Sally and I are hugely grateful to everyone for all their support, suggestions, action, etc. It has once again been a whirlwind week and we are now taking breath. Skye was admitted to hospital again yesterday, following a further consultation with a Consultant Paediatric Neurologist in the morning. We have been dashing about trying to assimilate all the information that everyone has kindly offered, but at the same time experiencing a real unreality about the whole situation because apart from this real weakness and inability to function on a mechanical level, Skye has been mentally alert, funny, witty, and a joy to be around. He is eating like a horse and has put on over a kg in a week. Thank you steroids. We keep joking about putting him up for Mastermind with his speciality subject being food!
We had a check up on Tuesday, which shed no more light on his condition, other than there might have been the slightest hint of improved strength on the right hand side. But apart from that - status quo! Our consultant continued researching and consulting but was coming up with nothing that might assist Skye. On Wednesday evening we received a call from our Consultant to ask whether we could come in for an appointment the following morning with the Neurologist. Hearts racing after another sleepness night we arrived at the JR for what we thought was going to be an hour. After going through the whole case history again and having had a chance to look at the most recent scans with the Neurologist and compare them with the scans of the 1st April, the decision was, that we would be admitted and an alternative form of treatment would be administered.
WHY? Skye's scan of last week Tuesday, was described to us as 'devastatingly abnormal' and having worked as a Neurologist at the JR since 1987, this is only the 2nd case he has seen which is anything like this. When he looks at the scan and then compares that to the assessment he has just done on Skye, it defies belief that he is functioning as well as he is. Had this not been picked up last Tuesday and he had not been started on high dose steroids there and then, Skye would have ended up in ICU very shortly thereafter. There is evidence of significant swelling of the White Matter in both the brain and the Upper section of the Spinal Cord. Lower down the cord looks to be normal. This could be consistent with the high dose boost of Radiotherapy which was given to those areas if this is indeed radio-necrosis. AMENDMENT 20th January 2015 It is now believed among the experts that it is likely the high dose Thiotepa was the primary cause of the Chemo-Radio Necrosis (which is also referred to as Chem/Radio Neuro-Toxcicity by some consultants). Children treated in France on a lower dose of Thiotepa have not shown any signs of severe side effects seen here.
MRI Scan - 1st April showed significant tumour reduction with no sign of any other unexpected abnormalities. Emergency MRI - 20th May showed 'devastating abnormalities'. Because of the rapid onset of this 'swelling', they are now doubtful that this is traditional radio-necrosis (sometimes seen as a much later side effect over a long period of time. It is possible but no certainty. It is consistent in that break down of tissues causes inflammation because the body floods the area with antibodies and cells to fight the break down and protect surrounding cells. If not radio-necrosis, then what? Some form of infection. Either something which has been dormant within his body and because he is immune suppressed it has reared its head, or it is the fact that the Thiotepa having passed through the blood-brain barrier and has flooded the brain and spinal fluid with toxins which the body is now fighting and causing inflammation. So, we had Infectious Diseases (ID) in yesterday taking bloods and demanding another Lumbar Puncture. ADDITION 20th January 2015 - This was ruled out and Skye's cause of death was registered Chemo/Radio Neuro Necrosis due to Toxcicity. Other children have also been found to have been effected in the same way.
So we have our team of Consultants pushing to start treatment with Intravenous Immunoglobulin (IVIG) and even higher dose steroids (methylprednisolone). ID want all the tests done before they start infusing anything. Next LP appointment was Monday, so significant delays to vital treatment. But, we have a great team and because they know we won't take NO for an answer they stuck to their guns, got the dose of steroids in last night, forced Skye onto the emergency theatre list and he had his LP today and his 1st infusion of IVIG. In a nutshell, it is a blood product pooled from over 1000 donors, where they take the best bits of the immunity cells and concentrate them all in a little bottle. Then shove them into Skye. He will be having 5 infusions of the stuff on each of the next 5 days. This is to prevent further spread and hopefully reverse what they are now calling:
Acute disseminated encephalomyelitis (ADEM) is an acute demyelinating disorder of the central nervous system, and is characterised by multifocal white matter involvement. Diffuse neurological signs along with multifocal lesions in brain and spinal cord characterise the disease.
So, our Roller Coaster continues - this offers us a potential speck of light at the end of what was an incredibly dark tunnel just yesterday. This means that Sally and Skye are once again in hospital, under a very watchful eye. If it works, then brilliant, if it doesn't then at least we gave it a try.
We are so incredibly grateful to everyone for their love, prayers and support. Skye continues to inspire us minute by minute. His ability to laugh through all of this has been extraordinary!
