Adult with ITP and exploring for treatment options after a few treatments failed

I’m 34, female. I started noticing unexplained bruises mostly at lower body since middle of 2015 and easily got tired, but I didn’t do anything about it until January 2017.

January 2017: Had unusually heavy menstruation flow and was instructed by an urgent care doctor to go to ER due to thrombocytopenia (8000/μL). I was admitted to hospital and platelet transfusions were administered. After reviewing bone marrow biopsy result, the specialist diagnosed me with ITP. During the hospitalization, two courses of IVIG were administered and I was discharged with 54K platelets counts. The specialist prescribed me with prednisone (60mg/day). After 2 weeks on Prednisone, I was given 3 high dosage of hydrocortisone infusions for 3 days because she believed that 60mg prednisone didn’t work on me. In late January, I was instructed to reduce prednisone intake to 50mg/day. The specialist also decided that high dosage of 3 hydrocortisone infusions didn't work on me either.

February 2017: I noticed a major bruising on my upper right arm. Just one big and deep blue bruise. I went to ER and was admitted to hospital due to low platelet count (8000/μL). I was given first administration of Rituxan and N-plate.

March 2017: My specialist prescribed me with another 5 sessions of Rituxan (total: 6 sessions). Honestly, I was confused since from what I read here only 4 sessions of Rituxan will be done for the first treatment. I completed all 6 sessions anyway. The prednisone level was varied from 20 – 60mg/day in 6 weeks. Had to take N-plate once while doing Rituxan due to low platelets count (49K). I didn’t experience any wet bleeding, just felt tired and I noticed some small bruises when I was at low platelets count.

April 2017: The prednisone level had been tapered at the order of the specialist; from 40mg to 10mg in 3 weeks. Yes. Crazy. I was miserable. After a few days on 10mg, I had severe muscular and abdominal pain and the specialist suspected that the symptoms were due to prednisone withdrawal but didn't ask me to do anything about it anyway. The pain became more severe that night, and without telling my specialist I began taking 20mg of prednisone again. The joint and muscle pains went away but the abdominal pain remained. I also had nausea, lack of appetite, bloating and lack of bowel movements. I went to an urgent care and ER; and was admitted due to the severe stomach pain. CT scan found thickening of adrenal glands. An endocrinologist indicated that I was adrenally suppressed.

End of April – The specialist decided that Rituxan didn’t work on me and asked me to choose either to continue with N-Plate or do a spleen removal. I was not ready to get my spleen remove, so, I decided to continue with N-Plate (I did once in April and once in May – I put the reason why I only took N-plate every 3 weeks as below, point no. 5).

Honestly, after my last appointment with the specialist in April, I was looking for another hematologist for a second opinion and I actually was ready to switch to a new hematologist because of a few issues I had with the existing specialist i.e.
1. They never contacted me for my blood work results. I had to call and chase them for a few days to get it every week
2. They don’t keep a proper record of my dosage of prednisone or any other treatments were given to me. I had to plot my own graph to monitor my platelets counts and to record all treatment given to me
3. Gave me 6x sessions of Rituxan instead of 4x
4. Crazy prednisone taper strategy that caused me admitted to hospital twice in 1 month
5. Gave me 250 mcg N-Plate for every time (4 times) without adjusting the dosage and find my ideal dosage as recommended by the drug manufacturer. My platelets count was sky rocketed up to 700K-1M after a few days taking N-Plate (for all 4 times – Feb, Mar, Apr, Jun) and it took about 3 weeks-4 weeks before the count dropped to below 50K and I had to get another N-Plate injection.

I asked for a recommendation list from PDSA and Carol provided me with Dr. Howard Liebman's contact. I contacted his office and sent all my medical records to him. I managed to get to schedule the earliest appointment to see him in June.

May 2017: Admitted to hospital again because of the same symptom of adrenally suppressed in April. Was on N-Plate and didn’t have any issue with platelets count since it will take about 3-4 weeks for the count to drop below 50K.

June 2017: Consultation with Dr. Liebman on 6/9. Immediately decided to drop the existing specialist and switch to him after 45 min of consultation. He instructed me to stop taking N-Plate temporarily until he sees my blood sample under microscope and all the tests he ordered came back. Scheduled to see him next week to review all the results and discuss for treatment options. He also asked me to check with my insurance on how much I will have to pay if I switch to promacta.

I read and did my research on all the options available i.e. promacta, n-plate, spleen removal and I’m concerned by the possible of side effects i.e. liver damage, myelodysplastic syndrome, leukemia etc. When I thought about my condition before was diagnosed with ITP (Jan 2017) – I didn’t experience any wet bleeding or anything, just bruises mostly at lower body and got tired easily, but, I didn’t know what was the lowest platelets counts from mid of 2015 to January 2017 since I didn’t do any blood work for that. So, I’m thinking if it is possible for me to go treatment free and just live with itp? Of course I will have to do frequent blood work and if the count is very low, can I go for ivig? This sounds stupid but I have to ask anyway.

Thanks.
Salina

Salina:
Hi...welcome. The best thing you did was research and get another doctor. I can't believe that I continue to be amazed at the way some doctors are treating ITP patients, but I am. Unbelievable! None of that should have happened. You got too much way too quickly and incorrectly, as you discovered. I don't understand why there is this huge rush to throw everything at a patient and get the counts up to unsafe levels. You're the second one in the past few weeks. Slow and steady wins the race.

To answer your question, yes, you can choose not to treat. Some people do, depending on symptoms. However, the side effects that you listed for N-Plate and Promacta are very rare and when they do occur, they have been reversible upon stopping the drug. We've been following N-Plate and Promacta users for years and have not been seeing serious adverse reactions or side effects. You might be able to use Promacta at a low dose and keep a safe count with minimal risk. The only way to know is to try. If you'd prefer to go treatment free until counts are low and then use IVIG, you could bring that up with Dr. Liebman and see what he says. Some people do manage ITP that way if they don't mind being hooked up to the IV's.

You are much better off with Dr. Liebman and I'm so glad that you made the switch! The treatments that you received and the manner that you received them were far more dangerous than ITP would be for most people.

Sounds like you've got options well figured out.
I assume insurance/cost is not influencing your course - which would cause me to have other comments. It will be interesting to see if you return to your pre Rituxan baseline count of 8 with no treatments. If it is higher, this too would cause me to have other comments.

8 is a pretty low number. Below the magic 10 number, there is increased probability of spontaneous brain hemorrhage. If insurance is paying why take the chance? As Sandi mentions, Promacta and NPlate carry little risk. That is especially true if you are sensitive to them and are taking a low dose. Your count was 700 - 1,000 range ! Rest assured, that IS sensitive! Further, if one responds to NPlate, it is very unlikely that one will not respond to Promacta. So pick either.

IMHO, don't underestimate science. Science does not stand still. It is very possible that in 5 to 10 years new drugs will be available which induce remission. So one shouldn't think Promacta and NPlate are 'forever' drugs - even though they are right now.

Hope this helps.