New to ITP, my story attached, have questions

No one has expressed anything about Coombs; I'm not familiar with it.

The definition of Hemolysis is more or less red blood cell destruction, and that appears to be the case with me.

I'll find out more details this week.

Coombs are the antibodies that cause red cell destruction, sort of the diagnostic tool for Evans. I'm just wondering why your current hemo didn't pick up on it on the CBC. Odd. Maybe I'm missing something.

My existing Hemo lacks a certain curiosity about my condition, which has been frustrating. I have just been biding my time until I went to Hopkins, and I have not gotten the lowdown from what they found yet, just the briefest of summaries.

My current Hemo is also an Oncologist, and I would recommend to anyone with ITP that they seek out a Hemo that is NOT also an Oncologist because sympathy will be hard to come by.

I also think that Oncologists, more than any other doctor, are taught not to get vested in their patients for obvious reasons. So it's a double whammy of not appreciating the gravity of ITP because it's being viewed comparatively to an often fatal condition, and a general lack of empathy for the same reason.

In my case add a near total lack of intellectual curiosity about the condition and you have medical stagnation, which is why Hopkins was so gratifying.

We'll see what happens next. I'll research Coombs.

Thanks

KO27, sorry to hear about your condition. Hope you get better.

I remember reading about a member who has ITP & later also experienced some hemolysis but its PNH not ES. Now I am confused, are both ES & PNH related:huh: . So us ITPers may have some link with both ES & PNH. I need to read up more.

Hello everyone,

It's been a while and I thought I'd write with some updates. Quite a bit has gone on since my visit to Hopkins and I'll try to cover anything I've learned and as always I am grateful for your input.

I got the results of extended bloodwork back from Hopkins in mid-June and while I have some slight hemolysis (which is being treated with Folic Acid), I do not have Coombs or Evans. There was, I think, initially a bit of a miscommunication on this. The hemolysis might be a remnant of the reaction I had to WinRHo and when I revisit Hopkins on 10/27 this will be re-evaluated.

The doctor at Hopkins recommended I try Rituxin after having a advanced liver evaluation performed. The reason for the liver evaluation was because I had previously been diagnosed with the anitbody for HCV (Hep C), and while I never tested to have any measurable viral load (negative quantitative RNA analysis), I did not want to take the chance of Rituxin reactivating it (possible side effect) and making things that much worse.
The HCV diagnosis had always bothered me because I never had any of the risk factors and had absolutely no idea how I would have acquired it.

So I had the liver workup (something like 22 vials of blood) performed at the transplant unit of Florida Hospital. I'm not normally a fan of FH because it's an Adventist hospital, and while I have nothing against Adventists, I'm not real big on all of the Christian iconography (Jesus statues, Jesus paintings, Jesus photographs - I'm not kidding, he's wearing Tevas in the photos) that pervade the place.
That said, the doctors at the transplant unit were first class and performed (among many other things) a new Elisa-3 test.
The previous diagnoses I've had over the past 8 years were based on an earlier Elisa-1 or Elisa-2 test and I was reactive for the antibodies for HCV.
HOWEVER, the newer, more accurate, Elisa-3 test showed no reaction for the HCV antibody. NONE. So after 8 years of thinking I had HCV, I... don't.

After nursing my first hangover in 8 years I got scheduled for Rituxin.

At the request of Hopkins I also had a series of CTT scans performed to rule out any occult cancers, and these scans were in fact negative.

Additionally, while I had been receiving regular treatments of IVIg to control the symptoms of ITP, the doctor at Hopkins had said (and I may have gone over this before) that he did not view IVIg as a treatment so much as a bridge to a treatment and felt it was safer and better to take Prednisone.
After several months of experimentation I've leveled off at 20mg/Day of Prednisone which has provided me with an average of 30K platelets.
I don't view these numbers as acceptable for the long term, but it's workable for the short term while trying other treatments.

I started Rituxin in the beginning of August 2011 and completed 4 rounds of it. Tomorrow is the five-week mark from my first treatment. As of last week, the four-week mark, I did not see any benefit from the Rituxin in my bloodwork. Rituxin is a slow-acting drug, but this is nevertheless not encouraging. Tomorrow will be more of a litmus test, so we'll see if anything has changed, but I don't have a general expectation that it will.

In the event that the Rituxin doesn't work, I am going to have to make a decision whether to live with a low platelet count or have a splenectomy. I am fully opposed to a splenectomy for reasons I have documented, but reality is what it is.
The choice comes down to the lesser of two evils. Live with a low platelet count on regular meds, some of which have bad long term side effects (Prednisone) and some which have unknown effects (N-Plate, Promacta) or have my spleen removed and potentially have everything stop there.

I promised myself that if it came down to this I would go to Europe for the Indium test because I cannot think of anything worse that having my spleen removed only to discover that it had no effect. If anyone reading this has had the Indium test performed I would be very interested to hear from you and your thoughts on it.

So as it stands now we have a few more weeks to evaluate any benefit from the Rituxin, and I am then meeting again with the doctor at Hopkins on 10/27 to reassess my situation.

I have also contacted the English and Catalonian hospitals that perform the Indium tests to see what is required to have the test performed.

Lastly, I am also more than halfway through a 30-day juice fast in an effort to reboot my system a little bit. I've dropped ten pounds (I only started at 159; I think you get more out of this if you start heavier. and generally feel and (apparently) look better, but there has been no positive platelet movement yet.

I will try to update my Rituxin results (if any) leading up to my next Hopkins visit and anything I discover about the Indium test.

I sincerely thank you for reading and am again grateful for any feedback.

Best Regards,

KO

Ann from this board had the indium test and it showed liver destructions so she chose not to have splenectomy. You can search the board for 'indium', she's told the story a few times.

So, after all that, it sounds like you have... ITP?

If you do get a remission from splenectomy, how many years of remission would you consider to make it worthwhile? We have members here who have had remissions of ten years and then counts dropped again.

Good luck with your path - Erica

Someone where I live went to London for the Indium test - it showed she would benefit from having her spleen removed. Yes she did benefit but not for long, her count went back down. Her story used to be in the Personal Stories section but I can't find it.

I'm still having trouble with getting beyond your description of why you don't like Florida Hospital. Were you able to see the little spider on the bottom of the sandal's heels and that's how you knew Jesus was wearing Tevas? Actually if He is wearing the ones like I have He is pretty smart, they have a good arch & are good for your feet. For heavens [no pun intended] sake if a hospital & its doctors are good does it matter what statues & pictures are on the walls?

Wishing you good luck and lots of platelets!

I hadn't meant to make it an issue, but I will explain. Some of the iconography at Florida Hospital depicts Jesus physically helping surgeons with their surgery. I am personally uncomfortable with this because a) I'm not Christian, and B ) I don't want my surgeon depending upon divine intervention for a successful outcome.

I'm not a religious person, but it sounds like you may be, and I respect that. However, assuming you are Christian, how comfortable would you be in a hospital full of say, exclusively Islamic iconography including a garden full of life size statues of the prophet Mohammed and paintings showing Mohammed helping surgeons perform surgery? That's how I feel.

The actual photos of Jesus, or rather what I have to assume to be an extremely self-conscious stunt double, are just plain tacky. The Jesus-double is standing in a field healing sheep (I'm not kidding) with a little-bo-peep staff and wearing clearly visible Teva sandals. And there's like 12 of them on a long wall. It's just bad on so many levels.
It's actually too unintentionally funny to be taken seriously, but it's pretty offensive on an aesthetic level.

Oh, and the cafeteria doesn't serve Bacon.

KO

I'm a human, I am not defined by my beliefs which I do not push on anyone. I will once more say: what does it matter what statues and pictures are on the walls if the hospital is good and the doctors are good. I am not prejudiced - Mohammed can be on the walls of my doctors or my hospital walls, Buddha can too, so can any Indian [as in India] gods.

Did you see that I mentioned the woman in my town who had the indium test or were you too focused on my comment about the Teva spiders?

I did see that, and I thank you. The decision whether to have a splenectomy has been a difficult one and I appreciate any input on the subject from anyone.

KO27, I have watched and read your journey from the beginning and I admire your determination to find an answer.
Splenectomy is a huge decision and I wish you strength in making it, can't really add too much as I am not yet in that position. It is hard to say from comments on here as I guess those that it has worked for are getting on with there lives and not on the boards anymore.
This was just a message to wish you well and keep that determination going.

Sorry, sorry, I couldn't help but get some chuckles over that discussion. I have some thoughts, but will keep them to myself so this subject will hopefully slip slide away.

KO - you asked for thoughts on splenectomy. These are mine: The Indium is a great way to tell whether or not a splenectomy will be unsuccessful. It can tell you where the destruction lies - in the spleen or the liver. If a patient finds that destruction is in the liver, they know it will not work and would most likely refuse the surgery. However, if it finds that destruction is in the spleen, success would be a bit more favorable, but that can end at any time. The liver can, and often does, take over destruction some time down the line. That cannot be predicted. Here are some stats:

"Among 98 RefITP patients, no response to splenectomy was noted in 23 patients. The remaining patients had relapses after splenectomy at less than 1 month (19 patients), 1 to 3 months (28 patients), 3 to 6 months (6 patients), 6 to 12 months (7 patients), 1 to 5 years (10 patients), 5 to 10 years (4 patients), and more than 10 years (1 patient)."

bloodjournal.hematologylibrary.org/content/104/4/956.full

I think splenectomies can be beneficial for some people, but there are recent studies showing that some patients end up with blood clotting problems. I've actually been surprised by the number of people reporting blood clotting issues after splenectomy. They end up trading one problem for another or dealing with both at the same time.

bloodjournal.hematologylibrary.org/content/114/14/2861.full

It's a tough decision. Make sure you do your research on it before making the decision.

Wow. That seems to indicate the success rate was 1 from 98. That's dismal.

My Hema seems to think the that 2/3's of people wind up in remission, but I am looking for statistics to back this up.

I would not even consider it unless there were a probability of a durable remission.

Any time a doctor talks about remission from splenectomy ask them - FOR HOW LONG. It's weird but it seems like that is not a tracked statistic. A surgeon walks away from the table, you lived, you have more platelets than when you started and they never check on you again. Are hematologists talking about their own experience? Their splenectomy patients probably do not return to them if they come out of remission - at least for me I'd find another doctor and never want to see that one again.

We do have members with enduring remissions from splenectomy, and of course if you are in that category you might not stick around on the discussion board. But the medical literature does not seem to address well the question of how long a remission the 'successes' can expect.
Erica

I was thinking the Indium test might have been a good place to start, to determine if I'm a candidate whom would benefit, but perhaps I should try to dig deeper into whatever stats are out there regarding the length of remissions.

The problem, of course, is that at this point there are no great options- i.e. a lifetime on Prednisone (with it's own side effect and problems) or a new drug with an unknown track record (N-Plate, Promacta) or a splenectomy.

I knew the probabilities were that I would wind up with these choices after a year of trying everything else, but it's still quite frustrating to only have bad choices to choose from.

Yes, there is a huge lack of long term follow up. Remissions after splenectomy are quoted as 2/3 or 1/3 or whatever, but they don't say for how long. Are the patients followed for a month, a year, five years, what? They don't seem to address that much.

As for your choices, you are assuming that this is a life-long condition with no breaks. Not necessarily true. I never, never thought I'd have a remission like I've had (6 years+). I struggled with ITP on and off for 8 years before that happened. Yes, I was sick of it at times, but life went on around me and I had no choice but to jump into it and deal with ITP as it came along. Everyone has a different experience, but I can tell you that most people adjust and find a norm that they can live with. It is considered a benign hematological disorder and unexpected remissions can occur out of the blue. It can take some time though.

For what it's worth, I've been on Prednisone for 7 years straight. I will probably always be on Prednisone (not for ITP). I do realize the long-term problems that may hit one day, but I can only deal with today, today. I function just fine with the drug (never thought I'd say that). Most doctors will not prescribe Prednisone long term for their ITP patients...it's unusual to do that for ITP since there are so many other options to try that eventually work.

Just my two cents; I was diagnosed in /04 and refused a splenectomy.
Finally agreed to one after two years and going through all the available to me treatments. It worked for maybe a month. I finally got off the Pred last December. Did Rituxan in Sept/Oct. of last year too. I think that is wearing off now.

All I can say, is read, study and really think hard about going with a splenectomy. One thing to remember is that what doesn't work for one might work for someone else.

Good luck with your decision.

This is a very confusing article. Those numbers look very bad, but I think they're just looking at a sub-population of ITP patients who failed splenectomy. I think those percentages just indicate how quickly a splenectomy is likely to fail if it's not going to work. This study wasn't looking at all ITP patients who had a splenectomy. It's assessing how people do who fail a splenectomy and the conclusion is that most eventually achieve a partial remission.

I sometimes think people here (and on the facebook page) are too negative about splenectomies. If you've failed a few treatments and your counts are really low, splenectomy might be quite a reasonable option. And I say this as someone who has chosen not to have a splenectomy.


Lily

so your main discomfort of the hosptial is that its christian based and you are not. And just a thought if people are questioning about what is appropriate to how Jesus is portrayed. The biggest difficulty I have is that being in the geography area he will have been most people would not have been white, but darker colour - tanned. Yet in all the pictures via the Roman Catholic Churches etc.... he is white. Just a thought if people disagree how Jesus should be portrayed in our every days lives....

I haven't an objection to you not being Christian and being uncomfortable in thus a Christian hospital. But any of us cannot expect them to hide a few pictures and icons based on just that one is not christian. However if you can find a more rounded reason why the picture, a particular icon should not be there then you could approach the board and state your concern without mentioning where you stand in your believes. For it may not be just you that it effects. If so many other patients are feeling uncomfortable with an image then it is in the hospital teams' favour to remove it - as I say, regardless of where one stands on religious believes. We have active patient groups in England and I would approach them first if you have that kind of set up in USA.

Moving on. I have had a splenectomy in 1994. It worked for that time because platelet count will I believe 0. The splenectomy raised it to normal levels. 18 month later or so 1997 the count dropped again and they discovered I had regrown the spleen. They removed that and the count recovered. I have had drops from time to time over the years. Short lived until this year and I think it may have been caught this time from my signs.... For now:-) A word of warning. If you do choose to go ahead and it is your own choice, regardless of what people say. Just make sure you are properly educated. I wasn't and so for one reason very personal to me is that I contracted meningitis which was a blessing in disguise for me at that time and recovered from that to contract Septic Shock - stage worse than septicemia 2006 (?) and very very luck to be here and minimal issues really. Took me longer to get over it mentally than physically. I don't want to be that ill ever again but well if I am then I am.... I have lived quite well without the spleen on the whole since 1994. No more extra colds than anyone else. Since septic shock I now take penicillin daily and at first that panicked me with when I should go to A&E and or the doc but coming out of the woods with that.

I don't know on the whole why people argue so strongly to keep it because i Haven't had one since 1994 and haven't really noticed apart from meningitis and septic shock. I could have been very lucky. I am not meant to catch maleria I been told. Yes I was niavely okay until meningitis or rather that didn't worry me so much as the after effects of septic shock and even then I seem to be more concerned at having hullicinations again than anything else it threw at me.... :-} They weren't nightmares or anything like it. I have told you honestly my experience of life minus a spleen. There appears to be some job restrictions but they don't affect me. though have started a teaching assistance course in May and going into classroom for a day a week and wondering if I get any more colds than normal. Though the rituximab might hinder that true result.

My biggest plea to you is simply make sure you are educated about vaccines and life long after care and if you intend on any serious travelling to countries that you need maleria treatment before you go and after etc... I do carry a medic card around though never needed it.

Please, if you have any questions I will try to answer them based on my own experience and am an ITP'er in England so insurance stuff I no nothing about. General medical care and how it affects me is what I knw about. LIke I say it was 1994 when I first had splenectomy and leave you to do the sums of how many years that is....

KO
I feel you might find the podcasts on itpeducation.com answer some of your questions particularly regarding medical management of ITP.(If you haven't already listened to them)
Kind Regards,
Edith

@asurok- Thanks! I was not aware of the podcasts. The problem is that I think I've pretty much tried everything out there that could bring potentially durable relief.

I haven't tried a lot of the daily stuff such as the aforementioned Promacta and N-Plate, or the off-label stuff like Vinchristine because I'm really looking for a durable solution, and because some of those products are a little scary or otherwise not as well tested as I'd like.

That being said, there's just not much left to choose from.

I'll check the podcasts and see what comes up.

I hope you find them helpful...I did. Particularly regarding the subject of splenectomy and the success rates.
Regards,
Edith

PDSA Guidelines:


Discussions on hot topics such as politics and religion should be limited as they can often spiral out of control. There may be times where we have to “agree to disagree”, and let it go at that.


www.pdsa.org/forum/rules.html

Sorry
I thought I had kept it limited as possible in that I well and truelly kept my personal opinion/believe out of it. I was basically saying how to go about getting things changed.
Though being Christian I do find it quite hard that am not allowed to talk about my believe, relationship with God anywhere I go. Not just here but just about anywhere. Seeing as we discussed religion on another thread and seeing someone asked for a prayer thread numberous times..... So now I know at least why we cannot have a prayer thread. Religion is not okay with the rules even though for some of us its major part of our lives and how we may handle ITP.
Sorry if I offended anyone but it does seem hard that christians are not allowed to talk about it anywhere. Like I say, not just here but anywhere in general. Sorry to have offended anyone

I'm not offended. It's not my cup of tea, but you have a right for it to be yours.

I do think I made an error in expressing my incredulity at Florida Hospital because it does distract from the actual issue at hand, so I thank Sandi for suggesting as much. Florida Hospital is still goofy though.

That being said, I did have my followup appointment to the completed Rituxin treatment today. My platelets were 18K, and while we'll still need a couple more weeks before ruling out any efficacy from it, the writing is indeed on the wall.

It also appears that the 20mg/day of Prednisone isn't doing much, or rather, enough. I'm jumping up to 40mg and we've ordered Promacta for me to stay on a maintenance drug until after the Hopkins visit at the end of October. Both my Hema and I were uncomfortable with me being on a high dose of Prednisone for much longer so I'll try Promacta for a few months.

I have contacted the Barts and London Hospital in the UK regarding the Indium test, as well as the hospital in Barcelona. I am particularly intrigued with the highly predictive results the Barcelona docs got in that most recent study and I want to see if they are using a different testing methodology than the London based docs.

I feel like my ITP is pretty aggressive in that I've gotten very little benefit from pretty much everything we've tried. Not happy with my current options.

Anybody care to share their experiences with Promacta? What am I getting into?

Thanks,

KO

Sorry, not my rule, but it is part of my job to temper it. Back in the day, this Forum was a mess due to heated discussions. I will not let that happen. Thank you for sticking to the topics at hand.

KO - I see you've read the Barcelona study on the Indium. While the 100% prediction rate is impressive, again, how long did those successes last? I really think they should state that they can predict failures 100%, not successes. If someone sees that differently than I do, please explain your theory.

Sandi, I think you are exactly right, they can predict failures 100%. Everyone they say will fail because destruction is primarily in the liver not the spleen will fail. But they do not know if the rest will succeed for a minute, an hour, a month a year or a decade or a lifetime.

I'm with KO, I would have the indium study before a splenectomy. When I was considering splenectomy I asked my mom if she wanted to come to London with me and she did
Erica

yes, sorry again because I could have easily strayed being used to talking about faith online but not else where and yes even on christian forums that gets very heated so I do appreciate that this forum on the whole hasn't been heated in my experience. So sorry for the hiccup and likewise I am glad you mentioned it because it went unchecked when 'we' were chatting about me getting 'Server gender wrong'. And yes I know that was about identity and this was a particular thought that possibly would upset many a ardent Christian. So, I am sorry for that and thanks for bringing it up Sandi.

Anybody care to share their experiences with Promacta? What am I getting into?

You'd be getting into taking 1 to 3 pills every day, depending on the dosage you need. They'll check for liver issues whenever a platelet count is done. Dosage may be adjusted until count level is stabilized.
Then again; it may not work for you at all, or it may work too well....everyone is different.
It has been working for me for nearly two years now. I ran through various treatments over the years. Promacta is far far easier to live on than 80mg of Prednisone or 40mg of Dex...and all of the other types of treatments I tried stopped working within 2 years.
I am currently on a 50mg Promacta dosage and holding steady around 40k-50k, with no liver issues. I was tested daily at first (platelet count and liver), then weekly, then monthly, then quarterly, now just every 6 months. While I may not go to Hopkins, The James Cancer Center (of The Ohio State University Hospital system) is a fairly respected facility and my hematologist is wonderful.

I read the comments a few pages back about possible lack of sympathy/interest from oncology folk for those of us who only have ITP....that hasn't been my experience at all. Everyone at The James has been amazing over the past 9 years. I'm treated so well at The James, that I often feel guilty for 'only' having ITP...when so many people around me there are really really sick.

As for the Indium test? It is wonderful doctors to have another tool in the bag. But I also view it as a means to predict failure, not success. Accurately predicting failure can be useful, but it is not the same thing as predicting a successful remission.
However, knowing in advance that the liver was destroying platelets and not the spleen, might prevent many unnecessary splenectomies. That's a good tool.

And success rates for splenectomy in general? I've read everything from a 60% 'success rate' to a 40% rate. Which to me just means there is a 60%-40% chance of failure right from the start. Since I'm older, that failure percentage just increases. I also see little to no documentation on how long these 'successes' last. Both 10 years remission and 2 weeks would equally be deemed 'success' in these percentages. Two weeks is not what I view as 'success'.
Now I am actually a 'glass half full' type of guy.....but I've yet to see solid documentation on how much is really in this glass.

YMMV

That is well thought out, and very helpful. Sincere thanks for your reply.