Diagnosing ET and scoring your ET risks

Back in April, the Cleveland Clinic added some updated info in handy chart form that might be useful to ET patients, including the scoring system used by clinicians to determine your prognosis. For those new to ET, this info may help you understand what tests your doctor will order and what factors they look at when they assess your risks.

Here's the first chart:

Diagnostic criteria for essential thrombocythemia

Current (2008) WHO criteria	Proposed Criteria
Major criteria
Platelet count ≥450 x 109/L Megakaryocyte proliferation with large and mature morphology Not meeting WHO criteria for CML, PV, PMF, MDS or other myeloid neoplasm Demonstration of JAK2 V617F or other clonal marker or no evidence of reactive thrombocytosis	Platelet count ≥450 x 109/L Megakaryocyte proliferation with large and mature morphology Not meeting WHO criteria for CML, PV, PMF, MDS or other myeloid neoplasm Presence of JAK2, CALR, or MPL mutation
Minor criteria
	Presence of a clonal marker (eg, abnormal karyotype) or absence of evidence for reactive thrombocytosis
Requires all 4 major criteria	Requires all 4 major criteria OR first 3 major criteria and 1 minor criterion

CML = chronic myelogenous leukemia; ET = essential thrombocythemia; MDS = myelodysplastic syndrome; PMF = primary myelofibrosis; PV = polycythemia vera; WHO = World Health Organization.

Groundhog Day and Simpson Oil

The local worthies pose with Punxsutawney Phil, in Punxsutawney,
Pennsylvania, which has one of the more famous Groundhog Day
celebrations in the northern United States. 

Happy Groundhog Day, everyone!

If you don't live in a wintry part of the U.S., you probably don't know or care about Groundhog Day. But the lore is that if the groundhog comes out of his hole and sees his shadow today, there will be six more weeks of winter.

Today is very cold, clear, and sunny, but I'm not prognosticating the end of the winter by it.

And neither am I publishing comments pushing Rick Simpson's cannabis oil as a cure for ET or Simpson's book, Phoenix Tears, or other unsubstantiated cure claims here or on the FB page.

Why your doctor doesn't think you have symptoms

Is this how your doctor looks when
you tell him you feel fatigued, have
headaches, have bone pain, or just
feel crappy? 

I've been at this blog and Facebook page for over a year, and ET patients commonly complain that their doctors don't believe they have symptoms ... even though our symptoms are pretty well documented in "the literature," as the scientists say.

In catching up on my ET reading, I have some theories about this:

ET patients are most often diagnosed in their 50s and 60s. The fatigue and aches that can be a symptom of ET may be waved away as age by both patients and their doctors. And, to cut the doctors a break, it can be almost impossible to determine whether our ailments are age or disease related.

Doctors may also be skeptical about our symptoms because we aren't complainers. I was struck by an MPN specialist in one of the many YouTube videos available about ET, who noted that his patients seemed to be asymptomatic before their diagnosis and then, after they learned they had ET, claimed to have ET symptoms. It was pretty clear that this doctor felt his patients were rather suggestible; they only starting having the symptoms AFTER they learned what the symptoms were. 

Incremental care and ET

Dr. Atul Gawande's article in The New Yorker
about "incremental care" should make all of
us with ET think about the care we need to
maintain quality of life ... and which health
care providers are most likely to provide it.

Dr. Atul Gawande has been making the media rounds recently promoting a concept called "incremental care," and I recognized at once that this is exactly the kind of care most of us with ET need more of.

Gawande notes that research and money tends to cluster around what he calls "heroic care"--months of the latest cancer cure, major heart surgery, organ transplants--the kinds of treatment needed to cure an acute problem and make it go away forever. As I researched Gawande's interviews and articles, it occurred to me that "heroic care" is the kind of care that spawns colored ribbons, 5K cure walk/runs, and singles out the brave "survivors." These "heroic care" efforts are great, and we all have friends and relatives who have benefited from them tremendously.

But we ET patients are never going to be "survivors." Barring an outright cure, we're all going to die with, if not from ET. So incremental care will continue to be an important part of our treatment, and I think we need to make sure that we get it by educating and enlisting our primary care physicians more.

2017 ET events

Happy New Year, everyone!

MPN Advocacy & Education has released its education events for 2017. Please note that there are several programs taking place around the U.S. and one in Melbourne, Australia, April 28. (I know we have readers from Oz here, so please spread the word.) I do plan to attend the program in Novi, Michigan, in March. So if you have questions you'd like me to take to the event, please post them here, or visit the FB page where you can message me.

Also, Rare Disease Day is coming up February 28. This is a worldwide observance, and there may be events happening in your area. Check it out at the Rare Disease Day home page. I do hope to attend Michigan's event this year. I missed it last year due to a combination of health and weather crises. (Planning things here in the Upper Midwest is dicey between December and April ...)

Shortly, I hope to come up with some ways for you to observe Rare Disease Day and impact lives positively in your area. So watch this space for details and ...

Be well!

More diversity in clinical trials needed and other stories

The New York Times has a flurry of
end-of-the-year stories of interest to 
you as an ET patients. Hint: One of 
them tells you to quit sitting around
all the time.

Newspapers often sit on some really great feature stories until the end of the year, when the news is slow and there's more empty space to run them. Here are some great end-of-the-year stories that you, as an ET patient, might want to be aware of from the New York Times:

Denise Grady did an excellent feature report on the fact that, when it comes to clinical trials for cancer in the U.S., patients are overwhelmingly white. Her story focuses on K.T. Jones, who has been looking for treatments for an aggressive form of Hodgkin's lymphoma for the past 15 years. Jones, who is an African-American, has had good results from experimental treatments, but he is not typical. The report underscores that wage disparities among ethnic groups in the U.S. lead to under-insurance for so-called "minorities," and that this makes learning about and participating in clinical trials more difficult.

Peg interferon vs. Hydrea ... again

Pegylated interferon seems to be the hot new treatment for high-risk ET patients. Dr. Rubin Mesa, MPN expert with the Mayo Clinic, offers the results of two studies presented at the American Society of Hematology (ASH) conference held earlier in December that studied the effectiveness of hydroxyurea (Hydrea) and pegylated interferon (Pegasys) in high-risk ET patients.

Those studies, which followed patients for a year, showed:

• Both drugs were equally effective at lowering platelet counts and reducing the risks of clotting and bleeding.
• Peg interferon has more side effects that patients found difficult as the study progressed.
• Peg interferon may better the progression of ET over time, but more study is needed.

If you've spoken to your doctor about peg interferon or are taking it, please weigh in here or on our FB page (link at right). Watch the video below:

Be well!

Related: Two clinicians talk about hydroxyurea vs. pegylated interferon

Related: Inteferon, another ET chemo

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