TOPIC:

Hello All,

First, thank you all for sharing so freely on this site. I've learned a great deal already and hope to be able to contribute by sharing my experiences.

The short version of my history is I suffered a severe blood clot 15 years ago and was diagnosed with antiphospholipid syndrome (APS) and have been on blood thinners since. Sometime after that I was also diagnosed with ITP, after monitoring for a while, decided to go with the 'wait and see approach'. This worked well for years. While not actively monitoring, during the last 4 years my platelets ranged from 100k to 40k, with the average at 60k.

In the past month however, I started suffering from spontaneous bleeding so additional action was needed. Platelets were tested at 25k, which apparently for me was too low when combined with my blood thinner. This bleeding also occurred after a relatively recent change in medication from warfarin to xeralto.

I have been taking 60 mg prednisone daily for several weeks. First test was encouraging as platelets increased to 90K, however, retested yesterday and they have dropped into 50's. My Hematologist feels additional action is needed as that doesn't give much of a safety net. We discussed 3 next best options in his opinion and he asked me to consider and research and he will await my decision. In the interim, he's keeping me on pred and off thinners so the clock is ticking. I feel very fortunate that so far I've tolerated the pred reasonably well.

I'm seeking another opinion and was relieved to see the doctor I contacted was listed in another thread here as THE ITP doctor in St. Louis (Dr. Blinder). I will of course wait on any final decision until consulting with him. For the record, I've been extremely happy with my current hematologist, but given the complexities and constantly changing treatment options I feel it would be responsible to talk to a recognized expert. My wife agrees 

I’m probably where a lot of you are at, looking at all the options and trying to make an informed decision. ITP is complicated enough; tossing in APS and limited renal function due to 1 kidney further muddy the waters by adding risk and limiting options. Not that any of us have easy decisions to make with ITP that’s for sure.

My initial thoughts were leaning towards spleen removal. While it has risks, which APS adds to, it also seems to have the highest probability of success in the short and long run. I need to get back on blood thinners, the sooner the better. Plus, not getting any younger (50) and if surgery is an option sooner may make more sense, as my health has shown things can change rapidly. But offsetting that, no putting the spleen back after you remove and all other options you can discontinue. Also, I’ve only tried one other approach, might make sense to try one other non-surgical option to see how I do.

I’m sure this line of almost circular thinking sounds familiar to a lot of you. I just found out yesterday afternoon, a lot to think about.

So still looking at other options, reading here and keeping an open mind, which I think includes back to a wait and see approach. Though it seems necessary at this point to at least have a “what next” plan.

Thanks again for all your support and please let me know if you have additional questions that could help you. And of course, very interested in what you all might have to share, if anything.

Mike

Mike,
You seem to have a great doctor lineup! Your main doctor giving you three top options and asking you to research - very unusual and very excellent. And a consult with another top doctor. It sounds like you have the best medical advice and support. Your case is complicated and I wish you the best.

I believe there is some recent research suggesting risk of clots increases with splenectomy - I believe Sandi can chime in with specifics, it's been posted here before. Something to keep in the equation.
Erica

Thank you Erica. I feel very fortunate with my doctors, especially after reading some of the stories here. I really like his approach and letting me make an informed decision.

I appreciate the heads up on the clots with the splenectomy, I'm slowly making my way through the recent research folder and may find it there.

Mike

Hi Mike. You sure do have a complicated combination of issues, although the two together are common. I have had elevated Anticardiolipin Antibodies but have not had a clot. I am supposed to take daily aspirin but usually forget because I have a lot of other meds in the daily lineup. Luckily, I have not had any issues with ITP for about 8 or 9 years.

It can be difficult to juggle the two; I have seen may fair share of it on the Forum in the past 15 years or so. One woman I know very well had repeated clots even with low platelets, so I do understand the need to get back on the blood thinners. As Erica said, there has been research done that suggests that splenectomy does raise the clotting risk, so that might be something for you to consider.

It's really difficult to find articles that discuss ITP, APS and splenectomy/thrombosis. Everything I found was really old. I do have some articles that discuss ITP and splenectomy/thrombosis.

www.ncbi.nlm.nih.gov/pmc/articles/PMC3674676/

www.bloodjournal.org/content/121/23/4782.abstract?ct&ct=edc0078f0223706ceb6453e28c53f19cb50ae40e71d3b442acf9a40c28f72c3622599d8d53bf161970897c9b7de283abb0295054cc9f469454f243cd2b2bc543

Why did they switch you from warfarin to xeralto? Seems a shame that you were stable until then. What are your three choices?

It is my understanding that Xeralto is given at a standard dose, whereas warfarin is titrated to achieve a target prothrombin time. Since you are needing to balance your tendency to clot vs. your tendency to bleed, it would make sense to me that warfarin would give you better flexibility and better precision.

More importantly, warfarin is the only anticoagulant that can be reversed in the ER, with a simple injection of Vitamin K. With any other anticoagulant, including aspirin, there is very little recourse in case of a severe bleed, especially a brain bleed, or an emergent need for major surgery. This would seem critically important for someone with a bleeding disorder.

Hello Sandy and Rob and thanks for your responses.

That's great news you haven't had any issues in such a long time Sandy. Overall, I've been very fortunate as well and have had years of not having issues, or any worry about these diseases. APS and ITP do complicate things as you need to balance clotting risk vs. bleeding risk. It's too bad they don't just offset each other It's clear that for me, adding blood thinners into the mix I can only allow my platelets to get down to 30k or so before I need to get them up.


Thanks for the article, I read through it and plan to reread it as well. It does appear to raise the risk of clotting even above the normal risk for surgery. As you said, something to consider, though for me at least just one of the many factors to consider.

The 3 options I discussed with my hematologist were Rituximab, Splenectomy and Thrombopoietin-receptor agonists. I think those are all pretty standard second line treatments. He gave me a description of each treatment, talked about the benefits and the risks and told me to take a few days to consider.

Rob and Sandi you both had questions about the change from Warfarin to Xeralto. The primary reason for the change was I'd grown tired of the constant blood draws necessary with Warfarin. Keep in mind I've been on blood thinners 15 years! While I'm relatively stable, there are times I am out of the range and adjustments with more frequent blood draws are needed.

Rob you brought up good points in favor of Warfarin and I am seriously considering switching back after this experience. While I was aware of those risks, there were several benefits I saw. I already mentioned that monitoring is not required and I also believed that meant I would be in the therapeutic range more consistently vs. Warfarin. Less doctor visits meant less risk of exposure to flu, etc. with my compromised immune system. And while Xeralto is not reversible (that I am aware of) it is out of your system very quickly, I believe 24 hours. When I'd required surgery, tests, etc in the past that had a risk of bleeding coming off Warfarin and back on took at least 7 days with constant blood checks. During that time, while it was hopefully small, I was at increased risk of clotting.


That said however, I am leaning towards going back to Warfarin after this experience. It was disconcerting that when I was bleeding spontaneously there was no way to know if the dosage of xeralto was too high or not. Plus the points you brought up. Honestly, I'd probably become too complacent with the risks of these diseases and was focused more on the convenience. After this experience, I hope not to make that mistake again.

Thanks again for the thoughtful responses.

Mike

One anecdote does not scientific evidence make... But I just want report my husband's experience with Xarelto. He was taking it to prevent clotting due to his a-fib. He also has an internal defibrillator (ICD) in place. The Xarelto did not prevent clots from forming on the leads of the ICD. His cardiologist reported it as a "failure" to the appropriate overseers.
DH is now taking warfarin for his a-fib. Sometimes the old-fashioned ways are better, even if they're less convenient.

Are you aware that it's possible to check the INR with a finger stick instead of a draw? DH went for at least four arm draws before someone mentioned another lab that offers the finger stick.

Thanks Janet, I appreciate you sharing.

Yes, they did switch me over to the finger stick vs. the arm draw. I'm such a wimp I still cringe when they pull the snapper back for the finger draw.

I think I'm going to look into how expensive home testing would be. I believe that's an option if it isn't too much money. It would save a lot of time and I would think overall save money, plus would keep me out of the doctors office. Especially important if I need to continue to treat ITP.

Mike

Mike - which APA do you have? It's more common to clot with the LA, so I'd assume it's that one although ACA is also a consideration. Do you have the antibodies tested often? I rarely do and am not even sure if I should. There's nothing I can do about it anyway. It's surprising that doctors don't routinely test ITP patients since it might influence the treatment decisions. Supposedly 30% of ITP patients have APA's. I asked my Hemo many times and he wouldn't do it. Finally, my Rheumatologist agreed, it was elevated and actually ended up giving me the clinching criteria for my Lupus diagnosis. I have a feeling it will come back and bite me at some point.

The TPO's also carry a clotting risk. You'd probably have to keep your counts on the lower end.

Sandi,

You'll have to bear with me, for someone that's dealt with this so long my grasp of all the technical details is lacking. You're definitely prompting me to learn more, which at this point is a good thing.

Looking at my most recent tests, I tested way over the reference range on the Plasma Lupus Anticoagulant test, the Serum Cardiolipin IgG and the Russell's Viper Venom Time test. I was in the normal range on the Serum Cardiolipin IgM and IgA. To be honest, I have no idea what those test results mean beyond confirming I have APS.

So you can imagine my answer to how often I am tested is...not often. I too tend to take the approach there isn't a lot I can do about it and try not to worry about it. For example, once I got past the initial ITP diagnosis I hadn't been testing on a regular basis. Though with all my doctor visits I was still getting tested multiple times a year.

I'm sorry to hear you are dealing with Lupus in addition to ITP. Seems you have complications as well. Last night in going through my tests I started wondering if my test results could indicate Lupus to. I recall being told I don't have it, but that was years ago and can't find any tests.

Regarding the clots and ITP treatments, I'm trying to see the good in that at least I'm already being treated for clotting. But it is something to be aware of for sure.

Thanks for your continued help.

Mike

Sorry, I assumed that you knew the acronyms. You seem to be so on top of things! LA is the Lupus Anticoagulant and ACA's are the Anticardiolipin Antibodies. You seem to have both. I have had the Anticardiolipin and a highly elevated VDRL which is another possible indication of clotting problems and is usually found in Lupus patients.

Ha, yeah, I've had my fair share of problems. If you don't show any symptoms of Lupus such as joint pain, muscle pain, extreme fatigue, feeling flu-like, etc., I wouldn't worry about it. They can't diagnose unless physical symptoms are present as well as diagnostic labs. It would seem for now that APS is your primary diagnosis with ITP as a symptom.

Thank you, but I'm feeling anything but on top of things at the moment! Honestly feeling a bit overwhelmed and uncertain trying to decide how to proceed. I'm sure some of it's the pred head, but as you all have experienced, there are so many options each with there own benefits and risks.

The good news Sandi is I'm showing no signs of Lupus. I'm just going to file that away as something to follow-up on at some point. I've got enough going on at the moment without worrying about that.

This has caused me to think about what my goals are with my treatments, what is it I want to focus on. Here's my list so far.

I want to survive 10 years - I have kids in school still and want to at least be able to support them and my wife through college. It seems the mortality rates for ITP are very low, most find something that works. However, treatments each have there own risks and I think I'll prioritize the shorter run vs. the longer run. Also, like many of you, I have other serious health challenges besides ITP. I need to consider my overall immune system health as it's already being stretched.

I need to work during those 10 years - My mind and body need to function well enough to work. Also, while my job and my boss are very understanding about all my appointments/health issues, I'd like to minimize the amount of work I have to miss.

I need to be able to afford my treatments - I not only can't afford to spend my savings on my treatments, I don't feel like I can afford even hundreds of dollars every month on my treatments. I'm lucky with excellent benefits now, but as we all know change comes fast and you can't be certain of your coverage in the future. Cost needs to be considered.

Hopefully the list above will help me as I try to work through the treatment options with my doctors. Maybe I can use it to evaluate my treatment options using the list. For example, while splenectomy may offer the best long term fix, it might offer the highest mortality risk, which may not fit my goals. BTW, not sure if that's true, I'm just using an example.

Have any of you all used a list like this before? Or how did you use some kind of rating for looking at your options?

Thanks for listening as I ramble through this process.

Mike:

I have also thought things through like that and it is sad, but practical. I've chosen ITP treatments that were cheap so I didn't have to spend my kids college tuition on meds. They got through college and I survived the steroids, although the physical and emotional cost was higher. I do not regret my decisions. The choices you make do affect other's lives when you have a family.

I never thought for a second that I would die from ITP, but I do realize that Lupus and the treatments could shorten my life. When I first found out about the APA antibodies, I was more scared than I'd ever been with ITP, even with counts under 5k. I had nightmares about it. I completely understand your fears, especially having to deal with both. You have a tough decision ahead because all of the treatments have risks that may be worse for you than the average ITP patient. If I can help in any way, I'm here.

I know you have a lot to sort through, but I wanted to point this out. If you'd go the splenectomy route and it fails, you're back to the other two choices. Either of those choices, along with being asplenic, could raise the risks even higher (clotting or immunosuppression). It is entirely possible that splenectomy could be the right choice, but not having a crystal ball, you cannot know in advance which would be more successful.

I was the same way with work....tried to miss as few days as possible. I scheduled doctor appointments at the end of the day and finangled whatever I could to minimize missed time. I went to work sick many, many times because when you have autoimmune disorders and are sick a lot of the time, you have no choice but to keep pushing through it. I also had to keep my health insurance, no choice there. I did get to a point where I was crashing about three years ago and had no choice but to finally give up working (two years ago next month). It took us a year of planning things out both financially and medically before I could put my notice in. It was the hardest thing I've ever done. The reasons are too long to go into, but we were able to manage things financially once my kids grew up. So yes, there are many things to consider.

I certainly hope that you far surpass your goal of 10 years. Somehow they manage to keep people hanging in there most of the time. I've known one other person like you who juggled low counts and blood thinners. She also had the LA and was on blood thinners. She had a few PE's and clots in her legs. She also had Lupus and ended up having a bone marrow transplant. Since then she has had no more problems with low platelets or blood clots although still has many other Lupus issues. It was a drastic treatment but something that could be a possibility for you down the line if things don't get under control. I'm betting that you'll be able to manage this though for quite some time.

PS - Would you mind if I moved this down to the APS section?

Sandi you have my admiration for having the discipline and concern for your family to make those sacrifices. I’m sure it couldn’t have been easy, especially considering the impact the steroids likely had on your emotional health. I’ve only been on them 4 weeks and have already had a couple of issues where I haven’t handled myself well. It’s ironic that in trying to do what’s best for our families overall, we end up subjecting them to some out of character behavior that doesn’t do a lot for our relationship with them. I worry about that and my feeling I’m not the same person on the high dosage of steroids.

Interesting, that ITP freaks me out more that APS. I think one reason is the treatment for APS is so straight forward, while ITP is anything but. Of course, being straight forward means taking blood thinners which complicates things.

I appreciate your points on the splenectomy. As I learn more about the procedure, I’m having more concerns about how the clotting risks and the overall increase in immune system risk.

That’s great you were able to manage to work through most of the time your kids were in school and that you were able to stop when you needed to. My wife and I are in the process of looking at our finances, trying to figure out how to best manage things now, as well as looking at what ifs.

I’m hoping to beat the 10 years and it seems very likely I will. It’s more of a guideline to help me figure out how to move forward. Basically I’ll trade some long term risk for shorter term results.

In terms of my decision about what next, I talked to my current hemo yesterday about 3 blood tests I found that might help us figure out how I might respond to treatments. He's looking into them and will call be back today. If anyone’s interested I’m posting details in another thread for someone else at the same point for their process.

Update: Heard from my Hemo, he thinks 2 of the tests have no value and one has such small value to essentially be worthless. Guess I'm feeling frustrated there doesn't appear to be ANY screening or tests used to help guide us in this process of picking our treatments.

Feel free to move this to the APS folder, or anywhere else you think is appropriate.

Thanks for all your help through this all.

BadBlood wrote:

Sandi you have my admiration for having the discipline and concern for your family to make those sacrifices. I’m sure it couldn’t have been easy, especially considering the impact the steroids likely had on your emotional health. I’ve only been on them 4 weeks and have already had a couple of issues where I haven’t handled myself well. It’s ironic that in trying to do what’s best for our families overall, we end up subjecting them to some out of character behavior that doesn’t do a lot for our relationship with them. I worry about that and my feeling I’m not the same person on the high dosage of steroids.

Ha - I never said I handled myself well! I did learn some tricks along the way though. My job was very fast paced with deadlines. I went nuts trying to do it before steroids, so during, I was a shaking mess. I couldn't speak coherently or think straight. I worked with money and if I screwed up, someone paid for it. You probably won't do this, but I would go into the restroom several times a day and just breathe or cry. I got away from the ringing phones and constant chatter to calm down. It helped. I also had a co-worker who was my wing-woman, she could always seem to read my mind and when I went blank, she rescued me. In retrospect, there were some pretty funny stories and I had to learn to laugh at myself. That made things much easier.

At home, I forced myself to maintain composure with my three kids. Just the noise of their phones, the dog barking, the TV and the arguing made me nuts, so I would chill in my room for a while and call them in one at a time to spend time with them. It was effective. My husband bore the brunt of the bad behavior, but he handled it well. He travels during the week so only had to put up with me on weekends. He survived it.

Too bad those three tests won't help. I was curious as to what they were, but I guess it doesn't matter. You are doing the right thing by researching and whatever you decide will be the best thing.

That's great you had someone to help you out at work Sandi. Fortunately, my job doesn't require constant face-to-face interactions, though there are still challenging times.

I'm learning the same things you did regarding your time at home. Even when I feel calm it doesn't take a lot to get me irritated, which is so different than when I'm not on steroids. Learning to get some away time when I'm really feeling it and leaning on my wife to have her handle some of the issues that come up. Not ideal, but better than the alternative at the moment.

Here are the details on the tests that I found:

Helicobacter Pylori - This infection has been associated with ITP, some studies have found eradicating it can help with platelet counts, though results are mixed.

Haptoglobin (HP) - Some research found that non-responders to splenectomy had significantly lower levels than those that responded. The researchers felt it could predict success 80% of the time

Thrombopoitin (TPO) - Some research found that if you already have high TPO (>95 pg/ml) you may not benefit from treatment with Platelet Growth Factors. Quest has a test for this #16336.

I had blood drawn for the Helicobacter test this morning, the other tests were denied. I’m getting a second opinion soon and will ask about the tests again. I’m going to keep pushing for these. I can’t understand why we wouldn’t do these tests, as they might provide some direction and help avoid costly and expensive treatments that might not work. The offset is of course they aren’t in the standard treatment literature and the may not be generally accepted. Even if the cost is wasted, it seems very small in the big picture vs. treatment costs, risks, etc.

I’ve continued to research and coming to the conclusion I am not going to get a splenectomy as it seems that APS significantly increases the risks. Specifically, the risk of clotting, but also the negative impact of sepsis and other infections that are more likely with no spleen. Infections can trigger critical APS. While I may need to consider it again, it doesn’t feel as if it should be the first second line treatment I try.

I'm not sure my current doctor considered APS and the risks it adds in his suggested treatment options, though I didn’t ask him. There are suggested ways to handle a splenectomy if you are at high risk of clotting. I think this just confirms, all of us with APS need to be proactive in doing our research for any surgery, treatments, etc.

In terms of treatment, I’m leaning towards discontinuing the pred slowly and seeing where my counts
end up. Last week I was 53k and I didn’t bleed until the 20’s, which seems to give me some room.

So this week I’ll continue to research my second line treatment and push my doctor to get me started on ramping down the pred. I’m still on 60mg after almost 4 weeks and really want to reduce that soon, even if I don’t know the next treatment for sure yet.

Thanks again for all your time and help.

Mike

Mike:

Just last week I came across the test to check TPO levels, a new article came out about it. Apparently it is a new test and you're right, it is not a standard test yet. This is actually the very first test that I've ever heard of that could predict the success or failure of a certain treatment.

I ended up with two Hemo's when I found out about the Anticardiolipin Antibodies. I asked my Hemo about it, and he said my Rheumatologist should manage it. When I saw her a few months later, she said that was my Hemo's area and back and forth I went. My regular Hemo finally referred me to another Hemo who was more familiar with APS so for a while I had one for ITP and one for APS. To manage both, you need a Hemo who is familiar with both and considers both when suggesting treatments. You are right about that! I wouldn't trust one who wasn't on top of both disorders.

You made a very good point about illness and APS. CAPS can be fatal and you want to avoid that risk as much as possible.

I have mixed feelings about H Pylori. I've only seen two or three people who seemed to benefit from treating it over the past 15 years. It seems that patients in China (I think) benefit more than we do here, possibly because it's a different strain. It never hurts to try though and even if it does not improve counts, you still have to treat H Pylori. Good luck with that!

Your counts are pretty good at the moment. I hope that continues so you can get back on blood thinners. Oh I meant to tell you....Plaquenil can help to prevent clots also. I don't know if your doctor would be willing to prescribe it for you. I've taken it for 9 years with no detectable side effects and am hoping it's helped with any clotting issues I may have had otherwise.

Sandi,

I probably found out about the test from the article you posted! Thanks for putting up all the recent research, with all the reading I've been doing I've lost track of where I found the articles.

Hard to believe, I haven't seen a Rheumatologist. That's definitely on my list of appointments to make soon. It's been challenging to find someone that's familiar with ITP and APS, hoping that the specialist I see in a few weeks is.

Right, I have serious concerns about pursuing a solution for ITP (which generally isn't fatal) that has a risk of fatality through clots, CAPS, etc. Though admittedly, it's difficult to quantify the risks in percentage terms. I am going with the thought it makes sense to avoid these risks until other less risky treatments have been exhausted.

I agree about the H Pylori being a very low odds possibility. I believe my doctor described it as an "infinitesimally small" chance. But what the heck, it's just an inexpensive blood test so might as well even if the only real effect is a little peace of mind.

Thanks and yes I need to get back on the thinners soon. Appreciate the tip on Plaquenil, I'll look into that and talk to my hemo.

Mike

I post all the articles that I find in the General Section under New Articles. I get updates from Bloodjournal.com and post those often. This is a very good article that I found a long time ago, from 2001. Not very current, but gives you some food for thought.

www.bloodjournal.org/content/98/6/1760?variant=full-text&sso-checked=1

Here's an article about Plaquenil. It is not a conventional way to treat APS and will probably not do all that you need, but it's an alternative.

www.circ.ahajournals.org/content/96/12/4380.full

This article is about half-way down the page:

Hydroxychloroquine

Hydroxychloroquine (HCQ) is an antimalarial drug, although the precise mechanism of its anti-inflammatory action is not known. In addition to its anti-inflammatory effects, immunomodulatory effects of HCQ include increasing the pH of intracellular vacuoles, interfering with antigen processing and inhibiting T-cell-receptor- and B-cell-receptor-induced calcium signaling.[109–111] HCQ also has antithrombotic effects by inhibiting platelet aggregation and arachidonic acid release from stimulated platelets (Figure 2)–.[112] In the general population, HCQ has been historically used as a prophylactic agent against deep vein thrombosis and pulmonary embolism after hip surgeries.

See more....

www.medscape.com/viewarticle/712112_4

Thanks again for the details Sandi.

In terms of updates, feeling like I need to get off the 60mg pred asap. Tired of how it makes me feel and concerned I've already been on this high dosage too long. Called the doc, he doesn't want to start ramping me down until I've picked my next treatment. He wants me to come in to discuss. I was hoping to meet with second doc before then, but that's still a week away for initial appointment.

I think I need to determine what is my target in terms of counts. Last tested I was at 50, which seems fine to me. I bled in 20's, so in my mind 30 - 50 is reasonable given challenges of treating and side effects. I haven't seen a lot of opinions about what range of counts should be in when being treated with thinners.

I'm going to propose, ramping down steroids getting back on thinners and see what happens to my counts. If that fails, then I think my next best non-emergency option is Rituxan.

Does that sounds reasonable overall?

Yes, I think it does sound reasonable. Keep in mind that Rituxan can take time to work, so it won't be a fast response. I think it could be manageable though. That probably would have been my decision if I were in your shoes.

Did you mean that you bleed in the 20's with or without the blood thinners?

I'm not quite sure why he'd want to keep you on such a high dose when it's not doing much. Seems like torture (or blackmail - decide now or I'm not letting you taper)!

Hi Mike,

It may be worth considering that anticoagulants are not an all or nothing medication, at least with warfarin. My 94-year-old dad takes warfarin for a-fib but since he is at high risk for falling they keep his prothrombin time at a higher level than they would otherwise. Balancing the clotting risk against the bleeding risk is a judgment call; I hope you have someone good to help you with it.

My dad gets his blood tested at home through medicare. He calls the technician "the vampire". You might check whether that is an option with your insurance.

I wanted to provide an update since my last post.

I met with my current hematologist and decided to go the splenectomy route. Even though my counts were mid-50’s he wanted to keep me on the 60 mg prednisone until treatment and off thinners. I wasn’t completely comfortable with any of these choices.

Yesterday I met with my second opinion hematologist and I have a new plan and a new hematologist. While I was happy with my prior hematologist, my new hematologist specializes more in my area of diseases vs. oncology. Also, he does research and teaches at an excellent medical school. In our discussion it was clear he was factoring in not only ITP but also APS.

He believes I have primary APS with secondary ITP. That’s important as it helps guide treatment decisions. As likely my issue is platelet destruction, not production. He believes treatment options that target platelet production are probably too risky at this point due to primary APS and clot history.

He recommended rituximab as my next treatment option and believes it may help both the APS and ITP. The drug not only reduces antibodies which may help with APS, it targets the t-cells which may help with ITP.

He wants to lower my prednisone dose ASAP (I’ve been on 60 mg for 5+ weeks) and also was somewhat concerned about no thinners for so long. After sharing I’d experienced some transient eye issues the past 2 days he put me on baby aspirin immediately.

He’s also researching a relatively rare, but possible cause of my low platelets. I can’t explain using the proper medical terms, so I’ll hold off explaining until I understand better. It’s something I’ve always thought might be related, but other doctors haven’t shown much of an interest in.

His assistant and staff were also absolutely amazing. Before I left the office, they had cleared the treatments with my insurance, setup my first treatment appointment for next week, drawn blood for a battery of tests, setup my follow-up appointment with him and also sent me off to immediately get my flu shot. His assistant also gave me her direct number and told me to call for test results vs. waiting for them to post as she’ll have them sooner.

So that’s it for now, I get my first rituximab on Tuesday and will finish up all 4 over the next month. They’ll be monitoring my platelets, but sounds like results could take 4 – 8 weeks so I’ll be patient.

Thanks again for all the information you all have shared. It’s helped a lot as I try to work through this.

Sounds like a good plan, Mike. I was also concerned about your being off of blood thinners for so long. It can be difficult to manage both of those and it seems wise to err on the side of clotting (treat that primarily) and worry about bleeding if symptoms occur.

I'm glad he is getting you off of Prednisone - it's about time! He sounds like he knows what he is doing. I've never heard of Rituxan as a treatment for APS though. I searched it when I was diagnosed in 2006 and couldn't find anything. I did just find the below articles from 2013. It appears that any studies conducted in 2006/2007 were inconclusive and perhaps prematurely optimistic (older articles that I found), but it seems as though it may prevent clotting on some level. These two articles clarify things a bit.

www.medscape.com/viewarticle/780417

www.mayoclinic.org/diseases-conditions/antiphospholipid-syndrome/basics/treatment/con-20028805

Sandi, as usual your concerns are right on track and that's definitely how I'd like to balance the two risks in the future.

As update, 8 blood results came back and what a mess. The good news is platelets are almost 80k, which is of course what started this whole thing off. Some results, are out of range but expected due to APS, etc. Others are just high or low and not clear why, for example of 20 measures on CBC 10 are out of normal range.

Probably my biggest concern is Blood D-dimer at 3,100 which seems really high. For now, not treating outside of the baby aspirin and hopefully starting to ramp me down from pred to see how I do, then look at getting back on thinners.

Everything else will just have to wait until main issues are dealt with.

Rituximab treatment delayed a week due to additional info needed from insurance company. Approved today, but won't start until next week.

Thanks for the additional info, it was helpful

Prednisone can knock white cells out of normal range, they will usually run high. Most of the time, my CBC's are all wacky too. If they are only slightly out of range, it's normally not anything to worry about. I've had a few flagged as abnormal cells in the past and if we wait it out, they will usually go back to normal.

As for the D-dimer, blood thinners would probably fix that. Why aren't they starting you back on it now? Counts in the 80's should be okay I would think.

My counts seem to do the same in terms of being in and out of range. As you suggested, nothing to be too concerned about now as the platelets are the priority. Though I do need to follow-up on kidney function test.

Regarding thinners, I think the plan is to start reducing prednisone, see how my platelets are then get me back on. Coordinating with doctors office now, as you know, takes some time.

BTW, looking at our insurance offerings for next year noticed 2 out of 3 plans we can pick from are adding separate out of pocket limits for prescriptions. For someone that has high drug costs this can double the amount they need to pay for. Definitely something us complex cases should keep an eye on, as in my case the extra costs could be up to $5,000. Ouch.

Yikes! What insurance company do you have?

My plan is pretty good right now and covers a good bit of my scripts. Even with a lower co-pay, it adds up because I have so many of them. Next year though, I will be on Medicare (disability) and I have no idea what to expect. I guess I'll need a good supplemental plan. I keep saying that I can't afford myself!

Did you try Promacta tabs? My wife tried many options (suffered 3 years) and finally happy with Promacta for ITP.