10/09/2008

Five years ago we sat in a hospital room and watched simple procedure gave us back our daughter. From that moment on she was no longer terminally ill, we knew that we had a long road ahead of us but we were full of hope and as long as we wake up to her smile every morning we would be ok. Well a lot has happened over the last five years but that feeling has not changed. Some moments I am overwhelmed by joy to have her in our lives she just takes my breath away.

We have just started a new school year and she has settled in really well. She did so well last year and she exceeded all expectations, she is popular with pupils and teachers alike. We had sports day at the end of the school year. Sophie refused to run holding the helper's hand and ran by herself. The whole school was chanting her name and the parents cheering. I found it difficult to hold back the tears.

Medically things are fine, she had back surgery in May and it all went well. She grew 1.5cm on the table. She should have her next spinal surgery in May. We did have a scare at the beginning of the year, she has a large cyst in her brain which our metabolic consultant thought was growing based on her last MRI scan. He got us appointment with a Neurosurgeon within a week who feels it is not growing just very large. She could have a operation to reduce the size but the operation is very risky. So he recommended that we wait to see if it causes any problems, the first sign would be her sight deteriorating, and then operate. As it might not cause any problems we decided that this is what we will do. As you can imagine it was not an easy time but it reminded us not to take things for granted.

We have added new pictures so you can see how much Sophie has grown. We have also created a slideshow here it takes a few moments to load but it's worth the wait. Turn your speakers down if you are at work as it has music.

On the 28th September we will be taking part in a sponsored walk for GOSH BMT Research Fund. We hope to raise as money as we can and if you would like to sponsor this very worthy cause we have set up a JustGiving website here

19/10/2007

I have just noticed that it is almost 6 months since the last update, the longer I put it off the more there is to write so I hope I don’t forget anything.

Sophie had an orthopaedic appointment following the rod lengthening surgery and everything is fine. The next lengthening procedure has been scheduled for February 2008 which will be 11 months after the first lengthening. Sophie had her regular hearing test and we were thrilled to hear that her glue ear has improved to such an extent that she now longer needs to wear hearing aids. Sophie had stopped tolerating her aids a few months beforehand so while we weren’t surprised it was wonderful to have her improvement confirmed. Sophie also had a test for carpal tunnel syndrome and there are no problems. In August Sophie had her annual blood tests and a full X-ray skeletal survey. Sophie was an absolute star throughout all the tests and the medical team did a wonderful job entertaining her. The blood tests which are normally very stressful (Sophie is usually very difficult to get blood from) went extremely well. We had our six monthly meeting with the metabolic consultant and the blood tests and X-ray’s were fine.

Now onto the non-medical news which thankfully there is a lot more of. In April we were contacted (via the MPS society) by a charity called Wellchild and asked if we would be interested in having a garden makeover for Sophie. The primary purpose of the makeover was to make the garden level and safe for Sophie to play in. In May, 11 volunteers organised by Wellchild came and in one day they cleared the garden, levelled the lawn and laid new turf. You can see the amazing results in the May pictures. Sophie spent all summer in the garden and can now come in and out from the garden by herself. It makes such a difference knowing that Sophie can play safely in the garden without being in danger of falling over and hurting herself.

We went to the MPS society’s conference in Northampton and had a wonderful time. It was nice to meet up with old friends and to make new friends with families of children with MPS conditions. Amy and Sophie went to a theme park for the day and had a great time. We were nervous letting Sophie off for the day but she had a great volunteer who had looked after Sophie at the last conference so it made it a little easier. In other news, the MPS society have updated the MPS-1 information booklet and Sophie’s picture is on the front page (the picture used may be seen in the May 2007 photos, it is the third picture from the top).

Sophie turned 5 in June and she really enjoyed her birthday. She now understands the concept of presents and birthday cake. Sophie loves singing “Happy Birthday to you” and often says it’s Daddy’s birthday so she can sing along.

In July we bid an emotional farewell to Sophie’s nursery. On the last day the children put on a show about the solar system and Sophie played her part as one of the planets beautifully. Her taking part showed us how far she had come in the two years she had spent there. We are deeply indebted to Rosie and all her team for the incredible support they have given Sophie in the past two years. Fortunately, Sophie’s one-to-one support at nursery was able to transfer to her primary school which was a great relief.

On the 10^th September, exactly 4 years after her bone marrow transplant, Sophie started school. This was a day we wondered if we would ever see and it proved to be very emotional. Sophie was very excited and settled in very quickly. Seeing Amy and Sophie together in their school uniforms made them both seem so grown up. Sophie’s new teacher was Amy’s teacher in her first year and we know she will take good care of Sophie. The school have been very supportive and have gone out of their way to make Sophie’s transition from nursery to school as smooth as possible. Sophie has settled well into school and has made looks of new friends. She loves the fact that she and Amy are in the same school and that she still gets to pick Amy up from school. Sophie stays for half a day at school and she recently started staying for lunch. We will build her time up slowly so eventually she will stay for a full day.

At half term we made a trip to Disneyland, Paris and we had a wonderful time. Sophie really enjoyed the “It’s a small world” boat ride and now sings the tune from it. Amy loved the roller coasters, she went on the “Thunder Mountain” and “Crush’s Coaster”, the next time we go she wants to try “Space mountain” which she is not tall enough for yet. We are going to Alton Towers next year with the MPS society so I am looking to a weekend of high speed spins, drops and loop the loops !!!

In the last few months the biggest change we have seen is in Sophie’s speech. She is learning so many new words every week and constructing longer and more complex sentences, it is amazing. Her understanding of the world around her has also improved incredibly. Sophie’s sleep pattern is also much better, she now wakes at about 6:00am which is much more bearable that the old 4:30am wake up calls. Sophie comes into our room whenever she wakes up and announces.. “It’s 6 O’Clock now, time to get up”, I think we have the best alarm clock in the world.

I think that’s all our news for now, I have added new pictures for July to October. Next time, I’ll try not to leave such long gaps between updates, the longer I leave it the longer it takes to write.

Love

Padraic

07/04/2007

Where do I begin, I am so sorry for the lack of updates, I won’t bore you with excuses there are many but mainly I kept starting and then got distracted and the next time there was more to write. Hopefully this time I will finish and you get to read this.

Sophie is doing really well and amazes us every day. Her language and personality has just exploded in front of our eyes. She gives us a running commentary of her life which can be very entertaining. She has discovered the concept of “NO” and “MINE” which makes life interesting . Dressing up is a passion and we went to the hospital for therapy one day with her dressed as a Doctor saying to everyone ‘Hello, I am a doctor!” to the great delight of everyone.

On March 23^rd Sophie had her first rod lengthening surgery and it all went very well. The surgeons were able to lengthen the rods by about 2cm which is fantastic. We saw the x-rays post surgery and her back is much straighter. Sophie was very sleepy and in pain after surgery but by Saturday morning she was running about and we were able to go home in the afternoon. She will have this procedure every year so it was a great relief to see her bounce back so quickly but it does not get any easier for us and I seem to get so angry with the world afterwards.

Late last year she had an assessment at the Wolfson Centre. They specialise in neurodisability and visual impairment. We went in with the question “How much can she see?”. We see Sophie, who watches TV; who can see colours; who runs around the house like any sighted child. Then we have appointments with the consultants who have re-tested her and basically say that she is borderline blind and the improvements we are seeing are her using her sight better. We also wanted an idea of how developmentally delayed she was and how much the visual impairment contributes to this delay.

The meeting was attended by her Visual Impairment teacher and OT therapist so they could give their input. Sophie was a star and worked very hard. At the end we were all tired it was an emotional day. They felt Sophie was more partial sighted and could see very small things within close range and has some sight up to a meter away. They felt developmentally she was about two years behind but if you compare her with a group of visual impaired children she was 18 months behind. We are going to see them again in a year so it will be interesting to see how much progress she makes especially with her sight. Who knows maybe she will see her rainbow, with Sophie everything seems possible.

Sophie is going to start school in September and she has a place at the mainstream school Amy attends. She will have one-to-one support and I have already had a few meetings with the team and head. I have to say they have been very supportive and we have had the great news that the one-to-one support at nursery will transfer with her which is such a relief. We will be having more meetings next term trying to prepare for a smooth transition period.

Finally, Great Ormond Street have just completed their 1000th BMT and wanted to use that feat to publicise the need for more donors. They asked if we would tell Sophie's story. Well she is on the front page of both our local papers. This one is online:

http://www.harrowtimes.co.uk/news/localnews/display.var.1260615.0.sophies_new_lease_of_life.php

We have also added some new pictures..

Love

Tina

21/09/2006

I am sorry it has been so long since we updated the website. We got a new PC yesterday so now we have no excuses. I will carry from the last update in May. Sophie's second hearing test showed that she has a moderate hearing loss and needs to wear her hearing aides again especially for nursery. We had an appointment with the orthopaedic team and Sophie's back is fine. She is scheduled to have her "rod lengthing" surgery in February 2006 which will be 12 months after surgery. She is spending more and more time out of her brace which is great as we had a very hot summer. She still sleeps in it and wears it whenever she goes out but when she is home she can stay out of it for long periods.

Sophie started back at nursery on Monday. We decided that although Sophie was due to start school that she isn't ready and would benefit from another year at nursery. This wasn't a straight-forward process and we had to have Sophie's statement of special needs amended to accommodate the change. There was a meeting with the council and we had Sophie's Montessori teacher, her visual impairment teacher, her hearing impaired teacher, portage, her occupational therapist, her physiotherapist and health visitor all supporting our request that Sophie could stay another year at nursery. It was a very nervous meeting for us but we are so lucky to have such a wonderful group of people who support Sophie's needs. After each specialist had expressed their support for Sophie's deferral we couldn't see any reason why our request could be rejected. Even so, we had a nervous few weeks until we got the news.

Sophie had a lovely summer. We went to Dublin to see Sophie's grandparents and we had a wonderful week in Cornwall. Sophie loved the sea and wasn't even remotely upset when she was knocked over by a "big" wave. As her sister Amy says, "Sophie isn't afraid of anything, she is so brave", too true.

We are taking part in a 10 mile walk for the BMT Unit at Great Ormond Street on Sunday in Hyde Park and we are looking forward to meeting some of our friends from Fox ward.

I will update again soon, as I am sure I have forgotten something.

Love

Padraic

P.S. I have finally added pictures for May to August 2006.

07/05/2006

Well once again I have to start by apologising for the delay in updating. Sophie is doing really well. We moved her into a "big bed" last weekend and she is really pleased with herself. The only downside is that she can now get out of bed by herself and we have had 5:00am starts for the past week. We hope it will calm down and she will go back to her more sociable waking times.

We had a follow up appointment for Sophie's back and everything was fine. Her scar has finally stopped weeping so she can now have baths with Amy which she loves. We also had an appointment with the surgeon regarding Sophie's umbicial hernias. He was in no rush to complete the surgery and we agreed that he would liaise with the othropaedic team and try to carry out the surgery when Sophie has her next back surgery which will be in the next 12 months. Sophie had an audiology test last week and her hearing is down on the last test. She has had a cold so will have another test in 6 weeks time to verify the results. If they are the same then Sophie will have to start wearing her hearing aids again. While this isn't a big deal it does feel like a step backwards and it is one more thing Sophie has to contend with.

The best news is that Sophie is now completing colour matching puzzles. We knew that Sophie could distinguish colours but we never knew to what degree. It is the most wonderful sight to watch her completing her puzzle, checking out the colours and even correcting her mistakes.

I have added some new pictures for February, March and April.

Love

Padraic

19/03/2006

We finally got home from GOSH on Tuesday evening. Sophie back is healing really well now, it is not going to look pretty but they will try to improve the scar with her final spinal fusion.

She is doing so well doing with everything, including bouncing in the hospital cot at 4:30am on Tuesday. She was so happy to be home it made me cry. She was so happy on Wednesday morning to find her ballet skirt in her toy box she wore on top of her brace all day.

Sophie had an audiology appointment last week. Her right ear is fine but they were unable to get a good set of results for her left ear. It may simply be that Sophie was bored and not interested in the test. We have a follow up appointment next month so hopefully we we'll find out a little more then. In the meantime we will carry on without the hearing aids.

Love

Tina

06/03/2006

Sophie's wound appears to be improving. She was due to have an ultrasound today but it hasn't happened as there were a number of outpatient clinics. We are waiting to see the spinal fellow tomorrow so we know where we go from here, my guess is that we won't be home until the weekend at the earliest as the wound is still weeping.

Sophie is fine but she is getting quite frustrated, she keeps asking to go home and she doesn't understand what is going on. Amy is really missing her little sister too.

Thank you all for you kind messages of support. It helps us so much to know that we have so many wonderful friends keeping Sophie in their prayers. I will try to post again once we have more news.

Padraic

03/03/2006

Sophie is still at Great Ormond Street. We were hoping she would come home on Wednesday but her wound has become infected. She needs to start a course of IV drugs to clear the infection and as it has not been possible to get an IV line into her hand or foot Sophie will need to have an IV line put into her groin under a general anaesthetic. This may happen tonight at about 12:00 or it may be delayed until the morning when there are more doctors available as Sophie is not easy to intubate. Sophie will stay in the hospital until the infection has cleared.

I will try to post another update tomorrow.

Padraic

25/02/2006

Sophie had her spinal surgery on Wednesday and it all went well. She was sick in the morning on the day we went into hospital so were worried that the surgery would be cancelled. Fortunately she wasn't sick again and her temperature was fine so the surgery could go ahead. She was in a lot of discomfort on Wednesday and Thursday but she was back to her usual self on Friday. She was on morphine and a fluid drip for about 36 hours. She started eating and drinking properly on Friday.

Sophie has had cast of her back taken and she will have her new brace on Tuesday. She will then be able to sit up properly and be given some well deserved hugs from her proud Mum and Dad. Once again Sophie has amazed us all with her powers of recovery. If you saw her yesterday morning you wouldn't have believed she was only 48 hours post surgery. She is still in a little pain which is being controlled with a combination of codeine, paracetemol and buprofen at regular intervals. If all goes well Sophie should be coming home on Wednesday. She needs to be comfortable in her brace (it normally needs to be adjusted a few times after she first wears it) and we need to have some X-rays done.

Thank you all for checking in on Sophie and for all your support.

Love

Padraic

19/02/2006

Well again we have to start an update with apologies it has been a very long time since our last update. In recompense there are new photos for you to enjoy from November, December and January. I hope you all had a lovely Christmas and this New Year is proving to be joyous.

Sophie is doing really well and growing up in front of our eyes. She is full of smiles and laughs and is such a joy. We are extremely happy to tell you that she seems to be sleeping better in the mornings. The 4:30am starts have been replaced with 6 or 7am starts. We think that a bottle of milk before going to bed may have been the culprit. Not sure why but as Padraic and I are beginning to feel human again we are not worrying about the reasons and are just enjoying the extra sleep.

Her spinal surgery is imminent in fact the 22nd February. We had the pre-op assessment back in December and it all went well. Sophie got quite bored after a while and kept saying "bye bye" to everyone she met on the ward. We spoke to the surgeon who explained the operation. Sophie will have two growth rods placed along her spine to straighten it. These rods will be attached to a "domino" in the centre of her spine which will be periodically adjusted as Sophie grows. The operation is not as long a procedure as the kyphosis surgery and we are not expecting to spend any time in PICU. She will need to wear a brace to protect the growth rods while she plays but thankfully she won't need to sleep in it or wear it when she ill or resting. She can still have a spash around in the bath. We were pleased to hear this as Sophie will need to wear the brace until her spine is completely fused, probably between the ages of seven and ten.

In December we saw a consultant about Sophie's Hernias and he also believes they need repairing. So we going to give her a couple of months to recover from the back operation and then schedule her in late May/ early June. They will keep her in overnight because of her history.

At the beginning of February Sophie had an appointment with audiology which went really well. Her hearing test without her hearing aids was normal. We have been advised not to use her hearing aids and she will be re-tested in a month. If that hearing test gives the same results we can forget about the hearing aids.

Her speech and understanding continues to grow. She is beginning to string words together in simple sentences like 'want telly', 'Dada at work' and my personally favourite 'want juice'. She can name all the characters in the Tweenies, which is her favourite TV programme. She received Milo and Jake dolls for Christmas and they have proved very useful in introducing role playing games with her.

We will endeavour to update as soon as possible after surgery to let you know how she did and if anyone knows how I could swap places with her for the next few weeks please let me know.

Love Tina

13/12/2005

It has been far too long since the last update so please accept my apologies. The sponsored walk for Great Ormond Street went very well, we managed to raise 1,250 pounds. We would like to thank everyone who sponsored us, you have all been very generous.

Sophie started nursery school in October, she goes twice a week for 2 hours each day. Sophie has her own support assistant who she really likes, as do we. It was quite an emotional moment when we left her at nursery for the first time. It was a day we sometimes wondered if we would ever see. Sophie has settled well at nursery and plays well with the other children. She doesn't kick up a fuss when she is dropped off, the second time Tina dropped her off she ran into the nursery. I have added some new pictures for September and October.

We had a break from hospital for a few months which was nice. In November Sophie had an MRI on her spine. Although she has some narrowing at the base of her neck, thankfully it doesn't require surgery at present. She will have another MRI in a few years time to monitor the narrowing. We were so pleased that she won't need this surgery as it is a major operation. We also had a meeting with Sophie's metabolic consultant and Sophie's recent blood tests are all fine, she is still 100% engrafted. He checked Sophie's tummy and she now has two hernia's. We are due to meet the surgeon next week as it looks like she will need surgery to repair the hernia's. This coming Friday Sophie will have a pre-op assessment for her back surgery. We will find out on Friday when the surgery will take place. We asked for to be after Christmas as there is no immediate urgency. Once Sophie has had the surgery she will have to wear a back brace for anything up to 10 years so we are stocking up on hugs while we can.

In December we has an appointment with the eye specialists. Sophie's eyesight has not changed which surprised us as we (and her visual impairment teacher) have seen dramatic improvements in the past year. We discussed this with the doctor who expained that although her eyesight hasn't improved she is now processing the information much better as her brain is getting used to being able to see (prior to transplant Sophie had severe cornea clouding which left her almost blind, the clouding cleared almost completely after her transplant). While we were disappointed to hear there was no improvement, we are happy that the hydrocephalus is under control and that there hs been no further damage to her optic nerves.

Generally Sophie is doing really well, in particular her speech is coming on in leaps and bounds. Sophie's vocabulary is increasing and she is developing more and more skills including being able to "escape" from her sleeping bag. She now runs around the house so quickly we spend most our time shouting "slow down Sophie", not bad for a child who only started walking a few months ago.

Christmas is fast approaching and our preparations are in full swing. The decorations are up and Amy performed in her Christmas show today which we really enjoyed. We are looking forward to having another wonderful Christmas with our two girls. I am sure Santa will be very good to them both, they certainly deserve it.

The past few months have been very difficult for our MPS/ML family. There have been so many angels and we are thinking of them all.

Love Padraic 

10/09/2005 

Another milestone in Sophie's journey, two years since we watched what looked like a simple blood transfusion give our daughter a chance to live. We look back on the last year and see what she has achieved, we are so proud. We look to the future and pray that again our star will bounce back from another surgery quickly.  Today we celebrated with chocolate cake and candles and as we watched her eat her cake with relish we felt such gratitude. 

I remembered the card we received from the donor and and the message inside. This stranger to us saved our baby's life, he is a true hero. 

Love Tina 

05/08/2005

This will be a long update, a lot has been happening in the past month. Sophie had an orthopaedic appointment as a follow up to her back surgery last year. The kyphosis (front to back curvature) surgery has been corrected but sadly her scoliosis (side to side curvature) has increased by 10 degrees in the past six months. Sophie has been scheduled for more back surgery early next year. She will have two “growth rods” inserted on each side of her spine to straighten it and then every 6 months or so she will have a minor operation to increase the length of the rods. The growth rods will ensure that the effect on Sophie’s growth will be minimal. The plan is then to fuse Sophie’s spine when she is about 10 years old. Sophie will need to wear a brace following surgery for at least 6 months. Depending on her bone density she may have to wear the brace until the final spinal fusion when she is roughly 10 years old. While we could see that the curvature had increased we hoped that Sophie could avoid surgery for a few more years. The idea that she may be in a full back brace for several years is upsetting, after all she has been through in her three years we just hoped she would be given a break from hospitals for a few years. On a more positive note we had an appointment about Sophie’s hands. We have been a little concerned as Sophie has been avoiding using her right hand. However, since she has been walking and also since we have been trying to gently force her into using her right hand things have improved. Sophie’s hands, hips, knees and ankles were checked and the doctors was quite happy that no intervention is needed at present. 

While we were at GOSH we met up with Nic and Mark who contacted us through Sophie’s website. Their beautiful daughter Hannah has Hurlers and had a BMT at GOSH about three weeks ago. It was so nice to meet other parents of a child with Hurlers and to talk about our experiences. We have added Hannah’s website to Sophie’s friends page, please drop by and say hello.

Last week we were in Dublin to see my parents. They were thrilled to see Sophie and Amy and were amazed at the changes in Sophie. She is walking really well now and likes nothing better that wandering around the house “investigating” and pointing out items of interest such as doors, shoes…..

We are still waiting to find out if a classroom assistant has been recruited for Sophie. She is due to start nursery school in the next few weeks so we hope that a suitable candidate will be found soon. Amy starts school tomorrow (Tuesday), we can’t believe that our little girl will be going to school, she looks so grown up in her new uniform.

This Sunday (11^th September), I will be completing a 10 mile sponsored walk for the BMT unit at Great Ormond Street. This year the bone marrow transplant unit at Great Ormond Street will be performing their 1,000th transplant, an incredible achievement considering on average only about 50 transplants are performed each year. If you would like to sponsor this very worthy cause please let me know by email at padraic@melroseroad.co.uk

Love 

Padraic

07/08/2005

Sophie is really walking everywhere, it is so wonderful I could spend all day just watching her. She keeps taking me by surprise, I suddenly see her walk into the room and I double take. I am so proud of her, it is almost a year to the day that she started crawling and in that year she had spinal surgery and wore a brace for five months. Anyway if you would like to see for yourself here is a link ..... Sophie Walking. If you can't see the movie you can download Apple's Quicktime software from here  Quicktime download.

Sophie's speech is improving every day and now demands milk in the morning and can tell me when she is hungry. In fact she is getting quite bossy and stamps her feet when she does not get her way. It is really quite cute but we have to be strong and not give in. 

We had a follow up appointment in Audiology. They were really pleased, her hearing test without her hearing aids again showed a moderate hearing loss with them she could hear a pin drop.  She is still tolerating them really well except when the weather was really hot. So we are going to carry on and will see Audiology again in February next year. 

We have a couple of orthopaedic appointments this month, one 19th for spinal check up and another on the 23rd to talk about her hands. She still will avoid using her right hand but it is a little better. 

Love Tina      

12/07/2005

Well I wrote an update on 16th June but I didn't upload it as I wanted to add some new pictures. We are now in July and I haven't added any pictures since April, I am very sorry and it won't happen again, May and June pictures have been added.

We had a wonderful time at the MPS conference last weekend. We were a little nervous as it was our first conference but there was no need to be, everyone was so friendly. It was so nice to meet our  friends from the MPS forum and to make new friends with other families of children with MPS conditions. Amy had great time, she had never stayed in a hotel before and she found it all very exciting especially the large jacuzzi which served as a mini-swimming pool for the children for the weekend. Sophie really enjoyed all the attention she received and spent as much time as possible dragging us all around the hotel. The weekend went so quickly, we wish it could lasted longer. We all had baby-sitters for the gala dinner on Saturday night and we had a great time on our "night out". The evening got even better when we won 4 tickets to Legoland in the raffle. 

The venue was excellent and the hotel staff, many of whom gave their time for free, did an incredible job catering for the 500 people attending. The volunteers were wonderful and we owe them all a big debt of thanks to them for all their hard work. We are also grateful to the MPS Society for doing such a great job in organising the event. 

Sophie's walking is going really well, she is now taking a few steps on her own steam and today she walked across the living room to the television. I think she has had enough of crawling and walking on her knees. 

Love

Padraic

16/06/2005

We have had a few trips to Great Ormond Street recently. We had the follow-up hearing test and Sophie did really well  with her hearing aids in, I don't think she missed any of the signals. We will stick with the hearing aids as Sophie is tolerating them well. I think she knows that when they are put in she will be able to hear Balamory better so she doesn't complain. Sophie had a set of blood tests to check her engraftment is still ok. The tests on Sophie's hands showed that she doesn't have carpal tunnel syndrome so we will have to carry on trying to find out what the problem is. Sophie didn't have the X-rays to check her hips and knees as they hadn't been booked. We will be back up in August for Orthopaedic x-rays so we will arrange to have them done then.

Sophie is going from strength to strength, her speech has improved so much in the past few weeks, I think the hearing aids are really helping her. We had a great trip to the beach last week for Sophie's birthday, she had a wonderful day and really enjoyed the picnic. She was so happy crawling all over the mat trying all the food from everyone plates !! 

We met a real sweetie on the MPS forum called Tyler. He is currently undergoing a Bone Marrow transplant, we have added him to Sophie's friends page, please drop by and offer him your support.

Love 

Padraic

05/06/2005

Things are going well and life is getting busy again. Sophie is doing well with her hearing aids I have only found her eating them twice and she has only lost them once ( we found them on a road in the local area). I have noticed her reacting to sounds more and she is certainly making more sounds. She is going to have a hearing test on Tuesday to see if there are improvements with the hearing aids in.  

Sophie just finished a block of OT sessions and has reached all her goals. I am so proud of her, she is focusing on a task for two minutes at a time (well almost) and is getting to grips with simple puzzles and posting. We start a block of speech therapy sessions next week lets hope they are just as successful. 

Her walking is improving and she seems more stable when she stands. She will now take a few steps on her own steam and still applauds herself when she reaches her target. 

I have been worried about her hands recently especially her right one. She is increasingly avoiding using it and some days she holds it limp against herself.  I have discussed it with the physio and OT and we talked about splints but we wanted to hold off as will affect her using her hands effectively and if we only use them at night it could affect her sleep. We discussed it with her Metabolic consultant and on Friday she is going to be tested for Carpal Tunnel syndrome and have x-rays on her hands.  She will also have x-rays on her hips and knees as well as engraftment tests. So it will be a busy day, we let you know as soon as we have the results. 

We have been busy with the Statement process and on Friday we got the initial version. Harrow council will fund a teaching assistant for Sophie for 12.5 hours a week. This is great news as we had been warned Harrow does not often statement a child so we were relieved. The next stage is to decide on the nursery. We would like her to go to the pre-school Amy is currently at. There are many reasons for this, but I have to confess that it's mainly because we know them and trust them. I feel confident they will look after my precious baby, let's hope Harrow council agree with us. 

Love Tina  

15/05/2005

We had a wonderful time at Disneyland but more importantly Amy and Sophie had a wonderful time. There were so many wonderful moments Amy shaking Woody's hand, Pluto feeding Sophie Ice cream, Amy dancing with Mickey and Sophie clapping so much at the Parade she was getting as much attention as the characters.

I know you all know how special this trip was for us. The memory of Sophie bouncing on my shoulders and clapping while the parade passed by will be with us forever.

We posted some pictures of our trip here................ Disney pictures.

Love

Padraic & Tina

p.s. A question for those of you who have been to Disneyland. How long does it take to get the tune for It's Small World out of your head ? It was Amy and Sophie favourite ride and we went on it every day. 

24/04/2005

It has been a little while since the last update so this is going to be a long one. Easter was fun, too much chocolate was consumed by all. We had a great day out at Legoland, Sophie enjoyed the rides and had a great time in the playground playing on the slides and swings. The following week we met up with Joan Cohen whose son Adam has Hunter's (MPSII). We met Joan through the MPS forum, she was on holiday in London from New York. It was nice to meet another parent of a child with MPS. We had a lovely afternoon in Regent's park swapping stories and experiences. Sophie was on top form and had a wonderful time on the swings where she was practicing her new favourite word AaaaWeeeeeee.... If we ever make it to New York I am sure we can call on Joan to be our tourist guide.

On the 13th April Sophie had another hearing test. She still has glue ear in both ears and still has moderate hearing loss as a result. We discussed Sophie's options with the ENT doctor. and decided for Sophie to try hearing aids. We will have to see if she tolerates them, if she doesn't then we may opt to have another set of grommets inserted. While grommet insertion would resolve her hearing the downside is that the they will eventually fall out and then we will be back to square one. Sophie's hearing loss was compared to the loss of hearing you get when your ears fail to pop on an airplane. For example, she can hear sounds such as normal speech level but very little below that level. On 5th May Sophie got her new hearing aids and she is tolerating them well. We will have another hearing test in 4 weeks time where we will hopefully be able to see an improvement in Sophie's hearing.
Sophie and Amy's Nana came over from Dublin in April and we all had a lovely few days together. We had a wonderful day out at Windsor, Sophie was delighted to have another friend to hold her hand while she walked around.

Sophie development progresses slowly but she is going in the right direction and every achievement is met with great joy. She recently has started walking a few steps unaided and each time it is greeted with wild appaluse. She now looks around ensuring all eyes are on her and leads the appaluse herself.

Tomorrow we are all going to Disneyland Paris for a week of celebration. We wanted to treat our two girls with a little magic because they fill our lives with magic every day.

Love Padraic & Tina

P.S. We have added some pictures for March and April 

21/03/2005

Normality rules in the O'Connor household, well as normal as it can get with two children under five. Sophie has been well and continues to take small steps in all areas of her development. She had her last Therapy group on Friday and we will now have a block of one-on-one therapy sessions. Sophie is easily distracted in group and is more interested in the other children. It is nice to see that she is becoming more social but we need to make the best of the small amount of therapy she receives. 

On Thursday she started her immunisations again. She did really well no reactions and only a few tears. It seemed so unfair that she had to have them again but I guess it is a small price to pay. The next set of injections will be in four weeks. 

Overall things are good. Sophie is becoming a strong and joyous little girl and lights up our lives. Whenever I worry about her future and what it will be like she blows those dark thoughts away with a smile, with laughter or downright cheekiness. She is our star leading the way.

May your Easter be a happy one.

Love Tina 

P.S. New pictures added

27/02/2005

Sophie had her orthopaedic appointment on Friday and we are pleased to report that she no longer needs to wear her back brace. Her spine is fusing well so she no longer needs the brace to support her. We have been giving Sophie lots of extra cuddles this weekend, it is so nice to be able to hold her without the brace being in the way. I have just figured out that this is first time that Sophie has been free of medical equipment since just before her transplant in September 2003. The Hickman line, PICC line, NG tube and finally the back brace are now all gone. Sophie is celebrating by showing off her tummy to anyone who is interested.

Love

Padraic

13/02/2005

I am so sorry it been over a month since we last updated. I suppose we been swallowed up by family life. The last three weeks Sophie has not been completely well. She had an ear infection which only shifted after two courses of antibiotics. Then she come down with a stomach bug which lasted for a few days.  Explosive diarrhoea and a back brace do not mix!!! She is over it all now and she is on top form again. 

Everything is progressing well with every day something new.  Taking the rubbish out of bin, pulling the toilet paper off the tube and generally empting cupboards. The world is her playground and secretly I am cheering from the sideline whilst trying to teach right from wrong. I think she has taken a leap in her understanding. She certainly understands 'no', 'take that out of your month' and 'Do you want some Wotsits?' 

We have started the process to get a 'Statement of Needs', a document which outlines the support she will need in pre-school/school. It takes about 26 weeks and we are still unsure of all the ins and outs but we are receiving great support from Portage so we will let you know how it progresses.

Yesterday we went to Jack's 2nd birthday party in Oxford. He is a little boy who had his transplant towards the end of our stay. Fynn another little by who had his transplant at the same time was also there. They are both doing so incredibly well and it was so lovely to see them having such fun. 

On the 25th we have an orthopaedic appointment where we hope to say goodbye to the brace. It will be five months and we were told it should be on between three to six months so fingers crossed.

We will update next with news from that appointment. 

Love Tina

p.s. I have added new photos.

09/01/2005

Christmas and New Year were fun, it was so nice to have time off from work which didn’t involve a hospital appointment. Sophie has been in top form over the past few weeks. She is eating and drinking really well, she now eats as much as Amy does at meal times. I never get tired of watching her enjoying her meals, you could never believe that only a month ago she was still being fed by NG tube. Sophie is more and more agile everyday and she doesn’t let her back brace get in the way. She is “cruising (walking holding onto whatever furniture, door or person is near)” all around the house and she climbs the stairs whenever we forget to close the stairgate.
Sophie has invented the new sport of “people climbing”, she will start at your feet and gradually work her way up your body until she manages to get her head higher than yours. She celebrates her achievement by shouting loudly in your ear.

The biggest change in Sophie in the past few weeks has been in her speech. She is making distinct sounds and associating them with objects. Most of her words sound like they begin with B so she can now ask for her bottle, bowl, bed and ball using the same sound. She has also taken to calling Tina “Mamma…my baby” which is just too cute. Sophie’s is now joining in with that childrens favourite “Wind the bobbin up”, she winds her arms up and then claps her hands together.

Please keep Sophie’s friend Jonathan in your prayers. He had a second transplant on Christmas eve and he not feeling very well. The good news is that he is 100% engrafted. Jonathan’s website is on Sophie’s friends webpage, please stop by and give Jonathan and his wonderful family your support.

Love,

Padraic 

02/01/2005

We had a wonderful Christmas, very emotional and filled with joy. We filmed and took photos trying to capture everything  but nothing could really capture the true joy of being together. It was made all the more poignant with the news of the earthquake in Asia on Boxing Day. So many families being torn apart, it is heartbreaking watching the news. 

So we look forward to 2005 with great hope. 2004 has had its ups and downs but the last few months has been nothing but good news. One thing I have learnt of the last couple of years is no one knows what tomorrow is going to bring so let's enjoy those days which bring us joy and hope that gets us through the rest.  

May this year bring you love, joy and peace.

Love Tina

P.S. We have added some new pictures

23/12/2004

Well I think we are all ready for Christmas. Lists have been sent to Santa and I think he will be very generous. Sophie has come so far in the past year, last Christmas Sophie was recovering from a serious fungal infection and she was being slowly weaned off TPN. She is now eating and drinking well and pulling the house apart. 

Thank you all again for checking in Sophie and for all your messages of support. We hope you all have a peaceful Christmas and happy new year. 

Love

Tina & Padraic  

12/04/2004

Christmas is coming and I am getting excited let alone the children, I am enjoying every moment. Last year our Christmas was different, very special but I felt I missed many things with Amy. On Friday was Amy's Christmas Concert, she was an Angel and when she sang I had to hold back the tears.  

Sophie is getting stronger and louder every day. She just takes my breath away. I am so privileged to have two such wonderful daughters, who constantly amaze me with their strength of character, resilience and determination.

Very soon Sophie's friend Jonathan is going to start his second transplant. He and his family have been fighting this battle for many months and to have to start again must be heartbreaking. Jonathon's parents are so amazing their strength as a family is an inspiration for us all.  We pray that this transplant is successful and has no majors problems.

His website is here Jonathan's Website.

Love Tina 

4/12/2004

I will start with the big news first.......Sophie's NG tube is out !!!!! Sophie had her NG tube inserted in August 2003 and it has been part of our daily lives ever since. She is now drinking enough fluid by herself and is taking her medicines orally everyday. It is so wonderful to be able to see her beautiful face without a plaster on her cheek. 
Amy went along to Sophie's therapy group this week and she thought it was much more fun than her pre-school, I think the snack of wotsits may have been a factor !! It was nice for Amy to see other children who are special like Sophie. We are now getting ready for Christmas and the preparations are in full swing. We are really looking forward to a nice family Christmas.

I have added some new pictures of Sophie enjoying her milk.

Love Padraic

26/11/2004

One year ago today, after a long and courageous battle, Mighty Max passed away. Sophie and Max were transplanted at roughly the same time and although we never met we became very close to Max and his family. They were both very ill at the same time and while Sophie survived, Max was given more than his body could bear. We always held onto the dream that someday Sophie and Max could meet, two kindred spirits with hearts as big as the ocean that separated them, but it wasn’t meant to be. Today as we mark Max’s passing we remember his beauty, his strength and the love he brought into the world. In Max’s all too brief life he touched the hearts of so many people most of whom never had the privilege of meeting him. We will never forget you.

We love you Max.

21/11/2004

Sophie’s Nana went back to Dublin last Monday and we were all sad to see her go.  I hope she got enough hugs from Amy and Sophie’s to keep her going for a little while. Sophie is continuing to explore her surroundings and she is not very happy that Daddy has put the stairgate back up. Her climbing expeditions will now be kept under strict parental control. Sophie has her first therapy session for over a month on Thursday and it went very well. She has now achieved all the goals which were initially set out for her so a new set of goals is being devised. Sophie is now using hand signs to join in with her songs, she is also developing her own signs but we have yet to work out what they mean.

On Tuesday we had two appointments at Great Ormond Street, cardiology and opthamology. We got good news from both appointments which I think is a first for us. The cardiologist was happy with Sophie’s progress to the extent that the blood pressure drug has been reduced from 3 times a day to twice a day. Her heart function has improved and he does not need to see Sophie again for a year. This was an appointment which we were very worried about even though Sophie hasn’t shown any signs of heart problems. We were shocked when she was diagnosed with cardiomyopathy and we are so relieved that her heart is improving.

In the afternoon Sophie had a eye examination. The consultant was pleased to find that although she has got major nerve damage, there is still some nerve activity. We told him about Sophie showing more interest in television and he confined that Sophie can see 2-D images by showing her a selection of cards with pictures at the bottom. Sophie has been booked in for another detailed eye test in 6 months (the same one she had in June where her head is connected up to a machine to monitor brain activity). Having reviewed the previous test result the consultant said that he thought the detailed eye test should show signs of improvement. While we knew that Sophie’s sight was improving it was great to have it formally confirmed. We know that Sophie is not going to regain her eyesight but we will be delighted with any improvement no matter how small.

On Friday we had an orthopaedic appointment and all is well with Sophie's back. We were shown X-rays of Sophie's back before and after the operation and the difference is incredible. I asked about the scoliosis and the doctor said that it isn't an immediate concern, he said that Sophie's spine is now well balanced (i.e. she doesn't lean to the left or right) and there is a possibility that she may not even need surgery. It will be monitored over the next few years to see how it progresses. We will have another appointment in two months time and there is a possibility that the brace could come off then. Despite the brace Sophie is doing more and more physically each day. She can stand up for long periods whilst barely holding onto a support and she can even move around the room with her hands on the wall. Her shoes will be fitted next week which should give her some additional support when she is standing.

Our next medical appointment will be in January so we will have two months free including the Christmas and New Year period  which is wonderful. Sophie's drinking is continuing to improve, she is now taking about 50-60ml at each mealtime so we have been able to reduce her NG feeds. If she carries on increasing the volume we will be able to take that nasty old NG tube out. It is not a major medical problem but it seems to be the first thing that strangers see when they look at Sophie and we are always conscious of people staring at Sophie. The attention she gets is not all bad; last week Tina was with Sophie in the park. A woman was admiring Sophie and told Tina how beautiful she was. She asked about the NG tube and Tina told her about the BMT last year. The woman then asked if Sophie was "one of our special children" to which Tina replied yes. Some people just know the right thing to say. It turns out she used to be a nurse and now runs music classes for children with special needs. She will be starting one local to us soon so hopefully Sophie will be able to go. Sometimes nice things do happen.

I have added some new pictures

Love Padraic

17/11/2004 

Sophie's friend Evline become an angel on Friday. Our thoughts are with her family, I cannot begin to comprehend their pain. I pray this will never happen again, but I know it will. I do not have the answers, all I can say is just hold your children and be thankful for every moment. We are the lucky ones. Her website is here Evline's Website.

We will update again after Sophie's orthopaedic appointment on Friday.

Love Tina

4/11/2004

Well the coughing season is coming to an end here so the house is a lot quieter. We did manage one doctors appointment in the last two weeks but this time it was for Tina. She is such a familiar face in the local pharmacy that the chemist assumed that the antibiotics were for Sophie and initially didn't charge for them. Sophie is now climbing to the top of the stairs at every opportunity (new pictures added to illustrate this). The moment she is left on her own she is up those stairs like a bullet. She has made it as far as halfway up  before being caught so I think it is time for the stair-gates to go back up. Sophie's feeding continues to improve and she is even showing a little interest in taking the bottle herself. She has become so determined in the past few months and she certainly lets you know when you are not doing what she wants to do. As well as climbing the stairs Sophie is pulling herself up to standing and "cruising" around the furniture.  While she is coping well with brace it is stopping her from standing up completely straight. otherwise I think we could be looking at a few tentative steps !!!

In the last few days Sophie has been showing more and more interest in the television. She likes to stand facing the TV and we have noticed that she often tracks the movement of people on screen with her eyes. She also like to place her hand on the TV screen and almost always places it on the person's face. It may be all wishful thinking but until Sophie can tell us how much she can see we will never know. We have an appointment with the eye consultant in a few weeks time so we will be able to discuss it further then, for the time being we are enjoying these little improvements.

We had a meeting with Sophie's metabolic consultant today. An MRI scan will be booked to check Sophie's neck as children with Hurlers often have problems with vertebrae in the neck. The MRI scan will also check Sophie's brain to ensure that her shunt is working properly. She will also have a carpal tunnel syndrome test on her hands.  Sophie's Nana arrived from Dublin today and she has noticed major improvements in Sophie in the last 2 months. We know that Sophie is improving but it is always nice when someone else notices the changes too. It's also nice to have my Mum here for a few days.

Please keep Sophie's friend Evline in your prayers, she is having a rough time at the moment. Her website is here Evline's Website.

Padraic

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24/10/2004 

Tis the season to be sneezing, or in our case coughing. Amy is just getting over it, Sophie in the middle of it and I just started. Apart from the coughing O'Connors things are going really well.  Sophie seems to adapting to her brace incredible well. She crawling everyway and even pulling herself up to standing;  walking around the furniture. This was something we were warned may take a while, not for our Sophie. She is a little slower but it gives me a chance to catch her.  Padraic added some new photos of her in her brace. 

A couple of days ago she drank a ounce of milk from a bottle. I hope this is the beginning of her starting drinking again. I just want to be able to get rid of her NG tube so I can look at her beautiful face.  Although as her hair as gone completely mad I not sure as you would really see anything. We are going to attempt a trip to the hairdressers as my attempts have only contributed to the problem.

We are in the middle of a two week period without a single hospital appointment, a rarity in these parts. I am just enjoying the normality while I can.

Love Tina

13/10/2004 T+399

Apologies for not updating sooner but we have had problems with our computer. I have had to dust down our old PC to get going again. It has been a very busy week for Sophie. While we were at GOSH she had another heart echo and as her heart function has not improved (it hasn't got any worse) her captopril dose has been increased. She will have another echo in months time which we hope will show signs of improvement. She was discharged from GOSH on Friday and it is good to have her home again. She had a beaming smile all afternoon so I guess she was glad to be out of there. She is doing well coping with the back brace which she will have to wear 23 hours a day for the next 3-6 months. Sophie's feeding is back to it's pre-surgery levels and she is standing with support and learning to crawl in her brace. We will be back at GOSH in about 6 weeks time for X-rays so we can see how Sophie's back is doing.

Yesterday we had an appointment with a new consultant at our local hospital who will be responsible for Sophie's developmental needs. After some simple assessments she said that Sophie's development is at about 15 months. She said that there were many positive things, her achievements in recent months are impressive and she has crammed 6 months of development into about 3 months. She felt that Sophie's personality i.e. her stubbornness, resilience, curiosity and good nature will stand her in good stead. Throughout her assessment of course Sophie laughed !!!

We had a visitor from the portage visual impairment team today and it went well. She brought along some kind of pole contraption which made noise whenever it was moved. Of course Sophie found this hysterical !! This afternoon we were signed off by our local consultant who was responsible for Sophie's post BMT needs. The BMT is now officially behind us, we will now be concentrating on Sophie's development and being a normal family again. 

Thank you all for your continued messages of support.

Padraic

02/10/2004 

Another good day, she still is some discomfort but it is controlled well with painkillers. Today they took her off the morphine drip as she has not needing any extra doses. She been written up for codeine but the paracetamol seems to be doing the trick.

She has stopped IV fluids and has just started normal NG feeding with water. If she tolerates this we can start the Neocate, Sophie's milk replacement.

After another x-ray she had the chest drain pulled out. I was warned that this would hurt, a short pain but very painful. Padraic said she was incredible, she cried but as soon at it was over was easily comforted. 

The nurse for the pain control team comes to check her every morning. This morning she said 'Sophie you are one brave girl'. I just wish she did not have to be. 

Love Tina

01/10/2004 T+387

Sophie is doing well. She seems much more comfortable and the morphine drip has been reduced. There was talk of taking out her chest drain today but after a chest xray it has been delayed until tomorrow. There is still some fluid in her lower lungs, but the fluid coming out is not bright red so its does not seem to be currently bleeding. Her blood counts are good also another good sign.

She has been less sleepy today and playing but is a little frustrated, trying to get up. Sophie's morphine "drip feed" has been reduced and she may come off the morphine tomorrow. The pain team are very impressed with her progress, she will still have regular paracetemol and codine as needed. We are down to one one line in her neck now, one stopped working, the other she pulled out!!! Sophie also decided that she didn't need any extra oxygen last night so she simply pulled the "blow by" tube off her face. Hopefully she won't try to remove the chest drain by herself !!

Padraic

30/09/2004 T+386

Sophie had her back surgery on Wednesday morning and it all went well. She went to PICU afterwards and came off the vent at midday today. She is now back in her room. She is on a constant drip of morphine and can be given an extra boost whenever we think she is in pain. She is very sleepy and has spent most of the last 24 hours asleep, we hope this will aid recovery. Sophie will be fitted for a back brace on Monday so until then she must stay lying down. We won't be able to pick her up and hold her until Monday which as you can imagine is very difficult for us. The surgeon said that the section of back repaired was very weak and would have almost caused her to lose the use of her legs if we hadn't proceeded with the surgery. We appreciate how lucky Sophie has been. 

Thank you all for your messages of support and for continuing to check in on our little star.

Love

Padraic

PS I have added some new pictures for September.

15/09/2004 T+371

Sophie's back operation which was due to take place today has been cancelled.
There were no beds available on PICU and as she is likely to be on the vent after the operation the surgery couldn't go ahead. While we understand and accept the reasons why it has been cancelled we have been mentally prepared and now we have to start all over again. We were given a new date of 29th September so hopefully in two weeks time we will be able to proceed.
Thank you all for your messages of support, they mean so much to us.

Tina & Padraic