Shay was admitted to Watford general Monday afternoon. He had a cough which can only be described as dry and slightly annoying for Shay. His temperture zoomed sky high and when it reached 38.9 we decided he needed to be admitted. We are getting so use to running around packing bags for our little man now; but this time knowing he would more than likely be spending the week rather than a few hours in hospital.
The Doctors and nurses who are all gradually getting to know Shay by sight have been fantastic. The care Shay has received has been outstanding. The hospital knew exactly what to do and in a matter of minutes they accessed Shay's port and had an IV antibiotic drip up and running. Blood was taken to culture which took 48hrs for the results to come back, this showed that Shay had a viral infection. We also discussed the possibility of Shay going on Tamiflu. Tamiflu for Shay would only shorten the illness by 24hrs and would in fact make him feel lousy. As Shay's symptons were only a a high temperture, slight cough and a snuffly nose it was decided not to give it to him as he was coping so well.
Shay's Hb levels also took a nose dive along with his neurophill and platelet count. He has had another red cell transfusion which this time around he fell asleep!!!
So after a very long week for our little boy he is now back home and it feels great to have him in his bed sleeping like the angel that he is.
Hb - 8.4
Neurophills - 0.05
Platlets - 58
WBC - 2.77
About Me
- Shay
- 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.
Friday 31 July 2009
Wednesday 29 July 2009
Results
On Monday Shay was at GOSH to see his Metabolic consultant. Alot of the test results are not back as yet. When Shay had his operation they took quite alot of blood to test the functioning of his major organs. We received fantastic news that at present they are working how they should be. No kidney leakage, no problems with his brain or heart.
Some of his levels they were checking have come back extremely low and they have taken more blood to check this wasn't a bogus result. One of the tests were for his carnitine levels. Carnitine plays a critical role in Shay's energy production. It transports long-chain fatty acids into the mitochondria so they can be oxidized to produce energy. What I understand is his carnitine levels could be why Shay is always clumsy when running, walking and is always quite lethargic. He will often be found hanging around our legs and asking to be picked up.
His consultant has also taken another sample of Shay's stool (he was able to offer this up on command). Shay's stools have been extremely loose the last couple of weeks to the point where his nappy is unable to hold it in. This test will see if his pancreas is working how it should be. It's hard to listen to Shay's doctors talking about his condition without becoming an emotional mess. When they are talking it is always 'that is not happening YET' sometimes I would like to hear hopefully that won't happen. Talking about Shay's Pancreas we were told in pearsons pancreatic involvement seems to be something that seems to always happen. Well!!! we just happen to think our son is the most amazing child with everything to live for. He is one very special boy who has touched the hearts of not just us his family but hundreds of people. We don't think Shay is a text book case so anything can happen. Maybe it is Shay who will be different because one child has to be for the future of the rest of the little darlings living in what can only be described as hell.
Some of his levels they were checking have come back extremely low and they have taken more blood to check this wasn't a bogus result. One of the tests were for his carnitine levels. Carnitine plays a critical role in Shay's energy production. It transports long-chain fatty acids into the mitochondria so they can be oxidized to produce energy. What I understand is his carnitine levels could be why Shay is always clumsy when running, walking and is always quite lethargic. He will often be found hanging around our legs and asking to be picked up.
His consultant has also taken another sample of Shay's stool (he was able to offer this up on command). Shay's stools have been extremely loose the last couple of weeks to the point where his nappy is unable to hold it in. This test will see if his pancreas is working how it should be. It's hard to listen to Shay's doctors talking about his condition without becoming an emotional mess. When they are talking it is always 'that is not happening YET' sometimes I would like to hear hopefully that won't happen. Talking about Shay's Pancreas we were told in pearsons pancreatic involvement seems to be something that seems to always happen. Well!!! we just happen to think our son is the most amazing child with everything to live for. He is one very special boy who has touched the hearts of not just us his family but hundreds of people. We don't think Shay is a text book case so anything can happen. Maybe it is Shay who will be different because one child has to be for the future of the rest of the little darlings living in what can only be described as hell.
Tuesday 21 July 2009
Cancelled visit
Shay's hospital appointment yesterday was cancelled due to his consultant having flu. This appointment was to talk through Shay's results, but now it will be a couple of weeks before we know anything.
Shay has very slight pain with his port now, but we are still very cautious about how we lift him up as this still causes him a great deal of discomfort.
He will gladly show of his scars to anyone that wants to see and when he puts a t-shirt on he will blow kisses and say 'bye' to it bless him.
Shay has very slight pain with his port now, but we are still very cautious about how we lift him up as this still causes him a great deal of discomfort.
He will gladly show of his scars to anyone that wants to see and when he puts a t-shirt on he will blow kisses and say 'bye' to it bless him.
Thursday 16 July 2009
Back Home
Shay is now back home from hospital and we are no further along with finding out what was wrong with him. As soon as his pain disappeared they said he can go home now. So what was the problem in the first place? some doctors seem to think if the pain has suddenly gone then the child must be better. It seems some in the medical profession get to a certain level in their career and they forget what basic bedside manners mean and what they can learn by listening to parents rather than their noses stuck in the air and pretending to know what is wrong with Shay; when in reality they have not got the slightest clue as even they have to rely on the internet for information on this horrible syndrome.
We were told Shay had constipation/wind then we kindly mentioned that an xray showed otherwise and that everything was normal as it should be. Shay constipated??? if only that was the case. Shay's bowels were soft to touch and his stomach was nice and soft, the only part that was rock hard with Shay guarding himself was around the liver region. Angry is not even able to describe how we feel about it all. Shay is back up at GOSH next week to see his lead metobolic consultant in his care so hopefully he will be checked out more fully than what he was at our local hospital.
He is in alot of pain and is very wobbly on his feet.
Just a quick reminder to everyone as it is so so easy to forget. Don't lift Shay up from under his arms this is extremely painful for him and the poor babe is now refusing to give cuddles just in case he is lifted the wrong way. Shay is so adorable he will now show his port to you so that he knows that you know not to lift him bless him.
We were told Shay had constipation/wind then we kindly mentioned that an xray showed otherwise and that everything was normal as it should be. Shay constipated??? if only that was the case. Shay's bowels were soft to touch and his stomach was nice and soft, the only part that was rock hard with Shay guarding himself was around the liver region. Angry is not even able to describe how we feel about it all. Shay is back up at GOSH next week to see his lead metobolic consultant in his care so hopefully he will be checked out more fully than what he was at our local hospital.
He is in alot of pain and is very wobbly on his feet.
Just a quick reminder to everyone as it is so so easy to forget. Don't lift Shay up from under his arms this is extremely painful for him and the poor babe is now refusing to give cuddles just in case he is lifted the wrong way. Shay is so adorable he will now show his port to you so that he knows that you know not to lift him bless him.
Wednesday 15 July 2009
Back in hospital
Tonight Shay has been admitted to our local hospital. At gosh today he was in so much discomfort from the LP and the Port op that he is becoming exhausted. From early this evening he has been cramping up in agony around his liver region. He is unable to stand or do anything that requires him to move. Phoning the hospital I was calmly told that they had no beds and really had to push my point and told them in an equally calm way that really there is no choice and I am bringing him in and to pull his notes so they can do a bit of reading. The doctors and nurses that I know are fab but when you speak to someone that does not know Shay it can get frustrating to the point where I had to start name dropping and was very willing to phone his consultants up personally.
Shay has had an x-ray on his chest and abdominal region. His chest was clear and his abdomin was a bit unclear. So to be cautious they have admitted him tonight to watch over him with Daddy by his side. Shay's pain seems to come in waves and on leaving both of them his stomach was making noises and his bowel was soft. He does have a slightly enlarged liver that they were able to feel eventually but no temperture. It looks like Shay will be having a scan in the morning just to see what really is going on inside him.
We want the best possibly care for our little man and his team of consultants have been absoultely brillant with Shay. Things have happened extremely quickly for Shay and we got a diagnosis quickly compared to others. The hospital visits are taking it's toll and I find it hard to keep the frustration out of me when I am asked the same questions over and over again regarding Shay. Simple silly things, how many times do I have to repeat what faith he is? What's his diagnosis? the whole point in having his special little green card I thought (with diagnosis blazened across the front) is to let others know to get the notes and read what's in them. Thank God that both our children has parents who are on the ball constantly because if we were anyother way who knows what might happen!!!
Shay was in GOSH all day with tests being done on his eyes. The jargon of it all was completely overwhelming and we are now having to get our heads around not only what is involved in hematology but now also the workings of the eye.
With one of Shay's eyes the muscle will move and the eye does not follow immediately, the specialist did mention that he won't at this time need an operation to correct this. They also noticed something unusual which is his left eye doesn't send messages over to the right side of his brain. The tests on his eyes were so far advanced in technology that we are now waiting for a report to fully understand what all this means. They think the pigmentation in the eyes could be that dad is pale and I am olive skinned and this could be our genes mixing to cause this. They now have a baseline of what his eyes are like and we will be going back in 6 months for the same tests to see if there are any changes. Shay was again the model patient today with electrodes attached to his skull and face. Even with the pupil dilating drops that stung him he did not complain, not even once. As most of you know Pearson syndrome attacks the eyes and Shay's risk of this happening to him is still extremely high along with him losing his hearing, todays news has not at the moment bought much relief to us. Until we get the report through and we can understand it more fully will we be able to take everything on board and look at the results alot more closely.
I know I keep saying this but he is such a strong child who just keeps amazing us, we are so proud to have the honour of being the parents of a very special boy.
Shay has had an x-ray on his chest and abdominal region. His chest was clear and his abdomin was a bit unclear. So to be cautious they have admitted him tonight to watch over him with Daddy by his side. Shay's pain seems to come in waves and on leaving both of them his stomach was making noises and his bowel was soft. He does have a slightly enlarged liver that they were able to feel eventually but no temperture. It looks like Shay will be having a scan in the morning just to see what really is going on inside him.
We want the best possibly care for our little man and his team of consultants have been absoultely brillant with Shay. Things have happened extremely quickly for Shay and we got a diagnosis quickly compared to others. The hospital visits are taking it's toll and I find it hard to keep the frustration out of me when I am asked the same questions over and over again regarding Shay. Simple silly things, how many times do I have to repeat what faith he is? What's his diagnosis? the whole point in having his special little green card I thought (with diagnosis blazened across the front) is to let others know to get the notes and read what's in them. Thank God that both our children has parents who are on the ball constantly because if we were anyother way who knows what might happen!!!
Shay was in GOSH all day with tests being done on his eyes. The jargon of it all was completely overwhelming and we are now having to get our heads around not only what is involved in hematology but now also the workings of the eye.
With one of Shay's eyes the muscle will move and the eye does not follow immediately, the specialist did mention that he won't at this time need an operation to correct this. They also noticed something unusual which is his left eye doesn't send messages over to the right side of his brain. The tests on his eyes were so far advanced in technology that we are now waiting for a report to fully understand what all this means. They think the pigmentation in the eyes could be that dad is pale and I am olive skinned and this could be our genes mixing to cause this. They now have a baseline of what his eyes are like and we will be going back in 6 months for the same tests to see if there are any changes. Shay was again the model patient today with electrodes attached to his skull and face. Even with the pupil dilating drops that stung him he did not complain, not even once. As most of you know Pearson syndrome attacks the eyes and Shay's risk of this happening to him is still extremely high along with him losing his hearing, todays news has not at the moment bought much relief to us. Until we get the report through and we can understand it more fully will we be able to take everything on board and look at the results alot more closely.
I know I keep saying this but he is such a strong child who just keeps amazing us, we are so proud to have the honour of being the parents of a very special boy.
Monday 13 July 2009
Port Op!
We are now back home (late) and Shay is tucked up safely in bed feeling a little bit sore and tired.
Today has been another emotional rollercoaster, with both of us feeling so many different emotions and in the back of our mind thinking if a port is the right thing for Shay.
Yet again Shay was an absoulte star letting the doctors and nurses do whatever they wanted. Our son is totally amazing with everything and even if he finds a procedure uncomfortable he will just keep still and let them get on with it.
Shay was down in theatre for 1.5 hours today, waiting in the ward the time seemed to drag by.
When we were called down Shay was fast asleep he opened his eyes for a few seconds looking disorientated then fell back to sleep. Even on the long trolley ride back to the ward his eyes kept fluttering open for a few seconds then he would fall back to sleep.
Shay is obviously in pain from the wound in his neck and chest as he wanted us to take the dressing off when we got back home. In his young mind he thinks if the plaster comes off then the pain will go away bless him.
The worst by far has been moving Shay around. Lifting him from underarm is causing him pain as well as the wound from the lumbar puncture on his lower back.
Shay cannot bath for 5 days and we have been warned that he could have a lot of bruising under his chest due to them tunneling under his skin for the port.
Shay has had no problems today apart from trying to catch a urine sample which he seemed to know everyone wanted but he wasn't giving up that easily. Cotton wool balls in a nappy catch this perfectly, that's of course with Shay participating with everyone :). In the end we manage to get a whole 16ml from our stubborn little boy.
Shay is back at GOSH tommorow for his eyes so hopefully we just might have some good news.
Friday 10 July 2009
Hospital appointments
Shay had his consultation at GOSH for his heart. He was a star when he was being examined and done everything that the doctors wanted him to do. Shay's heart on the scan is perfect and his ecg was normal. Just the news we were hoping for, which for once has made us extremely happy.
I did mention his liver to a nurse and asked what the 2cm edge meant and she said it did mean Shay might have an enlarged liver. We will know more by the end of July on this.
Shay has a big day looming with his port, lumbar etc it's been hard to try to keep everything normal and carry on like everyone else. There are so many things going on at the moment that sometimes the stress of everything just boils over. We have been noticing more and more Shay's eyes are very delicate and he now wears his sunglasses when he is out constantly. When he gets out of bed the light really affects his vision and he is sometimes known to have his glasses on when he is brushing his teeth. Again we will know the full extent of what is wrong with his eyes by the end of the month and hopefully he just has sensitive eyes.
Praying for a smooth day next week for Shay and hoping everthing goes as planned
I did mention his liver to a nurse and asked what the 2cm edge meant and she said it did mean Shay might have an enlarged liver. We will know more by the end of July on this.
Shay has a big day looming with his port, lumbar etc it's been hard to try to keep everything normal and carry on like everyone else. There are so many things going on at the moment that sometimes the stress of everything just boils over. We have been noticing more and more Shay's eyes are very delicate and he now wears his sunglasses when he is out constantly. When he gets out of bed the light really affects his vision and he is sometimes known to have his glasses on when he is brushing his teeth. Again we will know the full extent of what is wrong with his eyes by the end of the month and hopefully he just has sensitive eyes.
Praying for a smooth day next week for Shay and hoping everthing goes as planned
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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