aa and pnh

[3blankets]

Hi, I have posted here before but not in a while. I have had aa for 5 years and about a year or so ago started to show that I had the pnh clone. It is now up to 40% and I started soliris 2 months ago. I was on 100 mg of cyclosporine a day before the soliris and my counts were low but stable and generally I felt pretty good. Since I have started soliris my counts have gone down and I have needed transfusions. Luckily my ldh is normal now thanks to the soliris but I am pretty certain at this point my blood counts are not going to get any better, and will be needing more transfusions. So can I ask if there are others out there in the same boat as me, what kind of treatment has been successful for you? My doctor does seem to think that maybe the reason my counts were more stable before the soliris is because I had so many pnh cells, and I might possibly start going three weeks between soliris treatments to balance things out. any thoughts or advice would be appreciated. Thanks

[Relentless Against SAA]

Hi,
I am so sorry to hear that you are going thru this difficult time. I recently watched an excellent seminar on the Aplastic Anemia MDS Foundation website regarding PNH and Soliris. The doctor explained that the problem with PNH cells is they lack protection from Compliment which is a part of our immune system. Soliris stops Compliment therefore your PNH cells survive. This is why your LDH is decreasing- your Red Blood Cells are not being broken down as fast as before the Soliris. Your PNH cell number should actually be increasing and your Clone size increasing ( therefore increasing your RBC Count) because these cells are not being attacked by the Compliment part of your immune system. This is also why it is tricky to ever come off Soliris, because your Compliment would kick back in and your RBC be destroyed, lowering your counts to dangerous levels. Please question your Dr about this again. I am wondering if you did not understand his explanation. Is your Dr. a PNH specialist??? If not, I would strongly urge you to see one! This is a tricky diagnosis, as you know. Hardly any doctors have very much experience but it is well worth your time in finding one. I hope this helps and please know that I am thinking about you in this difficult time.

[3blankets]

Thanks for responding. I must have missed something in my conversation with my hematologist, because my post really doesn't make sense!! I am just so overwhelmed with all of this. Sometimes I feel like I cannot absorb everything I hear in my appointments. I meet with him again next week, so hopefully I will get some answers. This disease is so frustrating!

[Relentless Against SAA]

Erin,
I understand the difficulty absorbing everything the doctors have to say. Is there anyone who can go with you to the office visits? I try to always take someone. It seems I never hear all the information or understand it the same way my husband does. Please let me know what your Dr has to say at the next visit.