Day by Day - Daughter with ITP

You never know with ITP. I tell our daughter, Mia (17, dx at age 12) that ITP teaches us to live with ambiguity, to weigh the pros and cons and make the best choice we can when it's not always clear what is the best path forward.

Over the past 5+ years, Mia's tried just about every drug there is for ITP (ivig, anti-d, rituxan (4 rounds on 3 occasions), NPlate, promacta and, of course, the dreaded prednisone along the way.) Eventually, all the drugs have stopped working and, when they do, Mia's numbers always fall below 10. We are blessed that she's not a bleeder. Our doctors have never hospitalized her for low platelets (except to treat) so, even when she's in single digits, she does school and life, except for sports and any obvious risks. That said, when her platelets are this low, she's beyond exhausted. She can barely haul herself through her days, sleeps a lot and is understandably sad. We have a 504 in place at school for these times.

Last spring, the promacta Mia was on quit working. (We had pushed the hematologist to try promacta; she had never heard of it. Argh! But that's another story....) Mia's platelets plummeted, and the hematologist pushed very hard for an immediate splenectomy. We got a second opinion and ended up liking the new hematologist so much, we switched to her. (Now at Children's Hospital, Boston). Mia's new hematologist believes that splenectomies, especially for young people, should be the very last resort. But even she came to believe that a splenectomy was likely the best choice for Mia. Still, the doctor tried to "jump start" the promacta by combining it for a short period of time with a very low dose of prednisone. And guess what? It worked! Mia was on a pretty high dose of promacta (75 mg/day), but she had platelets.

After several months, her dad and I began to worry about the long term effects of promacta, especially at a high dose. We all felt it would eventually stop working again, like it had before, like every other drug has for Mia. It's hard to know what to do: risk a splenectomy, with its side effects, when it might not even work? Or keep pouring chemicals into your child's body?

Working together with her doctor and us, Mia eventually decided to go ahead with a splenectomy. We came to this reluctantly and only after a lot of thought. I'm glad Mia (and her dad and I) made this decision on our terms and didn't feel pushed into by our doctor. To get her ready for the surgery, the doctor slowly weaned her off promacta over a couple of months. But guess what? Her platelets didn't drop. In fact, they stayed at 200, way more than enough. About 2 weeks ago, Mia came off promacta altogether.

We went a few days ago to the hospital for her pre-op appointments. Mia was exhausted and certain her platelets had dropped. In 5+ years, Mia has never had numbers above 15 without some kind of drug intervention. Her latest CBC came through while we were at the hospital: platelets at 216! The surgery was called off. Officially postponed until at least June, after she graduates high school.

So we take it day by day -- as always with ITP. But she still has her spleen and, for now at least, she's not taking drugs. I know Mia would love nothing more than to have ITP all "wrapped up" and dealt with before college, and I think she hoped the splenectomy might accomplish this. (We try to remind her, without sounding like fatalists, that even if you are in remission, ITP is always with you). We will see what happens, and I'm praying hard that this lovely interlude lasts for her as long as possible. But again I'm reminded that this disease teaches us all to live with ambiguity, to constantly reexamine both the data and our hearts, and to try and make the best decisions we can, understanding there is no perfect choice. I wish for all of you high platelets!

- F. Reed

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