Steven-Johnson Syndrome???

Okay what a week and a half it has been. Just when you think things are progressing....The girls, Simon and I spent a long morning at the hospital today. At least the girls and Simon were rewarded with an Easter basket of goodies. The childrens hospitals are great about that. It was a worthwhile trip. We met with Allergy/Immunology doctors and I was very impressed with them. Its been a long time since Children's has had anyone in their immunology dept who said anything that was helpful. They hired a new head of immunology who we will meet next tuesday but today he sent his colleagues. After speaking to them, having simons skin examined and Dr. Mancini (our savior and lead dermatologist) consult they all think the same thing, Simon has erythema multiforme major. Basically one step away from Steven-Johnson syndrome, which is a very serious disease caused by a drug reaction. Since the only timing we could link his outbreaks to is the IVIG infusions we are concerned this is the culprit. Since his reaction has gotton worse over the last two months right after IVIG they are contemplating stopping those infusions. This brings up great concern for us all. IVIG is given because immune suppressed people cannot make their own and it helps to keep his immune system fighting against infection. Their thinking is that since simon has sores in his mouth and other areas it could affect his organs next time and that could be very serious maybe even fatal. So we are at a hard decision. Everyone is meeting, including our faithful Dr. Uzell in Washington, and they will discuss and come up with a plan. We have about 3 weeks before he is due any infusions. They are also tapering cyclosporine again in hope of allowing Marrianas immune system to start producing some IGG. Believe me I knew nothing about the immune system before simon and to look at counts and either get happy or worried is something I dread. We all walk around getting colds and things and don't even think of how our systems work to fight them off but when confronted with a situation like this every number sets the mood. Today our platelets are down. My stem cell drs say "don't worry you have bigger things to worry about right now" but my heart gets scared as these are the core of watching the immune system rebuild and Simons were great last week and down today. So we go back on Tuesday. Right now our biggest worries are that he will not drink or eat due to sores in his mouth and possibly esophogus and he is hooked up to his feeding tube which we will increase and lengthen tonight to keep him hydrated, otherwise his skin will get worse. He needs to have lotions applied 4-5 times a day and we pray all the blisters evaporate back into his skin and skip the popping otherwise we are open to more infections. So please, pray for these things along with the vntrs we drew today to be 99%+. I'll keep you all posted. Thanks for hanging on with us. Please pray for full recovery and no recurrent outbreaks along with if stopping IVIG there will be no side affects of infection. Thanks and God Bless. Tracy