My Story

I have read posts and articles on this support website and feel I should (finally!) include my story. There is a lot of detail and time that has passed since my diagnoses, but I will try to summarise things.

My name is Christopher Smid and I am a 41-year-old male who is married and has four active kids (ages 9 – 13). I live in Ontario, Canada. I was diagnosed with ITP 3 years ago in January 2017. It all started with swollen lymph nodes in my right groin area, which my primary physician thought was a hernia. He did the right thing and had me get an ultrasound. It was not a hernia, but swollen lymph nodes. Did blood work and there was a fear I might have a kind of leukemia -Yikes! Was fast tracked to a hematologist who ordered a CAT scan. It was okay and the diagnoses was ITP. Sounded better than cancer, but I had never heard of this before.

At that time my platelets where in the 40 – 50 range and so she decided to just keep an eye on my condition. Platelets began to drop and so we she started me on a 3 day shock dose of prednisone (65mg). At one point, I ended up in the ER because I was worried about a big bruise on my arm, which came out of nowhere, and had blood in my mouth and back end. Platelets where at 4, so we started the prednisone again.

So, over the last 3 years I have had short term prednisone treatments, longer term therapy (3 weeks and then tapering period) and IVIG. Platelets always began to drop after each successive treatment and both therapies worked less and less till they did not do anything. They decided to take my spleen out in August 2018. I got to 156. First normal report in a year and a half. Unfortunately, and rather quickly, my numbers dropped to 25 within 3 weeks. As it got even lower, she tried Danazol, then Danazol and Azathioprine combo. Did not raise my platelets much at all.

Started Romiplostim (Nplate) in the Fall of this year. After three weeks I got to 86. My Hematologist was delighted. Began to drop after that and she had to double my dose each week to the max. Eventually we quite that treatment as it was not doing the trick. On to the next one. Started Eltrombopag 10 days ago because my platelets went to 2, I wake up with blood in my mouth every morning (does not help that I am a mouth breather due to sinus troubles), and hematomas where I scratch (itchy skin). Went to the ER last night because I (and my family) were very worried. They put me on this clotting drug (something acid?) and boy did I get nauseated. Threw up an hour and a half later and do not wish to try that again.

In the end, my new normal keeps getting lower. Also, I developed Neutropenia as well, but she is not attacking that problem at the moment. I think she is running out of options, but she is very positive (and delightful) and does know what she is doing. She is a researcher and administers clinical trials for various drugs having to do with blood diseases. She wants to get me on Rituximab, but needs to get approval (a lot of regulations up here in Canada). She would also like to do an ultrasound to check for any lingering splenic tissue and wants me to try and get off my anti-depressant, which I have been on for over 15 years. SSRI drugs can affect one’s platelets. However, I have tried to get off of these meds in the past and the symptoms were unbearable. So, this concerns me.
This is the gist of my story and since I have benefited from others who have taken the time to post, I thought I would as well.

Sounds dreadful Christopher
Prednisolone works for me but only at doses higher than I am prepared to take unless it's for a quick boost.
Azathioprine & MMF did not help and Romiplostim stopped working after a year. I'n now on Eltrombopag which can take a while to kick in 10 days isn't really long enough. What dose are you on?

Hi Chris, welcome. When I was first diagnosed N-Plate and Eltromopag weren't available. I did IVIG every week for the first year after diagnosis; was on prednisone the whole time(at different doses) and splenectomy. Finally did Rituxan in 2011 and have been in remission since. My hema here in NB used it for his Cancer patients and had to get permission to use it. I actually asked him about it. Told him I would be a guinea pig for it.

Thanks for replying back. I did okay on prednisone and actually liked it because it cleared up a lot of allergy related inflammation that I have, like sinusitis. Eventually, and unfortunately, it did not do anything to elevate my platelets. I had one count of 86 after three weeks on Romiplostin, but it kept dropping after that even as they increased the dose to maximum. After 12 days of Eltrombobag (50mg), I went from 2 to 5 - not much of an improvement. I will go up to 75mg at the end of the week. I do not hold our a lot of hope for this drug However, since the Romiplostin did not do much to stabilize my platelets. She has put in a funding request for Rituxan (Rituxamab) and I hope that works.

That is great to hear Cindy that Rituxan put you into remission. It gives me hope because I feel I am running out of options. My mouth is bloody every morning, so is my behind, and my skin is just an awful mess. I have not had much worry about this disease until now and worry about a brain hemorrhage since blood seems to come out of every other place. I have more hope for the Rituxan then I do Eltrombopag.

Chris
We will have to agree to differ on Prednisolone. I think it is an evil drug but has its uses. As for Eltrombopag it is the best thing I've tried so far. But one has to adhere to the dietary restrictions and be patient before an effect is seen. Personal experience dictates that zero calcium for a minimum of 4 hours either side of dosing is advisable, as is waiting at least 4 weeks to see a response. Carcamoc10 on here was on massively high doses of it before she saw any benefit. It may be worth you searching for her posts . Happy new year
Anne

What happened to the lymph nodes? Have they gone down? It's not an ITP symptom so if still there, needs more investigation I'd have thought.

To you as well. Lets hope for some new break-throughs in 2020!

It was my swollen Lymph nodes (3 times the size) that started this whole mess. You bring up a good point in that I have not heard of anyone talking about swollen lymph nodes and ITP? It took 2-3 months for the swelling to go down. It seems that my ITP was caused or kickstarted by a bacterial or viral infection. I have had many infections in the past (cellulitis in the ears, and other infections, and antibiotics always did the trick. I have read the viral infections can lead to ITP, I believe.

That makes sense, that the infection caused the nodes and triggered the ITP. Romiplostim (Nplate) put me into a sort of remission with counts high enough not to need treating, and rituximab that I had a year ago for lymphoma seems to have put me into a better remission, with counts around 120. Good luck with whatever treatment you do.

If I could get counts over 50 I would be quite happy and then just keep on trucking. However, I have only been over 50 twice since August 2018: 156 the day after splenectomy and 86 this fall after 3 weeks of Romiplostim. Downhill ever since.

Csmid, you mentioned taking the max dose of Romiplostim/Nplate. Though the manufacture says max dose is a '10' that is not universally applied by the community. To confirm, was the dose of 10 given? And was it for 4 weeks?

Hopefully you have looked through user 'Carcamoc10' experience. Specifically, she went up to 150 mg dose of Eltrombopag/Promacta to achieve a platelet count response. Note that dose levels as high as this are being used effectively in difficult ITP cases.

First, I love your tag line under your username! I did take the recommended dose of Romiplostim of 10(?) per KG of body weight. It did get to 86 after 3weeks, unfortunately, went downhill after that even as they increased my dose to 10(?) for 4 weeks. Since December 20, I have been on 75mg of Eltrombopag. The highest it got was 10, but now is down to 7 this past week. I did read about 150mg doses being taken, but these seem to be more for Severe aplastic Anemia. My bone marrow works fine, but you may be right in that I may need to go a lot higher in order to get a better response. My Hematologist is really good, but has never had a case like myself. I admit that I do not like the concept of having to stimulate my bone marrow (which works fine, they say)to produce more platelets when my body continues to eat them up. However, having a healthy level of platelets is more important than me not liking the thought of something. I will inquire with the hematologist next week when I go. They called today and say I got approved for 4 rounds of Rituximab for a month. So thank you for your response/advice. I find this forum very helpful

Csmid12, 10 is good. I spent several months reading old posts here on this forum. I'm pretty sure I stole my tag line from someone else's comments, LOL.

AFAIK, higher doses of Nplate/Promacta can be needed with ITP when either platelet destruction is inordinately high, or, platelet production is hindered by the immune system attacking megakaryocytes. These are row 2 and row 4 respectively in my treatments table. Usually an Nplate 10 dose can overcome either situation, but sometimes not. High doses of Promacta are being found to work in these cases. If high dose Promacta is unavailable then Doptelet, another megakaryocyte stimulant/agonist, is an option. See the thread by 'Lizabeth1', link below. She recently started taking it. Doptelet has been found to work better for megakaryocyte issues then either Nplate or Promacta.
pdsa.org/discussion-group/7-treatment-general/30497-doptelet-dosing-qs.html#68119

I too don't like the idea of excess bone stimulation to overcome destruction. But for megakaryocyte problems, platelet destruction is not the issue. The problem is platelet production. Row 3 and 4 in my table are both platelet production problems. Destruction problems are row 1 and 2. With row 3 and 4, one can think of it more like Nplate/Promacta/Doptelet returning platelet production to more normal levels.

Note that Carcamoc10 was taking 75 mg of Promacta all through Rituxan treatments. IMHO, this combination is a more effective treatment when row 1/2 of my table are in play. Rituxan works for row 1 antibodies. Promacta/Nplate works for row 2 antibodies.

Hope this helps.