5 year old with hemophilia A & Sarcoidosis. Treatment unsuccesful

I have a five year old son (caucasian) that was born with Hemophilia A, eventually developed inhibitors & has been on Immune tolerance  treatment (Alphanate & feiba was recently added) to override the inhibitors for a few months now...He started showing other symptoms (nodules on lungs/inflamation, frequent fevers, enlarged liver & spleen with high liver enzymes & white blood cell count dropping drastically) about two years ago that were NOT hemophilia related about 2 years ago & we were constantly in and out of the hospital for sometimes weeks at a time...most of our visits went as followed:  They would admit him, do a blood culture to check for infection ( he has a port in his chest), started running other blood tests bc they suspected that it may be an inectious disease of some sort but both the cultures & all of the blood tests came out negative/normal... they would also pump him full of antibiotics through IV, I'm assuming for the fever, even though he had no known infection...and eventually his symptoms would start improving & when he was well enough they would send him home...& maybe a month or so later his symptoms would flare up again...this went on for about 13 months until October of last year (2011) they (Children's and Women's hospital in Mobile, AL) sent us to the children's hospital in Birmingham, AL & we were seen by a rheumetologist who after 4 days of tests/studies ended up diagnosing him with Sarcoidosis (autoimmune disease). They started him on 10 ml of prednisone by mouth, .03 ml of methatrexate once a week by mouth/mixed with juice, & zantac. His symptoms seemed to start improving, the next chest x ray showed that the nodules were still there but the inflamation (inflammation) had improved greatly & the fevers were staying away, etc...Once he was tappered off the prednisone & was just getting the methatrexate once a week he ended up starting to have flares again, so they started doing infusions of remicade every two weeks that didnt seem to be helping...they then started talking about the possibility of him having HLH, then back to the sarcoid...they also did an infusion of retuxin (a double dose back to back) & he's also had numerous bone marrow aspirations (the most recent being about 3 weeks ago) to rule out cancer & other things which have all been negative. They did do a lung biopsy last October as well to get a tissue sample of the liver since it had the nodules on it...Since then they have discontinued the methatrexate and the only thing he is getting currently is anakinra infused once a day & has been for the past 3 weeks....not only does it not seem to be helping, but his syptoms (symptoms) have since taken a turn for the worse & he has constant fevers every day (that I'm treating with tylenol) & now he's constantly coughing a real short of breath...I am not understanding why treatment isnt working & the disease is progressing...unless maybe the Sarcoidosis is a misdiagnosis...& if there was a treatment facility that would be better fitting for him to be in that have a better understanding of these types of diseases... I realize it makes it an extremely  difficult situation when you have both hemophilia A with inhibitors AND some sort of autoimmune disease at the same time...but this has been going on too long & am seeking any help I can get to try and get my boy well.

Thank you. ~L.

[margypops]

I have been looking through our list of expert/doctor forums to help you with any advice they may have , I cannot find a forum to cover it. I understand your concern and commend you for trying any and all avenues to help him, all you can do is keep asking get more opinions, research online .Good luck I hope you find some help and answers soon .