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London, United Kingdom
Welcome to Shay's story and thank you for taking the time to find out how Shay is doing. It is a place to share our hopes, sadness and the happy times that our little man has. Shay in April 2008 was diagnosed with Pearson's Syndrome a Mitochondrial disease with no cure. Pearson syndrome is very rare, less than a hundred cases have been reported worldwide. It characteristically present in early infancy with pallor, failure to thrive, pancytopenia and diarrhoea. Additional manifestations often include progressive external ophthalmoplegia, proximal myopathy with weakness, and neurologic disturbances. Multiple organ involvement is quite variable. Most infants die before age 3, often due to unremitting metabolic acidosis, infection, or liver failure. Those few individuals who can be medically supported through infancy may experience a full recovery of marrow and pancreatic function. These children eventually undergo a transformation from Pearson's syndrome to Kearns-Sayre syndrome with the development of ptosis, incoordination, mental retardation and episodic coma. Life as we use to know it I cannot remember. We have to live in hope that a miracle will happen for our son.

Saturday, 4 July 2009

What a day!



Shay had his 5th transfusion today and what a fiasco it turned out to be. Shay had his cannula fitted on Thursday as they had to take blood for a cross match anyway. Thursday evening a doctor phoned and informed us that not enough blood was collected for a cross match so we had to go back in at 9am Friday morning. The nurses were fab and saw Shay straight away. More blood should have been easy to take from Shay but as they were trying to flush his tube it was blocked. Throughout this Shay only had the cold spray which lasts 30 seconds and he was fantastic he was tensed up but he stayed still for the nurses, he is one remarkable brave little boy.

So cannula fitted again this time on his thumb sucking hand. We left the hospital as his transfusion was at 1.30pm so we thought. A phone call later informed us it was at 2.30pm as the blood was due in at 2.15. Still remaining calm and stress free at this point. We arrived at 2pm, Shay being Shay was a star and took everything in his stride.
At 3pm the nurse informed us that the blood did arrive for Shay but colindale sent the wrong type. Completely incompetent, at least it was noticed before it was too late. This went on for hours because Shay's blood has to be irradiated which is making sure it is bacteria/virus free this takes 1.5 hours to do.

We were moved onto the main ward into the high dependency beds until Shay finished the transfusion. Shay was having 190ml of blood which I asked earlier as it's good to know what is going into our little man. On a new ward with new staff I asked the nurse how much Shay was having again (just making sure they are getting it right)you've guessed it I was told a whooping 290ml. Towards the end of the transfusion the machine carried on feeding the blood to Shay even after his 190ml which I had to call the sister to stop the machine.
To end a very long day and story Shay eventually left the hospital at 10.30pm extremely tired, both of us exhausted and somewhat losing our confidence in hospitals. We realise to stop mistakes happening we have to be on guard constantly and we now know not to place our trust 100% in others but to take on a proactive role in Shay's care.

Results;
Hb - 7.7
Neurophills - 0.47
WBC - 3.25
Platelets - 122
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1 comment:

  1. KarenSun Jul 12, 01:36:00 pm 2009

    Love the picture - he really sounds like a poppet. I'm not sure I would be so understanding in his shoes!
    Mum says that Shay is having a hickman line inserted next week - I hope that make his life and yours a little easier. If there is any help you might need re wedding etc, then please don't hesitate to call. Alan has my number and E-mail- happy to help in any way we can and are looking forwrad to the Big Day!

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