New To ITP And Lost

Hi Everyone,
I’m not really a social media/forum guru so hang with me here. I’m a bit out of my comfort zone.

I’ve (34 M) been recently diagnosed with ITP. It started with a trip to the ER in the middle of the night due to strange bleeding in my mouth (early August 2019) and it has been a whirlwind of a few months ever since. I feel like my life has been turned on its head and I’m completely powerless. I went from a healthy thirty-something one day to having this strange, largely unknown disorder in the blink of an eye. I have a family with young kids at home who now constantly worry about me and I can’t do anything to help them.

I went into the hospital that first night where they found my platelet count to be 0.

After a round of IVIG and dexamethasone I was released a few days later with a count of 50.

A week later I was at 167 and things were looking up!

Until I went in for another draw and was back down to 38 a week and a half later. A few days later I was at 43. Then a few days later, 16 and was started on 100 mg/day of prednisone.

From there I went up again! A week later I was up to 147 and things were looking good again! I was cut to 50 mg/day of prednisone to start the taper.

Untill today I went back and was back down to 80… I’ll go back in another week where I just can’t figure I’ll be anything but low again.

It’s only been a month and a half, but the cycle of joy (ecstasy!) and depression is starting to wear thin on me and my family. So many questions and unknowns with no answers. My hematologist tells me that “steroids work on most people” so does that mean I’m in real trouble? If steroids can’t do it like they do on “most people” is anything going to work? His next step is Rituximab, followed by a removal of my spleen. Then if that doesn’t work, “it’s a very serious situation that may not end well.”

Do people die from this?

The more I read about ITP the more unknown it seems to be and the harder it is getting.

Thanks for letting me vent. I don’t really have anyone to talk to that isn’t already in stitches worrying about me and I don’t want to make them worse.

Have a great day.

Edit: I don't know if it's relevant but I guess I should add, for a few years my baseline platelet level at my annual physicals has been around 130-150, but stable and has never been low enough to concern anyone. And about two weeks before I first went to the hospital in early August, I had some sort of flu-like virus that I got over but the doctors initially said they believe is what caused the ITP in the first place. Who knows if that's true or not, but I figured I would add it.

I was surprised to see they cut your prednisone dose in 1/2 - that is drastic! That was done to me back in 1989 as they wanted me off pred before moving overseas and my count hit the gutter fast! [Did you see that it was 1989 when I was diagnosed? We moved to Tokyo shortly after diagnosis!] Steroids worked for me - I was on them a few years because I didn't want my spleen removed in Hong Kong where we moved after Tokyo. Because of that I did a very very slow taper and once we got back to the States I tapered off - I still have my spleen since it wasn't a guaranteed cure. Now my count isn't in the normal range 99.9% of the time - but prednisone did put me in a "remission", didn't need treatment and had a decent count. Most of us do not strive for a count in the normal range, but a decent livable count.

Quit reading about ITP unless you are reading good legit sites! I'm not dead yet so that should be a positive sign for you This was new to all of us believe me. The virus you had could have triggered your ITP - and before that your count was in the normal range, don't forget that a platelet count isn't a steady number as it does go up and goes down.

Stress isn't good - try not to stress! I'll bet you are going to do just fine - do read all you can, I suggest checking out the tabs at the top of this page - get to know your body and remember you have a say in what is going on with it [that's why I refused to go on prednisone 13 yrs after diagnosis when my count tanked & our son was getting married - didn't want pred face for the wedding pictures, had WinRho instead and that did the trick giving me a decent count again].

Others more knowledgeable will chime in with better responses than mine - in the meantime try not to worry and stress out! Keep us posted & good luck to you!!

Some of the things your doctor said like "it may not end well" is absolute nonsense! Really an insensitive thing to tell a patient along with it being totally not true! Most people can live fine with low platelets, You didn't die from having ZERO platelet count, right? Just remember that, you are okay. Its just something you need to manage. There are many more treatment options. If he didn't mention Nplate and Promacta, I would say your doctor is not up to date. These are drugs that are very effective and designed for ITP. My advice is you should take caution with this doctor's advice.

And educate yourself. Read the Mayo Clinic website and the PDSA "Disease Information"- very important to read all about the treatments. ITP is not a big deal as far as chronic diseases go, I know its hard to be healthy and then be diagnosed, but ITP for most people is very manageable. Many people go into remission esp. in the first year. I would never consider having a splenectomy in the first year and without trying some treatments. I have had ITP for 12 years, my counts have been below 10 many times, without any issues. I still have my spleen because its working just fine. I'm on Nplate which is a weekly injection. I have not gone into remission but honestly many people have. I do fine with counts above 20 and try to keep my counts around 50 which is a safe number- your counts don't have to be in the normal range to be safe. They can do open heart surgery with a count of 50, my doctor told me.

The old protocol was to try Rituxin as a second line treatment, but there is some new thinking that Nplate and Promacta might work well as second line to put people into remission. I don't know- I tried Rituxin and it didn't do much for me. But the important thing is that you can make your own decisions. I am on my third hematologist and he is the best. I had to change doctors to get someone I felt was very knowledgeable and up to date, plus easy to talk to. ITP is rare and for that reason many doctors have little to no experience with it.

Re: steroids- yes most people respond to them but very few can sustain higher counts. Counts go up and down with the dose. So you are totally NORMAL in having falling counts as you taper off of prednisone. But its good to know that you respond to them, you can then use prednisone as a rescue remedy. Its not a good drug for long term use but good to bump up your counts when you need it. As Mel said, that is a fast taper. Good luck- try not to worry, ask questions- theres a lot of good information on this forum, and thanks for sharing your story.

PS. I would get a second opinion, see if you could find a doctor that wasn't trying to scare you to death, and was more up to date on treatments.

Welcome. Yes, it certainly can be a roller coaster that can cause a great deal of anxiety in the beginning and even later on, depending.

IVIg and steroids is the standard emergency treatment and being released at 50 sounds typical as well. 167 is great. Glad you had such a good response. How did you feel on those medications? Any side effects? With the drops, have you had more bleeding in your mouth or other symptoms?

Steroids certainly can be a long-term treatment. It has been for many. Personally, I think steroids are over used and trying something else would be better in the long run. It is interesting to me that recently a few newly diagnosed people have written that their hematologists went straight to Rituximab as a first line treatment. I would think Nplate or Promacta would be more first-line, but perhaps they are trying to put an end to the ITP first, in the hopes that it won’t become a chronic condition.

I realize right now you just need to get stable, but you may want to look for a different hematologist. Being told, “it’s a very serious situation that may not end well” shouldn’t happen, especially since you are so newly diagnosed. You still have many treatment options, so don’t lose hope.

As with many diseases, there is much that isn’t understood, but lately there seems to be more ITP research being done and there are a few fairly new drugs/treatments that are very good.

I don’t know about the flu causing ITP, but have heard that thought from others. If it did, perhaps the ITP will be short lived.

Are there any ITP support groups near where you live? Even if you don’t want to attend a group, the leader of the group may be able to recommend a hematologist or two.

For the stress and anxiety, I’d say it’s a process and you and your family will get to know how the ITP experience is for you. You will get to know how various treatments and all aspects of life affect ITP and you. It’s totally normal to feel all over the place emotionally. It’s tough and there is definitely a mourning for the life one once had. However, you are by no means in a hopeless situation. Glad you posted.

1. Try not to panic or get stressed. Most people live perfectly normal lives with a low platelet count.

2. Get a different haematologist. This has to be a 2 way process. Read up as much as you can but make sure there are medical references to support what you are reading. Try accessing this www.ebmt.org/ebmt/documents/itp-guide-en . It is written by ITP nurses for nurses who know nothing about ITP (me 5 years ago). It's quite an easy read and not too technical. It is 8 years old but should answer a lot of your questions. If you can't access it message me and I'll send you a pdf copy. You need to make informed choices. Question everything. Haemo must have an evidence base to work from.

3. Keep your spleen. Splenectomy is rare these days and not recommended as an option this early. There are many medical treatments that you could try before sucummbing to anything so drastic as surgery. Promacta suits me.

4. Steroids are bad news. Yes they work but the long term side effects are horrendous. Make sure who ever is treating you sorts out a proper tapering schedule. Dangerous to come off them too fast. There is loads on this site about that.

5. Keep reading on here and posting your questions.

First of all, thank you so much to everyone who took the time to respond. I truly appreciate your words of encouragement, the knowledge you shared, and the time you took to reply.

Yes, as most of you mentioned, I need to try to stop stewing on it and stressing. I fully admit that and am trying. It gets easier with each new morning.

I was so discouraged by the words of my hematologist. He doesn't seem to be able to explain things very well, other than how serious everything is. I am trying to get an appointment to see another hematologist but it's taking a little while to get my medical records sent to the new doctor so they can schedule my appointment. As I said, the current one is recommending we jump from Rituximab to a splenectomy without even any mention of anything else. Up until now I just assumed there was a reason for that... Maybe I wasn't a good candidate for other treatments. But I will be asking about things like Nplate and Promacta when I see him again on Monday.

As for how I have felt on the steroids, to be honest, I have been extremely lucky and have hardly felt any side effects at all. I could barely even tell I was on IVIG/dexamtehasone. The prednisone has made me feel jittery and sweat a bit more, but other than that, nothing. And no, I have not had any more bleeding anywhere since the first night's bleeding in the mouth.

I appreciate all of the experiences you all shared. Especially with the other options such as Nplate and Promacata. I am reading up on splenectomy and while I'm not opposed to it if I have evidence pointing me to it, I prefer to try other things before jumping to that if the other options exist.

Thank you for the ITP nurse's guide, mrsb04! It is very good.

Regarding the recommended dosing of dexamethasone... The guide says 40 mg daily for 4 days every 2-4 weeks for 1-4 cycles. I received 1 cycle of 4 doses while in the hospital, along with IVIG. My current hematologist says that since that brought me up for a few weeks but did not keep me up, dexamethasone did not work for me and we cannot go back to try additional cycles; however, your guide says every 2-4 weeks for up to 4 cycles... Is there a reason we "cannot go back and try dexamethasone again since it did not work the first time."? It seemed to work well (brought me up to 167!) and lasted for about 2 weeks.

For my prednisone dosing, I was on 100 mg/day for about 1 week, then cut to 50 mg/day and have been there for a week and a half now. Is this usual? Did he give the high does enough time to work?

Thanks again everyone. Your knowledge and posts have really helped to calm us down and slow the stress down. Your time is certainly appreciated.

Glad the guide helped. Dosing of any medication is always going to be a contentious issue. Different doctors have different experience and preferences. Different patients have different expectations.
I've never done a Dex pulse so cannot really comment on that. Using NPlate or Promacta very early on is relatively new.

There are various factors to bear in mind.
:-The whole idea of treating ITP is to combat any symptoms NOT normalize a platelet count. You can have open heart surgery with a count of 50. The aim of any treatment is a count above 49. ITP patients have predominantly immature platelets, which are more juicy and sticky than mature ones increasing the risk of clotting.
:-The side effects of the meds can be worse than the symptoms of ITP.
:-There is no guarantee any treatment will work and that includes splenectomy.
:-Response times can vary considerably. Promacta for instance can be very slow to get going.

You know your body better than anyone else therefore you know which symptoms you can cope with. If I could hold a count of 30 without any medication I would be delighted. I bruise very easily irrespective of my platelet count. I've leaned to live with it. Below 20 I am always frozen stiff and exhausted, if in single figures I get blood blisters in my mouth and /or nosebleeds. A short sharp shock of Prednisolone gets my count back up.

This is a recent paper discussing current treatments that may be of assistance. www.ncbi.nlm.nih.gov/pmc/articles/PMC5737126/

I just came across Hal's chart of ITP types. I'm sorry that I have to ask this, but I don't understand where I fit. I seem to have had a strong response to IVIG and steroids, but it wasn't sustained for very long. Can someone explain it to me?

Thank you.

Don’t worry about it. It is a theory he devised. I have never received IVIg so fit in nowhere

Hey.
I understand the moment you went from a 34 yr old healthy man to a man with an unknown illness I was 21 when I had your issue and so you're lucky that you have it so late.
I see other people have explained some facts to you. So, I will tell my opinion in your next treatment options:
If I were you, I would try low-dose Rituximab regimen which has a fairly good chance of wrapping up this disease for a couple of years, but then If you failed (like most of us did) if you have good insurance coverage, switch to one of TPO drugs most people respond well to these drugs and are able to livetheir previous lives (but also being slightly more cautious).
Good luck.

Mrsb-
While I was getting IVIG in hospital, one reason that I was happy was that finally, I'm going to have some IVIG response data to fit in Hal's table

Ok, thanks mrsb04.

Thanks for the recommendation Lman. I'm fine with giving the Rituximab a try so we'll see on Monday how my numbers look.

I'm finding another hematologist who's not so gung-ho with the splenectomy option quite yet and will look at some other options with me...

Regarding the hematologist that is gung-ho on a splenectomy - remember it is your body and you have to do what you feel is best! I'd never tell anyone here what to take/what to avoid, that isn't my place (although I do give my opinions ) - but I will say just listen to your body & do what you feel is right. Your body is still new to this, you were only diagnosed 2 months ago.

running4cake wrote: I just came across Hal's chart of ITP types. I'm sorry that I have to ask this, but I don't understand where I fit. I seem to have had a strong response to IVIG and steroids, but it wasn't sustained for very long. Can someone explain it to me?

Thank you.

running4cake, on my ITP treatments table. The responses for steroids and IVIG columns is when one takes only a single medication at a time. You mentioned getting a 167 count after taking both IVIG and Dex (steroids). If so, then one can't use the 167 to place oneself in a row because two drugs were influencing the count.

Now that you have achieved a 147 count after a week on a 'full dose' of Pred, it looks like row 1 is possible/likely. 1 and some other row is also a possibility. Only this single steroid data point, so far, to estimate a potentially multi dimensional problem.

All though, with a handle that includes the word 'running', I have to wonder if there is a little bit of row 2 involved. Maybe 1&2. Do you run 2 or 3 times a week? This thread should explain the 'running', or exercise, aspect.
'No Response to IVIG, ACK !'
pdsa.org/discussion-group/7-treatment-general/30169-no-response-to-ivig-ack.html#64535

Have you given any thought about what may have triggered ITP? Some folks have no idea, and others have a suspicion. Maybe even a strong suspicion.

Hal..running4cake states in the initial post ”about two weeks before I first went to the hospital in early August, I had some sort of flu-like virus that I got over but the doctors initially said they believe is what caused the ITP in the first place. Who knows if that's true or not, but I figured I would add it” .

Yes, the virus I had a few weeks before is the only thing I can think of that happened to me this summer. Who knows...
As for exercise, it depends on the time of year. I go through cycles of being good about running 2-3 times/week, and then I drop off for a few months. I am in no way a diligent exerciser but I do try when I can.
I did run a 5k earlier this summer... Darn. I always knew exercising was bad for me!

I had an horrific bout of sinusitis 6 weeks before I was found to have a platelet count of 12.

I was probably the healthiest ever when ITP hit - had not been sick, nothing - only thing different was that gamma globulin injection we had to get before the company would let us go find an apartment in Tokyo, very short time later the red dots appeared.

We all have different stories to tell.

Curious - I haven't gone to your theory site Hal & don't plan to - but are you saying that running can make the platelet count go up or down?

Checking in... This morning's blood test had me at 85, so about the same as last Monay (80).

Hematologist asked me to cut the Prednisone from 50mg to 25 mg starting tomorrow.

I'll be enjoying the fact that I didn't decline this week

Good news. Bit of a drastic cut in steroid dose.

From what I've read, that was my thought too. I'm somewhat worried that he's focused on getting me off the Prednisone too quickly. Is that normal?

What does a typical taper schedule look like? I understand everyone is different but is it normal to cut it in half each time?

My docs, as I may have mentioned, were in a hurry to get me off before we moved overseas and they cut my dose from 60mg to 30mg and my count hit the gutter. I personally like a slow taper.

Running4cake
www.medicinenet.com/steroid_withdrawal/article.htm and www.nadf.us/adrenal-diseases/secondary-adrenal-insufficiency/ are essential reads in my opinion. GPs (PCPs to you folks across the pond) are much better at managing tapers than hospital docs believe me I've been there.

Your adrenals need time to cope with dose reductions. Here is an abridged version of the standard UK Guidelines.
Gradual withdrawal for people who have:
Received more than 3 weeks of corticosteroid treatment.
Received more than 40 mg prednisolone daily or equivalent for more than 1 week.
During withdrawal, the dose of oral corticosteroids may be reduced rapidly down to physiological doses (about 7.5 mg of prednisolone or equivalent) and reduced more slowly thereafter.
If the problem has resolved and treatment has been given for only a few weeks.
Reduce by 2.5 mg every 3–4 days, down to 7.5 mg per day, then reduce more slowly, for example by 2.5 mg every week, fortnight, or month.
If there is uncertainty about disease resolution and/or therapy has been given for many weeks.
Reduce by 2.5 mg every fortnight down to 7.5 mg per day, then reduce by 1 mg every month.

These are the latest guidelines from uptodate .com
-5 to 10 mg/day every one to two weeks from an initial dose above 40 mg of prednisone or equivalent per day.
-5 mg/day every one to two weeks at prednisone doses between 40 and 20 mg/day.
-2.5 mg/day every two to three weeks at prednisone doses between 20 and 10 mg/day.
-1 mg/day every two to four weeks at prednisone doses between 10 and 5 mg/day. (personally I would say 7.5mg)
-0.5 mg/day every two to four weeks at prednisone doses from 5 mg/day (personally again I would say 7.5mg) down. This can be achieved by alternating daily doses, eg, 5 mg on day one and 4 mg on day two.
It can take ages to get off them. I can't cope with more than 0.5mg/day /month otherwise I have horrific withdrawal symptoms.

Thanks mrsb04! That was helpful. I had no idea what a "normal" taper schedule looked like so I'm now a little bit more informed.

So when the UK standard says: "During withdrawal, the dose of oral corticosteroids may be reduced rapidly down to physiological doses (about 7.5 mg of prednisolone or equivalent) and reduced more slowly thereafter", my hematologist cutting me from 50 down to 25 mg is inline with this recommendation...?

I have no idea why this has appeared twice after I edited the original from imperial to metric. I was started on 45mg/day (1mg/kg/day). That took my count from 12 to 87 in 3 days. My initial haemo then dropped it to 30mg a day and my count plummeted. Experience has taught me that the USA schedule is better.

I was started on 45mg/day (I am 7stones). That took my count from 12 to 87 in 3 days. My initial haemo then dropped it to 30mg a day and my count plummeted. Experience has taught me that the USA schedule is better.

Hello, and welcome.

Excuse the analogy, but the best advice I could give you is one that may apply to running as well - keep your eye on the horizon.

If I could go 20 weeks in the past (when my wife and I began our journey), I would have told myself not to pin my hopes on the condition resolving in a day/week/month (although it is possible)... Rather, what I would tell myself to take a broad view on progress... Are counts in a very critical/very dangerous range? If no, independent of the count, are we symptomatic? When trended out over time, is there progression, regression, or stability? Do we have any active bleeding, or is the biggest symptom only minor bruising? Are the treatments or the condition materially affecting quality of life?

These have been some of our "measures of success". What we have found so far is that (for us at least) the stress generated from the condition and the side effects of the treatments have been worse than the condition itself. Once that becomes a fact in our mind, the threat of "ITP" fades into the background a little bit.

I can relate to the first steps being scary, but there are good people here who know your story well and can help provide hints on what future chapters look like.

Take care,
T

Running4cake.
Your story is very similar to mine. I never had any health issues until one day at my yearly Dr appointment in May 2019 at 48. The Dr was freaking out and sending me to a Hematologist with a 40 count that was in the 20's by the time my hemo appointment came around. I have only been through one Dex pulse, that didn't have good results, and a course of Prednisone, that I just finished a month ago, with decent results. I have been steady around 90 - 75 for the last few months. I maxed out at 118 while on Pred. I understand the roller coaster and I still get all worked up when I go in for blood work. I am slowly realizing that as long as I am above 40 or 50 then everything is fine. It is only when I dropped into the 20's that I had any symptoms (mainly bruising and tired). If and when I drop below 50 then I will have to make some decisions on what drugs to take next. I am currently leaning toward Promacta. Good luck and use this site for a support crutch, but don't overdo it. I feel the more I was reading on ITP the more I was stressing about it.