HAPPY NEW YEAR!!

Wishing you all a very Happy New Year and wish everyone of you the best for 2012!! It's been a busy time over the festive holidays so will update Shay's blog very soon.

Feeding issues!

Again, it's been a while. Life just passes you by and before you know it yet another couple of months has passed by. Shay carries on with life regardless of how he feels. In between never ending hospital appointments he needs to attend, Shay just wants to have fun like a normal 4 year old. He is now in full time school which most days he finds absolutely exhausting. Some mornings we let him go to school later and all he wants to do these mornings is to just lay down and watch movies.
The last couple of months we can see Shay's health deteriorating. Anyone who knows him only has to look at his lovely green eyes, he just looks so tired all of the time. When I look at him it is hard to stop your emotions spilling out. Automatic pilot is now a way of life for us, its the only way we find strength to cope with whats coming our way and being incapable of having no control over any of it.
Where do I begin with my little man? he is such a complex medical case its totally mind boggling at times. Between Alan and myself, somehow we get there at understanding how complex Shay is.
Shay has hit rock bottom with his eating. For two years now I have battled with Shay at times just to get him to eat. We have tried all techniques to get him to eat. There is nothing I have not tried. Someone said to me the other day "do you praise him?" We have learned to let comments like these go and just ignore them.
It now looks like Shay will have a feeding tube inserted directly into his stomach. We have an appointment in a couple of weeks to discuss this. Shay at the moment enjoys peperami's, sour cream pringles, vanilla ice-cream and not much else! I still manage to feed him his one weetabix in the mornings but he is even trying to stop that. I have took the pressure of his eating completely and if he doesn't want something then I will always find him something he will eat. His school has been fantastic with supporting him with his eating. He doesn't finish lunch at the supposed time and will only eat his two small peperami's. He will then have his tiny squares of sandwich late afternoon. We are pretty adamant that he needs this tube to survive and to take the pressure of him eating. All of his eating is supervised, if it was up to Shay he wouldn't eat at all (maybe just those dreaded peperami's). He will still be able to eat once he has the tube inserted but when he wants to. Once the tube is fitted we will hopefully see a big improvement in his general health and well being.

A Very Happy Shay

Shay was over the moon to have been gifted with this fab bike. A huge thank you to Fiona for thinking of Shay this way. Shay was jumping up and down with excitment when he saw it and it is constantly by his side when he is in the house. When he is not on it the ignition key goes everywhere with him. The other day he was in the drive just sitting on it watching people walk by and you could see he loved the reaction of the people when they saw it, such a poser!!!

Shay's mobility...

For the past couple of months Shay has been complaining about pain in his legs. On one occasion we took him to the hospital to check his lactic acid levels. Acidosis in a child with Pearson's can be fatal. Thank God it came back negative, but we are still no where near knowing what is wrong with his legs.

It is totally heartbreaking to see him in so much pain and trying to get on with things as best as he can. Since the start of the summer holidays there has been a dramatic decline in his mobility. He now favours his left leg for weight bearing when climbing the stairs, taking one step at a time and when he is coming down he is hanging onto the rails for security. Shay will start limping and more and more often his right leg gives way. This has happened a few times and he just drops to the floor and cannot move : ( I took Shay shopping the other day and within two minutes of us being in the shop I had to leave, he could hardly walk. I have noticed how Shay is always sitting when he is playing. Everyday he spends hours sitting down, sometimes he just sits and enjoys watching his sisters playing.

Something has happened/is happening to Shay. Something we cannot explain but it is obvious to us his parents that he is deteriorating in front of our very eyes. I can see it in his eyes and cannot explain the feeling to you of how I/we feel when we see our little man looking like that. Life is very unfair to do this to such a sweet lovable boy. All he wants out of life is to be like everyone else and do what other children are doing at 4 years old!!! We can't explain to him whats happening and even if he was old enough what would we say. Shay cannot remember what it is like to feel 'normal'. He was such a happy, energetic little baby who was always climbing and running around. He is still that happy little boy but so much has changed for him. It's the most frustrating feeling in the world when you struggle as parents to help your own child.

I would like to thank Fiona B for being there for Shay and coming to massage Shay's legs for him. Fiona is a physiotherapist and is extremely busy. We gave you no notice whatsoever and you came to him straight away. The massage worked extremely well for Shay and he only woke up with a little niggle of a pain in the morning. This lasted for four days before he started complaining that he was in a lot of pain again. Your a STAR!!!

Shay received a very special gift today from one of his friends today. He had no idea who the people in the photograph are but he was over the moon with it. The photo had his name on it and signed by all the people in it. We explained to Shay that the people in the photo were darts players and one was the current world champion along with the other two being previous world champions.

He really wanted to have the picture hung up next to his in the hallway, but we persuaded him to have it on his wall in his bedroom. This was a very kind gift from a good friend of Shay's. It just goes to show that Shay makes such an impact with people and that they are always thinking of him.

A big thank you for making our little man feel extra special again!

Pre-planned hospital visit.

After weeks of deliberation and worry, we decided to let Shay have his port removed. Shay went in on Tuesday as he had to have an ECG and bloods taken to make sure that he was well enough medically for the operation to go ahead.
Even on the way to GOSH it was 50/50 in our minds whether to go ahead with the operation. After talking to his doctors it was decided the best course of action was to have it removed. This was because he no longer needs it for red cell transfusions and also they are seeing alot more ports becoming infected and failing. They put it to us that they would prefer Shay to have the port removed in a controlled environment like GOSH, rather than him being rushed in as an emergency case. So the removal of the port was in place plus a lumbar puncture. Shay was slightly embarrassed when he had to put the hospital gown on, he did insist on keeping his boxer shorts on. For any parent it is the most heartbreaking thing to see your child put under general anesthetic, but for us it was even worse. Working on a patient with Mitochondrial disease is extremely risky and shouldn't be undertaken unless it is absolutely necessary. The entire procedure we were told would take around 40 minutes to complete. After the longest ever 40 minutes we started clock watching, an hour went by and we really started to worry. Worrying never gets us anywhere as we were called down to theatre just past the hour. Prior to Shay going under they wanted to also take a tissue biopsy which we agreed to. The tissue biopsy was taken from the port site so he didn't have this as an extra wound on his already marked body. The biopsy will tell us so much about Shay. It will take a very long six months for the results to filter back. We know Shay has Pearson's but this will tell us which complexes are affected; whether it will or is morphing into 'Kearnes Sayre Syndrome' amongst a whole lot of other information. Also it will quite possibly tell us what is happening with his legs as this is one area he always complains of being in pain. It's going to be the longest wait ever.
Shay is now back home and is recovering slowly. He misses his port as it is something he has got use to for two years and Shay will miss showing his port of to everyone that he meets.

Shay & The Royal Bodyguard

On the way to GOSH on Tuesday, Daddy and Shay were invited onto the film set of 'The Royal Bodyguard' to meet some of the stars and characters.

Sir David and Tim welcomed Shay onto the set and both of them made him feel totally at ease. Even though everyone was obviously having a very busy day, they took time out to talk to Shay. They were absolutely great with him. As usual Daddy was snapping away with the camera and Shay took everything in his stride. Shay thought Tim looked funny covered in oil and wanted to know why. He questions everything at the moment and can ask up to 20 whys before he is satisfied with the answer! The Bodyguard is being aired Christmas day, which I am sure Shay will enjoy watching.

2nd Hospital Visit!

Shay was recently tested to see if his body had developed antibodies against the childhood disease chickenpox. Back in March he developed some spots which the hospital thought was chickenpox. He was quickly put on antiviral drugs over a five day period. The test came back negative meaning Shay has not had chicken pox previously.
A couple of days later Shay wakes up one morning covered in spots his temperture was starting to rise, I really couldn't believe what I was seeing. A very Quick call to the hospital and we were on our way in. Shay was kept in for five days again, he was closely monitored as he is still neutropenic. His temperture went up to 40 and down again numerous times throughout his stay. The doctors did not know what effect the chicken pox was going to have on his body. Thank God all that is over!!! We are going to have his antibodies checked in approximately 8 weeks again. This should show us that it was indeed chickenpox. Also a very big thank you to Shay's community nurses who made it possible for Shay to come home for one afternoon. They were able to bring a mobile iv machine which enabled Shay to have his meds in the comfort of his own home. Little things like this makes all the difference to our little man.

1st Hospital Visit!

Shay has had a bad couple of weeks and is now well on the road to recovery. Firstly, Shay has been complaining of pain in his legs. He will wake up in the mornings limping and cannot walk down the stairs. This seems to gradually get better after a couple of hours. Lately however, it has been happening more throughout the day with no recovery in between episodes. It got to the point where we were so worried that I phoned Shay's paediatric consultant at his local hospital. She advised for us to bring him in straight away. We were extremely worried that Shay was showing signs of metabolic acidosis. The doctors were extremely quick at taking bloods and sending them to the lab to be tested. I am estactic to say that they came back negative showing no signs of acidosis. We don't know what is causing his pain but he still has it and complains constantly to us. When he is with other children he tends to hide how he feels as he just wants to be part of a group and be like everyone else.

Shay's special day.

Shay has just had the most fantastic day as a new fledgling police officer! He has been asking Daddy for quite a while to "go catch bad boys". A huge thanks to the Metropolitan Police Service for making this dream come true.
Shay was aware that he was going with Daddy and his friend Simon to work for the day. Shay was excited that morning and he even helped pick what to wear. What he was not expecting was to be issued with his very own uniform! The uniform services dressed Shay in police trousers, a police shirt, belt and utility belt and even his very own beat duty helmet (all customed made to fit Shay especially). Shay looked in the mirror and whispered to daddy "get bad boys dad". Shay was thrilled with his helmet it really made the day that extra bit special. Now all he needed was a set of wheels, so a short walk to the driving school and he was in his element. He enjoyed being driven around with lights and sirens on in the police armored truck. That was not fast enough for him, it was not long before he was in the marked BMW estate chasing a criminal around the streets of Hendon. The pursuit lasted about 5 minutes before the criminal stopped and tried to run off. Constable Shay Murray was overjoyed when he managed to catch the bad person and even arrested him.
Next, it was the skid pan. After the car spinning around the first few times Shay said to Dad "just like Mummy, Dad". Shay took all the spins, turns and stops in his stride.
Now, Shay loves being around dogs and it was a real treat for him to see the dog section at work. Shay was introduced to several dog handlers and a couple of the police dogs. Shay was shown how the dogs track naughty people and then shown how they catch them when they run away.

After a display the dog sergeant showed Shay his dog and let Shay groom him (which he thoroughly enjoyed). Whilst doing this Shay noticed a handbag on a chair close to him. A few minutes later Shay started to blow his police whistle as he had seen a criminal take the bag and run off. Shay was blowing his whistle really hard and started shouting along with the other officers present. The best part was for Shay to witness the police dog chase 'the bad person'. Shay witnessed the dog attack and knock the criminal to the ground. Most children would have been scared witnessing this. As Shay had his uniform on it didn't faze him one bit whatsoever. At this point in the day Shay was looking very tired but still wanted to do more.

After lunch and getting some much deserved rest. An important part of Shay's day was to meet with a senior officer. Shay took the oath and was proudly sworn in as a police officer. He was awarded advanced car driving certificates just to name a few. Shay had a fantastic day and as usual he didn't want to go home. We knew it would take him a few days to recover, but just seeing the amount of fun and excitement he had today has made it all worth it for our little man.

Shay enjoyed meeting some fantastic people and we would like to thank each and every one of you for making this day just so perfect. Don't tell Daddy BUT Now he wants to be a FIREMAN!

Hospital

Following on from my last entry - Shay was hospitalised for 5 days due to an excruciating pain high up on his right side. He had no coughs, colds or infections that we could see at this time. He has been complaining of being in pain for about 2 weeks prior to him going to hospital. Shay suffers from alot of stomach aches especially when he eats so naturally we put it down to this. It's very hard for such a young child to locate exactly where the pain is. That Monday evening while Shay was asleep, he woke up crying and just by listening to his cry you could tell that something wasn't right. So bags packed in a hurry and a quick dash to our local hospital. On arriving Shay's port was accessed, blood cultures taken and IV antibiotics started. When this happens we know he is going to be in for at least 5 days. His blood cultures came back negative meaning that any infection hasn't gone into his blood stream. Shay's CRP came back that he had an acute infection and inflammation somewhere in his body. Shay's tonsils where very large BUT causing him no ill side affects. That very night Shay had an xray which showed nothing unusual. As it was so late they decided to scan Shay the next morning. Morning, afternoon and early evening arrived and still Shay is waiting to be scanned. Shay is still being given IV antibiotics with no sign of the long awaited scan. Funnily enough his pain has completely disappeared. We mention to the doctors about a pancreas test, on Wednesday morning bloods are taken to test his pancreas function. (which came back clear). Late afternoon Shay at lasts goes for the long awaited scan which unremarkable shows nothing wrong. You can see a complete difference in the pictures...Shay is standing up for his xray the first night in hospital. He was looking very pale and tired. By the time he had his scan he had rosy cheeks and full of smiles.
Shay was administrated antibiotics on admission to hospital by IV. This is the quickest way to deal with infections and on previous admissions has worked very quickly with Shay. To me it seems obvious that any infection he had was cleared up very quickly by the antibiotics. I feel the scan, and pancreas function test was taken far to late for it to show anything. I believe Shay might of had an attack of 'Pancreatitis', talking to other parents with children with Pearson's this seems the most probable explanation for his pain. On leaving hospital he still had enlarged tonsils but this is believed to be only a secondary infection (without symptoms) and not the primary cause. So we have left the hospital with no knowledge or understanding of what was wrong with him. All we know is something has happened.....along with the pain, his speech became slurred and his eyelids droopy. It has taken well over a week for his speech to recover to how it was before and still he is not as clear as he was before.
Shay has a very special day coming up, which I will update everyone in a couple of weeks. He is going to be soooo excited and such alot of people have put so much of their time and energy into this for our little man. All we will say at this time is uniforms, dogs, cars, chases and a skidpan! Mix all that with Shay and he is going to have a day to remember.

Hospital visit!

Shay has been in hospital this week, which I will update you all later on. It has been a huge reminder of the daily struggles our son goes through. He is four years old, but has the aches, pains and fatigue of someone much much older. You look at him and feel totally powerless. His eyes tell a completely different story when he looks at you. The sadness I see from him at times is overwhelming it's hard seeing him like this. Tears are spilt countless times when he says 'I love you Mummy' 'I love you Daddy' . If he goes anywhere we don't like saying goodbye it's always see you soon. Both Alan and I have become very deep thinkers locking up that raw emotion just to get through everyday life and the struggles that brings with it. When people ask how he is it's always he's doing good, he's fine it's the only way to protect our emotions and to stop everything from boiling over. He isn't doing great, people see him playing and having fun! We see him falling over, dropping with exhaustion, not eating as he says 'me so tired, it's hard' or 'my bones are hurting', struggling with his mobility on a daily basis. BUT, take all that away and we have been blessed with the most amazing son, who has bought so much joy and laughter into our life's. He is an inspiration to us and to you, he is a fantastic brother who is so caring towards others. My little boy is fighting life daily and we are so proud he picked us as his parents.

Birthday Boy!

Shay was so excited as his birthday approached as he knew he was going to be four and he looks on this age as being a 'big boy'. He knew exactly what he wanted for his birthday; a motorbike, superman outfit and a skateboard! He will probably crash countless times, end up with a load of bruises but this is what it's all about, him having a very special 4th birthday.
It started as a very busy day for Shay. He was in GOSH nearly all day having his eyes tested. He is such a fantastic boy who gets pulled and prodded about until he is so tired that his eyes are closing with exhaustion. We are estatic to say there has been no changes with his eyes in the last six months. The information going into his eyes becomes very muddled but somehow it rearranges itself when it comes out the
other side as far as we are aware this causes him no problems at the moment.
After the hospital Shay wanted to go to Pizza Hut, he throughly enjoyed himself even though a mouse would have eaten more than him. Shay had a very long day and it took him two days to recover from a very busy day.

A special day out

I really need to be more organised and try at least manage to keep my little mans blog up to date! But life has been a little more than hectic to say the least. Shay had a fantastic 4th birthday and is now the very proud owner of a motorbike just like his Daddy. Of course he is exempt from any speeding or road traffic offences now :0)
A couple of days before his birthday Shay had a brillant day out at Camden town hall with Daddy. On the way he stopped to see his 'best friend' (as Shay calls him) Paddy Hughes. They enjoyed a late celebration in the pub for St Patricks day as you can see by the hats in the picture. After visiting, Shay and Dad both made their way to the town hall so Shay could watch his Daddy receiving a commendation. Shay was allowed to be next to Dad during the ceremony and must say gave Dad a tough time. Shay knows when to be quiet but his whispering is extremely loud! He was so proud to go up with Dad to receive his commendation by the borough commander and John Snow the channel 4 news reader. Shay had a fabulous day out and on the way back insisted on stopping at the pub again to see his 'best friend' Paddy.

Poorly...

Shay has been poorly since Sunday evening. It started with a high temperature and sickness. Monday morning he was put on antibiotics and Ventolin for his cough. Yesterday Shay was at hospital with suspected chicken pox, he only had the spots down the backs of his legs. It seems our little man has chicken pox but we won't be sure until his blood cultures are ready tomorrow. He is on double strengh Zovirax which will protect Shay from alot of the symptoms of the pox. At the moment no-one knows how his body will respond to the virus especially with him having Pearson's. It's getting more difficult to give him his meds as he is now refusing them point blank. So lots of persuasion is needed but even then it is a major battle.
Hopefully it will turn out to be an uneventful week and he gets better soon.

Growing up!

Shay is certainly growing up fast the last couple of months. He is looking older and developing how he should across all areas. He enjoyed a recent trip to Wembley stadium and as you can see by the picture he loved it. I think his favourite part was going on the train! Shay had his last transfusion on the 23rd September 2010 so it has been a while now. It seems his marrow is making red cells as his hb is now a whooping 10.8, nearly in the normal range. His platelets, WBC and neutrophills are still very low but stable. So hopefully in the near future these will recover also. It has always been a huge worry that Shay would go into complete marrow failure. They can transplant but it seems the survial rate for a child with Pearson's is 18mths post transplant. So thank God he is slowly recovering it's been a long old wait for it to happen. Can't believe our little man will be 4 years old next month he has had a hard struggle with life the last couple of years it just goes to show what a strong fella he is.

We'll miss you Katie!

Shay's favourite nurse is leaving GOSH at the end of the month to follow a career in nursing. Katie has been with Shay since day one and it was really sad to hear that she is going to uni. Shay will miss her lots when he is up at the hospital as he always looks forward to seeing her. We wish her all the best over the following 3 years and hopefully will catch the odd glimpse in the corridors when she is a student nurse.