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.