Recently diagnosed

I'm a 40 year old male recently diagnosed with ITP induced by an antibiotic.

At the end of July, I had a swollen lymph node in my neck. In the course of having it checked out by my primary doc, I was prescribed Bactrim, which I had never had before. Within a few days of starting the antibiotic, I noticed petechia all over my legs. Stopped the Bactrim, but the petechia didn't go away and bruises started appearing on my legs and torso. A few more days go by (I happened to be out of town on a vacation) and my primary wanted me to get a blood test. My platelets were at 2.

So, this lead to hospital stay #1, lasting five days. I ended up meeting with a hematologist within an hour of getting to the ER. We started with dexamethasone for the first three days, which didn't raise my levels out of single digits. Two IVIG's got me into the 40's, which was enough to get me released with a prescription for 40mg of prednisone.

Three days later, blood test shows I'm back down to single digits. Two days of outpatient IVIG's, which had little to no effect, so was admitted again to hospital for another five days, where I got more IVIG's for the first four days, which, unlike my first stay, had virtually no effect. Also had a bone marrow biopsy and started discussing second line treatments: splenectomy or Rituximab. The fifth day, got a platelet transfusion, just so the levels could get up enough to get me released. When I left the hospital, my counts were in the 40's. Next day, already back down to single digits. At this point my hematologist and I decide to go the Rixtuximab route.

And that was yesterday. Another platelet transfusion followed by 840mg of Rituximab. I have to say I feel very lucky, I didn't get hit with the normal side effects of Rixtuximab. I have three more treatments scheduled over the next three weeks, as well as the every other day blood draws and, if necessary, more platelet transfusion. The prelim bone marrow biopsy results came back without anything looking abnormal...just inflamed cells because they're working overtime to fight the platelet destruction going on.

As with a lot of people, I got frustrated with the numbers, especially because when I'm low, they're in the dangerous area, which prompted the long hospital stays as a precaution. Hard to sit around a hospital when you look and feel normal except for the single digit platelet levels. My physical well being was one thing, but it started wearing on my mental well being, as well. Reading through this forum, as well as other online resources, has helped greatly - puts things in perspective.

My biggest fear right now are the weekends and days in between treatments. I'm still so new to this I hope I can read by body's signals correctly - what constitutes an emergency and what doesn't? Like today...intermittent nose bleed (which hasn't been an issue the past few weeks)...is it bad enough to go to the ER? Or I get little twinges in my lower abdomen...not painful, almost gas like. Again, how do I know I'm not bleeding on the inside? I keep telling myself, I just need to make it to Monday, which means I can get treatments as outpatient and not have to be admitted to hospital...and thus save my sanity.

I'd like to hear your thoughts and encouragement!

Hi Matthew. Welcome to the group. I'm glad that reading the Forum has helped some. Keep it up. Makes us all experts in no time.

As for the bleeding question....you learn to gauge what is serious and what is not. That usually comes in time when you get to know your body's symptoms. Generally, if you can get the nose bleed stopped and it's not gushing, you don't need to go to the ER. As for internal bleeding, if symptoms on the outside are not too bad and you're not urinating blood, internal bleeding would be unusual. Most people can handle lower counts because people with ITP tend to have larger platelets and platelet microparticles which help with clotting.

Platelet transfusions generally don't for much for ITP and are very short-lived, so it's not used much unless there is serious bleeding. IVIG is pretty temporary too. I hope Rituxan works for you but if not, a next step could be N-Plate or Promacta.

Many patients with low counts are not admitted to the hospital, that decision is usually up to the doctor and the patient. Some doctors tend to hospitalize at the beginning of a diagnosis but that dies out in time.

You seem to have a great attitude and that will help tremendously. Keep reading!

Thanks Sandi, I appreciate the reply and advice.

I won't lie, the attitude waivers...I'm sure it does for anyone going through something like this, especially at the beginning. My personality tends to be very black and white, give me a definitive answer sort of deal. It's been an adjustment, to say the least.

I also feel lucky because...
- I feel like I have a good hematologist.
- I can't begin to describe all the acts of kindness and support from the nurses and support staff in the hospital or at my hema's office.
- I'm single and live alone, but social media is something that helps me feel support from friends and relatives. Just have to remember to always have a charger with me haha I quickly learned to have a 'bug out' bag ready just in case. Never know when I might have to get admitted to a hospital or what I'll need to keep me entertained during a long infusion.

Yes, ITP can be hard on Type A's who are control freaks. I got past that by pretending things were normal, went with the flow and kept going. You'll get there in time.

It's good to look at the positives, although that can be hard. Hopefully you do have a good Hemo, not all of them are up to speed the way they could be.

Hi Matthew,

Sorry you had to join our group but you came to the right place!

Social media is a life saver for ITP patients. In fact, all rare disease patients are thankful for the internet because even though we are few and far between, we can connect online and not feel so alone.

I was diagnosed on May 1st of this year, the feelings you describe about being in the hospital are spot on. I went from working 60 hours a week to sitting around waiting to see if I was going to kick the bucket. It was an extreme change and really weighed on me mentally. Especially being an otherwise healthy 27 year old who was active. Giving up my "life" for a few months while my doctors and I tried to get ITP under control was tough. You will come out on the other side much better off than before you were diagnosed. That I can promise you. It takes time, and everyone is different. What helps is reaching out and communicating with other ITP buddies. You don't feel like a freak when other people have experienced the same emotional turmoil brought on by the disease.

Thanks Lady Nole, appreciate the kind message...even though I'm a Gator

My blood counts this week are still in single digits - around five. Had my second Rituximab treatment this morning - slept through most of it, but felt well enough to do some work in my office afterwards. I think I'm lucky because I haven't gotten the side effects a lot of people get when on the treatment - although a fleeting thought crossed my mind: "if I'm not getting the side effects, maybe its not working". Odd thought, I know - but when you have a lot of time to think.

On a side note, I miss shaving my head. Before all this, I shaved it every day. Stopped a couple weeks ago when I got admitted to the hospital. I think tomorrow, as an act of trying to feel more normal, I'm going to (ever so carefully) start shaving it again. Maybe I'll go shaved head but leave a full beard...cut down on the real estate that could get nicked! haha

Welcome Matthew. Rituxan can take several weeks after the last treatment before it may start working. So do not get discouraged if counts do not respond right away. You are playing the waiting game right now. We all wait and see what treatment may or may not work. Good Luck!! Hope Rituxan works for you.

Hi Matthew,

Looks like you are going thru a lot of the stress we all know too well.
I'm having ITP now for 4 years and at the moment my life is completely normal.
I never heard of anybody how got into serious trouble by shaving. Small bleeding won't usually create a problem. You should have around 5 liters of blood. It will take ages to bleed that thru a small cut. Similar for nose bleeds or any other bleeding. For me even when I had single digits it stopped after a while.

Good luck and keep the chin up. You've get used to ITP and feel better soon.

Cheers
Joerg

Dean & Joerg:

Thanks for the words of encouragement!

Patience is part of my new normal, this I'm learning - believe me.

Had my third Rituximab treatment today. Before starting it, my hematologist sat me down and said that, if we didn't start seeing a response in the next couple weeks, he wanted to move forward with splenectomy.

My counts have not been out of single digits the past three weeks. In fact, since this all started about six weeks ago, my counts haven't come up out of single digits except for after the first couple IVIG treatments after four days of dexamethasonr - after which I was back in single digits in a few days (and any IVIG treatments after that never worked again). Also after two separate platelet transfusions - each of which got the count int the teens...for a matter of hours.

I've been on 60mg of prednisone (although starting a taper tomorrow...50mg and so on) to go with the three Rituximab treatments.

I think my doctor's concern - and thus the urgency of wanting to move towards the splenectomy - is that my counts are single digits. I think if I was even slightly higher...like 20 or 30...he may not be talking about the urgency of surgery.

I asked about Nplate, but he says those sort of treatments are used after a splenectomy has failed. They're not good for long-term treatment and can have damaging side effects. And that don't solve the underlying issue.

Some things maybe you, the group, can weight in on:
- Is Nplate an ongoing treatment for the rest of my life? Or could it possible stablize my counts at some point and then I could stop the treatment unless I crash again?
- Am I right in saying that the urgency towards surgery is probably do to counts having been in single digits for three weeks straight despite the steroids and Rituximab? I have read results can take weeks after the last treatment to show up, but does someone with counts in the single digits for several weeks (and really, since this started nearly six weeks ago) have the luxury to wait that long to find out?

Matthew:

Rituxan usually doesn't start to work until 4 to 12 weeks after the first treatment. You are still early in the game with that. When I started Rituxan, my counts for the first three infusions were 8, 11, 3. I wasn't using any other treatment. Suddenly on the 4th week, counts were 150. That was considered to be a fast response.

Platelet transfusions are not considered to be treatments, it's more like a rescue treatment for bleeding issues. They don't usually last for anyone.

N-Plate and Promacta are not necessarily saved to be used after a splenectomy. They can actually be considered spleen-sparing treatments. He is wrong about that. Many people use either of those two meds when they do not want to have a splenectomy. No, they are not always for life, and most of the side effects are reversible when the drug is stopped. Some people do and have attained remission which is something no one expected from these drugs. Splenectomy does not solve the underlying issue either.

While low counts are not the best situation, the symptoms matter just as much, if not more. Of course you'd want counts to go up, but the word 'urgent' does not really correspond with 'in a few weeks'.

Researching and thinking it through is your best bet. Be sure articles are new and credible. Feel free to ask as many questions as you want - there is a whole group here to help!

I just read this post from the start and the thing you should be taking from it is.....your counts have been mainly in single digits for a couple of months and YOU'VE BEEN OK! So, unless you start getting worrying bleeding, nothing seems to be an emergency. Wait and see if the rituximab works. And then maybe wait a bit more because the vaccinations you need for splenectomy won't be effective that close to IVIG and rituximab. Good luck.

Good point, Alison. The vaccines are not effective for a few months after using Rituxan.

I would give the Rituxan more time. As already mentioned it can take several weeks after the first treatment before it can kick in. I had two prior rounds of Rituxan several years ago and response was slow for me. I got discourged when counts did not increase. Counts bounced around during and after the treatments. Counts slowly started responding a few weeks after the last treatment.
I had counts of 0k - 8k for several weeks this past summer. Started with Prednisone, 4 Platelet transfusions, 1 dexamethasone, 4 IVIG and 2 Rituxan. Counts would not raise much and fall back to the single k's. Dr was pushing for Splenectomy as the treatments did not seem to be working. I had a consult scheduled with surgeon about the Splenectomy. I did some reading on H Pylori and chronic ITP and told Dr I wanted to be tested for H Pylori. He agreed to the request. Test was positive. 2 weeks of antibiotics was started. 4 days into the antibiotics counts went up, 6 days counts kept going up. 2 weeks and counts are higher than they have been in over 10yrs 293k. If I would not have had this test, I would be without a Spleen right now. Now I am not sure if the 2 doses of Rituxan would have kicked in or if infact the antibiotics did the trick. Another count to be done next week.

Rituxan can take as long as six months to work!

While delayed responses may occur in the lower dose regimen they may also occur with standard dose rituximab. Since maximal response to both dosing regimens may be delayed, a decision regarding splenectomy should not be made until at least six months after rituximab therapy.
Slow Responses To Standard Dose Rituximab In Immune Thrombocytopenic Purpura
Kevin Kelly, Mary Gleeson, Philip Thomas Murphy
Haematologica March 200994:443-444;Doi:10.3324/haematol.2008.001396
www.haematologica.org/content/94/3/443?ijkey=a92f69d450dd728ccf5abbb556f929a0cf3c9005&keytype2=tf_ipsecsha

A noted ITP researcher responded:

"We agree that, when possible, a period of at least six months of observation from rituximab therapy may be necessary before undergoing splenectomy since at present we still don’t have enough indicators predictive of brief and mid-term response."
Slow responses to standard dose rituximab in immune thrombocytopenic purpura.
Author reply, Francesco Zaja and Renato Fanin
www.ncbi.nlm.nih.gov/pmc/articles/PMC2649348/

There seems to be a misconception among many that because rituximab CAN work quickly, that if it doesn't work quickly then it won't work at all. There is ample evidence to the contrary.

Hmmm. I haven't seen the 6 month response time. Probably because most people do respond way before that and if they don't, they move on to other treatments that they do respond to. It does make sense to hold off on the splenectomy though, especially for vaccine reasons. And yes, it is surprising how many doctors expect it to work right away. I thought Rituxan was mostly out of your system by 6 months, go figure.

Sandi, I agree it doesn't make sense, but then none of it does.

As I misunderstand it, Rituxan should always work, and quickly.

I would love to find someone smart enough to explain it simply enough for me to understand it!

I used to have a really good explanation of it about 10 or 11 years ago, but it's long gone. I'll have to search it again.

I was one of the few (if not the only one) that I have seen on here who has said that it worked quickly for. My counts went up so fast that I was kinda surprised that I had the last treatment.

I appreciate everyone's input.

I've got my fourth Rituximab treatment set for this Friday. While my count is still in the single digits, it's at least trending upwards (albeit slowly) the last four times they've done it (which is every other day at this point)...so I'm cautiously optimistic.

Had my last Rituxmab treatment this morning. My count was at 2 when they started. By the way, I have not been getting low doses of it - I've been getting 840mg each time.

Hematologist is still advocating splenectomy. I told him I'd rather wait for a few weeks to see if the Rituximab starts working (although my impression is that he's skeptical that it will), then if nothing is happening, start NPlate treatments through at least the end of the year, possibly into February, which would put me at about the six month mark. He doesn't want to wait that long, but at least respects my choice enough to start the approval process with my insurance company for NPlate, which he'd like to start within the next couple weeks. At the same time, he also wants me to do the surgery consult, also in the next couple weeks.

For me, the reasons why I want to wait:
- If this is acute and not chronic (especially because it was a reaction to an antibiotic that started this), then removing the spleen seems like a long-term solution to a short term problem.
- I want to make sure we've covered every possible base in terms of finding out the underlying issue before taking a step to remove an organ.

For him, he's seeing that nothing is working and, with my counts stuck in single digits, is the clearly concerned that I could have a major bleeding issue. While I understand the concern, I think he's a little over the top with it (at times).

He also mentioned that doing NPlate before splenectomy lessens the chance removing the spleen will be successful - anyone else hear that?

I've also scheduled a second opinion with another hematologist who is Chief of the Division of Hematology and Medical Oncology at a teaching hospital here in town. I think it's time to hear another voice in the discussion.

Matthew:

I think you are on the right track. Your reasoning is very valid. I'm very glad you are going for the second opinion.

As far as N-Plate causing spleen removal to be less successful; I can think of no logical reason why that would be the case. Can you? I have never heard that before.

Unless there's a connection between how NPlate affects bone marrow

That's possible, but N-Plate usually takes time to affect the bone marrow and most people never have a problem. If they do, it usually reverses so it shouldn't be a long term thing. That's the only theory that seems even a bit plausible. You'd think that would be in the literature somewhere.

In the past week I've gotten a second opinion. The reviewing hemo (who's Chief of Hematology at a teaching hospital in town) concurred with my current hemo - except in regards to the next step of treatment. The hemo giving the second opinion is more willing to wait and see if the Rituximab treatments worked - although she'd also like to look into starting Nplate...probably sooner rather than later due to my counts being so low. This, of course, complicates things because it sort of overrides knowing if the Rituximab worked or not. At that point, we didn't get into a definitive plan until I could talk to my current hemo.

Which I did today (he's spoke to the reviewing hemo already). In short, he does not want to start me on Nplate because...
- He doesn't want to start me on, what he called, a 'life-long' and 'life-altering' drug treatment.
- He's worried that if I do Nplate now, then decide to stop it for whatever reason - including a splenectomy down the road - that the Nplate may not work the second time around.
...and that if I wanted to do Nplate instead of removing the spleen as my next treatment course (even after waiting two more weeks for the Rituximab to work), that he'd prefer I see another hemo to continue treatment, because he wouldn't feel comfortable doing it that way.

Considering that I, like many of you, would like to avoid having my spleen removed, it looks like I'm switching hemos. I'm waiting to hear back about squeezing in an appointment next week with the doctor who reviewed my case.

Questions:
- Is there any evidence that stopping then starting back up on Nplate makes it less effective the second time around?
- Would there be a difference in how my counts would react with Nplate on top of Rituximab in such close time frame? My current hemo mentioned this may cause my counts to REALLY skyrocket more than normal and would cause other issues (clotting, etc).
- If I get to the six month mark by using Nplate, then what? Do I stop that treatment to see if my counts hold? If they don't, do I elect for surgery at that point (given nothing else has worked for me)? Also, this goes back to my first question...am I risking making Nplate less effective?
- Also, I have an initial appointment scheduled next week with an acupuncturist/herbal medicine physician. Anyone had any experience with acupuncture while your counts are in single digits? Advisable? Not advisable? I'm looking for complimentary treatments to help with the condition and with the stress - and this sort of physician is covered by my insurance.

Any other thoughts you might have are welcome - this forum has been a great resource thus far!

Matthew:

I like your second opinion Hemo much better. The first does not sound like he knows much about N-Plate.

Answers as I know them:

- Is there any evidence that stopping then starting back up on Nplate makes it less effective the second time around? No, not that I have ever heard. Based on the way it works, I don't see how that would be likely.


- Would there be a difference in how my counts would react with Nplate on top of Rituximab in such close time frame? My current hemo mentioned this may cause my counts to REALLY skyrocket more than normal and would cause other issues (clotting, etc). Only if you'd happen to get a response to Rituxan while you were using N-Plate. N-Plate is used to keep counts around 50k. If Rituxan kicks in and your counts become normal, then yes, you'd be above recommended guidelines as far as counts. In that case, the dose should be lowered or not given during that time.

- If I get to the six month mark by using Nplate, then what? Do I stop that treatment to see if my counts hold? If they don't, do I elect for surgery at that point (given nothing else has worked for me)? Also, this goes back to my first question...am I risking making Nplate less effective? If you get to the six month mark, you see how your counts are doing. If you are maintaining counts around 50k and stable, keep going with N-Plate. It's not a bad way to manage ITP and if you are responding to it, then yes, something is working. If counts are higher, you can lower the N-Plate dosage and see how you do off of it. I wouldn't worry about any time periods like 6 months. With N-Plate, you just take things week by week. If it's working with few side effects, great! You have a treatment.

- Also, I have an initial appointment scheduled next week with an acupuncturist/herbal medicine physician. Anyone had any experience with acupuncture while your counts are in single digits? Advisable? Not advisable? I'm looking for complimentary treatments to help with the condition and with the stress - and this sort of physician is covered by my insurance. I don't know that I'd try acupuncture with counts that low. I wouldn't think you'd bleed to death or anything, but holes in the skin are usually not advisable. There are other ways to manage stress with that type of health care provider. Let him make the suggestions.

* N-Plate does not have to be for life. I don't know where some doctors get that idea. Studies have shown that people have gone into remission while on N-Plate. Also, many people have stopped N-Plate in favor of other treatment options. I also do not see how it is life-altering except for the weekly trip to the Dr. Many people have felt that when they started N-Plate, they got their lives back. Patients tend to have less side effects with N-Plate than they do with some of the other treatments. Some people even eventually get to the point where they can do their own injections at home.

I used Nplate for three years. In the end my count went high and I had to cut the dose down and down, then went to using it less often, fortnightly, then three weekly until I decided to stop and see what happened. My last count was 83 without treatment.

Before I started Nplate my count was at 1 and 2 without treatment. So it has been like getting my life back and it hasn't been for life for me. I may have to use it again if my count drops but I'm fine with that. It's an easy treatment, I self inject at home, and it gives me no side effects at all.

I would never have my spleen removed. To me, it's a barbaric old fashioned treatment, but that's just my personal view.

I will add to this discussion that I just had my first NPlate shot two days ago. I'm hopeful that the drug will raise and stabilize my count well enough that I can taper off the prednisone I've been taking steadily for 14 months. The nurse who injected it said their practice doesn't have a lot of experience yet with the drug, only 3 others have used it. But she said it worked to raise all their counts to safe zones. She also said that none of them have had to continue using it weekly... That all of them got to a certain place and were able to taper off it. That's very promising to me. Several studies on the "lifelong" aspect are finding it's not always the case, either.

Six doctors I've seen since January (2 hematologists, 4 other specialties) have told me that splenectomy is the standard of care and is what I should definitely do. One hema actually called it "a cure", which I know it is not. They've tried to make me feel ignorant and unwise for not having it. Like Ann, I too feel it's barbarism and will never agree to it as a treatment for ITP.

In your situation, I wouldn't give up on a rituximab response yet. And I certainly wouldn't be rushed off to an operating room, especially not so soon on the heels of the last rituximab infusion. You cannot build your immunity back up with the necessary vaccines until at least 6 months from now.

Just my 2 cents. I hope you can find the right answer to your particular situation.

That's unreal, Janet! Splenectomy used to be the standard of care. I wonder what they'd say if you simply told them to read the current protocol and studies.

Sandi, I was completely shocked when I heard the Wash. U. specialist so matter-of-factly say I should just have the surgery and would be "cured". I had every hope he would know better.
It is what it is, but I don't have to accept it.