Best treatment for chronic severe refractory ITP

This is my first post here and I was just looking for some support. My wife has had ITP forever (20 years). She had her spleen out 15 years ago and was in remission for about 10 years then had to be managed with intermittant dosages of prednisone whenever symptoms appeared. Now the prednisone isn't working, and her platelets are at 3000 and still dropping every day. SHe has had IVIG twice in the last two months. Platelets soar then tank again after a couple weeks. I I am actually in Japan which is my wife's home country. Here are my questions for anyone -

1. Does anyone else out there have chronic severe refratory ITP and if so what treatments worked for you if any?

2. Are any of you living a day to day like with almost no platelets or does no platelets mean you die?

3. Have any of you lived in another country while receiving treatment or gone to another country to receive treatment because of America's unforgivable medical costs. If so, what was your experience? Rituxan isn't covered here but I can probably get it cheap in Thailand.

I appreciate any comments. This is an absolute nightmare and I assumed that my wife's ITP was under control. Well you know what they say about assuming.....

Hi helldog,
We do have a number of people here with chronic refractory severe ITP, some live at counts of 10 or lower for years and years. Many have had counts of zero and didn't die! It seems that those fewer platelets tend to be larger and stickier and do their work pretty well. So it is possible to survive with those counts.

It does seem like your wife responds to IVIG - it is pretty normal for it to only last a few weeks. This means she has a 'rescue treatment' and can use IVIG to boost her platelets if for example she has a bleed or needs a surgery.

Have you tried nPlate or other treatments? Also expensive. But it works for some who have responded to nothing else.

Is your wife a bleeder when her counts are down or does she feel relatively ok?

Good luck, it can be scary but lots of people are living with itp and hardly anyone dies from it.
Erica

It is really important to know if she bleeds at low numbers. Another important consideration is age. When a person is over 60, the possibility of spontaneous bleeding and infections greatly increase in ITP patients. Be an advocate for you wife by reading up on ITP. Know what treatments are available and how they work. This pdsa website is a good source for this type of information. The more you and your wife know, the more in control you both will feel. Then you can be in partnership with your Dr to make the best decisions. Studies have shown that the more in control you feel the better the patient outcome. Keep us updated, we do care!

One more thing, when you have a CBC with a machine count, it doesn't get all the platelets. When it comes in as a zero there may actually be platelets. A manual count will be much more accurate. You might want to ask for it if the numbers are that low.

There are other treatments to try. The latest international guidelines list them all.. bloodjournal.hematologylibrary.org/content/115/2/168.full

Hi there. I have been on the ITP roller coaster for a year and a half and my platelets fall as low as your wife's. I too so far only respond to IVIG and then platelets fall. I recently saw James Bussel and asked about the risk of spontaneously bleeding to death. He does not recommend walking around with under 10k for that reason. He couldn't give me a percentage. Other treatments I've tried: rituxan, cellcept, and cytuxan. Hope you find something that works.

My son was more then 3 months with zero platelets and he is alive and well today. It was not easy, but he is fine. We did take pretty extreme precautions as he did bleed horrendously after a few doses of prednisone.

You might consider looking at India and a few very notable homeopathic doctors there. That is how we healed our son. I know it's not really close to Japan, but it's closer then the US.

yeah, I actually read about the sticky platelets that are common with ITP patients and I read somewhere that platelet fragment are still around after destruction which also helps with clotting.

I just want to thank all of you for your replies. It feels so good to hear responses from others with ITP. It is rare and I am happy to be able to find a place where others are willing to discuss this with me. It really is great and I totally agree with becoming as informed as I possibly can be. I have also been looking for a homeotherapist but it isn't so common here in Japan. I would love to try some alternative therapies, of which I have researched here and elsewhere, but I feel it is important to stabilize my wife enough that she can leave the hospital. I do have her taking noni juice, papaya leaf tea, pineapple juice, and chlorophyll everday, but that is just my own attempt at creating a body balancing remedy out of what I have read and what is available here in Japan.

Oh and one more thing. I am not really sure to what degree she bleeds at her current level or around 2000 or 3000. USually she will get a nosebleed that is mild but continuous, along with the usual petechia, wet purpura, and bruising, but the doctors have her on tranexamic acid pills which seem to stop the bleeding. I am happy or that but worry that she can build up a tolerance to these pills. Tolerance is a bastard and I wonder if that is the whole reason the prednisone doesn't work for her anymore. SHe might have built up a tolerance. Actually prednisone tolerance is a big question I have and I think I will start a new post about it.

Hello! I have chronic refractory ITP. I manage at low levels - work full time and have 2 jobs in the summer. I am very active and aside from the symptoms you described that your wife has (bruising, nosebleeds and petichae) live a very busy life. I run 1/2 marathons- last one with a count of 4.

I had some success with nPlate recently but all treatments seen to stop working for me after a period of time (prednisone, IVIg, spleenectomy, rituxan).

Wishing you good luck but want you to know that some can manage even at low counts.

wow, dyan! I will show my wife your post!