Quote:
Originally Posted by mola-tecta
I have spent a lot of time browsing the past threads on this forum and they have helped me understand a great deal more. I know they aren't very active but I see a few of you still floating around here, so I wanted to post.
I'll try to be short about it - my mom (69yo) had increasing weakness and bruising until ending up in the ER with Hgb of 4.6, platelets of 4, WBC 3.2, ANC 1, ALC 2; she spent a week in the hospital where they did a bone marrow biopsy and a million other tests to figure out the cause.
July 12th she was diagnosed with aplastic anemia, with bone marrow cellularity of 20%, reticulocytes between 0.02-0.03 (range 0.018 - 0.158 x10E+12/L), very tiny PNH clone (<1%) and started horse ATG on July 14th in combination with cyclosporine + Promacta. Had almost no issues with the ATG treatment but became refractory to platelets pretty quickly and thankfully HLA matched ones were found. She was released once they found the appropriate dosing for the cyclosporine to not be at toxic levels. Her doctor does not seem to specialize in AA but is at a premiere blood cancer center that I know sees a good number of AA patients.
Fast forward to today - my mom is still going twice a week for blood draws and is getting platelets about every 10-11 days (down from every 7 days) and blood transfusions about every 3-4 weeks. Her WBC have responded really well (ANC 2.4, ALC 2) but so far the lack of meaningful response for Hgb and platelets has concerned her doctor. I was under the impression that a response at this point would be considered fairly early but the doctor seems to be considering a very strict 6 month timeline to respond to the ATG or different measures will be taken.
Today's (Nov 22nd 2021) counts:
Hgb 9.4 (last received 1 unit blood 11/11), platelets 35 (last received 1 unit 11/18) ANC 2.4, ALC 2, Retics 0.54
She is currently taking 125mg x2 a day of Cyclosporine, 150mg Promacta, 20mg prednisone, along with acyclovir, levofloxacin, fluconazole, B12, Folic Acid, Vitamin D3/Calcium, Famotidine and metoprolol (for heart palpitations). She had been getting injections of Aranesp/darbepoetin alfa every other week but has asked to stop as they don't seem to be doing much but making her blood pressure spike incredibly high.
Right now my mom is having a lot of trouble with the side effects of her medications, specifically the cyclosporine and prednisone. I have not read anywhere that prednisone is valuable in the treatment of AA except for preventing serum sickness in the weeks during and after ATG but her doctor seems hell-bent on keeping her on it (and has tried to raise it) despite the side effects she is having, such as facial swelling, retaining water, muscle weakness, etc. She also is having trouble with tremors from the cyclosporine althought the first few months did not have this issue until her doctor tried to raise the prednisone.
I was hoping to know if anyone else was kept on prednisone for an extended time and how it impacting them, as well as if anyone has had any luck with treating cyclosporine-induced tremors beyond lowering the dosage. Both of these medications are making it very difficult for her to do things although she can mostly complete her ADLs with a great deal of effort.
Besides that, her doctor's adherence to a strict 6 month timeline for the response has me worried, as I've read it can take longer for some people to have meaningful response (especially if they are older) and have been told this disease is one of patience so it's making me feel like the clock is ticking away for something to happen and there is nothing I can do about it.
Thanks for reading if you are out there.
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Hi mola-tecta,
I am sorry to read of your mother's diagnosis. You are correct that prednisone is only used for serum sickness during ATG in the standard treatment of Aplastic Anemia. It MUST be tapered gradually.
Prednisone causes a false rise in WBC/ANC. So, do not be surprised to see those numbers fall once she is off the drug.
I think you should search out a specialist in AA in your area and set up an urgent consult with that person. There are many nuances to treating the disease so you want a doctor that has successfully treated many patients.
Your mother has a small PNH clone, which is a good sign that her aplastic anemia is immune mediated, but more medication isn't always the answer with this disease.
Here is an article from Dr. Young, an expert in the treatment of AA in the US.
https://ashpublications.org/blood/ar...plastic-anemia
Note the paragraph titled "What not to do" where it mentions that corticosteroids (prednisone) should not be used in the treatment of the disease.
Don't delay in seeking out an expert for a second opinion so that you can sort out her medications and dosages. Good luck!