What Are My Options? One Month from Dx

Hi Everyone,

Thanks in advance for your help. I am currently on the downhill side of my third round of high dose dexamethasone.

My short version story: I had a "chest cold". About a week into it I felt worse like I had influenza (was vaccinated against this year's 'flu six weeks before ITP dx). Started having some bruising, had a CBC done b/c I thought I was becoming anemic from becoming vegetarian three months prior (8/16). Platelets of 5,000, saw GP, sent to ER, platelets then less than 2 K and hematologist really didn't see many plts. on the smear. Spleen, etc normal on US and comprehensive labs all negative.

Hospitalized and given steroids and platelets increased to 8K, then over 100,000 K before I went home. However, platelets back down to 15 K about a week later. IVIG given, platelets about 115K, then dropped again but not below 66 K. Between the 15 K of 3 weeks ago and now, I have done two high dose four day courses of 40 mg dexamethasone daily. Waiting to see how my numbers are this week off the steroid. Doc mentioned may do fourth course if necessary.

Last week, my hematologist wanted to talk about what if my numbers don't stay up. Basically the options given were splenectomy and rituxin. I am mid 40s, so splenectomy isn't usually as successful, from what I have read. I am not yet ready to think about rituxin.

So, are there other options at this point? A slow taper of steroids, IVIG if needed? Anything else? I am not finding information that doing splenectomy or rituxin any sooner is any better or am I missing something?

Thank you.

The first thing you should know is that counts do not have to be normal. ANything above 30k is considered to be safe, so if you can maintain that, treatment isn't necessary. Some doctors over-treat and are only happy with counts above normal range, but that can cause unnecessary side effects from treatments that are not needed.

IVIG is usually always temporary. It can be a good treatment to get counts up in a pinch, but isn't a good long-term plan.

For now, I think I'd keep going with the Dex since you've already started it and are having good results. Remissions sometimes occur with the Dex protocol. There are several options to consider next, such as Rituxan, N-Plate or Promacta.

My wife, Ellen, had Rituxan with dexamethasone about 28 months ago. Since then she has not had normal counts, but her counts have stayed "safe enough" to avoid having to treat this whole time... until this week. Her counts fell to 14k, and now she is in the middle of a dexamethasone treatment, which she really hates. We hope this treatment will last, and other treatment will be unnecessary for a good while. 28 months was a nice "partial remission".

For those who can stand it, multiple courses of high dose dexamethasone is very often effective in achieving at least a partial remission, like Ellen has had. I hope it works as well for you.

Sorry to hear that, Rob. If Dex doesn't work, maybe she can become our Dapsone Poster Woman. What else would she consider? Rituxan again?

I think if you tell your doctor what you told us about a splenectomy, he will stop suggesting it. It seems that the insurance companies want doctors to push for that because of its relatively low cost compared to other ITP treatment drugs (except steroids).

Curious. Is your doctor suspecting your ITP was caused by the flu vaccine, or maybe the flu itself ?

Hard to say, Sandi. We aren't really thinking that far ahead, as we are very hopeful that the dex pulse will last awhile. We aren't totally sure whether it was the Rituxan or the dex that gave her the partial remission before, since she did both simultaneously.

Other strange symptoms --- perhaps related --- I will post separately...

I'm sorry to hear about your wife's low count Rob. I hope she can get back in the safe range quickly with minimal treatments! Stinkin' ITP just loves to keep comin' back for some of us...

Thank you for your kind thoughts.

Right now we are much more worried about another symptom coincidental to a drop in her counts. Ellen went to her internist regarding swelling in her left cheek, and that is when she learned of her low counts. The swelling is most consistent with a recurrence of a benign parotid tumor removed thirty years ago with a complete parotidectomy. Parotid tumors can (rarely) recur after very long periods of time, as cellular residue from the tumor can lie dormant. This also would explain the droopiness over her left eye (that we thought might be myasthenia gravis), because the nerve controlling that muscle runs through the lower right cheek where the swelling is. Ellen's droopy eyelid roughly coincides with her ITP. Also, rarely, ITP can be triggered by a tumor. Weird.

The doctor has ordered a CT scan.

Thank you for all your responses. Yes, Sandi, my hope is to stay at 30 K or higher and avoid treatment. My Doc seems ok with that.

I am sorry to hear about your wife's lower numbers, Rob. I hope the Dex is helping her and she is managing the steroid roller coaster easily.

Hal, yes, I told my doc I am not yet interested in splenectomy but agreed to get the pre splenectomy immunizations in case of some emergent need down the road to take it out. My GP and heme docs both think it was virally triggered by the chest cold. The timing makes more sense but as this is an autoimmune disease, I personally think the virus was a trigger but there may be some other issues with my immune system that I am working on with diet, medication (no one thinks my SSRI caused it, but I really wonder) so under my GP's guidance I am weaning down (while coming off steroids- my poor family!). I was also under an enormous amount of stress for over two years prior to this with another increase in stress with one of my kids having an injury (they are good now but high stress for four weeks) six weeks prior to ITP dx. I had started to work on my stress management already prior to all this and am building on that.

Thanks again.

Sorry to hear this Rob. If the current pulse isn't effective, do you suppose removal of tumor's regrowth, and a pulse, would be an option?

BTW, how's your shoulder these days?

I agree wholeheartedly with your refusal to have a splenectomy, but would think carefully about having the vaccinations at the moment.
It is well known that stress affect the immune system.
Which SSRI are you taking?

Rise, gosh you've got a lot going on. Flu, SSRI, vaccination, stress, and even diet.

Do you recall how many days it took (in hospital) to get your counts to 100k from day 1 of the steroids? Was that a Dex pulse they gave you in the hospital?

Also, how many days was it to get your count to 115k from day 1 of the IVIG? Just trying to get a sense if you responded better to steroids or IVIG.

Would it be safe to say that your doctor is thinking counts will go down after this pulse mainly because, with the Hospital pulse (or previous pulse), counts fell quickly?

Just off hand, if you aren't experiencing active bleeding, can't you take your time in deciding what you want to do next? Sometimes I wonder if these hematologists get in a hurry because they are treating a lot of cancer patients.

Rob16 wrote: Thank you for your kind thoughts.

Right now we are much more worried about another symptom coincidental to a drop in her counts. Ellen went to her internist regarding swelling in her left cheek, and that is when she learned of her low counts. The swelling is most consistent with a recurrence of a benign parotid tumor removed thirty years ago with a complete parotidectomy. Parotid tumors can (rarely) recur after very long periods of time, as cellular residue from the tumor can lie dormant. This also would explain the droopiness over her left eye (that we thought might be myasthenia gravis), because the nerve controlling that muscle runs through the lower right cheek where the swelling is. Ellen's droopy eyelid roughly coincides with her ITP. Also, rarely, ITP can be triggered by a tumor. Weird.

The doctor has ordered a CT scan.

No fun! I'll keep her in my thoughts and prayers. Maybe resolving the potential tumor will pop her counts right back to where they were during the remission. There are so many variables that make it so frustrating. I know you'll keep us posted on how things are going!

RiseAbove wrote: Thank you for all your responses. Yes, Sandi, my hope is to stay at 30 K or higher and avoid treatment. My Doc seems ok with that.

I am sorry to hear about your wife's lower numbers, Rob. I hope the Dex is helping her and she is managing the steroid roller coaster easily.

Hal, yes, I told my doc I am not yet interested in splenectomy but agreed to get the pre splenectomy immunizations in case of some emergent need down the road to take it out. My GP and heme docs both think it was virally triggered by the chest cold. The timing makes more sense but as this is an autoimmune disease, I personally think the virus was a trigger but there may be some other issues with my immune system that I am working on with diet, medication (no one thinks my SSRI caused it, but I really wonder) so under my GP's guidance I am weaning down (while coming off steroids- my poor family!). I was also under an enormous amount of stress for over two years prior to this with another increase in stress with one of my kids having an injury (they are good now but high stress for four weeks) six weeks prior to ITP dx. I had started to work on my stress management already prior to all this and am building on that.

Thanks again.

I would be a little bit leery of getting those immunizations as a "just in case." Certainly read up on them and get an idea about how immunizations might "ramp up" your immune system to eat more platelets!

Even if you do decide to go forward with a splenectomy at some point in the future, I don't think that it is ever performed as an "urgent emergency" situation. You would have plenty of time to get the shots before any procedure.

And I'll chime in to agree with everyone that anything above 30,000 is generally safe. Don't shoot for normal

Hang in there with your treatments and good luck with your research. As a failed splenectomy myself (got one done as a child, still fighting the ITP so many years later!), I agree with your refusal of that as a next option. There are lots of options nowadays.

This was posted on the PDSA FB page a few days ago. I think it could certainly apply to adults. Vaccinations have been known to trigger ITP.

www.empr.com/news/case-recurrent-immune-thrombocytopenia-after-flu-shot/article/571758/

Also, flu vaccines can cause the same virus that they are supposed to protect against.

Unfortunately, some people can become infected with a flu virus the flu vaccine is designed to protect against, despite getting vaccinated. Protection provided by flu vaccination can vary widely, based in part on health and age factors of the person getting vaccinated. In general, the flu vaccine works best among healthy younger adults and older children. Some older people and people with certain chronic illnesses may develop less immunity after vaccination. Flu vaccination is not a perfect tool, but it is the best way to protect against flu infection.

www.cdc.gov/flu/about/season/flu-season-2015-2016.htm

Of course, illnesses can also trigger ITP. It could have been a combination of factors.

I agree with the others; there is never an urgent need for a splenectomy with ITP. It is always a choice, not a necessity.

I had already been leaning against getting the vaccines now and decided to wait as my counts were normal this week and I agree, I do not want to ramp up the immune system now.

Yes, I did high dose dex in hospital and it took 3,days to hit 100 K but then fell to 15 K day 5 after finishing first round of steroids. Got IVIG the following day and plts of 115 day 3 post IVIG then round two high dose steroids and on day two on steroids were 211, then 2 days after finishing that course were 290 K. A week later were 66 K and started third course (already planned) of high dose steroid 48 hrs later. Plts 109 on day 2 of steroids, plts 229 3rd day off steroids, then 184 day 6 off steroids. Now waiting for recheck next week to see what happens and decide on possible fourth course.

Yes, aiming for 30 K or higher.

Whoa, you got your numbers down. That first 15k after 5 days doesn't sound so hot for steroid response. It will be interesting to see if the IVIG mixed in will help the current pulse.

Hope the number holds up...