A bit about my history:
This is my first post about my condition so I'll put everything down, you might find it useful.
I am a 37 year old male in the UK who was diagnosed with Lymphatic HES when I was 18 yrs old. They detected it during a blood test when I had Glandular fever, and I also had a beetroot coloured rash just on my shins.
My EOS count was regularly above 20 for no known reason. I have been to many hospitals (Sheffield, Manchester, Hammersmith, Sunderland and Newcastle) and undergone all of the standard drug and chemo treatments.
Until recently my only physical ailment with HES was a nagging skin rash that could be clear one week and turn to large red whelks/lumps the next. It can get so bad that even wearing underwear/clothes causes friction and extreme soreness and agony.
For the blood I am now on an infusion called Mepolizumab. This is an anti-interlukin 5 antibody that specifically targets the mechanism that produces eosinophils. GlaxoSmithkline make the drug which has been on clinical trials for the last few years.
My hematologist applied for compassionate use and we were accepted. Recently in June the powers that be didn't pass the drug to enable it to be available to all. I think they require many more statistics to be gathered, so GSK is keeping it available to those that are already on it. There are no side effects to the drug and it is given in a 30 minute infusion. My EOS are now kept at 1 or less and I only need to take the infusion every 6-8 weeks.
However the skin problem is not controlled by lower EOS. This is frustrating! I take around 20-30mg prednisilone to control the rash and it causes weight gain and cataracts. It also is bad for the stomach so need to take Lanzoprazole to help protect the stomach.
I've been on steroid creams, PUVA, UVB light treatment but they take so long to build up to a decent dosage that I am in agony for weeks until we get there. The PUVA is the worst as I don't tolerate the psoralin tablets which make you feel very sick for hours on each treatment day. Hence why I take prednisilone.
I'd say that either HES or the drugs that I've been on definitely make me more susceptible to viruses. I have had herpes simplex in both eyes which left scars. I started getting wharts and also toe nail infections (clear now!). I had labrynthitis which required a hospital stay and quarantine.
On three occasions I've had sever stomach pain, so debilitating that I couldn't move or take a breath. I went to A&E and they couldn't find anything wrong.
Anyway - I thought that my treatments were managing the condition but out of the blue in December 2008 I had a heart attack! To be fair the heart was monitored via ECG and ultrasound nearly every year since I was 18, but they didn't expect a heart attack.
They were quite surprised but have put it down 100% to HES. It seems that many years ago there might have been a tiny site in the artery that the EOS attacked, the body tried to heal it. This cycle went on and on and the mixture of repair tissue and EOS caused the entire artery to be blocked. The entire right coronary artery was fully occluded.
I had 5 stents put in to rebuild the artery and it looked as good as new. It was very traumatic, I had ventricular fibrillation during the procedure and even now I still relive the moment when I went unconscious on the operating table (I thought I'd died!) and they had to use the paddles to start me up again.
Unfortunately the stents became occluded within 6 months, an angiogram showed that no blood was getting through, but luckily some minor blood vessels routed blood around the problem in order to preserve the heart muscle.
I've since had two more heart attacks and it became more and more apparent that even though mepolizumab was controlling the blood count it was not stopping any end organ damage. Something needed to be done and after a long series of meetings with lots of specialists it has been decided to go ahead with a bone marrow transplant (BMT).
I had my second meeting with the BMT team this week and we are in to the second round of narrowing down the initial searches. I will be using an unrelated donor, of which there are only 1 or 2 known cases reported. The biggest risk is graft versus host syndrome which can affect the skin, liver, kidneys and pretty much anything in the body and has a serious mortality rate on it's own. Factor in to that the conditioning I will have to undergo before receiving the bone marrow and that opens me up to viral attack from the dormant viruses within my nerves. They have given me a 70% chance of surviving the transplant.
Well that's me done for now - if I can help anyone out please let me know if you want more information.
