Sleep disorder? Time for the "wallet biopsy"!

Had my yearly checkup December 23, which was a time to regroup with my GP's office about all the exciting diagnoses of the past 10 months, including ET.

It was all good news re weight, glucose, and blood pressure. Then I made the fatal mistake of asking if there was something I could do to alleviate fatigue.

Writer explores "the pressure to say you're OK"

Los Angeles Times writer Adam Baer writes about the exhausting pressure on the chronically ill to say they're fine: 

As a survivor of multiple cancers and rare diseases, I've had to write my fair share of group emails alerting friends and family to medical news. So I understand the impulse to sound upbeat. Diagnosis emails and social-media posts are now a genre with set tropes and expected turns of phrase.

We swear we'll morph into rays of light during the darkest times in our lives. We vow to battle our infirmities — as if that were possible beyond seeking treatment — and to become “well again.”

New York Times editorializes on drug costs

Saturday's New York Times editorial on the cost of medication in America was timely given recent FDA approval of Jakafi for some MPNs. The drug will set you (and/or your insurance carrier) back $7,000 per month.

Water, water everywhere. So drink it already!

Some patients with ET (including me) say they feel better when they drink more water. It's anecdotal information mostly, though the University of Maryland's Medical Center site encourages patients with all MPNs to move toward a healthy diet, including adequate hydration. The idea is that the better shape you're in, the better your body is able to cope with the added stress of disease.

So how much water is enough?

Two interesting reports on JAK-2 and CALR type ET

UPDATE: January 15, 2016: Ruben Mesa, hematologist with the Mayo Clinic offers a video summary of more ASH info. Click below to play.

Original post, December 16, 2015: The MPN Research Foundation (see links at left) puts out a yearly report from the American Society for Hematology (ASH). Research updates from this year's conference are online now. Among the papers presented were two that caught my eye about ET, and specifically about the differences between JAK-2 ET and CALR ET.

Caveat, caveat, blah blah blah: It's important to remember that most of these studies are are small and of relatively short duration. It's also important to note that the genetic components involved in ET are relatively new discoveries. So I'm not making life decisions around this info. But the message I got is that it's probably important for you and your hematologist to try to nail down the type of genetic mutation that causes your ET if possible.

Here's more:

JAK-2 or CALR?

In the olden days, before the discovery of the JAK-2 genetic mutation, the only way to diagnose ET was by process of elimination of underlying causes for an elevated platelet count. And even though JAK-2 tests may run false negatives (25 percent of the time, according to my hematologist), a positive JAK-2 helps your doctor initiate treatment and monitoring quicker.

But JAK-2 isn't the only mutation associated with ET.

Last year, Blood Journal reported a study of ET patients with two different types of genetic mutations, JAK2 and CALR (calreticulin). The bottom line for us patients is that those with the CALR genetic mutation seem to have a much lower thrombosis risk than those with the JAK2 mutation. That's signicant info given that the greatest risk for ET patients is clotting.

Here's more:

For Giving Tuesday: How much was your bone marrow biopsy?

On Giving Tuesday, I got the bill for my bone marrow aspiration and biopsy. Looks like I'll be giving a few hundred bucks to the pathology center in the near future!

While I realize that ET is one of the cheaper forms of cancer because it doesn't involve chemo infusions, radiation, or many oral meds (heck, I just take aspirin for mine), "cheap" in Cancer World is a relative term.

As researchers look for the elusive cures for these diseases, maybe somebody should be taking a good hard look at ways to control the costs of diagnosing and treating cancers, or at least providing some price comparisons for procedures and diagnostics so you know if you're getting ripped off.

You think you're fatigued now? Here comes Christmas!

First day of Advent, and, as Archie Bunker used to say, "The Christmas season is at our throats again." And that means increased fatigue. While doing research for my previous post, I learned that "cancer fatigue" is a real phenomenon that persists despite rest and sleep (though you certainly need both!). Patients with ET have that kind of fatigue. And that makes creating the "magic of Christmas" for your family a whole lot harder.

Fatigued? You're not alone

We live in tiring times. Google "Why am I so damn tired all the time?" and you'll get over 3 million hits. However, the results of a study in the journal Cancer in 2006 indicated that the fatigue factor for ET patients, even those considered "asymptomatic," is significantly higher than in the rest of the population.

Although the study is nine years old, drug therapy and treatment protocols for ET haven't changed much, so I think the results offer some interesting insights about how patients measure their fatigue and general quality of life. Certainly the study underscores the need for palliative care that improves quality of life as much as research in pharmaceutical cures.

So here's more about that study.

My self-improvement kick: Update #1

When I found out a year ago that my wonky mitral valve had progressed to moderate/severe regurgitation and the need for surgical repair was "probable," I started on a campaign to get myself in better shape before surgery might be needed. Then I got hit with the ET diagnosis a few months later.

Turns out, most of the things on my self-improvement list (below) are those the Mayo Clinic recommends for ET patients, too.

1. Lose weight (my BMI was 30.9)
2. Eat better (my glucose had spiked up)
3. Get more exercise to combat fatigue and back pain
4. Maintain healthy blood pressure
5. Reduce stress

Jakafi, oral chemo, and drug costs

Jakafi (ruxolitinib) is a relatively new drug on the market prescribed for some MPN patients who don't respond to other types of oral chemo. A recent study indicates it could be a good treatment for ET. (Right now, the FDA has approved Jakafi for myelofibrosis and polycythemia vera, two of ET's sister MPNs.).

Good as this news is, it raises concerns about the high cost drugs for rare diseases.

Welcome, fellow orphans!

Essential thrombocytosis/thrombocythemia is an "orphan disease," that is, a disease that is a) largely ignored by medical research because b) very few people have it, which means c) there's no money to be made treating it.

A National Institutes of Health article from the British Journal of Pharmacology explains the landscape for orphan diseases and treatments and why ET's designation as an orphan disease matters.

Need questions for your doc?

Don't know about you, but a trip to the hematologist at the regional cancer center usually gouges a good half day out of my schedule, what with driving in from the sticks and waiting around for the doctor to get done with the really sick people.

Writing down my questions ahead of time can get me the heck out of there faster. And, using my hyper-organized control freak super powers, I made a question tree to take with me to every visit. That way, my notes are all organized, and I'll be able to decipher them before my next visit.

You can make your own from the image provided here (click the image and it will enlarge). Or message me at facebook.com/ethrombo, and I'll be happy to send you a Word or PDF file.

Be well!

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Do you know what your platelet count means?

A platelet (aka thrombocyte) count measures how many platelets you have in a microliter (mcL) of your blood (which also contains red and white cells of various types). My last platelet count was 635. A normal platelet count is between 150 and 400 (sometimes the upper limit is set at 450 by different health centers and organizations). But here's the important thing: A platelet count of 635 means that you have 635,000 platelets per mcL.

Why is this important to know? Because if you don't understand what the number means and you learn that your platelets have jumped from 750 to OVER A MILLION you will be terrified and think you are dying. This happened to my dad because:

a) His lab tests were poorly explained to him, and/or
b) He was busy telling the nurse a story and wasn't listening.

... and I've got my brand spankin' new diagnosis

So the bone marrow and other tests done in October weren't a wash after all. (Just because you have access to your own online chart doesn't mean you can understand it.) Hematologist said today that the morphology was consistent with blah blah blah and etc. etc. Bottom line: essential thrombocytosis (aka thrombocythemia) is confirmed. I also learned some new things.

Bone marrow washout

A whole bunch of blood tests haven't yet confirmed the ET, so at my September visit with the hematologist, she asked if I would "be okay with" a bone marrow biopsy and aspiration in that way that makes you nervous about why you wouldn't be okay with it.

So that's what I asked: "Why wouldn't I be okay with it?"

How 'bout a dash of mutation with that genetic cocktail?

If you want to know all the ins and outs of genetic mutations and myeloproliferative neoplasms (of which essential thrombocytosis is one), get a Ph.D. in biology. But if your doctor suspects you have ET or some other MPN, s/he will probably look for a Janus kinase 2 (aka JAK2) mutation that causes your bone marrow to produce too many platelets, and that test is useful to understand.

How's your spleen?

Some patients with ET have enlarged spleens ("splenomegaly," which comes from the English word "spleen" and the Greek word "megas" or "big." Just in case you're going to a cocktail party where people enjoy word trivia.)

Your turn!

Tell us who you are and what your experiences with ET have been in the "comments" box. Browse the posts and add your own info and experiences, or just ask questions. If you can't find what you're looking for, try the search box in the top left of the nav bar. Your comments, questions, and stories will help others with ET.

We're all in this together.

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About ET

Essential thrombocytosis is a pretty rare condition in which your bone marrow produces too many platelets. According to info from the MPN Research Foundation, there are about 134,000 of us ET patients in the U.S. at any given time. If you live in a hick town like me, you're not likely to know anybody else with ET. If you live in an average sized American town, there might be eight other people who have ET, about the right size for a book group, holiday decoration committee, or a crack team of international jewel thieves.

About me and this blog

I'm a college teacher in the Midwest, in my 60s, and like many of you, my essential thrombocytosis was discovered by accident.