Questions for Those Experienced with ITP

6/10/2015:

Hello all.

You are correct, above, that I don't mind/care if my platelets are 20,000 or even 15,000 if I wasn't symptomatic. The problem is I've been crashing down to 2,000 for the past couple of months. I'd say I've got symptoms with the wet purpura that show up in the mouth.

I heard back from my doctor about the options we are going to discuss Monday, as a long-term type plan.

He laid out the following:

1. Rituximab -

2. Splenectomy

3. Orencia

To be honest, I'm a bit shocked about Splenectomy. I really hadn't considered this option because I thought that there are other more 'safe' avenues and that doctors are performing splenectomy less frequently these days.

I'm actually surprised that NPlate isn't even mentioned here by him, although he knows about it.

There is no way to think rationally or positively on high does steroids. Deln, hang in there. Once they lower your dose, you can at least start to feel like yourself again. The doctors have no idea how hard it is to deal with ITP and steroids. I have never been so depressed in my life. I cried all the time while on high dose steroids. I too have a much better understanding of the agony one must feel who has mental illness and depression.

Seem like odd choices. Maybe a second opinion is in order? Does he have some research regarding Orencia as a treatment for ITP. I haven't seen anything posted on this site or on the internet about Orencia as a treatment. I know about: Rituxan, NPlate, Promacta, and Cellcept as the most popular choices.
Keep us posted.

Deln,

Even if you have an HMO, you have the option of getting a second opinion. I had HealthNet last time my platelets crashed (HMO) and I opted for a second opinion and a "New" Hemo. I originally had a doctor who knew nothing about ITP (and she didn't seem to care); she was mainly a cancer treatment doctor. From what I've read, I would think that a splenetomy would only work if it was diseased or enlarged (saw this on PDSA FB site).

Did you ask you doctor about N-Plate? I have Medicare and I'm never sure if it is covered by it, but one person on this site said it was. You have a right to help make your choices.

I did have a relative and a patient advocate who helped me get that second opinion. Sometimes we need a little help in this area.

Hello everyone:

You all are very kind, supportive and informative. I appreciate being able to post my thoughts here, and also have this thread to serve as 'my story' for others to see how and what transpires.

So here's the situation in more detail regarding doctors. I currently go to a Doctor (Hemo/Oncology/Bone Marrow) at a large research/educational hospital. I also have a 'local' doctor that I'm corresponding with in the event I want to get infusions (i.e. Ritux, IVIG, NPlate potentially) so I don't have to drive for it.

NPlate has certainly been mentioned in passing, and we are I guess at the stage of potential long term planning.

Rituxin has been advocated by both the doctors. Yes, it's 50% success rate, but it might be worth the shot because it might solve this problem I'm having. I think they are focused more on this destruction aspect of my platelets right now (rather than a TPO). If the B Cell removal assists in eliminating the antibody destruction, then potentially that will solve it itself.

Also, it's 'okay' to eliminate the B Cells and suppress the immune system because the IVIG provides me coverage and protection anyway, so for the 4-10 week period that the Rituxin may kick in and work, I won't be too immuno-compromised.

To me, if the Decadron pulse doesn't work (and pending we don't do another 1-2 pulses) I'm leaning towards trying the Rituxin before NPlate.

I'm STRONGLY against getting my spleen removed. The success rate doesn't seem high enough, and some people's stories on here have made me discouraged.

I will have to find out on Monday 6/15 why my doctor isn't much in favor of NPlate though. He knows about it, perhaps he thinks that the Orencia is a better 'longer term' alternative. He has stated:

"Abatacept – this is a relatively new player; a weak immune suppressing agent that we have used in other patients with CVID who developed ITP and it worked. It is also very safe."

I agree about hanging onto your spleen.

So, your doctor is probably trying to deal with the CVID which the ITP is a result of having it. I have done some reading on CVID and know that it does cause ITP and the type of anemia that you have. Other than that, I don't know a whole lot about it other than I know that Caroline Kruse has mentioned that she has it.

So, Deln, you are probably dealing with a different version of ITP than some of us. It sounds like your doctor does want to get the CVID under control and that might resolve your other issues. I do know that Caroline hasn't had an issue with ITP for a long time (maybe 12-14 years?) and she did have her spleen out at the beginning. Does a spleen have anything to do with CVID? Was just wondering. I know I want to keep my spleen as I get a lot of staph infections which cause blood poisoning.

Keep us posted and glad to hear that you are know getting a plan in place.

The CVID issues are "under control" as they can be. CVID essentially leaves me more prone to infections or sickness in general. To supplement this, one can get IVIG or Sub Q injections that provide the immune globulin.

The ITP and CVID, in general is all part of immune system disregulation. Now, I've likely been combating ITP at many different years in my life, but I never noticed it because I never had wet purpura.

He is an intelligent doctor, and the research/educational hospital is one of the best cancer hospitals in the US. I don't doubt him, I'm just wondering his rationale about not wanting NPLATE right now.

Deln:

You have a tough decision ahead. I do not believe that any drug is safe....all drugs have potential side effects, some of which can be serious.

Orencia:

Respiratory side effects have included upper respiratory tract infection (5.8% to 12.7%), nasopharyngitis (7.8% to 11.5%), sinusitis (6.2%), bronchitis (5.8%), and pneumonia. Patients with COPD treated with abatacept (the active ingredient contained in Orencia) developed adverse effects more frequently than those treated with placebo (97% vs. 88%, respectively). These have included COPD exacerbations, cough, rhonchi, dyspnea, and pneumonia. Bacterial pneumonia and influenzal pneumonia have been reported in 0.4% of patients.[Ref]

Hypersensitivity side effects have included anaphylaxis or anaphylactoid reactions. Other adverse events potentially associated with drug hypersensitivity, such as hypotension, urticaria, and dyspnea generally occurred within 24 hours of abatacept (the active ingredient contained in Orencia) infusion.[Ref]

Oncologic side effects have included lymphomas and lung cancer. Other malignancies included skin, breast, bile duct, bladder, cervical, endometrial, lymphoma, melanoma, myelodysplastic syndrome, ovarian, prostate, renal, thyroid, and uterine cancers.[Ref]

General side effects have included acute infusion-related adverse reactions occurring within one hour of the start of the infusion. The acute infusion-related events have included cardiopulmonary symptoms, such as hypotension, increased blood pressure, and dyspnea. The most frequently reported events were dizziness, headache, and hypertension. Other symptoms reported as mild to moderate included nausea, flushing, urticaria, cough, hypersensitivity, pruritus, rash, and wheezing. Back pain and pain in extremity have also been reported.[Ref]

Immunologic side effects have included the development of binding antibodies to the entire abatacept (the active ingredient contained in Orencia) molecule or to the CTLA-4 portion of abatacept. No correlation of antibody development to clinical response or adverse effects has been observed. Streptococcal sepsis (0.4%) has been reported.[Ref]

Gastrointestinal side effects including nausea (6.6% to 11.5%), diarrhea (5.8% to 6.91%), diverticulitis (0.4%), peridiverticular abscess (0.4%), and dyspepsia have been reported.[Ref]

www.drugs.com/sfx/orencia-side-effects.html


Also, although IVIG does provide protection from infection, it does not provide complete protection. Please do not be lulled into a false sense of security.

Despite regular Ig therapy and the achievement of trough IgG levels thought to be protective (i.e. >7 g/l), PID patients continue to experience infections, particularly of the respiratory tract, as confirmed by the registry data presented above. The addition of prophylactic antibiotics does not prevent respiratory infection completely, which occurs predominantly with encapsulated organisms such as Haemophilus, Pneumococcus and Moraxella[54]. Rarely, patients on Ig replacement experience severe non-respiratory infection with organisms such as Neisseria meningitidis, even when trough IgG levels above 10 g/l have been achieved [55].

Polymorphisms in immune response genes and concomitant structural damage (for example, due to sinusitis or bronchiectasis) might confer ongoing risk for infection in patients on Ig therapy. Additionally there is a theoretical risk that Ig products do not always provide optimum levels of antibodies against encapsulated organisms. This could occur, for example, when plasma is sourced and used in different continents or different populations, either of which may differ in epidemiological characteristics. Alternatively, fractionation, purification or virus inactivation steps may reduce antibody levels quantitatively or affect their opsonic or complement-fixing function.

www.ncbi.nlm.nih.gov/pmc/articles/PMC2801032/

When making this decision, make sure you are informed and be sure to look at the big picture and how this choice may affect your entire future. Many patients and doctors fail to do that and only want to fix 'now'. I made that mistake and truly would like others to consider this when faced with a few options.

Thank you Sandi, this is useful information and should be brought up with my doctor. So helpful and appreciative.

Sandi, to me it seems you are a strong advocate of NPlate. Are you not? Are you seeing people just have better overall experiences with NPlate over the other alternatives?

Deln,

Sandy knows what she is talking about. You'd better think twice before taking the Orcenia as this can cause permanent lung problems. I think Sandy has experienced it with some drugs. I know my friend with RA has definitely experienced it with drugs treating her RA. Actually, the lung problems are worse than her RA now from these drugs.

They also seem to use N-Plate in a lot of different situation. Like in ITP patients getting ready for surgery.

6/12/2015

Good morning everyone,

Just another day. I'm in that "limbo" stage right now. I've been off Decadron (and all prednisone) since Saturday evening, which has been nice. However, I'm just kind of waiting for when I look in the mirror and see a blood blister on the mouth, then I know I've plummeted again.

I did some reading though, and it seems that Rituximab might be my next avenue. Patients with CVID have B cell issues, so it's likely that the Rituximab which will remove my B Cells could assist generally in sort of resetting this ITP issue?

Who knows really.

Deln:

Yes, I guess you could say that I am an advocate for the TPO's. Here is the reason. I've been on the PDSA nearly every day since 1998. I have read every single post and heard every single story. In all of those years, I have never seen a medication with as good a success rate as the TPO's, and I have never seen a drug with fewer side effects than the TPO's. Since they were released a few years ago, I have watched many people run down the line of treatments and many, after much time and frustration, end up on them anyway. That is when most people begin to report that their quality of life has improved and life became seemingly normal again. I wish it had been available when I was diagnosed and struggling. It took me 8 years of up and down counts, repeated steroids and Rituxan to achieve stability and remission. If the TPO's had been around, I would have gone that route. It seems much easier than the prolonged use of immunosuppressants and seems to have the least permanent side effects.

Rituxan is temporary for most. It has a 60% success rate and the average remission is a year. I got 13 months the first time, and counts were in the 60's/30's the last two months in that year. It kills off B cells and T cells, but they do reproduce eventually. You need the B cells because they are the memory cells that carry the antibodies that fight any viruses or infections that you've ever been exposed to. If memory serves, Rituxan only kills the mature B cells; the immature ones, or baby ones, survive, grow and repopulate. That is why Rituxan is generally temporary. During the window of time that most of the B cells are gone, patients are susceptible to infections. Most do fine unless they are already immunosuppressed for other reasons. Some people have had longer and even permanent remissions from Rituxan after undergoing a few rounds over several years time. But....some people do not respond to it at all.

I wouldn't tell you that Rituxan is a bad way to go, but when I say to look at the big picture, I'm saying that the accumulation of all of these immunosuppressants over a period of time can raise the risk for cancer and other organ damage down the line. It all boils down to benefit vs risk and the only way to decide is to seriously look at both sides carefully.

Thank you Sandi.

I am a complicated case though. I have CVID, and actually some 15 months ago I had a bout of Hemolytic Anemia. That went away on its own in a couple weeks without treatment.

I don't have good B Memory cells in general, because of CVID I believe, so losing them is not really a problem. The IVIG is there to provide supportive protection.

This is difficult, I am especially concerned now about the event of Hemolytic Anemia returning at some point.

What a mess.

Deln:

I understand that you are different. I am too because I have Lupus. I have to be careful of certain things also. Patients with Lupus also have an increased cancer risk, so after trying and failing several strong immunosuppressant meds, I decided to stop that Merry-Go-Round because I began to be afraid of acquiring cancer. I also ended up with lung damage from one of them which now restricts my activities.

I don't pretend to know much about CVID but if you already have few B cells, I don't understand how Rituxan would work well for you. I haven't been able to find any articles that report Rituxan as being beneficial for CVID. I'll keep looking though. Because you have CVID, I'd want to research the potential benefits or risks before proceeding (if I were you).

I did find this:

www.jaci-inpractice.org/article/S2213-2198%2814%2900236-0/abstract

I'm really finding it tough to stay positive. I feel like nothing will work for me, either Rituximab or NPlate. Even if NPlate works, I may be stuck on it for life and or it could potentially have an effect on my CVID issue, but nobody knows the side effect long term.

I'm just so frustrated right now. I have a feeling my platelets will be under 5,000 when I have my doctor visit on Monday.

Such a defeating feeling. It's tough too, when you have friends and family around that you see and they are "normal" with no health issues. I would love to feel care free again. That may or may not happen.

Deln
I totally understand how you feel. I see someone smoking and want them to get chemo and get so angry that they are choosing disease and I did nothing wrong and have ITP. I see people at the grocery store and get mad that they are all "normal." Of course I do not know what they are dealing with in reality, but it still makes me mad and sad. I feel cheated. The reality is that you still have to make it from sunrise to sunset every day and have to figure how to do that. Steroids mess with your mind. I know you will feel better once you can get them out of your system. Read your earlier posts. You are running a marathon, not a sprint. It all stinks, but you still need to get up every day.

You will be normal again. You will! I know you can't see it now, but in the scheme of things, ITP isn't as bad as it seems at first. I also felt the way you did, but looking back, I realize that it wasn't as bad as I was making it out to be. You only gain that perspective in time.

You know what? You can't judge a book by it's cover. I look normal too. But my face is permanently Cushing's and that will never change. People that see me probably just think it's weight. I went to a wedding tonight and had to watch people dance...I can't do that any more. I had to watch people karaoke. I can't do that any more either. I am facing losing the use of my hands. But I look normal. It's easy to fool people. I'm trying to find the positive in my life right now and I know there are people that have it worse than I do. It's not easy to do and yes, I'm struggling with it just as you are. Bad things happen to good people. It stinks, but that's just the way it is.

My niece got married tonight. She was born with cerebral palsy and has spent her entire life in a wheel chair. She needs help with the simplest of tasks. But she got a PhD in psychology, works every day, has gone sky diving, drives a modified van and got married today. None of us could have imagined all of that for her when she was a child. She made the most of her life and did not allow anything to limit her. If she can do it, I can. She does not look normal, but is one of the happiest people I know. Look for the inspiration. It is out there some where.

Thanks Sandi, you make good points. Everyone in life has struggles and hardships, I understand nobody is immune from it. Life has difficult.

I think the tough thing about ITP is the uncertainty and lack of predictability.

Tomorrow is a big day. I'll get more information about the next step. I imagine my platelet count will be under 10,000 platelets tomorrow when thru draw mg blood. I'm also getting IVIG tomorrow. It's funny but I had a theory. Maybe me getting sick would be a good thing. Maybe if would take my immune systems' attention away from my platelets and attack the new sickness?

I wonder if there's validity to that?

Some people do experience a rise in counts when they are sick, but it's usually temporary. Some people also drop during an illness.

Deln, you know what to expect for your next count, so I know you won't be surprised by it. You will find something that works. This is just a speed bump in life and you will get past it. Even if you don't get past ITP itself, you will get past the feelings of sadness, anger and despair. Every single person learns to live with it no matter what the outcome. It will become 'old hat'. I have not come across a person yet who has lived with fear forever because of ITP.

Nearly every illness has uncertainty. No one knows what is in store for them after any diagnosis. ITP is not known to be terminal. It is not known to be debilitating. It can usually be managed well (although not easily at first). It has some good points, if you can see it that way. Some people actually have learned things about themselves and about life that they wouldn't have learned otherwise. This experience will be what you make it.

Good luck tomorrow. No matter what happens, you will be okay. You will deal with it. Is there any other choice?

6/15/2015:

Met with Doctor today and got counts. At 17,000. So a small drop since last time's counts, though I imagine the Decadron is wearing off in my system and (like last time) I would expect that within the next seven days, I will be back down to single digits.

First, the Doc and I agreed we aren't treating me with anything unless I'm symptomatic. Thus, if I'm at 17,000 next week, or 10,000, or maybe even 5,000, I won't take anything unless I have blood blisters, etc....

Second, I mis-spoke in earlier posts. I was NOT taking 80 mg Decadron a day, it was 40 mg. We are going to reduce that to 30 mg. The plan then, is to take another round or two if I become symptomatic.

Third - After the Decadron, Ritux is the next avenue for us. It's worth the try.

Fourth - We spoke about Orecenia. This seems to be a very solid option. Unlike Ritux for instance, which targets the B cells, Orencia targets and "calms" or 'slows down' the T Cells. The doctor suspects that the ITP here is likely due to T Cell issues rather than B Cells. In that case, Orencia would likely work well. He said the drug is safe, and that many patients have been on this for awhile and there aren't any "major" side effects like cancerous, etc....

Fifth - NPlate was discussed. It's not that he doesn't 'like it' per se, but he said it really doesn't address the root of the problem. It just assists in getting the platelet count up, and certainly isn't opposed to it if I want to try this option. But, he said it doesn't really address the underlying problem.

We discussed the length of ITP. It is unknown. He said the pattern is that 'typically' this could last between 6 months - 1 year. However, there's no real prediction available. It could be lifelong, it could end next week. Each case is different and the body reacts differently.

Deln:

The treatments that supposedly address the underlying problem are usually just temporary solutions. You've chosen a fairly unexplored and unheard of route of treatment so this should be interesting to follow. N-Plate must do something more than just raising counts because patients are getting remissions. I haven't read anything about how long they last though.

Deln,

Does your doctor treat very many other ITP patients? I know your main problem is the CVID--but he also has to treat the ITP. I am saying that because most ITP experts (from PDSA Conference) do not use 30 mg of Decadron (at least I haven't seen it documented). This could cause you problems and cause your to relapse if you do not take the proper dose. Maybe Sandy can check with the doctors on the board.

I agree with Sandy, again, as I do not think you are getting standard treatment. I had to get off the FB PDSA site as it was really getting disturbing. Their treatment is all over the board.

Good luck to you and please do check into this.

Here's a good video from Dr. Michael Tarantino from the PDSA FB site: You have to copy and past in your URL:

pdsa.org/media-center/tv-a-video/item/1163-bleeding-and-clotting-disorders-institute-patient-support-for-itp-q-a.html

Hi Dee and Sandi,

Regarding NPlate, the issue is, even with other treatments, is that people's bodies may resolve these things on their own. Whether it's the treatment, a combination, or time, one may never know.

I think his view of NPlate is that it's only assisting in producing more platelets and raising numbers on the back end, but the T Cell / B Cell issues still remain, bogging the body down.

Don't get me wrong, he isn't against NPlate and if I said I wanted to try if, he'd do it. But addressing the T Cell and/or B Cell issues is actually addressing the ITP core problem at the root.

So, the T Cell drug ( Abatacept ) could be more useful in addressing the issue.

I'm not going to take that just yet though. To me, that is months away.

Regarding Decadron. The 40 mg is doable for me. I was just telling him how I was extremely grouchy and moody, etc... So he said 30 mg would be doable since I respond to Decadron, but perhaps 30 mg will suffice to boost me up for a 10-17 day period.

If I'm not mistaken, that other Galaxy's thread discussed her husband or boyfriend taking 8 mg for 4 days?

I'm not sure.... I'm obviously still in flux regarding when to try Rituximab. That is my next step and it's worth a shot. But maybe I can hang off for a couple months longer and hope the Decadron and or my body recovers

If you try Orencia, please be sure and let us know how it goes. Maybe it is a great option we should know about. I look forward to hearing how thing continue for your treatment.

I wish you all the luck in the world, Deln, but if B Cell/T Cell treatments always got to the root of the problem, they would would work 100% of the time and never need repeated. It doesn't work that way.

I'm the same as Deln with having CVID and the Nplate put me into remission as far as the platelet count goes so I'm not sure the doctor is on the right track there. The CVID is a genetic thing thre since birth and I can't see the body suddenly righting itself from that. I really do think that managing the condition rather than curing it is the realistic option.

Deln, do you see the same doctor for the CVID and ITP? I see an immunologist for one and a haematologist for the other, and I find that they really do not know each other's specialities in great depth. For example, the immunologist has sent blood off to look for a Zap 70 deficiency while the haematologist didn't know much about that.

Hi Ann,

So we are similar with our CVID and ITP issues, huh? What is your background with CVID? When did it come about for you, what symptoms were/are you having. What types of treatment or information have you discovered about your own, personal CVID issue.

That is great to hear that NPlate put you in remission from ITP. What was your NPlate treatment plan, if you don't mind sharing?

The Doctor I see (he is actually listed through the PDSA link when you 'search' for Hemos.) I primarily began going to him for my CVID, but he is also versed with ITP as well.

Here's my experience. ITP for 9 years. Nplate trial some years later. It took seven months to get stable on it and then remission followed later. That's why I get a tad frustrated when people give Nplate two weeks and give up!

Six years later neutropenia after a series of hepatitis B vaccinations, further investigation also found low IGs, along with almost absent IgA, and the haemo referred me to an immunologist who then also found very low CD8 lymphocytes as well as the usual lack of antibody response.

Symptoms - constant ear infections since childhood resulting in some hearing loss and tinnitus. Lots of chest infections. A type of cancer some years ago that is usually only seen in the elderly, when I was in my 20s. Interstitial cystitis on and off, once severely for almost a year. It's thought to be autoimmune. Now in the middle of having enlarged lymph nodes investigated. They've been enlarged for 6 months now.

There are a few genome studies going on in the UK just now. My blood has been sent off for this one. bridgestudy.medschl.cam.ac.uk/pid.shtml It takes ages and ages to get any results back though but we're hoping to get a reason for the lymphopenia which in some ways is more serious because it causes an inability to fight viruses which often can't be treated.

Enough...