When I was first diagnosed, I knew absolutely nothing about MPNs. And for a while…I was totally fine with that. But after a few months of not knowing what was going on with me, or my treatment, I decided to do some research. Some of what I learned was reassuring, and comforting. Some of it much less so. (I should add that the majority of my research was done on the internet because, honestly…where else does a freshman in college do research…) The internet is a wonderful place to find information; but I advise using large amounts of caution – the internet can also be a scary place.
One of the first things I saw when I started researching was that the disease could…change…and become something more serious. Like Myelofibrosis, or Acute Myeloid Leukemia (AML). This terrified me. I didn’t really have hard evidence to tell me that I would progress to MF, or AML…but it was in the back of my mind that it was a definite thing. While the likelihood of it actually happening is pretty low, it is difficult not to focus on. Toward the beginning of my diagnosis I was always paranoid or thinking the worst, and I had to force myself to stop jumping to those conclusions.
While at the Chicagoland support group, one of the questions I asked Dr. Stein was about disease progression. How likely is it really? He said that the risk of progression to MF from ET goes up to about 10% after 15 years, and for PV patients it was about 15% after 10 years. I felt a lot better after hearing him say that.
Even though 10% is pretty low, I was still curious about symptoms of disease progression, so I asked Dr. Ruben Mesa, of the Mayo Clinic in Scottsdale for some information. He very kindly agreed to help me. The following information is paraphrased from an e-mail I received from Dr. Mesa.
For patients with advanced MPNs, there tend to be many difficulties and symptoms which indicate progression. For example, a drop in blood counts; either bringing blood counts to relatively normal levels where before they required intervention thru phlebotomy or drugs, or bringing them so low they require transfusion. An enlargement of the spleen is often seen, which causes pain and discomfort. The bone marrow also becomes scarred.
There is still no definitive reason behind progression in MPNs. It is thought that JAK2 inhibitors might help to delay disease progression, and also that drugs like Interferon has been shown to help to delay it. Aside from that though, there is not a clear reason/form of prevention.
Another way patients progress is typically from MF to Acute Myeloid Leukemia. (it is less likely, but it is also theoretically possible to see progression from ET or PV to AML).
As a patient I still fear that progression from ET to MF one day, or even possibly AML…but I try really hard not to let it nag at me. When something strange happens (A new or different symptom, for example) I do wonder…but I don’t automatically assume the worst anymore the way I used to.
I know I’m repeating myself when I say this; but I strongly recommend being an informed patient, and a partner in your own health care. Things are much less frightening when you know what they mean, and it is much easier to take care of yourself when you know what is going on.
You are your own best advocate. If you do not take care of yourself, who will?
Until next time,