Update- 1 year after diagnosis

I just wanted to post an update about my dad who had a sudden onset of ITP last thanksgiving, almost a year ago now ( read the previous saga on the forum thread here )

He had a sudden onset of ITP with bloody nose and platelet level down to 1. He was treated with IVIG, Prednisone, Rituxan, daily platelet transfusions and finally Nplate during a hospitalization that lasted 7 weeks. Following the discharge from the hospital he was treated with Azathioprine and Heparin (in the form of injections of Lovenox for a minor blood clot in his leg)

That's a lot of drugs, but the good news is 9 months after he was discharged he not taking any drugs and his platelet levels are stable in the 200's. A specialist who we saw at USC medical center diagnosed him as T-cell dominate and the hypothesis is that the combination of Nplate and Azathioprine may have induced a remission. According to the specialist there's a 1/3, 1/3, 1/3 breakdown for patients who follow this regimen. A third show no improvement, a third have a temporary remission and a third have a permanent remission. So it looks like he is in one of the latter two camps.

A few takeaways for those who are newly grappling with ITP:
Everyone is different when it comes to ITP, but here were some lessons we learned-

-See a specialist: Most Oncologist/Hematologists are 95% oncology and 5% hematology, and a lot of oncologists don't know much about ITP. Most likely the best bet for a specialist is going to be your nearest teaching hospital.

-Be your own advocate: If we hadn’t he wouldn’t have a spleen right now

-The new treatments work: Not necessarily for everyone, and not necessarily permanently, but in my dad’s case, Nplate led to a remission.

-Be your own expert: Get involved on the forums (lots of knowledge here), read the blood journal, paper your doctor with articles, whatever you need to do, stick with it.

Great news! Thanks for the update! So glad to hear you father is in remission- that is awesome! Also good information about Aza + Nplate. I will look into that for myself- Rituxin didn't work well for me so I assume its not my B-cells causing ITP.

Newer research shows that Rituxan also targets T-cells, so I wouldn't necessarily assume that. I would gander that you might have production problems as well as destruction which is why you are unresponsive to most treatments. It's just a guess though. There are a lot of different processes and variables with ITP.

I am glad to hear the news about your dad and hope he stays in remission. We are coming up on the one year anniversary for our daughter, age 13. We've done AntiD and Ivig several times. Nothing has stuck. for past 6 months have followed the "european protocol" of just watching carefully. She is not a big bleeder so we are lucky and can do this. That said, she does get a lot of bruising. When she is low (or what I imagine is low since we do fewer cbs's too) she gets pettichae and some small blood blisters in her mouth -- which, as her mom, make me very nervous. but I've become much more calm about all this. We have a great hemo who is indeed as you recommend a hemo. She's been on board with no treatment but now is talking Rituxin or spleen removal. Ugh to both. Since our daughter has relatively few symptoms, I'm interested in people's thoughts. My husband, who has mostly let me lead the way on this, has read about Rituxan and really doesn't want to do it given her relatively few side effects. I'm with him. So hard to know the right thing to do. I am grateful beyond measure to Dougie's mom, Ali: a voice of reason and calm.

I'm really interested in other people's thoughts, esp Sandi's, although I know in the end this our family's decision (or non-decision decision).
THANKS for this wonderful site.
Firkins

ps My daughter btw just had her bat mitzvah and received a couple of checks which she was able to direct to a charity of her choice. She chose PDSA. Even though she never comes on the site, she knows how important this site is and the work and support it enables.

Firkins,

I don't know how far you've gone back on the boards, but this is my reasoning for not even considering the splenectomy approach for Dougie.

www.pdsa.org/forum/14-social-chat-for-pa...moval-of-slpeen.html

Answers would come out different for everyone, and so would the end result, but I think they are good questions.

Hope the link works

Ali

OK, it doesn't seem to work - topic is five down in Social Chat for Parents

P.s. I can't take credit for my reason and calm - I inherited it from Donna (Lucidawn), mother of Tim who calmed me down in the days before I chilled out!

Well I credit you, Ali! I will look through old threads. I need to, but other daughter is struggling to with her own health issues and I'm feeling tapped -- oy. I'm really very sanguine about Mia until i see the blood blisters inside her mouth. Some kind of trigger for me. My tipping point. I told Mia we might need to go to MGH (the hospital to see doctor this weekend) and she announced fiercely: "Well, you can go, but I'm not. I'm going to Vermont to walk in the woods."

Oh, out of the mouths of babes, as they say. So off to Vermont we go with Ali and Dougie's spirits to guide us.
Best, F

Love that response - the girl's got style!

Firkins:

Tough choices. I do have opinions on splenectomy but that is really all it is, my opinion. I can only tell you what I think and I'm not sure how much that is worth; I'm not a doctor. However, I am someone who has followed the people on this board since 1998. I have seen it all. I used to be able to remember each person and their situations, but over the years there were just too many to keep up with. Anyway, this is what I think:

First of all, I would recommend reading Alison's post. She has made some excellent points and if I were the parent of a child with ITP, I would consider what she has to say. Ali has a way with words and has a good grasp on understanding ITP. I'm sure you do too, but since you're asking for thoughts, her post is a good place to start.

My personal thought regarding splenectomy is that it should absolutely be a last resort. I never considered it for myself because I tended to look at the big picture. Part of the problem with ITP is that doctors want to fix it now. I understand that; that is the reason we go to them, to help us get counts up. We ask for their advice and that advice may include the suggestion of splenectomy. Due to the nature of ITP, the automatic response is to focus on the moment....now. The problem is that no one looks ahead. A teenager has about 60 years of life ahead. Can they get through that time without a spleen? I tend to look at the future. "What if's". What if the patient develops another autoimmune disorder and needs to use immunosuppressants? What if the patient develops cancer and needs chemo? The spleen would be very handy to have during those instances. What if the patient develops APS (33% of ITP patients do) and has clotting issues? Being spleenless can contribute to the risk of blood clots. What if they become young adults and do not keep up with the necessary post-splenectomy vaccines? Sepsis is a possibility even with the vaccines.

I don't know.... you can play the "what if" game all you want, but are those risks greater than the risk of bleeding with ITP? Only you can decide that. Splenectomy isn't as popular as it used to be and is falling slowly out of use. Very few doctors know that though and are still following the old protocol. The UK has a very different way of treating certain disorders and I tend to think they could be right in a lot of circumstances.

My daughter was diagnosed with Graves Disease a few years ago. One of the most popular treatments in the U.S. is to radiate and kill the thyroid. Her Endocrinologist recommended that and we were all set to go for it. Then I began to research and found out that in the UK, they do not radiate the thyroid, especially young women. They believe that it can be managed with medication, which is also an option here. When I started to read about the radiation, I found that a certain percentage of patients who were radiated end up with Thyroid Eye Disease years later, which can be disfiguring and affect vision. My daughter and I agreed that protecting her eyes were a priority for us, and using the medications gave some protection against that. So, she manages her thyroid levels with medication with relatively minimal side effects. She knows that the radiation is always an option that is out there, but has not reached a point where she thinks it's necessary. Point is, some of the most popular options for treating autoimmune disorders are antiquated, and do not take the future into account. Of course no one can predict what might happen later, but you can weigh the pros and cons and go from there.