On 30th May of this year (2014) our brave knight 3 year old Cameron, lost his 9/10month battle with this disorder.
This is why it is our aim to fund as much research as possible to stop little boys and their families from going through what Cameron and our family did.
Cameron was perfectly normal, healthy little boy until September last year (2013) when he appeared to have caught a cold, with a nasty temperature. It didn’t go away, and after spending 2 weeks in the local hospital (Kettering) with test after test, he was transferred to Leicester Royal Infirmary because of abnormal blood test results. On the 5th October we were told that they suspected Cameron to have HLH (Hemophagocytic Lymphohistiocytosis) Syndrome and with this suspicion we were to be transferred to Great Ormond Street Hospital, which we were within a week.
There are 2 types of HLH Syndrome, Familial HLH or secondary HLH. Familial HLH means there is an underlying genetic problem/disorder. This diagnosis was confirmed by the lovely immunology team at Great Ormand Street Hospital. Cameron had HLH Syndrome secondary to XIAP Deficiency. Our lives were completely turned upside, with the shocking discovery of just how poorly our little boy was and the prospect of having to face a bone marrow transplant to save his life. We battled through and picked ourselves up, and were ready to fight. We got to go home for the first time since September in time for his 3rd birthday in November. He was doing so well and we thought he might just get away with not needing any chemo therapy.
Then in December he took a turn for the worst and we ended up back in Great Ormond Street Hospital, where we stayed until February. He had relapsed, which meant needing the chemo therapy we thought we had avoided and a bone marrow transplant was a must. In January we found out that Cameron had a match for his transplant, which was fantastic news. We didn’t, and still don’t, know much about the hero that donated for him other than he was a 24year old male from Germany. And we thank him immensely for giving Cameron the chance he so desperately needed. We only made it home for a week in February, and really, didn’t come home again. Cameron kept on catching infections, ranging from shingles to line infections. Which with not having an immune system working properly was quite dangerous, he was on medicine after medicine and antibiotics galore!
He went through so much that even the staff were saying “it could only happen to Cameron!” Despite all of this he was such a happy, smiley little boy and didn’t let it get to him and quickly became a favourite on the wards he was on. We were allowed home for a week and a half before having to go back down for his transplant. On the 22nd of April the transplant process started, which didn’t seem to affect him until he was about a week in. The 1st of May saw him receive the donor cells, effectively transplant day. He continued to do well, although was feeling very poorly and his need for oxygen was getting greater and greater. On day +12 of transplant we found out it was working, he was already 30-40% donor after 12 days of receiving the cells. Fantastic news! All this battling, tears, stress and worry was finally looking like it was coming to an end.
On day +18 he went to intensive care, his need for oxygen support had got too great for the normal ward. On day +20 the need was far greater than we could’ve imagined and he had to be tubed and ventilated. But he wasn’t strong enough to be on a conventional ventilator and needed to be on an oscillator (high frequency ventilator) which was devastating news.
He started off in intensive care on a CPAP mask which meant he didn’t need to be sedated, he hated it! But we still smiled, played lego, watched films and had Buzz Lightyear laser the naughty things away from his lungs! Day +23 saw him have a very bad day, with not nice conversations with doctors and horrible phone calls to family at home. Looking back on that day we all knew what the outcome was going to be, but we didn’t believe it and weren’t going to stop fighting.
He seemed to improve over the next couple of days, to a point where they attempted to change his ventilator to a conventional ventilator, it didn’t work and he had to fight very hard and remain on the oscillator. He made tiny improvements and the PICU team and the BMT team both worked very hard and did everything they possibly could to save him. On day +29 Friday, 30th May 2014 we were due to have a meeting with both teams to discuss a plan going forward, this meeting didn’t go ahead. Cameron couldn’t fight anymore.
This doesn’t mean his bone marrow transplant didn’t work, in fact it worked a bit too well. He suffered from Graph versus Host Disease (GvHD) on his lungs. The donor cells sort out a place in his body that was unhappy, and wanted to help fix it. Cameron’s cells didn’t want the donor cells to help, this resulted in them having a big fight on his lungs.
Our Brave Knight is aiming to help research in to these sorts of things, find out why these things happen to XIAP patients and help with stopping it happening to other families. If we, as Cameron’s family, can stop at least one other family from going through what Cameron and our family had to go through then we have achieved our goal.