Promacta and Cyclosprine?????

[Grifmat]

So my mom is now refractory to the transfusions....so they are starting her on crossmatched or HLA platelet transfusions. All her blood work is normal except for those pesky platelets. Her platelets were at 12 and then she got 2 units which brought her up to 21 but then they gave her 5 units which brought her down to 15...not good. She has been on cyclosporine for just about 2 weeks and now they are starting her on promacta. Anyone had any success with this? I'm feeling very hopeful. She still looks and feels great and has had no spontaneous bleeding or complications. Any info is so appreciated! Thanks.

[Sally C]

Hi Grifmat,
My husband had no response to Cyclosporine but had an unbelievable response to Promacta. Please read about his history and success under "Clinical Trials" and if there are any questions I can try to answer regarding Promacta don't hesitate to ask - either on the forums or my personal e-mail - shcalvert3@aol.com.
I wish you amd Mom well.
God Bless,
Sally

[Grifmat]

Sally,

Thank you so much. I'm so glad that your husband had such a good response to the promacta. My mom is very new to this disease. It's only been 4 weeks and she is already rejecting the platelet transfusions. It was so scary. I will definately be in touch and I will read your husbands story. Thank you again.

[marmab]

Hi,

I was refractory for platelets right from the get go (first transfusion) 18 months ago and thus have received HLA matched platelets since then. I get a good bump in my Plt number with every HLA matched transfusion.

Marmab

[Grifmat]

Wow, your first transfusion. It's so scary. If you don't know much about this disease you would wonder...what am I gonna do now? I'm glad you are getting a good bump from the HLA platelets. I know they can bring the counts up higher but do they last longer too? I see that you had a minimal response to the cyclosporine. How are you doing now? I hope you are doing well. It's a very strange and scary disease, although it's manageable. You just never know from one day to the next.

[marmab]

Hi,

Hopefully your mom's problems will be confined to her platelets, and the doctors will figure out what's going on -- perhaps an ITP-like condition? As Hopeful said, the thinking is that there is a strong autoimmune component to AA, hypoMDS, and other similar syndromes, so strategies and treatments take this into account. I responded to your PM separately.

What's happening right now is that, after 6 months of transfusion independence (low-ish but stable counts), I had what the doctors think was some sort of autoimmune "event" that caused my counts, particularly platelets, to tank all of a sudden beginning in late March. So I started needing Plt tx again. I would get a very good bump (70-100 points) after one unit, and then 2 or 3 days later the plts would be down to 2 or 3. After 3 weeks of this, the doctors debated trying ATG again (I truly am an overlap of hypoMDS/AA -- these syndromes are not as clear cut as we would like them to be), but then decided to try prednisone and a 4 week course of Rituxan again. I had my 3rd Rituxan treatment yesterday, and my platelets ARE hanging in there a little better. They were at 37(!), rather than 2 or 3. And my Hgb, which was down in the low 7s early this month, was up to 8.9, the highest it's been in many months, which is great because I am trying to avoid more PRBC transfusions. I have iron overload (ferritin 1598) and have developed many antibodies.

BMT is still lurking over the horizon, though. But trying to determine the tipping point -- when to do it -- is difficult. I am healthy other than this disease, so I am managing, and am very fortunate and thankful for that, but on the other hand, it seems that my marrow is not really recovering with immunosuppressive therapy the way we hoped it would, so new marrow may be the only answer. I just had my 4th BMB, and there has been no improvement seen since my dx almost two years ago. And that weighs in favor of BMT sooner rather than later, especially because I AM "healthy", and not getting any younger. I really fear the substantial risks of BMT, which my doctors understand and respect, but as they remind me, I am having more trouble and problems with this disease as time goes on, and the chances of a good BMT outcome depend partly on one's health going into it. I would love to be able to do some of the physical activities that I used to, if I am so lucky as to have it all work out. I was extremely athletic my whole life, and miss it. I joke that my default setting used to be "active", but with low hgb and plts, my setting is now sadly "inactive"; actually I still do walk, swim, canoe, kayak, etc. and ride a stationary bike, rather than a real one -- anything, really, to keep moving -- when my hgb is high enough.

Sorry to blab on at length -- I'll quit now. Wishing the best to your mom. Keep us updated. This forum is an invaluable resource! Many knowledgeable and caring people here.

Marmab

[Grifmat]

Hey Marmab, I just realized you had posted. I know what you mean by these diseases overlapping and being inconsistant. My mom's situation is literally just her platelets. She hasn't gone below 7 and that was just for about 12 hours. Other than that she has been at 9...that seemed to be her go to number no matter how many tx she had. This past week we've had an 11 and a 14!!! Strange how those numbers would have put you in a state of terror a few months ago but now they seem like the best numbers in the whole wide world. It's nice to hear other's stories...no matter how long they are. It makes me and my mom feel a little less alone. She will be getting the HLA platelets next week and she will be starting on the Promacta. I'm hoping this helps. My mom was also very active...not quite as active as you but she was in very good shape and very healthy..which I do think has helped her situation. Please keep me posted on how you are doing and I wish you all the best.