New to ITP, my story attached, have questions

sandi thanks for that info

Paul - you might get more info if you started a new thread.

Hey all, just a short update. Ok, it was supposed to be short...

10 days with a 2/3 dose (doc was cautious due to WinRho reaction) of IVIG, platelets are at 99k, which is down from 126 a week ago, but I also have a mild cold. Not complaining at all about the results.

I am scheduled for a full dose of IVIG on 12/17 and we are going to measure my platelets frequently to see where the drop off occurs. Hopefully I get +3 weeks and high counts out of it.

Thanks to the forum participants (Thanks!) I did inquire about several items when I saw my hemo:

Quinine- As a former moderate but regular drinker of vodka tonics I did ask about the possibility of quinine as a cause and the availability of a quinine antibody test. My hemo expressed that quinine is most frequently linked to TTP (which, if you haven't read about it, is really bad news).
My hemo wasn't necessarily aware that quinine can cause ITP and he had never heard of a quinine antibody test.

Iridium test- My hemo had "heard" of it, but wasn't aware of anywhere it could be obtained, and the impression I got from him was that it would be a waste of time.

Rituxin/HepC- My hemos interpretation of B-cell activity was different that what was presented earlier in this thread, thought he said the general thought was possibly valid. He still felt as though the risk of HCV reactivation (with neg viral loads) was incredibly small and possibly worth the risk.

Yes, I found all of these answers to be disconcerting. Yes, my hemo has answered a frustrating number of my questions with "I don't know", and has even appeared to be mildly exasperated with my questions at times.

Here's the catch- this guy (who I like personally, and who does appear to legitimately care) is supposed to be the best guy in town for platelet disorders. He's also light years ahead of the first (full-blood idiot) hemo I went to.

I'm beginning to think I am simply expecting too much out of an individual hematologist. I don't mean to suggest that I ever thought they had the capacity to fix me (us) just by waving magic platelet wands, but I perhaps naively thought they would have a greater curiosity regarding cause.
Rather, it seems (in my moderate experience with two hemos) that their general scope of practice is really limited to picking from a list of available medicines and for the most part prescribing palliative treatments as so little of what is available could be described as curative.

I suspect I've watched too many episodes of House, but I am really astonished by the absolute lack of curiosity about the cause of ITP in an individual, and particularly the specificity of an individual's symptoms. It surely must mean something that some people bleed while others don't, and that some people bruise or have purpura and others don't, etc., but for whatever the reason these are considered idiosyncratic rather than being relevant data points.

I really am beginning to think that ITP treatment is in the absolute stone-age compared to what I've seen Western medicine be capable of in other circumstances.

Sorry, not meaning to rant, but I'm tired of being told "I don't know" to questions that appear reasonable to everyone except hematologists.

Real-time update on my quest for a solution:

The medical records relating to my first autoimmune issue (1993) have been delivered to my GPs office and I am going over them with her on Thursday (12/16). I am hoping that they, combined with my HCV and ITP records with provide enough data points for something meaningful to be concluded.

With these records compiled I am going to make my appointment at Johns Hopkins for early January and push like hell for somebody to get curious about it.

Thanks for listening and thanks again for the input. I'll update again on Thursday after I find out what I had in 93'.

KO

Are you kidding me? Your hemo isn't aware of quinine/ITP? There most certainly is an antibody test and quinine induced ITP is widely known. Yes, I know about TTP and it is a very, very scary disorder that makes ITP look like a walk in the park.

www.psbc.org/lab_platelet/test03.htm

The Indium is done in the UK, not in the U.S. It can be a waste of time and the only thing it can do is save the spleen if it's determined that the liver is destroying platelets. One woman here had it and did not get a splenectomy for that reason. If the test does determine that platelets are destroyed in the spleen, that doesn't mean that the liver won't take over that destruction in the future and the splenectomy can fail.

KO - again, the cause doesn't matter. They can't fix it. As I said before, none of the 88 autoimmune disorders have known causes or cures. ITP is no different. All that is available are treatments. I don't like it either, but after researching for 13 years, it is what it is. What other illness are you seeing that have better treatments? Actually, ITP treatments have advanced tremendously in the past 10 years. For the longest time, the only things available were Prednisone, IVIG and splenectomy. ITP has come a long way. I have Lupus and my daughter has Graves and those treatments are just as nasty and barbaric.

I posted this on the old forum; it might be helpful:

When diagnosed with ITP, most of us keep asking why this happened. We blame ourselves, blame medications, blame foods, etc. We can't accept the fact that there is no real explanation or fix. The summary below partially answers the question why and shows why ITP, and most autoimmune disorders, are more complicated than we realize, and why you can't just cure it.


"Gene copy number variation (CNV) and single nucleotide polymorphisms (SNPs) count as important sources for inter-individual differences, including differential responsiveness to infection or predisposition to autoimmune disease as a result of unbalanced immunity. By developing a FCGR-specific multiplex ligation-dependent probe amplification assay we were able to study a notoriously complex and highly homologous region in the human genome and demonstrate extensive variation in the FCGR2 and FCGR3 gene clusters, including previously unrecognized CNV. As indicated by the prevalence of an open reading frame of FCGR2C, Fc receptor (FcR) type IIc is expressed in 18% of healthy individuals and is strongly associated with the hematological autoimmune disease idiopathic thrombocytopenic purpura (present in 34.4% of ITP patients; OR 2.4 (1.3-4.5), p<0.009). FcRIIc acts as an activating IgG receptor that exerts antibody-mediated cellular cytotoxicity by immune cells. Therefore, we propose that the activating FCGR2C-ORF genotype predisposes to ITP by altering the balance of activating and inhibitory FcR on immune cells."

bloodjournal.hematologylibrary.org/cgi/content/abstract/blood-2007-03-079913v1?ct

Sandi wrote:

KO - again, the cause doesn't matter. They can't fix it.

I think you can say this as a matter of personal statement - but there are those who have treated it successfully without drugs that disagree. ITP can be fixed (as in cured - not remission) and there are many people that have been on these boards that can attest to that. As can other auto-immune diseases (I know too many personally that have fixed them).

So the only thing I can say KO is what I said above. With 80% of the immune system in the gut, that's where you start. Triggers can be food allergies, chemical exposure (environmental), drug exposure, etc. Benadryl use was a pretty large factor in our case. As was food allergies. Both of which mess with the gut.

Anyways, don't give up. I understand your frustration though. But what you're finding is exactly what allopathic medicine is. They don't really care what the cause is - just throw a drug at it and treat the symptoms. It is what it is.

Best wishes,

patti

KO - I had to stop watching House. He is very amusing, but the show was so full of medically incorrect drama that I got too frustrated. There was a show once about ITP. It was laughable.

Patti - just exactly what would you see a doctor for? I've been curious about that. Would you treat everything naturally or would you actually see an MD for something?

If IVIG works, DO NOT get a Splenectomy... Honestly, there's no need.

What does it mean to say '80% of the immune system is in the gut'? How is the immune system measured to come up with a statistic like that? What percentage of the immune system is the white blood cells? What percentage of the immune system is our tonsils? Is it by weight? By volume? I saw that statistic repeated over and over on non-scientific internet sites. That doesn't make it true.

I happen to agree that gut-related issues can make immune problems worse (or fixing them might make them better) - but I object to sloppy statements like that. Also we don't use just 10% of our brain but a lot of people have certainly repeated the statement that we do. Repeating it doesn't make it true.
Erica

Referencing the idea that "cause doesn't matter because they can't fix it-"

Here's my logic, lest anyone think I'm on some kind of Quixotic quest.

For many people ITP appears to be an isolated diagnosis- they had blood platelets and now the don't. In the absence of any other issues (which I respect that many others do have), this is the diagnostic equivalent of Vanna turning over one letter in a four word phrase on Wheel of Fortune. Yes, it increases the possibilities for discovery of the phrase, but not in a statistically meaningful way.

However, with a few more letters being turned, or in my case data from two past autoimmune conditions (HCV and the 1993 mystery), the possibilities for discovery of a cause or at least a meaningful research direction increase exponentially. This is what I was saying about data points; they are pieces to the puzzle and the more you have the more clear the picture becomes.

I am not naive; I understand that many diseases are like avalanches; it doesn't matter what started them because that information isn't useful in stopping it. And this may be where I wind up.

That's a realistic possibility. However, I am going to keep pushing until it's clear this is the case. I think there is enough about my situation that is atypical (this doesn't mean that I think I'm 'special') that I'm hopeful that I can make some headway with it.

To put it another way, I don't have anything better or more important to do. I have three kids under 6 years old, and I feel like it's incumbent upon me to go war over this and not just sit back and appease it with mediocre palliative treatments.

I'm going to investigate every possibility, and I think there might just be enough data points for a sufficiently skilled and interested doctor or team to get a foothold.

We'll see. I don't have any particular pride over this approach; I understand it is still statistically unlikely but it's what makes logical sense to me.

You only get one life; this isn't a dress rehearsal for something else, and I'm not going to sit back and let the quality of mine wane without a fight.

KO,
I don't believe that cause doesn't matter (at least sometimes). For example, my ITP is secondary to lupus. Therefore if the lupus is under control, the ITP might be controlled. I wish the drug companies would break down results by primary ITP (idiopathic) and secondary (known or believed to be a result of some other condition), I suspect certain treatments are more or less effective based on this cause information. I've asked drug company reps at the PDSA conference if they can tell me a breakdown of results for patients with lupus and they can't.

The mechanisms of the ITP treatments are so different, it makes a lot of sense that there could be interactions between the mechanisms causing the disease and the mechanisms of treatments that work and don't work.

I'm not aware of any lupus patients with secondary ITP who have used the platelet stimulating drugs (nPlate, Promacta). One thing I watch for as I read this board are 'patients who seem sort of like me' in terms of their medical condition, and what works and doesn't work for them.

You might want to explore support groups for your other medical conditions, they might have sub-folders like we do that cover paired issues (like lupus/itp or for you, HepC/ITP, etc.)
Erica

KO:

Technically, HCV is a virus, not an autoimmune issue. A virus however, can trigger an autoimmune issue.

I wouldn't discourage you from pursuing the answers to your puzzle, but we all have puzzle pieces here. I can also dig though my entire history starting in 1982 (my first odd test result) and find pieces that would certainly fit my situation today. I've been to many doctors over the years and the general consensus is that yes, those pieces do fit my current diagnoses. There's my answer.

Dr. House's goal every week is to find the diagnosis. He does that in an hour and sometimes he nearly kills the patient with his unethical diagnostic tools. He doesn't always cure the disease.

I am very interested in your story and would love to hear the steps you take and the information you learn. I think this discussion is interesting and it does make us all think. Of course giving up on your health is not an option and I always advocate for people to push for themselves and research for themselves. As I've said before, good luck! Keep me posted.

Ok, just received the medical records from my stay in the hospital in 1993.

It was a suspected autoimmune issue. Erosions in my esophagus that were compatible with herpes esophagitis. The report reads that in the absence of any known immunosuppressive illness, this would be a very unusual illness unless I were immunosuppressed.

From this, the doctors, rather shockingly (to me) concluded I must have AIDS. I had no risk factors, and was in fact in the army reserves at the time and was tested regularly. I knew I didn't have AIDS/HIV, but the doctors were insistent at the time that I must have it in order to explain the esophagitis.

Quote from report:

"Things that need to be considered in this situation are HIV related disease with resultant immunocompromise, other things that can cause immunocompromise and lead to this problem are diabetes or other underlying malignancy."

When the HIV and herpes test came back negative the doctors ultimately concluded that this was in fact pill esophagitis from Doxycycline. The only problem was that the Doxy was administered to treat the esophagitis I reported...

In re-reading this it sounds like the doctors were ultimately at a loss to explain it, but it went away and the HIV and herpes tests were negative, so they let it rest.

It seems clear that there was an immunosuppressive issue present, even if they were not able to identify in it with the technology available at either this time, or at this hospital.

It will be interesting to see if Johns Hopkins can make any use of this.

Patti and Sandi,

I realize I'm new here, but I am trying to get some serious input from people experienced with ITP, yourselves included, but this dispute you are carrying on is distracting from the focus of my thread.
While I welcome any input either of you have, I would respectfully ask you to please perhaps start a separate thread where you can continue discussing the merits of your obviously differing perspectives.

I hope you find this to be a fair request.

KO: when that happened in 1993, did you have low platelets?

This what the report says:

WBC- 6700
HCT- 47.3
Platelets- 182K
Electrolytes and liver panel normal
Absolute Lymphocyte count- 1000

Historically as I've looked through my past medical records my platelet count appears to consistently be on the low side of normal (150- 190K).

I wish I could explain it, but it doesn't make a whole lot of sense, I agree with you on that one.

It will be interesting to see how you do on a full dose of IVIg -- (as you know) that is typically short acting stuff, but it has sometimes caused longer remissions. At least you know that, in a pinch, you can get a quick (if frustratingly short) boost in counts if you need it. IVIg is the only treatment I have ever found that gives me a boost. My boosts last for usually 2-3 days before the counts drop.

I totally agree with your comments about the state of ITP care. Most hemoncs don't run across it enough to be doing much more than trial & error on treatments. And, I think that is compounded by something Erica said -- ITP is probably more than one disorder that all get lumped together under one name because we don't understand them well enough.

Unfortunately sometimes you have to move around before you find a hemonc you "click" with. I've had a bunch over the years (including a really, really good one who retired). My current hemonc is not so much an ITP specialist, but he consults with the big ITP specialists (including Fogarty at UCSF) and he is very "curious" to use your word, and that's a good thing.

Best of luck tomorrow.

PS: I had heard a rumor that Fogarty had moved, and looks like that is true:

penn-medicine-physician-announcements.blogspot.com/2010/11/division-of-hematologyoncology-welcomes.html

He is a really smart guy (ignore that he looks like he is 12 in that picture) and does consultations.

KO:

Sorry to high jack your thread. Sometimes debates are a good way to learn other views, but I will refrain from continuing the homeopathy discussion. I don't have much more to say about it anyway. Been there, done that.

Posts have been moved.

KO, have you been to the site ITPeducation.org yet? There are podcasts there by hematologists who routinely research ITP and its treatments. The podcasts are not for patients, they are for other doctors, but I think you'd find them interesting. You do have to create a logon to access the information.

Re: the quinine issue, here's a snippet from an article that my hema just emailed me (at my request, since methylprednisolone does cause my platelets to drop).

Methylprednisolone-induced immune thrombocytopenia
Drug-induced immune thrombocytopenia (DITP) may occur after
exposure to many medications and is sometimes indistinguishable
from idiopathic thrombocytopenic purpura (ITP).1-3 When suspected,
the causative drug must be stopped and the platelet count
returns to normal in less than a week. Quinine is probably the most
common trigger of DITP in outpatients and vancomycin in
hospitalized patients.4 Typically, thrombocytopenia and hemorrhagic
symptoms occur after one week of exposure to the drug, but
they may occur within one day if the patient has been exposed
intermittently over a long period.

Great info. Much appreciated. Will follow up on. Thanks

You're very welcome. I hope the info is helpful. Dr. Leibman's webinar on pathogenesis looks particularly good, but I've only watched about the first 10 minutes of it.

Not sure what your hema quibbled with in my description of how rituxan works (I'm assuming it's my description he disagreed with). Here's some info that supports at least some of the info I provided. You can find more by googling rituximab memory b cells.

www.genengnews.com/gen-news-highlights/memory-b-cells-linked-to-ra-and-response-to-rituximab/36720271/

www.ncbi.nlm.nih.gov/pubmed/16447239

Hello,

I'm not sure what the tiny typeface is about, or if it will post this way or not, but here's my most recent update.

I received a full dose of IVIG on 12/17/2010 and my platelets were at 165 on 12/20/2010. One week later on 12/27/2010 they were actually at 175, though my hemo said not to get excited that they were higher because this was within the margin of error.

I have yet to experience any side effects from IVIG and the treatment is actually pretty pleasant as the prophylactic Benadryl hits me like a Wild Kingdom animal dart and I sleep for 6 hours.

I am having another CBC tomorrow, which will be at the 2.5 week mark, and we'll see how durable of a treatment IVIG actually is.

I made my initial appointment at Johns Hopkins and the staff (so far) has been excellent and responsive. I did type up what I thought would be a short relevant medical history for the staff to reference, but it wound up running 4 pages. They did say it was extremely helpful, if verbose.

The only bummer is that JH is in such demand that my initial appointment is, um, May 5th, 2011. They have promised to try to accelerated this so perhaps I'll get in sometime in Feb through April, but I'll not hold my breath.

The IVIG is keeping me wonderfully stable and full of energy, and at this point I don't see a reason to consider a splenectomy.

I thank everyone for their support and will continue to update.

Thanks,

KO

Thanks for the update. I was wondering what happened to you!

Platelets at 127 this AM, so at 2.5 weeks the IVIG is losing some efficacy, but my platelets are still strong. We will be checking them again on Friday 1/7/11 to see where they are at and then have scheduled another treatment of IVIG for Tues., 1/11/07 with the anticipation that it will be needed at that point (3.5 weeks from last treatment).

KO27: I read your story and the thread.. seems that we have something in common, I'm also father of 3 kids, And determine to know some answers to your questions. What really cause ITP? I know there's gonna be cure for it maybe not now but soon...it's just that I don't have the money to go to different doctors and hospital to seek for more options,and internet is my option. I even emailed almost 30 different of clinical trials/clinical study for ITP for I to be come part of the study voluntarily and hope find a cure not just for myself but for others with the same disorder. Like you,when I asked my Hematologist I got the same answer. I feel that my hema doesn't really care and just want to make earnings.
For me,someone really could help me(us) is the person who's actually has ITP because he/she understands what it feels like to have such a disorder with a lot of questions.. Not like my Hematologist that all he do is smile everytime I ask questions that I think he doesn't know the answer. GOOD HEALTH to all of us!
keep us update for best answer..

KO,

Are you planning to get IVIG next week no matter what your platelet count is?

What is the lowest your count has been?

My hemo explained that IVIG typically stays in the body about 3 weeks, at which point it's efficacy drops. This appears to be the case as my platelets have dropped by almost a third in the last week.

I am having another CBC on Fri 1/7, and expect them to have dropped somewhat further.

If they have not, then I'll hold off, but assuming they are in the +/-60K range then it's only a (short) matter of time before I'm running on fumes. I know from experience that my human performance drops rapidly from 40K on down and I don't have a desire to go there.

If somehow my platelets are stable and above or near 100, then I will hold off until they drop.

My lowest platelet count has been 16K.

Below 40K I experience fatigue and brain fog and I have to be conscious of avoiding this.

Do you have any thoughts on IVIG?

Thanks,

KO

I do have some knowledge about IVIG but my hema suggested winrho but I can't afford both.. My lowest count was 2K..
I was admitted in the hospital:
Dec. 7, 2010 platelet count is 2,000 and had 8 units of platelet transfused.. Dec. 9, 2010, 8 hours of nosebleeding and had immediate 10 units of platelet transfused... Dec. 15, 2010 platelet count before I was discharged from hospital is 11,000... Dec. 22, 2010 platelet count is 18,000 and now got my latest CBC Jan. 5, 2011 platelet count is 17K..

I have not had IVIg, so can't comment specifically on that. There are a lot of things no one can predict about ITP, least of all just from reading someone's story online. All things being equal, it seems overly pessimistic to schedule a treatment when platelets are still in a very safe range.

Also, it's not uncommon for steroids to make you feel terrible, even days or weeks (or months) after you are off of them, so I guess I'd re-examine whether you're sure having a 40K count is the cause of the low energy you describe.

40K was my threshold for treatment for 2 years. I had had a couple of experiences where I dropped from the 40s to the teens quickly, and figured that was my MO, so treated at 40K. I also blamed low platelets for the mental fog that seemed to set in 6-8 weeks after WinRho, when my platelets slowly wandered down to the 40s and 50s.

Now I know I had some faulty assumptions. Understandable, but faulty nonetheless.

Knowing that you want to conquer this quickly and get back to your life, I'm just thinking maybe you don't want to schedule treatments based on assumptions.

I've been under 20K several times, and down to 3K once, if that helps put my opinions into perspective.

I'm glad WinRho helped you. It might be a stretch to say it almost killed me, but it wouldn't be much of a stretch.

I'm trying not to make any assumptions regarding when the next IVIG treatment will be necessary, but I am as well trying to be realistic with my expectations. Believe me, I'm as unexcited by the prospect of sitting in a chemo room for 7 hours as any sane person would be.

My problem is that, without sounding full of myself, my livelihood depends upon my ability to make sound critical thinking decisions and requires a lot of energy. I don't sit in a cubicle where, if I were fatigued to the point of paralysis, no-one might notice, either physically or results-wise.
I am a successful entrepreneur, have been self-employed for 11 years, and I can't fail to perform.

Now, whether the cause of my fatigue is a low platelet count or not, I cannot say with greater than circumstantial evidence as there is no Litmus test for this, but the correlation in my mind is absolute. When my platelets crater, I can identify a 'bone' tiredness, and shortly thereafter, when performing tasks that can be measured objectively, a cognitive sluggishness, which is frankly alarming.
This has seemed to be directly and inversely proportional to my platelet count, at least as it drops below 45K or so.

The only mystery I have is why I can have periods (1 to 2 hours) of 'bone' tiredness on days, such as today, when my platelets are more or less normal. I notice no brain fog and it only happens every 5 days or so, but it is an unexplained anomaly, perhaps attributable to diet.

I'm not going to even pretend to judge other people's choices (such as they may or may not exist), but I personally cannot imagine living with a platelet count of +/-30K. My life, and my family, require more from me than a platelet count such as that appears to permit.

I will take your caution against assumptions seriously and try to ensure the methods I'm using to evaluate my experience are as objective as possible, however.

I do think there are some unexplained aspects to my condition, and this is why I am proceeding to Johns Hopkins as soon as they will have me.

Your input is much appreciated. Thanks