day 7 onwards.

Padraic

14/09/2003 T+4

Sophie was still tired and had a temperature today but she was happier. She has an infection which is the cause of the temperatures and they started the right antibiotics so it should be under control soon.  

Her Haemoglobin count was low today so when I left they started a blood transfusion. I have just called Padraic and her energy levels have been given a boost, so bedtime has been postponed. 

Still not eating much but a bowl of wotsits were a great success again. It was great watching her and today's nurse Mari had to stay to watch for awhile. 

Tina

13/09/2003 T+3

Sophie had a good night and slept well. She was on a supplemented milk feed overnight and I think she may have had a little too much because she was sick as soon as I sat her up in her cot. She had a mild temperature so she has started another course of antibiotics. She has been a little warm since her previous antibiotic was stopped so some investigations will be done to determine the source of her increased temperature. She is really off her food at the moment but the feeds she receives through her NG tube are maintaining her body weight. Sophie was however quite enthusiastic when I presented her with a bowl of wotsits (cheesy puffs), she managed to lick each and every one of them and even ate a few. She also had a milky way bar so not all her taste buds have changed. Sophie has been quite sleepy today although when she is awake she plays very well and is still giggling. 

Please keep Sophie's friend Max in your prayers, he is slowly improving but he has just been diagnosed with moderate GVHD (Graft versus host disease). His website is www.mighty-max.com

Padraic 

12/09/2003 T+2

Sophie was still very tired today but happier in herself and her skin is improving. The results from the x-ray of her Hickman line shows it is in the right position. The IV specialist checked it and was able to draw blood from both lines so no further investigation will be required. 

The BMT consultant saw her today and is happy with her progress.  He told us that the Bone Marrow she was given had a high cell count which should improve the chances of engraftment. Which is really good news.

I spent the day with Amy today and she is doing ok. We went for a walk and she told me today that she wished Sophie was with us and that she is missing her. She also told me she wanted a big ladder as she was going to the moon. I want to be like Amy when I grow up.

Tina

11/09/2003 T+1

Sophie wasn't to happy today. Her skin seems to have become really sensitive and even her nappies are making her skin red and sore. The doctors seem to think it either a reaction to one of her many drugs or a reaction to the actual Bone Marrow. Nothing to worry about though and they are going to give her some cream to help.

Sophie has to have bloods tests once, twice, sometimes several times a day which they take via the Hickman line. It unfortunately is not cooperating and it is taking a long time to extract any blood. Today they tried to see if it was blocked and she had a chest x-ray  in her room to see if there is a problem with the position. We will find out the results tomorrow, hopefully she will not have to another operation.

She is hardly eating now and is mainly being fed via her NG line. I think it is time for the Chocolate cake to be brought in!

Tina

10/09/2003 Transplant Day

WBC 0.07

Today was the day we have been waiting for since April. Sophie had her transplant at 6:00pm. The transplant itself is simply a bag containing  bone marrow which is diluted with a saline solution and then given to Sophie through her Hickman line. The whole process takes about 5 hours as the bag is flushed several times to ensure that Sophie gets every last bit of marrow. She has been in quite good form today although she is getting increasingly tired and likes to lie in her bed playing. Her temperature has not changed all day so hopefully the mild fever she had has gone. Her eye examination will have to wait until Sophie is able to leave her room as it needs to be carried out in another part of the hospital.

From today Sophie's recovery from the whole process starts. Her white blood cells count (WBC) will hopefully start to increase in about two weeks time. This is the first indicator that the new marrow is producing cells. Days 7 to 21 are the critical days as any serious problems are likely to appear during that period. After about 4 weeks blood tests will be carried out to determine the percentage of cells are being produced by the transplanted marrow and by Sophie. We need Sophie to produce 100% of her cells from the donor. 

Finally thanks to everyone who takes the time to keep up to date with Sophie's progress and for all the nice guestbook entries. It really does help us to know that Sophie has a great deal of people praying for her. I have added some new photos to the site.

Padraic

09/09/2003 T-1

Sophie had a busy day today. She was up with the lark at 6:00am and ready to play. A yoghurt at 8:00am and it was back to bed for a well earned rest at 9.00am. Or so she thought. It seems to me that the doctors here wait for the children to fall asleep before they start their rounds. The BMT doctor arrived at 9:15am and said that Sophie had a mild fever, this was nothing to worry about but it explained her temperature spikes over the last few days. At 10:30am the physiotherapist and occupational therapist arrived with a new chair for Sophie. The chair is very comfortable and the designers have added a "rocking horse" feature which Sophie enjoyed very much. She had an echo scan on her heart at 12:45am, yes you've guessed it 20 minutes after she had fallen asleep. It was all fine, the leak in her valve has not deteriorated since the last scan. She was due to have an eye examination but that never happened, we will chase it up tomorrow. 

Sophie started two new drugs today, Cyclosporin which will help guard against Growth Versus Host Disease (GVHD) and Acyclovir which fights off viral infections. I also got some details of Sophie's blood counts, her counts on 01/09/2003 (admission date) where

Her current counts (09/09/2003) are

My understanding of these figures is as follows, but please remember I am not a doctor.

Post transplant we will be looking for an increase in these counts showing that the bone marrow stem cells have migrated to the bones and are producing red cells, white cells and platlets. 

Sophie's transplant is due to take place at about 5:00pm tomorrow, I will bring a camera and hopefully post some pictures tomorrow.

Padraic

08/09/2003 T-2 

Sophie is now beginning to get tired, this is to be expected but she is doing very well. In between her frequent naps she is still happy and playful. Her latest game is throwing things out of cot and laughing as Mummy and Daddy try to catch them before they roll under something and become lost forever. 

Her eyes have been flicking more then normal so somebody from the neurosurgery came to check that the shunt was fine and as she has no other problems and her head feels fine he was happy. Her eyes are going to be checked tomorrow.

Congratulations to Uncle Alan who completed the Nike 10K breaking all records, not saying which ones!

Tina  

07/09/2003 T-3

First sorry I did not update yesterday. I swapped with Padraic last night and by the time I put Amy to bed I was so tired. 

Sophie was fine again but is not drinking any milk or water which means she has to be feed through her NG line. I hate this, I know it is very important she gets a litre of fluids a day to flush out the drugs but I want to feed her, I want to hold her and feed her. She is still eating though, especially her yoghurt. 

The doctors believe her fevers were a reaction to her drugs and not an infection which is great news. She should be finished her chemo today.

Back to the washing!!!

Tina

06/09/2003 T-4

Sophie had another good day. She is being given lots of fluids by her NG tube which she has now stopped trying to remove. Today is the last day for the Campath and Treosulphan, her other chemo drug Fludabarine will stop tomorrow. Sophie will then start the drugs directly related to her transplant. I will get details of her latest blood counts tomorrow so we can see the counts decreasing and hopefully increasing post transplant. Sophie's sister Amy came to see her today and we were able to take her into the corridor outside Sophie's room. Sophie almost jumped through the window when she saw her which left Tina and I a little weepy eyed. Amy left the Barbie cake from her birthday party for the nurses to have with their tea.

Tomorrow Sophie's Uncle Alan will be running in the Nike 10k to raise funds for Great Ormond Street Hospital. It's not to late to sponsor him. His email address is alanoconnor@btopenworld.com, thank you Nero (my much too infrequent golfing partner) for your generous contribution. 

Padraic 

05/09/2003 T-5

I had my first full day at the hospital today. Sophie had her final chemo drug (Treosulphan) late last night. She didn't have any adverse reaction and she had a reasonably good night. I however was woken about 10 times in two hours as the alarm on the IV pump kept going off, I had to leap (ok crawl quickly) from my bed hoping it wouldn't wake Sophie. We had quite a good day today until teatime when it was decided to put a nastro-gastric (NG) tube down Sophie's nose as she hasn't been taking a lot of fluids. This is something she would have done next week anyway so it's no big deal. Sophie was less than impressed and the NG tube lasted about 10 seconds before she managed to remove it from her nose (I must say I would have done the same thing). The second "fitting" was much more difficult as she knew what was about to happen. This time lots of tape was used to stop her repeating her previous trick. Sophie was running a temperature at teatime so she will be put on an general antibiotic in case she has an infection. Her bloods will be tested for infection tomorrow. The NG tube can be used to administer Sophie's oral medicines and top up her fluid intake so it will make things a little easier.  Sophie will have her second dose of TreoSulphan tonight. 

Padraic

04/09/2003 T-6

Sophie had another fever today so she was seen by ENT who rechecked her ears and they are clear now. They are going to keep taking cultures from the Hickman line to make sure there is no other infection. She started chemo proper today.  I was feeding Sophie her tea and in walked a nurse with gloves up to her elbows and a face visor, I was warned but it still takes your breath away. 

I (Sophie's Mum) noticed Sophie got a new tooth yesterday, she now has eight teeth all going in different directions but I think they are lovely.  

03/09/2003 T-7 

Sophie had a bit of a restless night. Her blood pressure was a little low and she had a slight temperature. By lunchtime however her temperature had gone and her blood pressure returned to normal. This was probably just a reaction to the chemo. She had her second batch of Campath and seemed fine. She ate well at lunch and tea and went to bed at about 8:00pm. She will have more meds at 10:00pm and 12:00am so she will probably be woken up a few times. We will be moving to another room tomorrow which will Sophie's home for the rest of her stay. From next Tuesday (T-1) Sophie will be kept in isolation for a few weeks, so on Saturday we will bring Amy (Sophie's big sister) up so she can see where Sophie has been for the last week.  I will get Sophie's blood counts tomorrow so we should be able to see that the chemo is lowering her immune system. We have to enjoy this little "honeymoon" period because we know it is about to get difficult in the next few days. Tomorrow will be my last day at work for a few weeks so Mum (Tina) will have the night off to play with Amy who is missing her Mum and baby sister. 

02/09/2003 T-8

After another seemingly endless batch of pre-meds Sophie had her first course of chemotherapy (Alemtuzumab (Campath)). She will be closely monitored in case she has an allergic reaction. She has been out of sorts today, probably a bit upset at been given different medicines every few minutes. At teatime she was a little warm so she was given some medicine to reduce her temperature. She went to sleep after her night-time bottle. 

As a result of Sophie's condition we have made a lot of new friends through the internet. One of them is really sick now and we need you to pray for him. His name is Max and he is 19 days post BMT. Max's parents Mike and Margaret have given us great support in the last few weeks. His website is www.mighty-max.com,  please drop in and add a guestbook entry.

01/09/2003 T-9

Sophie was admitted to Great Ormond Street today to begin the transplant process. Writing T-9 at the start of this entry has made it all very real and very scary. She started on a course of pre-meds, mainly antibiotics which help fight a variety of infections. All was going well until one of the meds was in tablet form. The crushed tablet went in and then came out along with her tea. Next time she will have this medicine in liquid form. 

30/08/2003

Sophie had a reasonable night's sleep and she is slowly returning to her usual self although I think she is still quite sore from the operation. We returned home today and tomorrow (Sunday) will be our last day together as a family for a little while. 
I have added some new pictures to the website. I have also started to archive some of the older diary entries as this page will start to become very full from next week.

29/08/2003

Sophie had her "back-up" bone marrow harvest and her Hickman line fitted. As she was under anaesthetic for a few hours she was very sleepy for the rest of the day. She has something to eat at teatime and promptly brought it all back up along with the milk she had earlier. We were shown how to care for the Hickman line and what to do if there are any problems with it. At least now it will be easy for Sophie to have all her blood tests.  

28/08/2003

Sophie was admitted to Great Ormond Street in preparation for the insertion of a Hickman line. 
(A Hickman line is fine plastic tube which is inserted into a large vein in the neck and then tunnelled under the skin (to prevent infection) until it exits the body at the side of the chest. There are two tubes attached near the exit point. One will be used to administer chemotherapy drugs and the other to take repeated blood samples.) 

She had some blood extracted but not without some difficulty (she doesn't give up her blood willingly). We had a meeting with Dr Veys from the bone marrow transplant unit. The new set of drugs has been approved for use in Hurler's children. It is hoped that this new set of drugs will improve the chances of a successful engraftment while also reducing some of the side effects of chemotherapy drugs. Sophie will be the first Hurlers child to use this new criteria although the drugs have been used in the treatment of other conditions. As a result of this, our schedule has changed a little. Sophie will now be admitted on Monday 1st September, she will have an echo scan on her heart to ensure that her leaky heart valve is unchanged and then the chemotherapy will commence on Tuesday 2nd September. The transplant date is still 10th September. 

27/08/2003

We had a follow up consultation for the grommets which were fitted on 29/07/2003. Sophie has a small infection in her left ear. She has been prescribed a course of antibiotics and ear drops to ensure that the infection is cleared prior to the commencement of the chemotherapy. The dates for the transplant are now almost 100% certain and we should receive final confirmation this Friday. Sophie will be admitted to hospital tomorrow and have a Hickman line fitted on Friday. We hope to come home on Saturday and return to Great Ormond Street on Wednesday 3rd Sept to begin the transplant process.

26/08/2003

In the midst of all these hospital appointments and operations it is very easy to forget one thing. This transplant would not be taking place without a suitable donor. There is a young man living in the UK who has never met Sophie but nevertheless is prepared to undergo surgery in order to give Sophie a better chance in life. We are incredibly indebted to this man and we owe Sophie's entire future to his selfless generosity. Please consider becoming a bone marrow donor, you really can save someone's life.

15/08/2003

Sophie had a back X-ray and we had a meeting with Mr Tucker the orthopaedic consultant. In order to deal with the kyphosis in Sophie's back she will need to have a major operation to fuse her spine once she has recovered from the BMT. We will hopefully have an MRI scan on her spine prior to the BMT to minimise any delays.

13/08/2003

Another busy day at Great Ormond Street. We started with a dental examination and Sophie was given the all clear (we kept quiet about all the chocolate cake Sophie likes to eat). We were then given details of the BMT schedule, although it has not been confirmed it is a starting point. The current dates are

                                                        28/08/2003     Operation to fit Hickman line

                                                        04/09/2003     Chemotherapy starts

                                                        10/09/2003     Transplant day

Sophie finished up with a chest X-ray, the results of which will determine whether a sleep study needs to be completed. We were given a quick tour of "Fox" ward which is where we will be during the BMT. Having the dates has made it all very real and very frightening. It is only 2 weeks until we start the countdown.

10/08/2003

A fun day was had by all as temperatures reached the magic 100 degrees here in London. We spent the afternoon camped in the bedroom with the air conditioning unit working overtime. I have had enough of this global warming, I'm Irish and my wife is half-Norwegian, we struggle when the temperature is in the 80's.

05/08/2003

Sophie had an echo scan on her heart today. This was a more detailed scan than her previous one as various measurements of her heart had to be taken. During the scan the radiographer noticed than one of the valves in Sophie's heart is not functioning 100% correctly and that blood is leaking back into the originating chamber. This is not a problem, just something that we have to be aware of and which will be monitored closely. It was 33 degrees Centigrade in central London today, so we had great fun looking after a baby who was teething and who is not very good a regulating her body temperature (yet another wonderful aspect of Hurler's Syndrome) whilst travelling in a car with no air conditioning. 

We had a call from the BMT unit and Sophie will have a chest x-ray and dental examination on Wednesday 13/08/2003. The results of the chest x-ray will determine whether there needs to have a sleep study. Sophie's breathing has been fine so this shouldn't be necessary. Sophie will start a course on anti-fungal medication from next week in preparation for the transplant.  

31/07/2003

We had a meeting with the neurosurgeon today to discuss the results of Sophie's CT Scan (post shunt insertion). We were shown the CT scans before and after the operation and they show that the shunt is working well and there is no longer any fluid build up on the brain. Dr Solanki believes that the problem was probably caused by a cyst on Sophie's brain ( this is not necessarily related to Hurlers). There is no need to operate on the cyst as the shunt is working, however if for any reason the shunt has to be removed (i.e. if it becomes blocked or infected) then the cyst will be examined and dealt with if necessary. 

Sophie will have another check up in 3 months time.

29/07/2003

Sophie had an operation to have grommets inserted into her ears today. It all went well, Sophie was a bit grizzly afterwards but was back to her old self by the time we got home and made up for her fasting by polishing off her tea and demanding more !! She will have another hearing assessment at the end of August.

The insertion of grommets should ensure that Sophie is less likely to get ear infections which would be a concern during the bone marrow transplant.

24/07/2003

We had a call from the transplant team and they are trying to arrange the transplant for the 2nd or 3rd week in September.

We have also read more details on Genzyme's Enzyme replacement therapy for Hurlers. It seems that the therapy helps bone joint function which is one of the things that the bone marrow transplant does not improve. Sophie will hopefully be able to receive the enzyme replacement therapy once she has recovered from the bone marrow transplant.

23/07/2003

We had a meeting with Dr Veys from the bone marrow transplant team. He explained that there were two possible donors for Sophie and that they were 10 of 10 matches which is almost as good as a sibling match. Further tests will be carried out to determine which donor is most suitable for the transplant. Dr Veys outlined the three phases of the transplant process. Firstly, Sophie will have a Hickman line fitted under general anaesthetic and she will then have 7 days of chemotherapy. Secondly, the transplant will then be carried out and we will know in 14 - 21 days whether donor cells are being produced. The third phase is the recovery phase as Sophie's immune system returns. Sophie will be kept in isolation during the transplant process as she will have no immune system and so will be unable to fight off any viruses. It is estimated that Sophie will be in hospital for about 2 months. There is a 30-50% possibility that the transplant will fail and will have to be redone. There is also a possibility that she may not survive the transplant, the most dangerous time are the days following the transplant as she is vulnerable to infection and Graft Versus Host Disease (GVHD). There is a 10-20% chance that Sophie may not survive the transplant but we feel that we must take this chance as without the transplant Sophie is not likely to survive beyond her 10th birthday and will start to deteriorate from her second birthday. We are awaiting some information on the transplant process and we will add more details to the website when we receive them.

16/7/2003

CT Scan following the shunt operation. This was a lot easier that last time as Sophie did not have a general anesthetic she was sedated instead. We will get the results of the scan on the 31/07/2003.

09/07/2003

We had a call from Great Ormond Street today. They have found a donor for the bone marrow transplant. We will have a meeting with the bone marrow team on the 23rd July. While this was the news we were waiting for we know that we have a difficult road ahead.

26/06/2003

More appointments: 16/07/2003 CT Scan to check that the shunt is working correctly. 29/07/2003 Operation to fit grommets

25/06/2003

We had a meeting with Dr Vellodi at Great Ormond Street. Following the full skeletal X-rays Sophie has been referred to the orthopaedic department as there are two curvatures in her spine. Scoliosis (sidewards curve) and Gibbus (back to front curvature). She may be fitted with growth rods with can be adjusted at regular intervals to straighten her spine. There is some concern following the developmental assessment. It has been shown that children with Sophie's level of developmental delay have more developmental problems following a Bone Marrow Transplant. However the Hydrocephalus and her other physical disabilities will have affected the results of the developmental tests. Sophie may also undergo Gene Therapy after the bone marrow transplant if required.

24/06/2003

Sophie had a developmental assessment at Great Ormond Street. Her developmental delay is close to what we expected. It is difficult to tell how much of the delay is caused by the Hurler's and how much is related to the Hydrocephalus and Sophie's problems with her vision and hearing.

23/06/2003

No news on a bone marrow donor yet, tests are still being carried out. The bone marrow team are confident that they will find a suitable donor (and a reserve if possible) in the next few weeks.

4/06/2003

Sophie is in much better form today. I got smiles and cuddles when I came home from work and she seems much more settled.

3/06/2003

Sophie has been a little out of sorts for the last few days. We spoke to the neurosurgery department who said that she would be getting used to the shunt for the next few weeks.

30/05/2003

Sophie came home today. She has been in much better form since the operation and is showing few side effects so far. Her eyesight is definitely improving and I now expect to lose my glasses each time I pick her up.

26/05/2003

Sophie had her operation to have her shunt fitted. It all went well and she spent most of the day sleeping.

24/05/2003

We received a call from the neurosurgery department asking to come in the on the next day to have the shunt fitted as there was a concern that the increased pressure in Sophie’s brain could cause some damage.

22/05/2003

Dr Vellodi said that the CT Scan showed that there was evidence of raised pressure in Sophie’s skull and that she would need to have a shunt fitted. A tube is placed in the skull and the excess fluid is drained into the stomach. The tube has a valve which opened when the pressure is too high.

Some good news. The BMT team phoned to say that they had found a number of possible matches. We arranged to bring Sophie in for some additional blood tests.

21/05/2003

The BMT team at Great Ormond Street phoned to say that Amy was not a match. While we had prepared ourselves for this we were devastated. The BMT team will now carry out a search on the bone marrow donors database. We were told that as Sophie’s tissue type was not unusual we had a good chance of finding a match.

1/05/2003

Sophie had an Echo Scan (which showed her heart was fine), a full skeletal set of X-Rays, and a CT Scan to check for Hydrocephalus ("water on the brain")

29/04/2003

Blood test for the family to test for a tissue match.

16/04/2003

Dr Vellodi phone to say that the test results had come back and confirmed that Sophie had Hurler’s Syndrome. We asked that tests be arranged for us and daughter Amy to determine if we were a match. Amy has a 1 in 4 chance of being a match.

9/04/2003

We had a meeting with Dr Vellodi of Great Ormond Street Hospital, London. He examined Sophie and agreed with Dr Hyer’s prognosis that Sophie probably had Hurler’s Syndrome. He talked in great detail about the condition said that if Sophie did not have a bone marrow transplant she would die before the age of 10. We said that we had discussed the transplant and decided that even though there are risks that we must try to give Sophie every chance of a normal life by giving her a bone marrow transplant.

8/04/2003

We returned to Northwick Park for blood/urine tests to confirm the diagnosis.

7/04/2003

This was the day that every parent fears. Following our concerns about a "bump" on Sophie’s back and her slow physical development our GP referred her to Northwick Park Hospital, London. Dr Hyer examined Sophie and asked us lots of questions. He then said he believed that Sophie had a condition called Mucopolysaccharidosis Type 1-H (or Hurler's Syndrome) and that the only treatment was a bone marrow transplant.