Information help

Hi , I am new to this page , but not new to itp . My name is Amanda , my story is way to long to type , but after spending nights I can't sleep on research pages it arose some disturbing questions . I was diagnosed when I was 15 out of no where and maintain the count of 0 unless treated due to the anti bodies in my body that kill my platelets (not a production problem) . Throughout my teens I spent at least three days in the hospital or doctor and not much beside self management , trial and error treatments, and acceptance has happened since then . I am now 25 , other things in my health have been going sour over some time , for example stomach issues which are most likely from the millions of milligrams of steroids I've eaten over the years . Getting to the point now lol, I read in studies that because of the constant ness and my age when this all started that my life expectancy is only until I'm near forty . No one has ever really had this conversation with me before and I'm curious and just want to know if that's true , not sensitive and if someone knows anything at all about that , that they can share .. I would like that . Appreciate your time

Hi Agervase,

I doubt strongly that anyone can seriously predict your lifespan. I know somebody who has ITP for more than 25 years. I'm also convinced that long before you turn 40 some alternative treatment has been developed. There are at least three companies I know off that have promising ITP treatments in the pipeline. With all the progress possible now due to medical, biological and chemical progress now is the time to have ITP.

Keep your chin up!

Cheers,
Jorg

I agree..Which are the companies with new treatments in the pipeline ?

Hi Ager:

I'm happy that you found us!

I can assure you that having ITP does not shorten a lifespan. People with ITP live as long as any average person. Treatments can cause damage to the body over time though and yes, some people do die from bleeding or infection but that is very rare. Most people with ITP are healthy; they just have lower than normal platelets.

I'm curious to know what studies you've read? I've been reading about ITP daily since 1998 and have never come across anything like that.

I'm happy I have too !! I wish I remembered the page to send you but I didn't save it , wasn't the best to read. I just remember the name Cohen.

Definitely not true! These articles are a bit older...from the late 1990's and early 2000's, but the outlook for ITP patients is even better than it was back then with the newer treatments.

In conclusion, most adult patients with ITP have a good outcome with infrequent hospital admissions and no excess mortality compared with the general population. However, patients with persistent severe thrombocytopenia not responding to therapy within the first 2 years have considerable morbidity and mortality.

www.bloodjournal.org/content/97/9/2549?sso-checked=true

Abstract

ITP (immune thrombocytopenic purpura) is generally considered a benign disease. Despite what may be severe thrombocytopenia, most patients with ITP do not suffer significant bleeding episodes. Mortality is rare, and the majority of cases are managed successfully with conventional treatments. For patients who maintain a platelet count of > 20,000/microliters, aggressive immunosuppressive therapy is usually unwarranted. There are some patients with ITP who are at increased risk of serious morbidity and mortality from their disease. Fatal cases of ITP are rarely mentioned in published reports. In this study we review the incidence of mortality and describe the deaths of seven patients with ITP, with the aim of identifying and improving management of high-risk cases. Based on our review of the literature and analysis of cases at our institution, fatal cases of ITP fall into three groups: death from intracranial hemorrhage with severe thrombocytopenia, death after splenectomy, and death due to infection after cytotoxic treatments. Patients who appear to carry increased risk include: 1) older patients; 2) chronic, refractory patients with a history of hemorrhage; and 3) patients with concomitant bleeding diatheses such as uremia and hemophilia.

www.ncbi.nlm.nih.gov/pubmed/8172178

Agervase wrote: I'm happy I have too !! I wish I remembered the page to send you but I didn't save it , wasn't the best to read. I just remember the name Cohen.

I read that study and several others from back then. They are disturbing, and still often mentioned on blogs and personal sites. Fortunately, as Sandi pointed out, they are quite out of date. There has been substantial progress, and little reason to plan an early demise. Importantly, a small subset of ITP patients, who can now be identified early, are at greater risk, and so can be monitored more closely and treated more aggressively, which should improve their outcomes too.

Keep in mind that a study reporting outcomes of ITP patients that was published in 10 to 15 years ago is based on data from the 1970s through 1990s. That's just too old to be useful at this point. Instead, we need studies looking at data from the 2000s onward, and the ones I've seen strongly suggest that life expectancy is not notably affected.

This one *is* from 2000, by Cohen.

www.ncbi.nlm.nih.gov/pubmed/10847256

"Idiopathic thrombocytopenic purpura with persistent low platelet counts carries a grave prognosis."

Nice!

In fact it is now known that more people die of the treatments than the ITP.

This is a reply to Cohen. www.bloodjournal.org/content/98/7/2282?sso-checked=true

I haven't read it all but it looks interesting.

This may also be relevant. In the the Feb. issue of the journal "Blood" I found:

Platelet transfusions in platelet consumptive disorders are associated with arterial thrombosis and in-hospital mortality.

While platelets are primary mediators of hemostasis, there is emerging evidence to show that they may also mediate pathologic thrombogenesis. Little data are available on risks and benefits associated with platelet transfusions in thrombotic thrombocytopenic purpura (TTP), heparin-induced thrombocytopenia (HIT) and immune thrombocytopenic purpura (ITP). This study utilized the Nationwide Inpatient Sample to evaluate the current in-hospital platelet transfusion practices and their association with arterial/venous thrombosis, acute myocardial infarction (AMI), stroke, and in-hospital mortality over 5 years (2007-2011). Age and gender-adjusted odds ratios (adjOR) associated with platelet transfusions were calculated. There were 10 624 hospitalizations with TTP; 6332 with HIT and 79 980 with ITP. Platelet transfusions were reported in 10.1% TTP, 7.1% HIT, and 25.8% ITP admissions. Platelet transfusions in TTP were associated with higher odds of arterial thrombosis (adjOR = 5.8, 95%CI = 1.3-26.6), AMI (adjOR = 2.0, 95%CI = 1.2-3.3) and mortality (adjOR = 2.0,95%CI = 1.3-3.0), but not venous thrombosis. Platelet transfusions in HIT were associated with higher odds of arterial thrombosis (adjOR = 3.4, 95%CI = 1.2-9.5) and mortality (adjOR = 5.2, 95%CI = 2.6-10.5) but not venous thrombosis. Except for AMI, all relationships remained significant after adjusting for clinical severity and acuity. No associations were significant for ITP. Platelet transfusions are associated with higher odds of arterial thrombosis and mortality among TTP and HIT patients.

PMID: 25588677

Although this article is looking at outcomes in TTP, HIT, and ITP, note that at the end of teh abstract, the authors state that "No associations were significant for ITP". In other words, platelet transfusions did not lead to an increased risk of mortality for people with ITP.

I'm still looking for a current article on ITP and life expectancy, but it may be that the treatments are evolving so fast that such an article is difficult to produce.

Ann:

I'm stunned! This sentence takes the cake: " A 30-year-old woman remaining thrombocytopenic due to ITP was predicted to lose 20.4 years (14.9 quality-adjusted life years) of her potential life expectancy. At age 70, predicted loss was 9.4 years (5.0 quality-adjusted life years)." Unreal. Now I have to go and read the second article.

Aoi:

I have seen that article repeatedly, but didn't make much of it since ITP didn't seem to be included. It is nice to know just in case another diagnosis were to come at some point.

At any rate, they are only talking about severe, refractory patients. That excludes most of the people here. Even refractory patients can do quite well with few symptoms.

"Portielje et al1 now show that these patients do not have their life expectancy (LE) compromised. But they also show that patients with refractory ITP with persistently low platelet counts (below 30 000/μL) have 4.2-fold increased mortality risk in comparison with the general population. In this population the authors calculated the death rate of 0.019 cases per patient-year at risk. They compared this estimate with our estimate of the fatal bleeding rate in patients with refractory ITP of 0.0162 to 0.0389 cases per patient-year at risk. Our estimate, based on pooled data available in literature up to year 1998, showed a considerable compromise in LE in patients with refractory, untreated ITP. But by analyzing the cause of death, Portielje et al1 pointed out that half of the deaths were due not to bleeding but rather to the lethal infections likely related to the treatment."

And yes, treatments contribute to mortality more than bleeding does. That is actually good to know.

Sandi wrote: At any rate, they are only talking about severe, refractory patients. That excludes most of the people here.

I like hearing that - now I don't need to run to the cemetery this afternoon and set things up.

What exactly do you mean when you say refractory ? Unmanageable like nothing works longer then a few weeks refractory ?

Agervase wrote: I read in studies that because of the constant ness and my age when this all started that my life expectancy is only until I'm near forty.

If you are thirty and have a life expectancy of forty years, that means you are expected to live 40 MORE years, to the age of seventy. It does not mean that you are expected to die at the age of forty. Google {life expectancy definition} and you will see what I mean.

I made the same mistake as you at first. I always thought life expectancy meant the expected age at death, and I have seen the term used that way, but the correct usage is as a measure of the expected number of remaining years of life.

Agervase wrote: What exactly do you mean when you say refractory ? Unmanageable like nothing works longer then a few weeks refractory ?

"Refractory" is a relative term: it means that the ITP is unresponsive to a particular treatment, as in "refractory to steroids" or "refractory to splenectomy" or "refractory to the usual treatments". When reading an article one always must try to ascertain how the term "refractory" is defined by the author.

Often, "refractory" means that neither steroids nor splenectomy results in a lasting remission.

'Refractory' usually means that a person does not respond to any treatment and they have continually low counts. 'Severe' means that symptoms are present when counts are down. So, severe refractory includes patients who have bleeding and cannot get counts up.

Using the wrong terminology can cause issues, that said even doctors have been known to use the wrong terminology. Wrong terminology used on me.

After 1st treatment counts up Doctor said I was "cured" nope just in "remission".

After many years multiple treatments Doctor said I had "refractory ITP" nope just "chronic ITP" because I respond to treatment.

Counts dropped 6 weeks after Winrho Doctor said treatment "failed" nope just "wore off".

Here is a blue ribbon panel attempting to define various terms related to ITP:

www.bloodjournal.org/content/113/11/2386
March 12, 2009; Blood: 113 (11)
Standardization of terminology, definitions and outcome criteria in immune thrombocytopenic purpura of adults and children: report from an international working group
Francesco Rodeghiero1, Roberto Stasi2, Terry Gernsheimer3, Marc Michel4, Drew Provan5, Donald M. Arnold6, James B. Bussel7, Douglas B. Cines8, Beng H. Chong9, Nichola Cooper10, Bertrand Godeau4, Klaus Lechner11, Maria Gabriella Mazzucconi12, Robert McMillan13, Miguel A. Sanz14, Paul Imbach15, Victor Blanchette16, Thomas Kühne15, Marco Ruggeri1, and James N. George17

"Refractory patients should fulfill 2 criteria. First, they should have failed splenectomy or have relapsed thereafter. Second, they should either exhibit severe ITP (see Table 1) or have a risk of bleeding that in the opinion of the attending physician requires therapy."

These definitions are used inconsistently.

Bonbon was refractory - IVIg worked to get her counts up to decent but only a very short time. And she had to have the same brand of IVIg each time and I recall that was getting hard to come by.

Refractory, refractory - same as remission, remission. It's all in the eyes of the beholder so to speak.

What I don't like is what I'm going to call the "scare factor" - the you are going to die earlier than normal because you have ITP bit. Seriously - if I was going to lose 20+ years off my life thanks to ITP I'd be dead already.

"Idiopathic thrombocytopenic purpura with persistent low platelet counts carries a grave prognosis."

I'm not going to read that article - refuse. But what is "...low platelet counts"?? I have persistent low platelet counts. Are they talking a count of 1k, 100k, 52k, what is this article considering "low platelet counts"?

It is bad enough we have this to deal with (and some of us have added health problems) - but to be told we are going to lose some years off our life because we have ITP - we don't need. There are some here that will roll over and think their life is over because they have ITP when it isn't over.

Julia - that was an LOL post! My chuckles for the day. :woohoo: You are so right and cracked me up!

Melinda - I think "low" counts in this instance refers to "low enough to cause severe symptoms". In that case, it can be more serious. Thankfully, treatments are more successful these days for those who used to be considered refractory, but there are still some people who do struggle with very low counts. Yvonne may very well have responded to the TPO's and it's too bad we will never know. She was still here when they came out because I remember her arguing that her doctor said she didn't have a production problem, therefore, the TPO's were not an option for her. That couldn't be farther than the truth (as we now know). I really wish we could know how she's been doing.

Two ways ITP can shorten life:

1. Having severe, refractory ITP that results in bleeding to death. Very rare. I think I read .019%. That would abruptly end life.

2. Toxicity of treatments. They talk about dying from infections from treatments. Splenectomy + immunosuppressant = higher risk of something such as sepsis. We are aware of that risk and know to watch out for it. Another example of a quick, unexpected death. But what no one mentions is the wear and tear on the body from years of treatments. This could possibly shorten the life span. All of these immunosuppressants that we pound ourselves with weaken the immune system which lowers the body's defense against cancer cells. You treat ITP for five years and twenty years later get cancer. Coincidence? Maybe not. It's definitely something to think about when making treatment choices and over-treating just to keep counts normal. I don't think there are any studies about that. But yes, I do believe in that manner, a lifespan could be indirectly shortened because of having ITP.