The phases of ET

Phase Zero:
Blissful Ignorance

ET is an "indolent, progressive, chronic myeloproliferative neoplasm," which means it is a slow-going cancer with no cure that does get worse over time. The issue of disease progression--how ET may worsen over time--came up on the Facebook page the other day, and I realized that a lot of ET patients don't really think about this, so let's dig in.

Doctors don't seem to talk about disease progression much. Instead they talk about risk factors. There are probably a couple of reasons for this. First, doctors are busy, so why invite a bunch of questions or get ET patients all upset by doping out progression possibilities that might never occur? Risk factors help assess where the patient is right now. Second, the progression of ET and other MPNs is scantily researched, and doctors can't tell you when or if you will progress to another phase.

But I think part of dealing with an chronic and progressive illness is understanding where it might go and be better prepared. So here are the phases I think about when I think about living with ET. These phases aren't "official" or come from a doctor; they just capture my understanding of what progression means.

Phase Zero: Blissful Ignorance. You don't know you have ET. Sure, maybe you're kind of tired and brain foggy, but the typical ET patient is a middle aged woman, and everything's going to be blamed on age, the Change, and low thyroid. So basically life is good, tra la la.

Phase Zero can last for years. I had a slightly elevated platelet count six years before I was actually diagnosed with ET.

In some ways, Phase Zero is a dangerous phase because you are not receiving monitoring and treatment that might reduce your clot risk.

Phase One: Early Diagnosis. You find out you have ET, an incurable cancer. You recall everything you know about treatments for acute cancers, and you start to freak out.

But ET isn't an acute cancer. Your platelets aren't that high. Your doc tells you to take an aspirin every day, get periodic blood counts, and come back every six months to a year. This phase can last years or even decades. Life is good again, Tra la la.

In my view doctors should be talking to patients about how to reduce their overall clot risks in Phase One. Now is the time to work on weight loss, lowering cholesterol, and improving your circulation with exercise. It won't make any difference to the progression of your ET, but it will help improve your overall health, and that's going to help your general quality of life.

I know. Easier said than done in late middle age, but even small changes are better than none.

Phase Two: Clots and Chemo. Some people will progress from Phase One to Phase Two in the normal course of the disease.

Other patients will jump from Phase Zero to Phase Two because they developed a clot and or some other ET-related ailment that wasn't diagnosed in the early phase. Or perhaps they just haven't had a blood count in years, so nobody noticed the platelets creeping close to or over a million.

However you get to Phase Two, you will need to take chemo, probably for life.

The word "chemo" conjures up visions of your hair falling out, throwing up, and feeling tired all the time. But chemos for ET are milder than the infused chemos that acute cancer patients get, and they're oral (or injectable in the case of Interferon), so you take them at home.

In this phase, you and your doctor figure out which chemo works for you and establish the lowest dose that controls your platelets.

Once the doc starts talking chemo IT IS CRITICALLY IMPORTANT that she or he knows about any other health problems you have. For instance, anagrelide (Agrylin) can worsen some cardiac conditions. (I know because it was nixed as a possible drug for me by my cardiologist who monitors my wonky mitral valve.) So ask lots of questions, find out what the side effects are, learn how you can deal with the side effects, and discuss what side effects might need immediate or emergency attention.

Patients in Phase Two are often eligible for drug trials. Discuss this possibility with your hematologist to see what's involved if you are interested

Sometimes your chemo works fine for many years and then stops working. Your doc may want to increase your chemo dose, try another med, or try a combination of drugs. At this point, you may want to see one of the hotshots at a specialty clinic to see what the latest new thing is.

I have heard of a few doctors letting patients take "chemo vacations," going off the chemo for a time once their platelets are stable in the normal range. I don't know much about how this is decided, so let us know if you have info or experience with this.

In Phase Two you continue periodic blood counts, and you may visit the doctor more frequently than in Phase One. But you realize your hair is still there, you're not throwing up all the time, and the fatigue is intermittent. Tra la.

Phase Three: Morphing. Most people don't progress to this phase. Let's all breathe deeply and say it again together: MOST ET PATIENTS DON'T PROGRESS TO PHASE THREE.

But sometimes ET will morph into a more serious MPN such as myelofibrosis (MF) or, in rarer cases, chronic or acute leukemia.

If your disease morphs, you will need more monitoring and perhaps different medication. This is definitely the time to get second and third opinions. Many of the Phase Three diseases can be managed, and you are also in territory where there has been more research and there are more medical options.

The Bottom Line: I'm offering my thoughts on disease progression in hopes that you will have a better idea of how to take care of yourself now and in the future. I also hope it will help you think about things to ask your doctor at different periods in your treatment. ET may be incurable, but there are things you can do to improve your general well-being.

Be well!

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