Update - 10 months Post-Splenectomy

Hello All,
I have not posted in a while because I wanted to wait a bit after my splenectomy to assess my overall health and give some feedback.
For all of you who do not know my history here it is:
Diagnosed just 3 months prior to turning 40 (male): 5/22/17 with platelet count of 16.
Dexamethasone 4 day: Up to 120 and down to baseline within 1 week followed by IVIG up to normal then back down again in 2 weeks
On periodic dexamethasone pulses for a month until I was approved for Promacta.
7/3/17 - Promacta 50 daily did nothing / 75 mg daily did nothing. Hit 0 platelet count twice during this time. IVIG was my bailout.
10/1/17 - Stopped Promacta.
10/6/17 - Prednisone comes into play via 40 mg daily with taper down to 20mg = counts hovering between 15 and 25k
11/3/17 - Rituximab x 4 weeks = nothing. (No combo treatment with Dex. Only Rituximab by itself). Counts never moved above 30k.
12/8/17 - NPlate began - nothing as I went from dosing levels 1 through 7.
1/24/18 - Bone Marrow Biopsy done. Results = no lymphoma, leukemia, or myelodysplasia
3/1/18 through 6/17/18 - Nplate dosing levels 7 through 10 yielded inconsistent counts and eventually lost total response even with being paired with Prednisone 15mg to 20 mg daily. (By the way, I was on prednisone since 10/6/17 all the way until I had my splenectomy every day.)

6/18/18 - Fostamatnib (Talavisse) 50 mg followed by 100mg dosing schedules yielded nothing.
9/15/18 - Trip to London for Indium Platelet Scan - St. Barts.
Results = Spleen / Liver Ratio 3.24
Average Life Span of Platelets = 1.2 days
Since my Spleen/Liver Ratio is above the key number of 2.0 (3.24), this means that most of the destruction was being done in the spleen. (Predominantly splenic). I also visited Dr. Drew Provan in London on my last day there to consult about next steps. Dr. Provan said Mycophenolate Mofetil- MMF combo treatement.
Pair it with 8 weeks on Promacta 75mg and 20 mg prednisone daily and .........if no response.... then follow with 8 weeks of MMF with 20 mg of prednisone daily and weekly NPlate level 10 dose shot. .....................................If all of this fails with poor platelet counts..do the splenectomy.

2/1/19 - Got all immunizations done after my doctor recommended Splenectomy due to all other options being deemed refractory. No response.
3/13/19 - Requested Haptoglobin test from Hemo and results were 132. (Refer to haptoglobin as a marker in a study for a higher levels = successful splenectomy outcome. I believe anything that is 120 or higher is yields a higher success chance)
3/20/19 - Splenectomy done. First count back was just over 200k in hospital post surgery
4/3/19 - Counts up to 551k
4/17/19 - Count went down to 41k
4/24/19 through 9/1/19 - Counts hovered between 30k and 45k
9/10/19 - CRASH..(Family Get together = alcohol consumption = learned lesson)..down to 6k - Hemo gives me Level 5 Nplate shot paired with 50mg prednisone for 3 days.
9/13/19 - Count went to 62k plus referral for accessory spleen scan. (No accessory spleen found). Prednisone taper complete later that week.
9/18/19 - Count over 600k off of the single shot of Level 5 Nplate from week prior. (I guess it works now that I have no spleen). No more NPlate.
10/11/19 - Count 100k. Monthly visits. Flu shot given.
11/8/19 - Count 74. Monthly visits.
12/4/19 - Count 42. Monthly visits.
1/10/19 - Count 66. Next Visit is April 3. Quarterly visits. No medication.

I will continue on periodically with my post-splenectomy status updates. Please keep in mind that I had to do a splenectomy because I tried everything up until this point in time. The people on here and know my history know that I have tried alot. My decision to do the splenectomy was based on the following decisions: The Indium scan result in London, My Haptoglobin result, IVIG worked great initially, my age 41 at time of surgery, and Dr. Provan's recommendation.

Also keep in mind that at my most desperate time, I was on IVIG, 100mg of Fostamatinib, Level 10 NPlate and Prednisone at the same time with no meaningful response. I was a zombie. I went from 152 pounds to 175 pounds on prednisone until I had the splenectomy. From 10/17 to 3/19 was the weight gain period despite exercising at the gym doing running cardio for 1 hour per day 6 days per week at the gym yet.... gaining weight. You can't lose weight on prednisone no matter what you do. I could not lose a pound. The decision was a no-brainer at that point.

My current weight is 161 pounds as of today. My goal is to get to 155 by the summer. I continue to exercise at the gym with 50 minutes of cardio paired up with 15 minutes of strength training. My platelets are not normal but hopefully this partial remission is sufficient enough. I feel good but I can still tell when my platelets dip up and down. The good thing is that they hover in a range 45k to 75k where my own personal homeostasis is ok. I still get tired and fatigued but nowhere near pre-surgery. They have a lot of new stuff for ITP that are in phase 2 or phase 3 research. I still think the C1 esterase inhibitor (Sutimlimab) will be the answer to alleviate and hopefully cure ITP. I pushed my doctor to give me a similar type of C1 inhibitor, Etanercept, during my desperation times but he said no.

ash.confex.com/ash/2019/webprogram/Paper125341.html

www.clinicaloptions.com/oncology/conference-coverage/hematology-2019/nonmalignant-heme/capsule-summary-slidesets/898

In addition, I thought this was very important to be knowledgeable of the following.
Apparently, people with ITP all have abnormal mesenchymal stem cells in their bone marrow as where healthy people do not. When the people afflicted with ITP received these umbilical cord stem cell infusions in China, they all went into remission. 4 people went into complete remission in this study below.

www.ncbi.nlm.nih.gov/pmc/articles/PMC5443306/

I will keep everyone updates periodically. Thank you.

Thank you for sharing the information. So helpful. I am happy for you that the outcome was great after the nightmare.

Holy cow Jason. That is fantastically good news. You made it !

First thing. Might want to take a look at this post by JayInChicago who has a partial remission going, much like what you've got going now. He has a similar statement about alcohol consumption.
pdsa.org/discussion-group/7-treatment-general/30084-last-nplate-shot-was-on-feb-22nd-2018.html?start=30#67253

Then there is this other post by JJ. After she achieved a partial remission via Nplate, went on to take Rituxan. Note that Rituxan is often taken soon after being diagnosed. Taking it after a partial remission may be a first on this forum. Rituxan lifted counts higher. More into the normal range and out of the ~50 count partial remission range.
pdsa.org/discussion-group/7-treatment-general/30412-nplate-loss-of-response-antibodies-to-nplate.html#67352

Also of interest. User Carcamoc10 was treated with 75 mg Promacta (no response) and then combined it with Rituxan. Failing that, went on to 150 mg of Promacta. This worked in short order. Nplate never entered in the picture. She now appears to be in full remission after just a few months. I have to wonder if you would have responded to the higher Promacta dose. Who knows...

I went to the PDSA conference this past summer. They may have mentioned what you have for new treatments, not sure. Will look through the links you've given. Personally, I suspect most of these auto immune diseases will go away when they figure out how to rid the body of all the different Herpes viruses. That these viruses hiding in immune system B cells and T cells, taking over certain cell functions and causing them to do the wrong thing, is the root of the problem. I have also learned that EBV can hide in bone cells - which kind of aligns with your statement on mesenchymal stem cells.

You didn't mention getting off Prednisone. I take it you didn't have much problem, or ?

Hello Hal,

Thank you for the good wishes.
In regards to your question, yes. I am off of prednisone since my surgery on March 20, 2019. The only time I needed prednisone so far was for that 1 week when I crashed because I drank too much alcohol at a family gathering. It was a blessing since the hemotologist gave me a level 5 NPlate shot to skyrocketed my counts into the 600's after the 1 and only dose administration. NPlate never did that for me before, so it is good to know that I have a safety net in case I fall out of remission.

By the way, I know most people do not want to get rid of their spleen and neither did I but when you feel like you have run out of options, this was what needed to be done. Oh and by the way, my doctor did have me on 100 mg of Promacta daily paired with MMF (never went to 125 or 150) right before I had the splenectomy. The TPO's never had an effect when my spleen was in. I reviewed all of the studies about the likelihood of a successful splenectomy so I did all of the relevant tests (Indium Scan, Haptoglobin levels right before surgery) and analyzed relevant statistics (under age 45, initial response to IVIG). All of these factors assisted me in choosing to remove the spleen.

I went to my Surgeon for a post - surgery follow up and he did tell me something very interesting about my spleen when it was removed. The report said that my spleen was exhausted. My Surgeon said that the red pulp was all used up. My spleen had been working on overdrive.

The best way that I can explain my current part of life is that I feel "in the moment" now. I do not feel like I am in a fog due to medications or low counts. I also have energy that I have not felt since pre-diagnosis in 2017. The fatigue is gone unless my counts fluctuate down to the low 30's.

Hal, I feel the C1 inhibitors are the answer as well. I really do.

I will keep people updated and I will try to be as detailed as possible because it is important for people who need a comparison.

Jason, about this study.
"1087 Serum Complement Levels in Immune Thrombocytopenia: Characterization and Relation to Clinical Features"
ash.confex.com/ash/2019/webprogram/Paper125341.html
Specifically this part.
"
ITP patients have significantly lower serum C3, C4, and CH50 than healthy subjects, with the overall difference driven by the one-third of ITP patients with substantial reductions. We observed a relation between low C4 and more severe disease and low C3 and reduced platelet counts.
"
If I'm understanding, about 1/3rd of folks have statistically lower Complement levels. With that, I wonder if Complement is not a pervasive/universal problem with ITP. It would be reasonable to expect the same percentage to respond to Sutimlimab when the clinical trial results come out.

It is interesting about low (baseline) platelet counts being correlated with low C3 Complement. That makes a lot of sense. Also, I suppose low C4 implies no/poor steroid and/or IVIG responses.

I was reviewing video of this summer's PDSA conference. One doctor mentioned that Complement activation on platelets implies destruction in the liver (and thus not the spleen), primarily.

As a side note, I found this study on Complement and the drug Sutimlimab, aka BIVV009. Figure 6 is good to help one understand how the drug works.
"C1s Inhibition by BIVV009 (Sutimlimab) Prevents Complement-Enhanced Activation of Autoimmune Human B Cells In Vitro"
www.ncbi.nlm.nih.gov/pmc/articles/PMC6360260/