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.

Happy New Year!

Shay at last was well enough to enjoy Christmas this year. After weeks of colds and coughs he was given respite from all of this and thoroughly enjoyed Christmas day. It is the first year that he knows what Christmas is and that Santa is coming to visit. Shay is manic about toy story and buzz at the moment as the picture shows.
Shay had his bloods taken just before the New Year and with a fantastic 9.4 for his hb he doesn't need a transfusion. It has now been 15 weeks since his last transfusion which he is really pleased with. Next week we will be seeing his lead specialist and lots of questions to ask regarding this change in Shay's health.
Happy New Year everyone!

Life is very busy...

The last 7 weeks have been hectic to say the least. Shay is now a very proud big brother. His sister 'Cearney Shaylee' (pronounced Car-nee)is now 7 weeks old and is adored by Shay. Our household has become manic! Shay has taken to a new addition really well and has grown-up in many ways.

For the last 4 weeks Shay has been at school only 4 times. Since September he has had more coughs and colds then ever before. He has had one visit to hospital for a medical assessment but so far we have been able to keep him at home. Shay is now at the age where he gets really upset when he cannot go to school and is starting to dislike hospitals.

As Shay has grown his port is now very uncomfortable for him and having bloods is requiring two or even three attempts with the needle. Our son shows so much bravery and knows that if he moves it will hurt him more. You can feel him shaking and tensing up as his port is accessed.

Shay's last transfusion was in September. It has now been 13 weeks since this transfusion and at his last count his hb was 9.4. At last his marrow is starting to make red cells. To most this would be brillant news but to us it's just as his doctor predicted and now we are waiting to see what else changes in our little boy. Any changes with Shay is not good, it is a sign that the pearsons is progressing.

Something to celebrate, maybe....

Shay has been having transfusions for nearly two years now and the majority of the time he has needed one every four weeks the last couple of months has been every 6 weeks. Today he is now six weeks post transfusion and his Hb is 9.1. Fabulous news the nurses are saying, for me I feel anxious, panicky, hating change of any sorts when it comes to our little boy. It certainly looks like his red cells are starting to reproduce once again and at least one of his blood lines is on the road to recovery – but at what cost is a question that refuses to go away and one that I cannot answer just yet. I know I critically look at everything when it comes to Shay. I look too far into the future sometimes but to me what help can I be to my son if I only take one day at a time.
Shay is looking very pale, tired and his eyes are red rimmed. Saying all this he still has a gorgeous smile and is turning into a real Dennis the Menace.

Hb - 9.1
Platelets – 66
Neutrophills – 0.61

Someone who seems to know what they are talking about...

Shay was at GOSH today seeing a child physiologist who specialises in children with metabolic diseases. At last someone who understands what we are going through and more importantly what Shay might be feeling. We have waited a very long time for this appointment. She has two other children in her clinic she helps, maybe children is not the right word but TEENAGERS! Both have been diagnosed with Pearson’s/Kearns Sayre syndrome. She spoke more of the boy than the girl but it did come across to me the girl has far more complex health issues than the boy. He is 15 years old, lives an independent life even though he does have sight, hearing problems and growth issues. It has made me deliberate further into the future, which I don’t do and prefer to take each week as it comes.
We discussed Shay in general with the only real problem that needs to be tackled is his eating and growth. She said he is very complex as he is a very social eater and he will be up at a buffet table along with the rest of us loading his plate up, but then he doesn’t eat any of it. Shay gets very territorial over his food if anyone tries to take it away, but still he doesn’t eat. I am at a loss as to how he sees food, how it tastes to him and why he shows this sort of behaviour. Metabolic diseases can make food taste differently to individuals but Shay is too young to be able to tell us.
We have to tread with caution so as not to make an issue out of it and just keep trying to do the best that we can for our little man.

Growing...

Shay at last is in a junior bed and out of his cot. Last night he slept in it for the very first time and insisted on a story before he snuggled down. I thought we would have major issues as he doesn't cope very well with change. The fact that it is a toy story bed and all the paraphernalia that goes with that has helped. He has grown 3 Cm's so far this year, not a major growth spurt but he is growing and the cot was getting to small for him. Shay has gone toy story mad at the moment and we are just waiting until he has fully recovered from his cough to take him to see toy story 3.

Sometimes I speak to soon..

Shay has been poorly for the first time this year. He has been battling a cold and cough this last week but he is coping extremely well with it. Shay is on his ventolin inhaler every two hours plus antibiotics which seem to be working.
Today Shay enjoyed his first swimming lesson, it was hard handing over control and watching others with him in the pool. It has taken a while to find the right swimming pool but we managed to find a luxury heated one with one to one instruction. Shay smiled and swam widths with his arm bands for 30 minutes this morning, they even had him swimming on his back! The swimming instructor has been fantastic and went out of her way to find out all about Shay's health and what would be best for him before Shay started swimming. Shay's mobility is extremely important to us and we have to do all that we can to maintain and improve his strength and stability for the future, what better way than swimming.
Shay had his Audiology appointment today at GOSH and we are really pleased that his hearing is perfect. He will not need to be seen for another year now.