TOPIC:

Thank you so much for your reply.

My doctors don't have any plans to drop the eltrombopag dose below 25 mg per day, since my platelet was out of normal range in last two blood test. At this moment, everything remain unchanged will be the best option.

On the other hand, I am trying to change my lifestyle not to make me feeling stress.

My doctor Dr. Raymond Wong and others has done an research on using eltrombopag for ITP patients, but not for my case.
journals.sagepub.com/doi/full/10.1177/2040620717693573

My treatment is doing in two ways:
1. Boost bone marrow to produce more platelets
2. Compress strength of immune system and try to minimize the system wrongly attack the platelets.

My doctors concluded that the possible cause of my case was immune system disorder, which is wrongly attacking the platelets, rather than insufficient production of platelets.

That's interesting. Questions for you: 1. How did they determine that your ITP is due to destruction only and 2. Why are they using TPO's for you to boost production if you only have destruction going on?

Thank you for your questions.

1. My doctors performed different body checks on me in 20 days, they tried to sort out the most possible cause of ITP in my case, but the result of different tests were normal, even my spleen and bone marrow. Therefore, my doctors concluded that the most possible cause of my case was due to flu and made the immune system disorder.

2. I have tried different medicines to compress the strength of the system, but most of them even prednisolone have no impact on me. Cyclosporine A is the one have impact on me, but it can't stand alone at the minimum dosage in my case.

My doctor suggested me performing splenectomy, but the successful rate is 66%.

drbean, I was reading through your thread. Perhaps I missed it. Did your doctor ever try Cyclosporine without Promacta? And if not, why? Too many side effects with higher doses of Cyclosporine?

My doctor didn't try Cyclosporine without Promacta.

Since my platelet count didn't back to normal range, that didn't convince my doctors to reduce the doses of Cyclosporine, then promacta afterwards.

At the same time, my doctors didn't want to take risk which may make my platelet crashed again, and the whole treatment back to starting point with high doses of Cyclosporine and 5x-6x bottles of ivig.

drbean7218 wrote:

... At the same time, my doctors didn't want to take risk which may make my platelet crashed again, and the whole treatment back to starting point with high doses of Cyclosporine and 5x-6x bottles of ivig.

drbean, I find your case, as I understand it, extraordinary.
There seems to be a class/set of ITP folks who have no steroid response and a weak IVIG response (eg 49). You appear to fall into that group. From PDSA member reports on this forum, many of those in that group have found relief with higher dose NPlate/Promacta, or, regular IVIG treatments when/if NPlate fails. But your doctor seems to have found another alternative - the Cyclosporine and low dose (25 mg) Promacta combination. Nice!

The bad thing is that the combination doesn't seem to lead to remission. Tapering off Cyclosporine is leading to collapsed counts for you. There doesn't seem to be a way to get the immune system to unlearn antibodies against the Thrombopoietin Receptor .

Has your doctor considered going to 50 to 75 mg of Promacta only?

drbean7218 wrote:

My doctor didn't try Cyclosporine without Promacta.
Since my platelet count didn't back to normal range, that didn't convince my doctors to reduce the doses of Cyclosporine, then promacta afterwards.

But counts shouldn't be in the normal range with Promacta. The dose should be adjusted to keep the count around 50k.

I have done another blood test on 7 Aug 2017. No of platelets is 194

My platelet count as at 9 Aug 2017 - 194

Unfortunately, I was hospitalized again due to Hemoglobin dropped to 6.6. My doctor said that they find antibodies in my red blood cells.

The combination of my treatment:
a. Cyclosporine A - 50 mg daily
b. Eltrombopag - 25 mg daily
c. Prednisone - 50 mg daily

With a count of 194 can't you start reducing both Prednisolone and cyclosporin as side effects of long term use are pretty grim?
Promacta could be increased if platelet count drops below the recommended target count of 50.

Prednisolone was included yesterday for boosting Hemoglobin.

My doctor said the reason of high platelet was due the effect of cyc-a and promacta. He didnt have intention to reduce the dose this time.

Is the cause of your low Hb known? Cyclosporin can affect kidney function which in turn will affect the body's own Epo production.

The results of different tests are fine including the function of liver and kidney.

My doctors attributes to the extension attack by the immune system based on the foundings of antibodies in the red blood cells.

drbean, have you ever tried MMF (Cellcept) before? Using MMF instead of Prednisone might 1) be an effective ITP remission agent when combined with cyclosporine, 2) allow for a lower cyclosporine dose to maintain counts. Isn't your ITP refractory to steroids?

Combination immunosuppressant therapy for patients with chronic refractory immune thrombocytopenic purpura
www.bloodjournal.org/content/115/1/29.full?sso-checked=true
"
The rationale for combination immunosuppressants is to target multiple pathways to inhibit the pathologic platelet autoantibody with minimal overlapping toxicities. In that way, lower doses can be used.
"

I haven't tried MMF (Cellcept) before. We have tried Prednisone for ITP before, but it seems that no impact on me. This time we used Prednisone for low hb.

Just a quick update of combination of my treatment after discharge

a. Cyclosporine A - 50 mg daily
b. Eltrombopag - 25 mg daily
c. Prednisone - 15 mg daily
d. Folic acid - 5 mg daily
e. Famotidine - 20 mg daily

I tried MMF, 3 dose increases, was on the maximum dose for 3 months & I only achieved one count above 33. It made me very depressed indeed to the point of consultant offering to add in Prozac in an attempt to cheer me up. I gave up the MMF instead.

My platelet count as at 2 Sep 2017 - 216

Hemoglobin rose to 12.3.

My doctor said that my CT scan and bone marrow biospy were normal. He diagnosed of Autoimmune Hemolytic Anemia (AIHA) with unknown reason of the immune system disorder.

The combination of my treatment:
a. Cyclosporine A - 50 mg daily
b. Eltrombopag - 25 mg daily
c. Prednisone - 12.5 mg daily
d. Folic acid - 5 mg daily
e. Famotidine - 20 mg daily

My platelet count as at 23 Sep 2017 - 200

Hemoglobin rose to 13.9.

I will go to UK in the mid-Oct for two weeks, so that my doctor suggest the treatment remain unchanged.

The combination of my treatment:
a. Cyclosporine A - 50 mg daily
b. Eltrombopag - 25 mg daily
c. Prednisone - 12.5 mg daily
d. Folic acid - 5 mg daily
e. Famotidine - 20 mg daily

My platelet count as at 11 Nov 2017 - 195

Hemoglobin rose to 14.5.

The combination of my treatment:
a. Cyclosporine A - 50 mg daily
b. Eltrombopag - 25 mg daily
c. Prednisone - 10 mg daily (reduced 2.5 mg)
d. Folic acid - 5 mg daily
e. Famotidine - 20 mg daily

My platelet count as at 27 Dec 2017 - 192. Hemoglobin is stable at 14.X

The combination of my treatment:
a. Cyclosporine A - 50 mg daily
b. Eltrombopag - 25 mg daily
c. Prednisone - 7.5 mg daily (reduced 2.5 mg)
d. Folic acid - 5 mg daily
e. Famotidine - 20 mg daily

My platelet count as at 24 Feb 2018 - 215. Hemoglobin is stable at around 15.

The combination of my treatment:
a. Cyclosporine A - 50 mg daily
b. Eltrombopag - 25 mg daily
c. Prednisone - 5 mg daily (reduced 2.5 mg)
d. Folic acid - 5 mg daily
e. Famotidine - 20 mg daily

My platelet count as at 24 Mar 2018 - 8.

My doctor didn't find any possible causes related to the platelet count crashed. I have stayed at hospital for 5 days and injected 5 round ivig. My platelet count increased to 156 after 4 rounds of ivig.

The combination of my treatment:
a. Cyclosporine A - 50 mg daily
b. Eltrombopag - 25 mg daily
c. Prednisone - 10 mg daily (increased 5 mg)
d. Folic acid - 5 mg daily
e. Famotidine - 20 mg daily

My platelet count as at 6 Apr 2018 - 36.

After injection of 5 rounds of ivig, my platelet count dropped from 156 to 36 after a week, I was back to hospital again. However, my doctor didn't arrange any treatments for me this time. He only increased the dosage of Cyclosporine-A, then I was dispatched yesterday.

The combination of my treatment:
a. Cyclosporine A - 200 mg daily (increased 150 mg)
b. Eltrombopag - 25 mg daily
c. Prednisone - 10 mg daily
d. Folic acid - 5 mg daily
e. Famotidine - 20 mg daily

drbean7218 wrote:

My platelet count as at 6 Apr 2018 - 36.

After injection of 5 rounds of ivig, my platelet count dropped from 156 to 36 after a week, I was back to hospital again. However, my doctor didn't arrange any treatments for me this time. He only increased the dosage of Cyclosporine-A, then I was dispatched yesterday.

The combination of my treatment:
a. Cyclosporine A - 200 mg daily (increased 150 mg)
b. Eltrombopag - 25 mg daily
c. Prednisone - 10 mg daily
d. Folic acid - 5 mg daily
e. Famotidine - 20 mg daily

IVIg is definitely temporary, so it's not a surprise that your counts dropped back down after that treatment. It looks like you are on 3 different treatments for the ITP. You are on two immunosuppressants (Cyclosporine and Prednisone) and a TPO-RA - Eltrombopag. That is a lot of simultaneous treatment. Do you know which of those three treatments are actually doing anything for you. Prior to your last crash, it looks like your counts were quite high (higher than they should have been while on a TPO-RA like Eltrombopag). Do you think it was some kind of a virus or infection that triggered your crash? Whenever I get a crash I try to wait it out as long as I don't have active bleeding and most of the time my counts will rebound after some time passes.

I would want to figure out which of your treatments are actually working if I were you. Having 3 treatments going simultaneously is a lot of side effects that may just not be necessary. Regardless, a count of 36 is just fine, so hopefully you can keep the doctor from going crazy increasing everything until you get some kind of a trend going.

Dr. Bean, I, too, had my platelets crash on Feb. 27. They went down to 3 and stayed there for a few days. I've been in remission for 7 1/2 years. I had a really bad year in 2017 and stress (from the hill behind our home eroding/falling from rains) as we just recently moved from Calif. to Arizona. I sort of knew they were going crash and was not surprised when they did. I was in the hospital for 5 days (had to drive from Arizona to Calif. since I didn't have a new doctor). My doctor did four IVIGs on a slow drip and I also did three Decadron pulses. The Decadron pulses made me feel worse than my platelets falling. Since then my platelets went up to 213 and I'm doing good. I'm like you "mom"; I need a little time and then they start to go back up. But I did get a virus the day they fell--so that was the trigger this time. My doctor wanted me to start on Promacta to help bring them back up. But, the cost of Promacta would cost me at least $10,000 a years as my co-payment. That's too much for me. I'm more for a conservative treatment. In 2010 when they fell, they stayed at 2 for almost 6 weeks--then started going back up. Good luck to you and try to be patient.

My platelet count as at 20 Apr 2018 - 5.

I was back to hospital again with hematuria. My doctor decided to use 5 rounds of ivig injection again and suggest me performing splenectomy.

I have read different articles, papers and other members' sharing, splenectomy isn't an favorable option. I am trying to think about the platelet and Hb crashed before may be due to stress & emotion.

The combination of my treatment:
a. Cyclosporine A - 200 mg daily
b. Eltrombopag - 25 mg daily
c. Prednisone - 10 mg daily
d. Folic acid - 5 mg daily
e. Famotidine - 20 mg daily

I agree with you Dr. Bean. I know the stress caused mine to fall. I will never let them do a splenectomy on me since I am in my 60's and remission rate isn't very good. Also, I am prone to blood infections when I get run down. So, you need to really think about it. My new doctor has a patient who's platelets never go above 5 and she is still doing good. She's tried every treatment around (not sure what happens to our body at this point). Good luck to you and please take good care of yourself. This is what helps me to heal when my platelets crash.

Dee Dee

drbean, I've always been impressed with your doctor for coming up with what was a low dose combination of drugs to keep your counts up. Now, I wonder that your doctor is mentioning splenectomy because he is trying to get you off prednisone, and has been unable to.

I've mentioned it before. MMF might be good. Or even Danazol if your liver is tolerant to it. Danazol is the safer of the two as far as I know. I wonder if either of those could replace two of the drugs your taking: Promacta and Prednisone.

In any case, good luck with it.

It may well be due to stress. It sends my counts down.
Your adrenal glands are probably not producing any cortisol to help you because of your steroids.
Maybe up your pred dose for a few days to see if that brings your count up. I've found that works for me. Then I drop the pred back to where I was.

I'm not parting with my spleen. Like Dee Dee Marie I'm over 60 so not risking it.