3rd crash in 2 months

My 19 year old son was diagnosed with ITP in Sept
Platelet count was 14000.

Did IVIG for 4 days and count rose to 1000000
But within 2 weeks came crashing to 5000.

Did methylphenidate and dexa for 4 days and counts were 176000.
However in 2 weeks came crashing down to 1000.

Next treatment was Revolade and Prednisone tabs
Counts climbed to 245000
Dr advised tapering of revolade and prednisone.

Sadly in 2 weeks counts are back to 1000

Now DR is advising
Prednisone 40mg
+
Revoladesign 50mg
+
Mycophenolate 1000mg

Need advice if this entire cockatail of drugs are to be had or not?

Pl suggest

Badami

It would probably be better to ask your doctor why each of the three drugs are needed - presumably at the same time.

I'd be looking for a new doctor

I would be questioning the doctor as to why he is doing multiple treatments at once? how does he know which meds are working and which meds are not?? Some medications produce short term results and some long term results. Some medications should be started at the lowest dose and some depend on weight.


Examples: IVIG works in the short term so when the counts went up and then crashed. The drug did its' job. That should of brought the question out do we do IVIG every couple of weeks?

Revolade is a drug designed to bring counts up out of danger zone to about 50,000 and stay there. I believe it is one that you start out low dose and go up from there. If the count gets too high you risk cloting. Prednisone is design to bring counts up to normal range. The dose is decided by weight and then weened off slowly. Many people find their numbers drop as they drop the drug. To me it works if you ween off of pred and your counts stay up.

I have a question/ need to clarify did you mean Dex as in dexamethasone and methylphenidate?? Because Dexamethasone is a steriod used for treatment of ITP, but methylphenidate is used for treatment of ADHD it is not a drug used for ITP. Methylphenidate is given to children with ADHD to help them stay focused and concentrate.


I strongly suggest you scroll to the top of the page and click the link for information about ITP and treatments. Please research as much as possible. Then discuss between you and your son what plan of action you two want to take.

You mention numbers but no symptoms what are his symptoms???
Decisions should be based on symptoms, numbers, life style, medication side effects (short & long term). Please never blindly follow a doctor do research all options. Make your choices informed choices.

I also agree with mrsb04 a new doctor is probably needed this doctor does not sound like he knows anything about ITP and the treatments that are used.

Thanks guys so much for the info.
I had a typo error in the message sent earlier.
He was on methylprednisolone for 4 days.and followed by 4 days of dexameth.

I'm also very sceptical giving a cocktail of drugs.
My son and I are deciding to go ahead with revolade 50mg and prednisone 40mg (slowly taper prednisone after 3 weeks or so)
We shall question the doc on his logic of using mycophenolate.



Thanks again for your inputs
Really appreciate this

Badami

I agree with the others and have said as much in my e-mails to you. Sorry I didn't get back to you. I have way too much going on this week. My son is getting married on Saturday and my mom is in assisted living and needs a lot of my time right now. I am hardly ever home and trying to juggle 20 things right now.

Anyway, I agree that a new doctor might be a good idea. This one seems to be combining meds that do not need to be used together. Revolade alone might be enough to do the trick if the doctor doses it correctly. Some doctors over-treat and there is no need for that. It places unnecessary side effects on the patient and also, you have no idea which treatment is working.

Thanks Sandi
Will consult for a 2nd opinion.

Congrats on your sons marriage.
God bless your family

Visited the haematologist today who insisted all the 3 medicines
Prednisone 40mg
Revolade 50mg
Mycophenolate 1000mg

He says that the platelets need to go up first and his plans to subsequently ween off prednisone by 5mg every week.

Count today was only 5000.
That too after 48 hours of the medicine cocktail.

I'm trying for a second opinion but here in Mumbai it's difficult to get another doc who is experienced with ITP

Monday we go again for a cbc!!!

Curious. You mentioned Revolade tapering. Was the taper from 50mg to 25 (which failed) and now back to 50 ?

The last time revolade was weaned off from 50 mg daily to 50mg for 5 days a week then to 50mg thrice a week. Then came the crash!!!

This time his plan is to only wean off prednisone later(once platelets counts come up and maintain ) by about 5mg a week

Ah, ok. From what I've read it is a bad idea to skip days with Revolade. Bad in the sense that counts can crash - which appears to be what happened.

Sounds like the doc has a better plan going forward. Good luck...

pdsa.org/forum-sp-534/7-treatment-general/29026-my-promacta-journey.html?limitstart=0
pdsa.org/forum-sp-534/5-newly-diagnosed-a-frequently-asked-questions/28574-my-journey-crazy-emily.html?start=270

Badami

Have a look at the above threads they might be helpful

Thanks so much for those threads.
Really helpful.

We go in for a cbc on Monday
Will post the outcome.

I am often very skeptical of ITP doctors, but I believe that your doctor might be following a rational - albeit unconventional - strategy for treating your ITP. An article from highly respected practitioners indicates that they also use combination therapies:

www.bloodjournal.org/content/128/12/1547.full#T2
Adam Cuker and Cindy E. Neunert Blood 2016 128:1547-1554; doi:10.1182/blood-2016-03-603365
How I treat refractory immune thrombocytopenia
..... We use a tiered approach to treat patients who require therapy to increase the platelet count. Tier 1 options (rituximab, thrombopoietin receptor agonists, low-dose corticosteroids) have a relatively favorable therapeutic index. We exhaust all Tier 1 options before proceeding to Tier 2, which comprises a host of immunosuppressive agents with relatively lower response rates and/or greater toxicity. We often prescribe Tier 2 drugs not alone but in combination with a Tier 1 or a second Tier 2 drug with a different mechanism of action. ...

It is important to note that the authors recommend combination therapies only when single treatments have failed. The following articles are also targeted at refractory cases:

onlinelibrary.wiley.com/doi/10.1111/jcpt.12421/full
Rashidi, A. and Blinder, M. A. (2016), J Clin Pharm Ther, 41: 453–458. doi:10.1111/jcpt.12421
Combination therapy in relapsed or refractory chronic immune thrombocytopenia: a case report and literature review.
"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."

Also similar:

www.bloodjournal.org/content/120/21/1095
Cyclosporine-Romiplostim-IVIG Combination Therapy in Refractory ITP

The following link is to a long thread pertaining to a group member in Hong Kong who goes by drbean7218.

pdsa.org/forum-sp-534/6-general-itp-discussion/27398-8-oct-2016-my-platelet-count-is-168.html?start=60#34306

Drbean had a particularly difficult case of ITP. He ended up being treated fairly successfully with a combination therapy much like yours. He is taking eltrombopag (Promacta or Revolade) and cyclosporine A (known internationally as ciclosporin). Cyclosporine and mycophenolate are both immunosuppressants, but act somewhat differently, so his treatment is similar to yours..

One benefit of using combination therapies is that they might lessen the dose required for the more expensive drugs, like Revolade. Also, using alternate day treatment strategies (example: using 50 mg every other day rather than 25 mg every day) can result in less treatment cost.

Hal9000 wrote: ... From what I've read it is a bad idea to skip days with Revolade. Bad in the sense that counts can crash - which appears to be what happened....

HAL,
On this site we (myself included) have recommended skipping or alternating doses of eltrombopag in order to fine tune the platelet counts to a safe level near 50k. In particular, we spent a lot of time on that with EmilyK and momto3boys.
I cannot find any recommendations not to skip days (as part of a dosing regimen). Do you remember where you read that?

I can agree that cocktails are sometimes needed, but generally after it has been concluded that one drug alone doesn't work. That is just my opinion and based on my thought that accumulated toxicities and side effects are way too often overlooked. I see that you did state, "It is important to note that the authors recommend combination therapies only when single treatments have failed". Exactly.

As far as the alternate day dosages, that occurred with Emily only after she had been able to get to a low dose and still hit the target count. I don't remember MOM3's doses.

Rob, as I recall, not skipping days recommendation was a comment here on PDSA.

Several weeks ago I did a search on 'Promacta' and read most all of the threads on that topic - which was a lot! I think someone's doctor had said that he didn't want to taper that way as he had bad results with that method - counts crashing. The context may also have been someone's counts had crashed while tapering Promacta and couldn't figure out why.

As Sandi points out, Emily's case is different as she was forced to taper to zero because of high counts.

HAL, I found it:

Sandi wrote: I don't think Promacta would make your counts drop unless you are not taking it properly. Skipping doses could cause counts to drop.

Hi Friends
My sons platelets today are 115000.
This was after the crash last Tuesday.

So now platelets are over 100000
; after 5 days of

50mg of revolade
40mg of prednisone
1000mg of mycophenote

Doc now is planning to slowly taper only prednisone by 10 mg from tomorrow.
Cbc again after a week.

Will keep you posted

Rob16 wrote: HAL, I found it:

Sandi wrote: I don't think Promacta would make your counts drop unless you are not taking it properly. Skipping doses could cause counts to drop.

Okay, wait - to clarify this....yes, skipping pills could cause counts to drop in a person who is not stable. However, for a person who is ready to taper the drug because they are stable, alternate day doses can work to get off of the drug. Of course if counts start to drop though, you'd want to get back on daily doses.

Rob, take a look here...

www.pdsa.org/forum-sp-534/7-treatment-general/27604-counts-with-promacta.html#36424

Just to chime in, I think there is a difference in meaning between "skipping" a dose and titrating to achieve the required counts. Because of the limited available pill dosages, sometimes alternating daily dosages is a way to keep the counts in the target range near 50. Whenever I start getting too high (above 80, but certainly 100 is too high) I decrease my dosage very gradually. First one day a week at the new, lower dosage with all remaining days staying the same. If the counts are still too high, then 2x a week at the lower dosage, etc.

Currently I'm at 75mg daily because I've been fighting some viruses, so I won't change anything unless my counts creep into that "too high" danger zone. If they do, I'll gradually titrate down as needed.

My hematologist recommends this method of adjusting the medication and there are no contraindications that I know of in "skipping a pill" in the sense of titrating a dosage down because counts are too high.

momto3boys, very well explained!

As an aside, we have seen where doctors have skipped a weekly injection of Nplate, resulting in plummeting platelet counts.

Okay, we all agree. N-Plate is definitely another matter and another discussion.

Wait - an add to what I wrote above. If a person is on Promacta and other meds and counts get too high, that is again, another matter. It can be messy when a person is newly diagnosed and they have too many meds thrown at them. You don't know which one worked so you can't possibly know which one to taper or how fast to do it. Tricky situation which could result in a crash.

NOW ONLINE
Posts: 15
We have been advised a cbc next week.

Present count is 115000 and medication is
50mg revolade
40mg prednisone
1000mg mycophenolate

We have discussed with doc on tapering prednisone by 5-10 mg per week.

Quick question
If the count remains UP next week
Do we also reduce the dosage of revolade along with the tapering of prednisone? ??

To clarify the situation for someone else to answer.

Is it correct to say that the 245 count he had (original post) was after two weeks on 50mg Revolade?
Similarly, is the current count of 115 after being back on 50mg dose for one week?

I'd get off the steroids first if it was me

Badami wrote: NOW ONLINE
Present count is 115000 and medication is
50mg revolade
40mg prednisone
1000mg mycophenolate

We have discussed with doc on tapering prednisone by 5-10 mg per week.

Quick question
If the count remains UP next week
Do we also reduce the dosage of revolade along with the tapering of prednisone? ??

Regarding the Revolade, the doctor should be concerned that the count is that far above the required count of 50.

I don't know why they are doing such a high dosage of Prednisone along with the Revolade because it seems like that would make it more difficult to get an idea of how much Revolade is needed to achieve counts in the 50s.

I would be pushing that doctor to taper off of the Prednisone as quickly as possible if it were me (I would not take no for an answer). I get VERY nervous if my counts ever go above 100 on Revolade because of the increased risk for clotting and strokes.

As you decrease the steroid you will be able to more accurately gauge whether the Revolade is helping with the counts or not, and then you can figure out what dosage of that medication is needed to get a count near 50 consistently.

Good luck, and keep us posted!

Sandi wrote: Wait - an add to what I wrote above. If a person is on Promacta and other meds and counts get too high, that is again, another matter. It can be messy when a person is newly diagnosed and they have too many meds thrown at them. You don't know which one worked so you can't possibly know which one to taper or how fast to do it. Tricky situation which could result in a crash.

I definitely agree with Sandi in this scenario Badami. There are too many medications being administered at one time, and it isn't clear which ones are having an effect.