TOPIC:

DeeDee wrote:

Hi Dr. Bean,

You are doing very well! It might not be a good idea to stop the Cyclosporine A and reduce
the promacta at the same time. Is this what you did before? Maybe try one at a time and see how you respond. Good luck to you! And, hopefully you will not crash this time.

Dee Dee

We removed Cyclosporine A and remain promacta last year, but the platelet count crashed after 1.5 months.

My platelet count as at 11 Apr 2015 - 205

As my request, the medical treatment remain unchanged

a. Cyclosporine A - 25 mg daily
b. Promacta - 25 mg daily

My platelet count as at 4 Jul 2015 - 215

We found this combination of medicine can make my plate count become stable, so that the treatment remain unchanged

a. Cyclosporine A - 25 mg daily
b. Promacta - 25 mg daily

My platelet count as at 3 Oct 2015 - 155

My doctor said that it may be normal fluctuation, so that the treatment remain unchanged

a. Cyclosporine A - 25 mg daily
b. Promacta - 25 mg daily

My platelet count as at 14 Dec 2015 - 9

My doctor said that it may be due to flu

a. Cyclosporine A - 250 mg daily
b. Promacta - 25 mg daily
c. IVIG - 60 bottles were injected in 5 days

Hi Dr.bean,

Yes, it could be possible that your count went down due to the flu. Until now, you've been
doing fairly well?? Hopefully, you will get those platelets back up soon.

Does your doctor always feel you need the Cyclosporine A? Was just wondering. It would
be nice to just have to take Promacta by itself.

Take care and hope you get those platelets up real soon.

Dee Dee

Hi DeeDee,
Thanks for your reply.

My platelet count raised to 140 after injection 48 bottles of ivig and 4 bags of platelets.

Cyclosporine A is needed for my case, especially when the platelet crashed to single digit. It is because the effect of IVIG only last for a week, but we need another thing to compress the activity of immune system, that is Cyclosporine A.

We have tried different dose of Cyclosporine A, the dose below 250 mg daily was insufficient to make platelet count stable after the effect of ivig.

I have tried to take promacta itself and without Cyclosporine A, but the platelet count crashed after 1.5 months, we will keep on trying in 2016.

Bean when you tool only promacta, without the Cyclosporin A, what did your count go down to?

My platelet count dropped from 256 to 126 in two months after removal of Cyclosporin A, then it dropped from 126 to 6 in two weeks.

Hi Dr. Bean,

Overall, you did do fairly well for the year. It's just too bad that the Promacta doesn't work by
itself. I'm sure your doctor will get back on schedule soon. Being sick probably did bring you back
down. It does happen to some of us.

I know that the IVIG is only a temporary fix--but sometimes it does help your body to heal--at least
it did for me. Good luck in getting those platelets back up and hope you are feeling better soon.

Dee Dee

Although my treatment back to starting point, I find a possible flow that trigger my immune system to attack platelet.

The flow could be:
Dust/ chemical/ smell in vault -> nasal allergy/ symptoms of catching cold -> trigger my immune system to attack the platelet

In the mid of 2012, my first time working in a vault, then I caught cold and my platelet was crashed first time.

This time of crash is similar to the one in 2012, since I didn't enter the vault in last 12 months and my platelet count was stable in around 2xx.

However, I back to the vault frequently since this summer holiday, then my platelet count dropped to 150 in the early Oct 2015.

In the end of Nov, I entered the vault twice, then I feel like catching cold, and it can't completely recover after two weeks. Ultimately, my platelet count crashed last week.

At this moment, I didn't have strong evidence to prove that the environment of vault was the source that trigger my immune system, but I will protect myself.

Dr. Bean, I agree with you. The environment does play an important part in some of our immune systems. The vault could very well be the culprit, especially since your platelets seemed to crash after entering it. Try to protect yourself, if possible. Just alone, the dust and chemicals in the vault could cause some of us to relapse. I'm that way. It can be stress or even a spider or some other bug bite that crashes my platelets.

Good luck and hope you able to get yourself back on track, rest, and get your platelets back up.

Dee Dee

My platelet count as at 23 Jan 2016 - 130

My doctor said that the effect of ivig has gone and it may be a normal fluctuation, so that he reduced the dose of Cyclosporine A and my next blood test is arranged after 5 weeks.

The combination of my treatment:
a. Cyclosporine A - 200 mg daily
b. Eltrombopag - 25 mg daily

Hi Dr. Bean,

It sounds like you are fairly well! 130 is a very good platelet count. Your doctor seems to have
you on a treatment plan that works for you. I hope you are doing well and feeling well too!!

Good luck to you and keep those platelets up!

Dee Dee

Sandi
How can I get in touch with you?? I am having some trouble navigating the website to post a question.
Joanne

What's the problem? You did fine with the anti-depressant question.

My platelet count as at 27 Feb 2016 - 183

The report is looking good this time. My doctor reduced the dose of Cyclosporine A.

The combination of my treatment:
a. Cyclosporine A - 150 mg daily
b. Eltrombopag - 25 mg daily

Hi Dr. Bean,

Really glad to hear from you and so glad that you are doing well!!
Really great news. I am also glad that your doctor was able to reduce
the dose of Cyclosporine !.

Take care,
Dee Dee

I wear a face mask around 8-10 hours per day to minimize the chance of getting infected.

My platelet count as at 16 Apr 2016 - 189

The combination of my treatment:
a. Cyclosporine A - 100 mg daily
b. Eltrombopag - 25 mg daily

Hi Dr. Bean!

With a platelet count of 189, you are doing great! It seems you are presently holding
steady, and I am so glad to hear this. Your doctor seems to have the right treatment for
your case, which is really good.

Thanks for checking in and updating us! And, hoping your platelets stay up there!

Dee Dee

Hi Dr. Bean,

I don't often reply, but I am always glad to see your updates. Looking good!

Dr Bean
Nice count..is the reduction in cyclosporin continuing?

DeeDee wrote:

Hi Dr. Bean!

With a platelet count of 189, you are doing great! It seems you are presently holding
steady, and I am so glad to hear this. Your doctor seems to have the right treatment for
your case, which is really good.

Thanks for checking in and updating us! And, hoping your platelets stay up there!

Dee Dee

Dee Dee, thanks.

I am trying different methods to avoid getting flu.

Rob16 wrote:

Hi Dr. Bean,

I don't often reply, but I am always glad to see your updates. Looking good!

Rob, thank you.

I just want to show all of you that my medical treatment may help ITP patients.

mrsb04 wrote:

Dr Bean
Nice count..is the reduction in cyclosporin continuing?

Thanks.

Cyclosporin-A was reduced from 150 mg daily to 100 mg daily and I will see my doctor again in mid of June 2016.

Dr. Bean,

Thank you for thinking of others! I figured you wanted to keep us posted to let us know that
your treatment plan is working. Thank you again!

My platelet count as at 18 Jun 2016 - 224

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

My platelet count as at 8 Oct 2016 - 168

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

Dr. Bean,

I have a couple of thoughts about your treatment:

• Your platelet counts typically remain much higher than the levels recommended to minimize the risk of blood clots.
• Your eltrombopag dose has not been lowered below 25 mg. per day

Except in times of illness, your counts have remained well above the recommended target count of 50k - increasing your risk of blood clots - as long as your cyclosporine dosage was not dropped too low. One thing that has not been tried is dropping your dosage of eltrombopag below 25 mg per day. The tablets should not be split, but 12.5 mg Promacta tablets are available, at least in the US. You could make the change very gradually, substituting 12.5 mg. tablets once per week at first, then twice per week, and so forth. If you can't get 12.5 mg tablets, you might try dropping a full dose one day per week, then if your next count looks good, drop a second dose. (All of this, of course, after consulting with your doctor!) This might help keep your platelets at safer (lower) levels, and might also be a cost savings (although the 12.5 mg price is roughly the same as the 25 mg. price in the US, if you can skip days that would be a savings).

Another unrelated thought (based purely on conjecture):
You were taking prednisone for a long time - long enough to develop adrenal insufficiency, which could mean that the ability of your adrenal glands to produce cortisol could be impaired. I can imagine how your adrenal glands might produce enough cortisol for daily life, but not enough to deal with times of stress - like THE FLU. You have mentioned at least two cases where you thought that your platelet levels crashed as a response to the flu. People with adrenal insufficiency require additional prednisone at times of stress like with illnesses including the flu. I wonder if taking prednisone when (and only when) hit by stressors like the flu might help protect you against platelet crashes. An ACTH stimulation test would check for adrenal insufficiency.

Of particular interest to me with the case of drbean7218 is that it appears that combining a TPO-RA with cyclosporine allows for a very low dosage of both medicines to be used, while achieving effective control of platelet levels. These lower dosages, one might presume, could result in fewer side effects than with the dosages that might be necessary for monotherapy.

For those interested in the topic of combination therapies (which look very promising!), I am providing links to a couple of articles.

I did not find any studies combining Promacta (eltrombopag) with cyclosporine-A [Dr. Bean, your doctor should really publish a case study!]. I did find this study which combined romiplostim (NPlate) with cyclosporine-A. IVIg was used as needed as a rescue therapy, just as it has been with drbean7218.

www.bloodjournal.org/content/120/21/1095
Cyclosporine-Romiplostim-IVIG Combination Therapy in Refractory ITP
Emily Leven, Allison Miller, Brittany Cooper, James B Bussel
Blood 2012 120:1095;
Conclusions: These results suggest that cyclosporine can be used as part of an effective combination with romiplostim and IVIG to manage patients with difficult ITP. The 3 agents were chosen to target different mechanisms of disease pathobiology: FcR “blockade” (IVIG), stimulation of platelet production (TPO-A) and inhibition of T cell activation (CSA). The efficacy of added CSA suggests that activated T cells contribute to refractoriness in difficult ITP.

Another recent study, reviewed available literature and looked at combination therapies in general for ITP. It was especially impressed by the potential for combinations of TPO-RAs and immunosuppressant therapies.

onlinelibrary.wiley.com/doi/10.1111/jcpt.12421/full
Journal of Clinical Pharmacy and Therapeutics
Combination therapy in relapsed or refractory chronic immune thrombocytopenia: a case report and literature review
Authors: A. Rashidi MD PhD, M. A. Blinder MD First published: 8 July 2016 DOI: 10.1111/jcpt.12421
"Combination immunosuppression can potentiate the effect of TPO-RAs. This mechanistically reasonable strategy could result in a more rapid response than the more popular, sequential, single-agent strategy. Stepwise tapering can be successfully implemented. Comparing sequential single-agent therapy with early combination approach warrants a more extensive study."

My platelet count as at 15 Jul 2017 - 130

The platelet dropped from 143 to 130, it may due to infect with flu last week.

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