Feedback requested after Several Failures

I cant imagine enduring IVIG every week. I had just 2 treatment toward the end of my first hospital stay. The day after I was home, the body pain and headaches were intolerable. I am a bit of a wimp I suppose because I rarely have headaches and was not accustomed to my body feeling that off. The IVIG body aches, for me at least, were exponentially worse than any I had ever experienced from a flu. When I went in to hospital I had more blood draws over those first 48 hours than I had in my entire life before hand.(which were 6) Those many blood draws as well as the IVIG and the Platelet transfusions left my arm trashed as well.

As I have stated, there are other members on here who have dealt with for much longer and have a much better grasp and understanding of ITP and the various treatments. As someone who never went to the DR before hand and who had not been in the hospital as a patient in over 40 years I assumed they all knew what they were doing. I had no experience with or desire to question anything they suggested because who was I but the patient. They were the experts right? When I was being told what should be done and when it should be done those first 6 days in hospital I was in no position to question or doubt their recommendations. It wasnt until I was home that I found this web site and began reading and listening to real world experiences. Thankfully there was no recommendation for spleen removal in my case in those first weeks of hospitalization. If there had been I would have been like "ok lets do it". I now know, thanks to the good people here that its important not to rush that sort of decision. Luckily my hemo fills the same. He has never said never but he has said as a last resort and only after many months of non responsiveness. Luckily my body has been responsive to ritux and Nplate so at least for now removing the spleen isnt even anywhere near on the table.

Has your doctor explained why she is so quick to recommend splenectomy since the standard is to wait I think 6 months to a year to see how one responds to the various other treatments? Could it be that the treatments themselves are aggravating your wifes preexisting conditions requiring the epidurals? I dont know if thats possible but if so maybe in her case they are thinking splenectomy earlier than normal since, of all the treatments, it has the highest rate of success in increasing platelet levels without the potential side effects of the other therapies. I dont know. I also dont know why a CBC would not be ordered before any treatment in order to see where one is at.

The one bad thing about ITP and the various options for treatment is that everyone responds differently. Some respond positively to a particular treatment while some do not. The same with symptoms experienced from various treatments. Its quite impossible to know which options are best without actually trying them to see if they work for you.

I am sorry your wife is having to deal with so much pain. I was fortunate, serious pain was never an issue for me. I was always able to honestly state that my pain level was 0 everytime I was asked.

I did IVIG every week the first year I was diagnosed.

I have done a bit of digging around. It seems that an epidural isn't recommended with a count below 80.

omg

Hello -
The hemo doctor seems to have a scripted 3x5 card. Try prednisone, platelets, IVIG, nplate and then throw hands up with splenectomy. The metropolitan area in which we live is around 500,000. I just don't think there's a sufficient population of ITP sufferers for her to have developed a precise plan. The surgeon does a handful of spleens a year but admits that they are mostly in the ER when he's assigned duty and gets the car or sports accidents. Yes, clearly the lack of spinal epidurals is manifesting now as prednisone, Nplate and Rituximab have lessened their impact. Her hemo doctor is focused only on platelet count and doesn't show any interest in anything outside of her area, which is probably 98% cancer patients. This week will be interesting as wife is canceling her weekly IVIG. We may not even have the PICC line done. She needs to decompress. Hopefully the anesthesiologist will get her in for an epidural soon.

Hello -
That could be the case, haven't had call back from anesthesiologist so don't know what he will require?!
Sincerely.

Hello -
We just met with the hematologist (phone call due to covid) and were informed that the bone marrow showed no disease but an excess production of platelets. She used the term hyperplasia to describe the platelet production. We consider this data "good news" from the perspective that she does not have a platelet production problem, but rather, a destruction problem. It also helps to narrow the problem. I'm now wondering why she could not have (definitely, did not) ordered this biopsy at the outset to better direct the treatment. Our 2nd opinion experts are analyzing the bone marrow and we expect that feedback very soon, as well as the next course of treatment.
Of course, the hematologist immediately identified the spleen as the culprit. She dismissed any other antagonist such as the liver, etc.
In addition, I pinged my insurance company and they confirmed coverage for an "indium" test as was mentioned previously in the UK.
I suspect that the 2nd opinion will suggest a medical drug treatment plan and not immediate surgery.
We feel upbeat today.
Sincerely.

Well that's good - and it is good to hear you & your wife are upbeat!

Your insurance will cover a trip to London and the indium test? That is a surprise. It is expensive and even if accurate that the spleen is doing its job and destroying the platelets doesn't mean that a splenectomy will do the trick.

Hello -
I just know that insurance here says it will pay for such a test, unsure if it can be administered. Previously, the local surgeon stated there was nothing to test the spleen to identify it ahead of time as the culprit. The hemo doctor was snarky yesterday since my wife wanted to skip one week of IVIG yesterday due to her pain and her needle-tortured arms. So the hemo threatened us that "we should get another doctor" if we weren't going to follow her directions and get an IVIG this week, which we relented (despite having a PICC scheduled for next Tuesday). Of course, her team failed to log platelet counts last week so she didn't really have data to state that "you really are going to die if you're at 2,000, and it's exponentially worse for spontaneous bleeding if you're under 20,000." Of course, these statements scared the daylights out of my wife and she readily bows down to the gods of western medicine. So....I knew if I said anything that appeared challenging such that the doctor said she was dropping her as a patient, my wife would be even more upset at me as well.
More research for the weekend.
Sincerely.

I understand that the indium scan is not available in the US, so your local surgeon was right. I'm not sure where you live or how big a city but if I had a doctor threaten me like that I'd say "so long, adios, that's it" ! There is nothing wrong with western medicine - however there is something wrong with a doctor threatening a patient, there is something wrong with a doctor who will not listen to & work with their patient!

As to the hematologist's team failing to log in her platelet counts for last week YOU keep track of those counts, and the treatments. My husband made charts of my counts - date/count/dose of pred I was taking/any illness I may have had - we would get my count and he would put it on the chart (still have them). I'd take that chart with me every time I had an appt with the hematologist.

I have been doing, until the pandemic hit, acupuncture for peripheral neuropathy - it has helped a bit, and with PN anything that helps a bit is great. I do love acupuncture - had asked her if it could help with ITP, she said I had had it too long so it probably wouldn't, but she did tell me of someone who worked there who had ITP and it did help but to what degree I don't know. With Chinese medicine as with western medicine you have to watch it as some meds/herbs/etc could cause a drop in count - my acupuncturist who also is an RN recommended a Chinese herb, I looked it up and it wasn't for someone with ITP.

I would never see that haemo again, absolutely outrageous behaviour.
Over here in the UK we have the GMC (General Medical Council) that covers the whole of the UK. If I had been treated like your wife I would be reporting the haemo to the GMC as a bully who is unfit to practice. As I understand it in the USA each state has its own medical board. Your profile indicates Idaho as your state. I strongly suggest you report this haemo. See link below. bom.idaho.gov/BOMPortal/AgencyAdditional.aspx?Agency=425&AgencyLinkID=650

My haemo asks all her patients to have a book in which they keep a record of all tests and medications and any other notes the patient wishes to make. It goes to every clinic with me.

That's a terrible way for a doctor to behave!
When I was first sent to my hema, he was very patient with me and answered all my questions and explained why a certain treatment wouldn't work.
I will admit, I was surprised when I got the 4 dose of Rituxan as my counts were in the normal range.

Hello -
Great input and appreciated. All the data and numbers are available 24x7 on the portal, but yes, it's a good idea for me to have a spreadsheet. I have a powerpoint that I update weekly that I print and take to other of her doctors.
Before the catastrophic platelet diagnosis on 6/5/2020, she was in physical therapy, had tried acupuncture, trigger points, ketamine injections (a few times) and has relied on a super-wonderful anesthesiologist for timely epidural (steroidal) spinal injections to address the pain in back/torso/neck/etc. We also have a visit in a few weeks with a local naturopathic practitioner who is a former MD but has a successful and highly recommended practice in non-Western treatments. As Forrest Gump said, "life is a bowl of cherries." One never knows if a practitioner will help or hurt, but you have to try. And I'm always seeking non-drug treatment to improve my wife's quality of health, even if it's our money and not covered by insurance. She has a gut/digestive problem along with a chronic cough so I diet and environment has to be explored.
Regarding doctors: wow, what to say? We've been both blessed and cursed over the years! I believe my wife could write a book on her experiences.
I should stick to the facts and omit certain topics but obviously emotions run high the past few months. My basic methodology is:
* insist and obtain the best outcome for my wife
* understand and invite perspective from those who are involved and can help
* be proactive with research and be data driven

I do feel that the hematologist has the best interests of my wife at heart. I have to be a pragmatist and look through everyone's eyes, walk in their footsteps. Reality is that her patients are probably 99% cancer patients, my wife is an outlier. There are too few doctors here, the population is exploding and she is clearly overworked. Yes, she came across as harsh and running an hour behind schedule which is not the norm. Seeing cancer patients every day, seeing them not show up and knowing they passed...it has to be a difficult job. Who knows what's going on in her personal life, with covid, her family? I feel I must be quick to listen, slow to speak. The basic rule is that no harm be done. On the flip side, she pauses to ask for questions and has been open to us providing feedback on our 2nd opinion and, thus far, implement those suggestions from another medical practitioner. Is my wife getting the best of care? Does she feel she's getting the best of care? I'm playing the middle-man attempting to remain logical, calm and helpful. I must be able to sift the wheat from the chaff, so to speak. The flip side is this: is there a better hematologist out there, better than what we have? It would be easy to drop someone but I've been through situations where we ended up (temporarily) with someone worse! This must be weighed. I've learned in life that it's most often the best to not rush a decision unless it's life-threatening.

New treatment options will be shared with us in coming weeks, PICC installation next week: I remain positive and resolute.

Hello -
Thank you for your perspective. I just replied to a thread covering this. We're focused on the 2nd opinion coming in within 2-3 weeks and are hopeful now that the production of platelets is sufficient, and that it's really the destructive side of the problem that needs addressed. Someone introduced me to the Wimhof method so trying to breathe accordingly each day.
Sincerely.

Hello -
That is a great point! They almost always check the counts BEFORE the weekly IVIG, yesterday it was over 20,000 whereas the doctor speculated that it would be around 2,000. Could the Rituximab be starting to work?! It's a suspicion. The doctor told us that we should probably give up after the 3rd treatment as the platelets didn't move upward, so we did. Was that a mistake???? Unfortunately, with the IVIG yesterday, next week's count will be much higher so we will not empirically know if Rituximab was starting to work. I suspect that the 2nd opinion may come back with instructions to finish that treatment and see it through.
Sincerely.

Seeing cancer patients every day, seeing them not show up and knowing they passed...it has to be a difficult job. Who knows what's going on in her personal life, with covid, her family?. This is not an excuse for being unprofessional.
Regarding gut/digestive problems. Have you read anything regarding the gut microbiome?

Im newly diagnosed as of July this year. I had never heard of ITP or Rituximan etc etc. I did receive the 4 weekly infusion treatments back n July and August and my Hemo and all the nursed told me at the beginning that it could take up to a few weeks after the final treatment before major results were seen. As it happened I had a big jump after the second infusion but this included weekly Nplate which I began receiving the day of my first Ritux infusion and have been receiving every week since then.

Sandi used to point to a response she got from a well respected haematologist who explained why a bone marrow biopsy can't confirm adequate platelet production. Anyone rememer the details?

JJ is this it?

Actually, the bone marrow biopsy does not show whether or not production is adequate. That is believed by many doctors and is untrue. I asked Drew Provan and I questioned the PDSA medical advisers about this a few years ago, and this was the response:


"I have stated quite a few times that a bone marrow biopsy does not determine adequate platelet production, regardless of what we are hearing from our doctors. I have not been able to find any publications that state this. I asked the PDSA if they knew who could answer the question, and it was passed on to 4 of the PDSA medical advisors. This is the answer that one of them gave, the other three fully agreed with the answer:

"I dont understand why anyone would use a bone marrow test to determine whether a patient is a candidate for Nplate or Promacta. My guess is that the doctor making this statement believes that if the bone marrow is normal (normal number of megakaryocytes) or is consistent with ITP (normal or increased number of megakaryocytes) that this shows that the bone marrow is making an adequate number of platelets and that production is therefore "normal" and that the patient is therefore not a candidate for a class of drugs that increase platelet production.

Nothing could be further from the truth. A bone marrow is a static sample and does not tell the doctor whether the normal or increased number of megakaryocytes is actually producing platelets. A large number of studies over the past 9 years have suggested that the normal or increased number of bone marrow megakaryocytes in the ITP patient bone marrow is not producing platelets adequately. Rather these megakaryocytes are probably being attacked by the immune system and undergoing programmed cell death (apoptosis) and die before they have a chance to make platelets.

The bone marrow test cannot be used to determine whether Nplate or Promacta will be effective.

With regard to when to do a bone marrow biopsy, the new (and the old) guidelines suggest that in adults it is rarely recommended except in those who have not responded to initial therapies, those over 60 (to exclude myelodysplastic syndromes) or those contemplating splenectomy. I rarely see a need to do a bone marrow biopsy in most of my patients except for those who fail to respond to initial therapy and those older patients who might have MDS (again reserving it mostly for those who do not have a robust response to initial therapy or those who might have other cytopenias). In a patient who has responded well to initial therapy and will then go on to splenectomy, I dont usually see a need for a bone marrow."

I guess its all a bit of a gamble when choosing a Dr or specialist. THose who have a lot of experience working in the medical field have an advantage. Those who follow trusted recommendation also have advantages. I dont have much experience dealing with Dr's or specialists. I dont have a personal Dr simply because I never go. Of course that ended this past July with my ITP diagnosis but even now I only see my Hemo.

When I was in the hospital the first time back in July for 6 days I had like 4 different Hemo's come in and consult and explain my diagnosed condition and what steps were being recommended and contemplated. I was given the choice to decide which one I wanted as my own personal Hemo and I decided on Dr Inal based solely on his personality and demeanor. He seemed more laid back and easy to talk too. That was my criteria. I guess I chose right since things are progressing nicely up to now but in the end it really is a crap shoot.

Hello -
I appreciate this new data. I'll confirm that I heard correctly during our phone call with the hematologist three days back. Your data would contradict what she had said to us. I generated a list of twenty questions for our 2nd opinion and we'll see how they respond!
Sincerely. BTW, I found the "Thank you" button finally

Many years ago I worked at a Radiopharmaceutical manufacturing facility that produced In-111. I’ve been in the PET Radiopharmaceutical side for almost 20 years now so lost track of the regular nuclear medicine products. I am going to look into this.

Hello :

We received second opinion from expert on east coast about one week ago which disagreed with local hematologists definition of treatment "failure" and also recommended fostamatinib as another potential treatment option. We met with local hemo last week and they have given up. Upon communication of the potential of fostamatinib, they replied that "it's outside the standard of care." We were immediately referred to another peer hematologist for another local second opinion and we expect that to occur in mid-to-late December. It's apparent there is a big gap between local expertise (albeit with seeing only one hemo face-to-face) and specialists at academic institutions.

Yesterday we had a tele-conference with a hematologist on the east coast whose job it is to communicate results of the second opinion "expert." He, himself, we could tell was quite skilled, personable and articulate. He level-set our anxiety and expectations towards the disease, as well as expected responses to various medical treatments, underscoring that it can take a couple of months or so for a response to be observed. Also, this expert conveyed hope, that one of these treatments could "cure" the ITP, in the sense of stabilizing it or returning to "normal" for a period of months or even years. Each person is unique and reacts differently. Unfortunately, we realize now that the local professional has never conveyed any sense of hope. Spouse has only been viewed as a number, a platelet count.

Last Thursday spouse maintained 38,000 count versus 36,000 count from the previous week, so it was the first week where neither platelets nor IVIG was required. Friday will be next draw and curious to see if there will be a precipitous drop as seen in the past or a similar count. The response to IVIG was horrible two weeks back with terrible pain, swelling and other side effects not before seen as so harshly. Spouse plans to only prefer platelets and avoid IVIG if possible.

While waiting for local second opinion, we are considering splenectomy and researching experts in this area. Major reason is that the side effects have been nasty and the weekly all-day in-patient IV therapy is debilitating and frustrating. Ultimately, it's her decision.

Sincerely.

Hope you get some good feedback with another second opinion. I had a follow up with my hematologist today. Watchful waiting to see if Rituxan has a positive effect. Platelets went up into the 180s following IVIG two weeks ago, but today are at 87. Fortunately side effects from IVIG are mild in my case.

Platelets should never be given for ITP, except in emergency. It can cause far more harm than good.

Hello -
What is your definition of emergency?
Sincerely

Not JJ here, but... An emergency is catastrophic bleeding that can't be stopped. Platelets are not a recognized accepted treatment for low counts from ITP alone.

Edited to add: Also, consider that splenectomy has by no means a guaranteed response. You may be interested in this opinion on the subject. pubmed.ncbi.nlm.nih.gov/10676920/
I decided very early on that this would be my approach. At my age and duration of disease, the positive response rate is less than 40%. Not good enough to justify the potential for harm from the surgery itself.

I ruled out splenectomy as well as at my age the success rate isn't high enough to offset the negatives. In the article midwest cited it notes that a splenectomy could also become *in*eligible for anti-D globulin. I hadn't seen that in the more recent articles so perhaps that has changed since 2000?
Roger
Edit to correct “eligible” to “ineligible”.

I cannot understand anyone contemplating a splenectomy whilst the COVID 19 pandemic is still rife.

I have had ITP for over 6 years and refuse point blank to part with my spleen. It is a perfectly healthy organ doing its job properly i.e. removing the rogue antibodies which unfortunately have platelets attached to them.

There is no guarantee a splenectomy will work. The liver and reticuloendothelial system could well take over platelet destruction. Accessory spleen growth cannot be ruled out.

Risks of infections, sepsis and blood clots are increased

Vaccinations will be required for
-pneumococcal infections, such as pneumonia, with regular boosters at least every 5 years
-flu jab every autumn
-Haemophilus influenzae type b and meningitis C (Hib/MenC)
-Meningitis A,B, C, W & Y
-Childhood vaccinations may well require a booster

The possibility of life long prophylactic antibiotics (or more courses due to infections) in conjunction with the increase in antimicrobial resistant infections is another factor that needs to be taken into consideration.

I guess I'm lucky. I don't have any of the issues mrsb has mentioned. Nor do I get any of the shots, other than the pneumonia one when due and the flu shot every year. I had my spleen out in 2006.