I need some hope. I feel destroyed from ITP. Fostamatinib next.

Hello All,

I am a 40 year old man diagnosed with ITP in May of last year after excluding all else. Treatment list is as follows:

May to June : Hemo started me on high pulse dexamethasone. I immediatey went from 13 to 115 on a count but on next pulse after a crash...I only hit a 37 and then crash again.

June to July: Hemo puts me on prednisone ranging from 40 to 70mg daily and numbers can't get beyond 20, 000 platelets. IVIG was provided twice during this time with a good consistent response but does not last beyond 2 weeks at a pop. I get readings over 100k after I am provided IVIG but it is a quick fix.

July to October: I get approved on July 10th for Promacta. Starts off at 50mg and nothing. Bump to 75mg and I got only 1 reading that was good in mid August (58,000) and then it fell to 7,000 the following week and it never went back above 20,000 again.

November: Rituxan (4 weeks) with a prednisone combo after the Promacta failure. Nothing. Count stayed below 20,000 again.

Mid to late December: N-Plate was started in December after a bone marrow biopsy which revealed nothing but ITP. I quickly went up the first 5 levels of the N-Plate dosage list without any response while pairing up with a daily 40mg of prednisone dosage.


January 22, 2018 - I had to rush to the Hemo with a platelet count of 1. I was provided IVIG and had a fabulous February with normal counts over 80k for 4 weeks. At same time of my good counts, while being on 40mg of prednisone since beginning of NPlate treatment in late December.

March - After my IVIG crashed down from 100's to 20k, Hemo put me up to level 7 out of 10 on the NPlate dosage scale while on 40mg of prednisone. I get good numbers. I tell hemo I want off of prednisone. Hemo tapers me at 10 mg less each week. Once I am down to 10mg of prednisone and level 6 of N-Plate, I crash with a platelet reading of 23,000.

Late March - Hemo ups my N-Plate to a level 8 and keeps me at 10mg of predinsone daily. I get a 46,000 reading followed by a 50,000 reading back to back weeks. Hemo said lets continue the taper down to 7.5 mg of prednisone daily while staying at level 8 out of 10 on N-Plate.

April - I crash the last week with a reading of 13,000. Hemo ups me to level 9 out of 10 while remaining at 7.5 mg of prednisone daily.
Today - I get a reading of 14,000. Hemo ups me to the final level 10 on NPlate while remaining at 7.5 mg of prednisone daily.

Hemo said that if things don't improve over the next 4 readings while on the highest dose of N-Plate..he will discontinue it.
Next option is Fostamatinib.
I doubt this will work since only 18% success rate in clinical trials. Beyond that ..all that is probably left in the treatment list is a splenectomy which I am really trying to avoid.

I feel that I am refractory to everything but IVIG. I cannot stay on steroids. I can't deal with the massive frontal headaches I get once I go above 10mg of prednisone. I can't lose not even a pound while exercising 8 miles of eliptical machine per day burning 700 calories a session with only 1 off day of exercise each week. I do this just to keep the prednisone from making me a balloon. I currently weigh 165. I am depressed because I really wanted NPlate to work and I am afraid of what is beyond level 10 of NPlate. Please provide me support because everyone says that they find a treatment that works for them but I am beyond my wits end with my search because nothing seems to be working. Remember, I am trying to get off of steroids because of the long term effects and my hemo agrees with me. I have been on prednisone since September. Any advice will help.

Jason:
Hi, I'm sorry you had to join us. How are your symptoms when counts are low? All I can say is...hang in there. It can be frustrating. Has your doctor mentioned Win-Rho? It isn't used for ITP much these days but is a valid option. It used to be used all the time until a black box warning came out, but even IVIG has one of those. At least you know that IVIG works if needed and you could manage ITP with that until something starts to work. It's not ideal, but it's an option.

I know that you want off of the steroids, but 7.5 isn't all that much. I've been on doses between 10 mg's and 15 mg's for 12 years straight (not for ITP). It took a very long time to start having long term side effects.

I have seen some stubborn cases like yours before and eventually, something did start to work. Keep the faith!

My sympathies regarding steroids. Horrid drugs.

Some treatments take time to work.
There are other medical options open to you such as Rituximab, Azathioprine, MMF, Cyclosporine has your haemo suggested any of these?

ITP can be really depressing and it sounds like you have been through a real roller coaster as many of us have. The steroids are rough, I am having those awful headaches now at 10mg. I can only empathize and say keep your spirits up. Hopefully the fostamatinib works for you!

Jason, note my embedded comments below.

jasondema wrote: Hello All,

I am a 40 year old man diagnosed with ITP in May of last year after excluding all else. Treatment list is as follows:

May to June : Hemo started me on high pulse dexamethasone. I immediatey went from 13 to 115 on a count but on next pulse after a crash...I only hit a 37 and then crash again.

June to July: Hemo puts me on prednisone ranging from 40 to 70mg daily and numbers can't get beyond 20, 000 platelets. IVIG was provided twice during this time with a good consistent response but does not last beyond 2 weeks at a pop. I get readings over 100k after I am provided IVIG but it is a quick fix.

Poor steroid response and week IVIG response. Sounds like row 4 in my ITP table. Probably getting two weeks out of the IVIG, instead of one, because you are taking steroids with it.

July to October: I get approved on July 10th for Promacta. Starts off at 50mg and nothing. Bump to 75mg and I got only 1 reading that was good in mid August (58,000) and then it fell to 7,000 the following week and it never went back above 20,000 again.

After my experience with Danazol, I have to wonder if folks who don't respond to Promacta don't respond for the same reason - the liver. That some folks metabolize Promacta faster than others. That it is metabolized and cleared from circulation before it has had time to stimulate bone production of platelets. Just a theory.

November: Rituxan (4 weeks) with a prednisone combo after the Promacta failure. Nothing. Count stayed below 20,000 again.

Mid to late December: N-Plate was started in December after a bone marrow biopsy which revealed nothing but ITP. I quickly went up the first 5 levels of the N-Plate dosage list without any response while pairing up with a daily 40mg of prednisone dosage.

January 22, 2018 - I had to rush to the Hemo with a platelet count of 1. I was provided IVIG and had a fabulous February with normal counts over 80k for 4 weeks. At same time of my good counts, while being on 40mg of prednisone since beginning of NPlate treatment in late December.

March - After my IVIG crashed down from 100's to 20k, Hemo put me up to level 7 out of 10 on the NPlate dosage scale while on 40mg of prednisone. I get good numbers. I tell hemo I want off of prednisone. Hemo tapers me at 10 mg less each week. Once I am down to 10mg of prednisone and level 6 of N-Plate, I crash with a platelet reading of 23,000.

Late March - Hemo ups my N-Plate to a level 8 and keeps me at 10mg of predinsone daily. I get a 46,000 reading followed by a 50,000 reading back to back weeks. Hemo said lets continue the taper down to 7.5 mg of prednisone daily while staying at level 8 out of 10 on N-Plate.

Rituxan fail and high doses of Nplate/Promacta needed to get a response. Both help to confirm row 4 response.

April - I crash the last week with a reading of 13,000. Hemo ups me to level 9 out of 10 while remaining at 7.5 mg of prednisone daily.
Today - I get a reading of 14,000. Hemo ups me to the final level 10 on NPlate while remaining at 7.5 mg of prednisone daily.

Hemo said that if things don't improve over the next 4 readings while on the highest dose of N-Plate..he will discontinue it.
Next option is Fostamatinib.
I doubt this will work since only 18% success rate in clinical trials. Beyond that ..all that is probably left in the treatment list is a splenectomy which I am really trying to avoid.

Might want to look at 'sachmo16' story. He took Fostamatinib and had good results. Note that, before taking the drug, he had regular IVIG treatments to keep counts up. Sound familiar?
For me, I wonder if you are in that 18% group.

I feel that I am refractory to everything but IVIG. I cannot stay on steroids. I can't deal with the massive frontal headaches I get once I go above 10mg of prednisone. I can't lose not even a pound while exercising 8 miles of eliptical machine per day burning 700 calories a session with only 1 off day of exercise each week. I do this just to keep the prednisone from making me a balloon. I currently weigh 165. I am depressed because I really wanted NPlate to work and I am afraid of what is beyond level 10 of NPlate. Please provide me support because everyone says that they find a treatment that works for them but I am beyond my wits end with my search because nothing seems to be working. Remember, I am trying to get off of steroids because of the long term effects and my hemo agrees with me. I have been on prednisone since September. Any advice will help.

Yes, absolutely. Fostamatinib is the next logical step. The drug is safer than taking one of the other immunosuppressants. Let me guess. You really don't have much bleeding symptoms and because of that your doctor isn't recommending those immunosuppressants. Yes?

If the Fostamatinib does't work, if it were me, I'd try Cyclosporine as shown in my ITP table after that.

Hope this helps.

Hello All,
Thanks for all your responses.
Hal, I probably am row 4. Its sad but true. I am intrigued by your cyclosporine suggestion. I first have to get through these last 3 weeks of NPlate level 10 while paired up with a 7.5mg daily prednisone regimen. Hemo will take me off NPlate and push the Fostmatinib, I guess. Just to let you know here is my last few lab results from my hemo every Friday. This is 100% accurate. All other numbers on CBC are normal. Hal, you are right. I am not a bleeder. Start in inverse order.

4/20/18 - I get a 14k count - Doctor keeps me at 7.5 daily mg prednisone but increases to level 10 NPlate
4/13/18 - I get a 13k count - Doctor keeps me at 7.5 daily mg prednisone and increases to level 9 NPlate
4/6/18 - I get a 50k count - Doctor decides to taper to 7.5 mg daily mg prednisone and keeps me at level 8 NPlate
3/30/18 - I get a 46k count - Doctor keeps me at 10 mg daily mg prednisone and keeps me at level 8 NPlate
3/23/18 - I get a 32k count - Doctor keeps me at 10 daily mg prednisone, but increases to level 8 NPlate
3/16/18 - I get a 105k count - Doctor tapers me to 10mg daily mg prednisone, keeps me at level 7 NPlate. ***Doctor says that it has to be more than just the IVIG that is helping at this point*****
3/9/18 - I get a 80k count - Doctor tapers me to 15 mg prednisone, keeps me at level 7 NPlate
3/2/18 - I get a 21k count - Doctor increases me to 20 mg prednisone and increases to level 7 NPlate (IVIG probably lost its effectiveness here)
2/23/18 - I get a 168k count - Doctor reduces me to 10mg prednisone, keeps me at level 6 NPlate
2/16/18 - I get a 142k count - Doctor reduces me to 20mg daily prednisone, keeps me at level 6 NPlate
2/9/18 - I get a 94k count - Doctor reduces me to 30mg daily prednisone, level 6 NPlate
2/2/18- I get a 73k count -Doctor says to stay at 40mg daily prednisone, level 6 NPlate
1/22/18 - IVIG treatment with a count of 1k followed by a bone marrow biopsy 3 days later that came back ITP only. All else normal.

Sandi, thank you for your wisdom. I have been pressing the doctor to get me off of the steroids. Hopefully, Fostamatinib or possibly cyclosporine would help as per Hal's recommendation. I still have the splenectomy in my back pocket but I have a feeling that a spleen removal might not help me because of my limited responsiveness to prednisone, TPO's, and IVIG as well as my non-responsiveness to Rituxin back in November.

Mrs. B,
In response the following "There are other medical options open to you such as Rituximab, Azathioprine, MMF, Cyclosporine has your haemo suggested any of these?" I have tried Rituximab (Rituxin) already. It did not work. I have not tried the rest of them. Apparently HAL9000 thinks that Cyclosporine might be the best shot if Fostimatinib fails to deliver a good result. I still have my spleen by the way.

Dru, I hate the steroids for 2 reasons only. The frontal headaches starting fro 10mg daily and up as well as the inability to lose at the very least 1 pound regardless of the amount of cardio exercise I do. Not even 8 miles for 1 hour burning 700 calories a day on the eliptical machine helps. Now that I am under 10mg of prednisone a day helps with the absence of headaches, I feel better. The cost for feeling better is a 14,000 current platelet count.

Jason
It's soul destroying treatment after treatment failing to help.

Apart from Prednisolone (which I'm still tapering off 4 years down the line) I've tried Azathioprine [Imuran], MMF [Cellcept] , Fostamatinb, Romiplostim [Nplate} & Eltrombopag [Promacta]. The first 3 didn't work. Romiplostim did for about a year, then it stopped working quite abruptly.

I've been on Eltrombopag for just over a year. My counts aren't that high which suits me, anything over 80 and I start to twitch incase they keep climbing.
So long as count is over 30 I'm asymptomatic. I'd be quite happy to run at a count of 30 with no meds but unfortunately the only thing that does that is more prednisolone than I'm prepared to take long term. Quite frankly I'd rather bleed to death than be in the living hell of high dose steroids.

I have discussed Rituximab, which will be probably be my next choice should Eltrombopag let me down.

My spleen is staying put.

Anne

Jason. Yes, this ITP game is no fun.
When I failed Rituxan it set me off on a long study about what drugs do and do not help someone with a row 3 response - which I have. About the same time a study came out claiming that they could only find 4 types of ITP antibodies. So with that, modified my study to try to identify and include those other responses too. That is how the table was created. Hopefully to make ITP treatments less of a 'rolling the dice' game when considering a harsh drug. Rituxan made me do it.

About steroids. There are some ITP drugs that are considered 'steroid sparing' drugs. Drugs which can perform like steroids but can be taken for a longer period of time. Just off hand, I think those include Danazol and Dapsone. But you've got Fostamatinib on the horizon. Doesn't seem like there is a need to go into those, or Cyclosporine, right now. For that matter, Nplate could well get you to where you want to go in a few days. I can assure you that those reading are anxious to hear a good news report.

Thanks for the detail on your counts. It really shows your good response to high doses of steroids. I didn't expect that. Let me ask about some earlier detail. When you had that count jump up to 58 last August. When going from 50mg of Promacta to 75. Do you remember how many days it was between going to 75mg and that 58 count blood test? I assume it was less than 7.