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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Two clinicians talk about hydroxyurea vs. pegylated interferon

Yup, age matters. In fact it's the most important factor that two
Italian hematologists consider when they prescribe treatment
for ET patients.

Drs. Elisa Rumi and Mario Cazzola, two Italian hematologists, discussed how they treat ET patients in this fall's issue of Blood Journal.

While the docs are clearly up on all the latest info, and they "slice and dice" patients by mutation, risk, age, familial factors, etc. ... they pretty much come up with just three three different first-line treatments: Baby aspirin only, aspirin + hydroxyurea (Hydrea), or aspirin + pegylated interferon (Pegasys). Age seems to be the determining factor in who gets what.

I've joined the CALR-positive club

My CALR test bruise is a beaut! I learned that an ice pack will
take down swelling and relieve discoloration quicker. Also that
it's really hard to take a photo of your own arm with a tablet.

Last week I went to a specialty lab to get tested for CALR. The specialty lab was like something out of a David Lynch movie and resulted in the worst case of swelling and bruising I have ever had from a blood test. The upside is that it put me in touch with my inner fifth grader, allowing me to show my prodigious bruising to all my friends to astound and gross them out. (Tip: If this happens to you, icing the swelling will reduce it quicker.)

Anyhow, today, the results are in, and I am now officially in the CALR group of patients with ET, specifically CALR exon 9, which I realize, sounds like something Scotty would need to add to the dilithium cyrstals in order to move the U.S.S. Enterprise into warp speed.

There was also a whole lot of blah blah about the test on the results I got that I will ask my GP to translate when I see her for my regular check-up in a few weeks. Since my treatment (a baby aspirin every day) is not likely to change, I'm not going to try to get past the army of schedulers to see Dr. Blood about this before next year's appointment.

Anyhoo, now I'm busy relearning all the info I amassed in the past year about CALR-related ET. IMPORTANT CAVEAT: The CALR mutation and its link to ET has only been known for a few years. So saying anything definitive about it is impossible. However, short-term studies show the following:

Sleep again

You can catch Americans catnapping all over the country. Sadly,

the real cure is more and better sleep.

Yesterday I headed off to the grocery store to buy the turkey for Thanksgiving. My menu is going to be super simple (and the recipes are at the end of this post).

But in trying to pick up the items in the supermarket, the aisles seemed packed with people who seemed loggy and unaware. Carts were blocking aisles, people were calling their families to remind them what ingredients they needed, and one woman was holding two large cans of pumpkin as if she were trying to read the label in a foreign language.

It was like one of those zombie films. Or maybe I was just looking at a lot of sleep-deprived folks trying to get up the energy to deal with another holiday.

I am not a hypochondriac junkie! I am a '54 Chevy Bel Air!

1954 Chevy Bel Air. Maybe runs hot, needs a few valves replaced, a little
chunky by today's standards, but still has some good lines. Turns over in sub-
zero weather. Yes. That's me!

OK, that title will make sense in a minute, I hope.

Annual check-up with Dr. Blood today. Good news is that all those 50- to 100-point fluctuations in my platelet count she considers "stable." She checked my spleen and other blood work and said those seemed fine.

Doc said to continue with having blood draws every three to four months and to call in if platelets jump to 900 or higher (currently my high readings are about 750). She said I should keep taking a baby aspirin every day. She noted that enteric coated aspirin would help with any stomach upsets. Most baby aspirin is enteric coated (look for "E.C." on the bottle), so just passing this along as a tip for anybody else on aspirin regime.

She also said I could stay stable for a period of years, and I'll take that, though, in reality, even the hot-shots don't really fully understand how quickly your ET might worsen or why it might morph into some other disease.

But then things took a strange turn.

... and now some thoughts for those concerned about Obamacare

Whatever your feelings about the recent U.S. election generally, if you rely on the Affordable Care Act (aka Obamacare) for insurance, you probably have some concerns about your coverage given that many of those elected ran on platforms that included repealing and replacing it.

As someone who relies on Obamacare myself, I am offering two lines of thought here that might offer some practical info. First is how quickly the repeal and replace effort might happen. Second is what you can do to anticipate gaps in your coverage.

Hydrea's side effects?

Ever feel like this when you start taking
some new medication that your doctor
hasn't clearly explained to you?

This question comes up a lot on our Facebook page and on cancer discussion groups: "I just started taking hydroxyurea (Hydrea). Is it normal to ...?"

My mother is a cardiac patient who has had open-heart surgery, persistent atrial fibrillation, and a host of complications. I will spare you my rant on doctors who prescribe medication without explaining a) what it is supposed to do for you, and b) what side effects you might need to report.

Instead, let's pause while I a take a nice cleansing breath and scream at the needless worry and confusion that our doctors create because their offices are chock full of sick people and they don't have time to explain things.

Ah, that's better.

Now let's take the bull by the horns and get some info that might help us reduce needless worry.

First off, here's the link to the Mayo Clinic's page that lists Hydrea's side effects.

My self-improvement kick #4: Tricking myself into exercise

It's trick or treat season. Here's our jack-o-lantern, which reminds
me I'd really like one of those pumpkin muffins every coffee shop
sells right now. I'm trying  to trick myself into more exercise and
fewer of those muffins. 

So, this summer was hot and muggy up here in the Midwest, and don't care much for summer in any case. So I did a lot of sitting on my fanny in the one room that has air conditioning and trying not to eat too much macaroni salad. As a result, I gained back a few of the pounds I lost.

I'm also coming off a couple weeks of Prednisone for a lung infection, so didn't get a lot of exercise for about a month. The Prednisone always zips me up, taking away a lot of the ET-related fatigue and with the added side effect of making all the all the back and neck pain I have from a severe spinal curvature away. For two weeks I felt 45 again! Now that the course of Prednisone is done, I feel like I've been hit by a Peterbilt.

About that high LDH and enlarged platelets (and more) ...

Global Handwashing Day. It's a thing! It was October 15.
But EVERY day can be handwashing day. And should be
if you have ET. 

Sunday I met with five other folks in my area to ask questions about ET blood tests and research.  Dr. Robyn Scherber, MPN expert from the Mayo Clinic, spoke to us by phone for about half an hour. MPN Advocacy &Education International (link at right; please check it out!) sponsored the meeting and the call with Dr. Scherber.

I've been puzzled by two things that show up in my bloodwork, and if you have ET, you've probably noticed it too. I asked Dr. Scherber about high LDH (lactate dehydrogenase) levels and the exciting news that "enlarged platelets detected" now appears on my CBC. Randomly googling these things will scare the hell out of you because you may think you're in liver failure or that your platelets have stopped working.

This is not the case.

ET group forming in Michigan; questions for the experts?

MPN Advocacy and Education International (see their link at right) will host an MPN patient support session October 23, 11-1 p.m. at Gilbert and Blake' in Okemos, Michigan. Please make this session if you can!

An "MPN expert" is promised (live or via phone). If you can't attend but have questions you'd like me to take on your behalf, please post them on our Facebook page.

Not sure if this group will be ongoing or is a one-off, but am excited to meet in person with others in my area who are in the same boat.

Will have a full report (and maybe photos!) after the event.

Be well!

Presentation videos available from Women and MPNs conference

MPN Advocacy & Education International wrapped up its Women and MPNs conference in New York recently. I am slowly working my way through the videos, but especially recommend Dr. Gail Roboz's "How To Be Your Own Best Advocate as an MPN Patient."

Roboz offers some really good questions to ask your hematologist and suggests ways to open up better lines of communications with your doctor.

I hope to get through the videos soon and share some thoughts about the conference presentations as soon as this pesky bronchitis subsides.

Meantime, though, here's the portal page for the presentations. Please do watch some of these and let us know what you found helpful here or on our FB page.

Be well!

Related: Need questions for your doc?

Related: Does your doctor CARE?

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What ET "fatigue" feels like

Ever have one of those days when you just
wish someone would hit your "power off"
emergency switch?

In our latest poll, I asked people to describe what ET fatigue feels like. For 80 percent of respondents, ET fatigue is a "lack of physical energy, not wanting to move," but "feeling 'burned out'" ran a close second with 70 percent of respondents.

These two responses tell me that what we often feel is not only physical but mental as well.

Case in point: Yesterday was the first day of classes for the fall term for me, and by the end of the day, I could hardly sorm fentences.

I mean form sentences.

Notes from the Virtual Roundtable on MPN Awareness

The MPN Research Foundation just released its roundtable on MPN Awareness on Facebook. You can access it on the foundation's FB page here.

As I listened, I took a few notes that some of you might find helpful. But please watch the program yourself. The sound is a little wonky at times, but generally all the information is intelligible.

1. MPNs are cancers in the leukemia family. In my encounters with other ET patients, I hear too many patients say that their doctors do not consider ET a cancer. This should be a red flag for patients. Either the doctor is not up-to-date enough to know that in 2006, the World Health Organization reclassified MPNs as "cancers" rather than "disorders." Or the doctor objects to calling MPNs "cancers," which means he may not take your ET seriously.

2. ET and PV have less affect on life expectancy and quality than MF. Only a small number of patients with ET and PV will see their diseases progress to MF or to AML. However, ET and PV do require management and monitoring to get the best quality of life possible. Sometimes this involves oral or injectable chemotherapy. It's not a fun prospect, but oral and injectable chemos have fewer side effects than the infused chemos for acute cancer patients.

3. The cause of the mutation for MPNs is not known. A few people with ET have high platelet counts but do not show one of three mutations associated with ET (JAK2, CALR, or MPL). Dr. Srdan Verstovsek in the video notes that the mutation might be caused by something inside the body (perhaps a genetic trigger) or something outside the body (possibly an environmental cause). More research is needed.

4. The severity of an MPN ranges from the relatively benign to the life-threatening. Most of us with ET can live relatively normal lives, especially if we stay well-informed.

5. The American Society of Hematology can help patients find a hematologist in their area who is knowledgeable about MPNs here. I was happy to see my hematologist on the list!

6. Info on MPNs has proliferated tremendously in the past 10 years. More research on effectiveness of treatment and disease progression has helped doctors better understand MPNs and care for patients. However, the biology of MPNs still needs more research, particularly to find out why some people with ET and PV will progress to MF or leukemia and others will not.

7. Patients should be part of their own care team. Doctors should be willing to answer questions about MPNs and to consider information that patients bring to them about their cancers.

8. MPN patients can help the MPN community. Sharing information on forums and blogs can help, just by helping patients feeling less alone. Because our disease is rare, it's easy to feel like a hypochondriac, so participating on forums reassures other patients that, no, they're not crazy.

9. MPN Foundation will roll out a patient registry to increase the amount of information available about patients with MPN. That registry is expected to go live sometime around the end of this year. (Watch this blog for more info!)

10. No two ET patients are the same. Disease progression is different for different people, even with the same diagnosis. Patients with ET will also respond differently to different treatments. Information from other patients is good, but when it comes to your treatment, your doctor should have the final say.

Be well!

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Our poll: Fatigue is the biggest problem for ET patients

Here's my cat Edgar. He's not
fatigued. He's just thinking about
how to get the gopher in my yard
out of its hole. 

Our last Periodic Poll revealed that 100 percent of ET respondents found fatigue the most bothersome part of dealing with their disease. Headaches were also mentioned by about half of the respondents. Bone pain visual distortions were reported by 18 percent of respondents.

No one reported itching or spleen enlargement as their most bothersome symptoms.

Because fatigue seems to be the worst problem faced by ET patients, our current Periodic Poll takes a look at how you define fatigue. Is it mental? Physical? Do you feel sleepy? Burned out? Run down? Talk about how you define fatigue (all responses are anonymous), and I'll report the results in a couple of weeks.

If you've found some good ways to cope with your fatigue--or ways to generate some energy in order to do what has to be done--please share them on here or on our Facebook page (link at right).

Be well!

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News about the VA and MPNs

Today I received a statement from MPN Advocacy & Education International, which is working to get MPNs on the Veteran's Administration list of "presumptive diseases." The MPN group has collected data that seems to show a link between Vietnam vets who were exposed to Agent Orange and the development of an MPN.

Getting MPNs on the "presumptive list" means that the VA assumes that a particular disease is related to a vet's military service and will provide benefits that cover disease treatment. (See diseases on the presumptive list for Agent Orange Exposure here.)

The statement from MPN Advocacy and Education International spokesperson Ann Brazeau reads:

Heatwave hiatus

Hey, fellow ET orphans. The heat index here in the Great Lakes region is hitting the mid to upper 90s, and I am beset with fatigue and brain fog. It's been a long hot summer here, especially for those of us not used to relentless heat.

So, until I can make sense again, I'm neglecting the blog until cooler weather arrives and I feel zippier, by week's end, I hope.

Meantime, I've posted some items on our Facebook page in the run up to September 8, which is MPN Awareness Day. Link to FB is in the column at right.

Hope it's cooler where you are.

Be well!

Mayo Clinic wants to hear from you

The Mayo Clinic is doing a survey of MPN patients and their symptom burdens. Specifically, they're looking at alternative treatments and how patients use them.

This is an international study, so please consider taking the survey here.

Be well!

EM and the weekly poll report

EM is rare, but ET can be a cause. It starts with
a burning sensation in hands, feet, ears, or face,
and can spread. 

File this info in your "something to keep an eye out for" folder: Erythromelalgia, EM for short. It's a rare condition that can be caused by ET.

A friend sent me an article from the Washington Post last week about a woman in Maryland who had a mysterious foot problem:

Along with the sensation that her feet felt unusually warm, the skin on the second toe of her right foot looked inflamed. Weeks later, she noticed a small blister. ... Changing footwear didn’t help. Sometimes her toe would itch and feel tingly. At other times, the redness seemed to lessen, but it never disappeared entirely.

New feature! The weekly ET poll

This blog and Facebook page are doing well enough to try a new feature: The weekly poll.

At top right each week you'll see a new question. You can answer and see the poll results in progress. This week's question: What do you worry about MOST as an ET patient?

The poll closes in a week, and I'll update this entry with the final results. Then a new question will be posted. 

If you have ideas for future questions and answers, please suggest them in the comments box or over on our Facebook page.

Be well!

Does your doctor CARE?

Doc lacks empathy? Try asking different questions!

Interesting article in the Washington Post today that shows patients may actually get better quicker if they have doctors who rate high on the CARE scale. ET patients have a chronic illness that doesn't get "cured," but how might understanding the scale improve communication with your doctor?

Let's take a closer look.

The CARE scale asks patients to rate their doctors from 0 to 5 (5 being best) on these 10 questions:

Ischemic digits and circulation exercises

Squeeze the foam ball for one second ...

... and release for one second, spreading
your fingers

I recently wrote about stroke risks for ET patients, but there is another risk you might want to be aware of, and that's ischemia in the hands and feet.

Ischemia is restricted blood flow, often caused by small clots in the hands and feet or further up the "line" in an artery or vein.

I don't have hard numbers of the incidence of ischemia in the hands and feet, fingers and toes of ET patients, but it seems to crop up fairly often in the medical literature. So pay attention to the circulation in your hands and feet.

Some tips:

Interferon, another ET chemo

Patients taking pegylated Interferon
often do so by giving themselves
subcutaneous injections at home.

While Hydrea (hydroxyurea) remains go-to drug for ET, some patients are taking Interferon, which has been a known platelet reducer since the 1990s. It still seems to be an experimental drug; studies of ET patients and Interferon have been small and have not yet covered long-term effects.

Here's what I've been able to find out about it:

There are two types of Interferon, alpha and pegylated. Pegylated Interferon stays in the body longer and seems to be more effective and have fewer side-effects.

A study in 1993 (before the three ET mutations were discovered), 51 German MPN  patients (26 of whom had ET) participated in a limited study. All patients in the study experienced reduced platelet counts, 78 percent of them lower than 450, which is the upper level of the "normal" range established by the World Health Organization.

Forty of the MPN patients were treated for more than three months with Interferon. Ten of those patients discontinued used within the first year or so because they could not tolerate side effects: nausea, fatigue, dizziness, fever, headache, diarrhea, weight loss, heartburn, hair loss, bone pain, and anemia. However, the study concluded that for younger patients, especially those with a history of thrombosis, Interferon treatment should be considered, and most research in the 1990s called the drug "promising.

TP, a temporary measure for reducing platelet counts fast

Therapeutic plateletpheresis (impress your friends with that term!), or TP, is basically a process that "vacuums" out the excess platelets in your blood and returns the blood with more normal platelet numbers to your body. Blood goes out one tube and in another, so it's not the same as a transfusion, in which you get someone else's blood.

TP is not ordered routinely for ET patients, and there isn't a lot of info on it. Johns Hopkins and the Cleveland Clinic both note on their ET info pages that TP is largely an emergency treatment. For example, if you have had a stroke or other severe clotting incident and your platelet counts are over a million, the docs may decide your platelets need to be lowered before a drug like hydroxyurea or anagrelide can get to work on the problem.

According to the National Organization for Rare Disorders, plateletpheresis is the same process blood banks use to collect platelets from donors, and it will take platelet counts down immediately. NORD says that TP has not been studied much in ET patients, though the procedure has been used for decades in emergencies.

Anybody have any experience with this? Please chime in.

Be well!

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Ice! The hot new anti-inflammatory treatment

Cheap vodka and a couple of Ziplock bags can be used to make
a flexible ice pack!

I recently had a six-month blood pressure check with my family doctor and started whining about my periodic sciatic and neck pain due to arthritis. I told her I was taking low-dose aspirin for ET, and that I didn't want to take a bunch of pills. She suggested ice.

And, by golly, ice is my new best friend! This also might appeal to those of you interested in alternative therapies for aches and pains.

I have an ice bag (the kind you used to see on the heads of people with hangovers in old-fashioned cartoons). They're pretty cheap and widely available at your local chain pharmacy or big box store in the medical supplies aisle. Some even come in designer patterns.

Thinking about stroke risks

I read a moving piece in the Washington Post last week about a woman trying to teach her mother how to read following a stroke that left her with alexia, the inability to read. As a voracious reader, it reminded me that those of us with ET are considered to be at higher risk for blood clots (aka "thrombotic events") of all types, and one of the most serious of these types of events is stroke.

So what does the stroke picture look like for ET patients and what can we do about it? The research can be confusing, but here are some recent research and practical ideas I hope are helpful.

My low-energy pot garden

Some of my pot garden plants: geranium, nasturtiums, mariachi
peppers, rosemary, gerbera daisies and some anemic basil. Never
have much luck with basil for reasons that elude me.

Ah, the Fourth of July weekend, often the laziest and most sultry weekend of the summer here in the Upper Midwest. It's a good weekend for watching birds and bunny rabbits, eating strawberry shortcake, reading a book in the lawn chair, and admiring the garden.

I used to have a big garden with vegetables and herbs. It had a fence around it and everything.

However, I don't have the energy for that any more, so I garden in porch pots. I used to be a snob about using only clay pots, but, frankly, those things are heavy to move around, and they suck up a lot of moisture from the plants. So as they busted, I started replacing them with plastic.

Positive thinking good for you? WOOP! Think again.

Strong positive thinkers often don't have an edge over more

realistic thinkers. 

I like "The Hidden Brain" segment on NPR, and this report explored the topic of "positive thinking," something many cancer patients try to (or are constantly being exhorted to) cultivate. 

While negativity can make you (and everybody around you) miserable, it turns out that strong positive thinkers may be worse off than those with a dash of more realistic, strategic thinking. 

Be well!

Musing on the latest platelet count

Platelet counts can bounce around like these kids on
pogo sticks.Remember those? I sure do. They were fun! 

Today's a good day! Got my quarterly platelet count results, and the platelet count was down a bit. Counts do bounce around, and it's a reminder not to get too excited about your periodic tests.

You may recall that I was up to 735 in March, an uptick of nearly 100 points from the previous quarter. My main concern was that the platelets would spike again this month and I'd need to talk to the doc about going on hydroxyurea (Hydrea). But for now, I'm back into the 600s (670), and, since no other blood counts are off, I'm still in the mild to moderate risk zone.

The blessing of a good night's sleep

It's the Archangel St. Raphael, 
patron of sweet dreams! Doesn't 
he look peaceful? Maybe he's 

watching over your sleep.

I woke up this morning after an unbroken eight and a half hours of sleep, and the difference in my energy levels and mood were noticeable. You may notice that a good night's sleep improves your alertness, clarity of mind, energy, and mood.

And you would be right; many scientific studies show that these are all benefits of a good night's sleep.

Sleep is especially important for those of us with ET because a) lack of sleep makes fatigue worse and b) good sleep reduces your chances of a stroke, something we already have a higher-than-average risk for.

Preparing to take hydroxyurea?

This photo has nothing to do with Hydrea; 

I just want to show off my cat, Flora, who 

is pretty, affectionate, and well behaved,

but does not want to pose for pictures.

This month I have my quarterly CBC test. At my March test, my platelets were up about 100 points, to 735; if they've risen, I'll need to contact my hematologist to see about starting hydroxyurea (Hydrea).

In getting myself prepared for the possibility of starting oral chemo, my first question is: How well does it work in reducing platelet counts that contribute to blood clots, the main danger with ET?

That question was answered 20 years ago in an article in the New England Journal of Medicine. Researchers followed 114 ET patients for a little over two years. They found that, among the control group (those taking no Hydrea), the incidence of a clot was about 24 percent. In the group taking Hydrea, the incidence was about 3 percent. The platelet count for those taking Hydrea stayed around 600. Those not on Hydrea had blood counts that varied between about 450 to 1.5 million.

Only 4 patients developed bleeding, just one was in the Hydrea group.

So, yup, it works, and that's why Hydrea remains the go-to drug for patients with ET.

Congress passes updated chemical safety bill ... and more

U.S. lawmakers sent a bill to President Obama yesterday to update the Toxic Substances Control Act.

For those of us with ET, this bill is important because it goes some way toward more strictly regulating toxic substances and makes provisions for studying "cancer clusters." Cancer clusters are areas that have unusually high numbers of cancers that could be linked to an environmental cause.

"Natural treatments" and ET

Turmeric! It's what makes your curry powder yellow

and your chick peas nice and spicy. Can it help with ET? 

Several people on this blog and the Facebook page have an interest in "natural" treatments for essential thrombocytosis. Turmeric and resveratrol have come up in conversation, but the hard scientific evidence about how well these substances work is sketchy.

But since there's a lot of interest in these treatments, here's what I found out, but first, a few caveats and disclaimers. (You can see from the number of "buts" that I'm a little hinky about this topic.)

Cancer deaths rose during Great Recession; is there a cause or a fix?

Cancer deaths in industrialized nations went up during the Great Recession. The Lancet, a British medical journal, estimated that about a quarter of a million more people died of cancer than expected between 2008 and 2010, and that the rate was higher in countries without universal health care. 

A Washington Post article published yesterday examines the Lancet study more closely and suggests that perhaps those without health care insurance postponed expensive diagnostics and treatment. But establishing a cause and effect between cancer deaths and access to health care is dicey. And even if there is a connection, what, if anything, do we do about it? 

As cancer patients, those of us with ET have more than a passing interest in this issue. Delaying diagnosis, monitoring, and treatment will affect our general health. So please read and ponder.

Be well!

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Better late than never: Michigan poised to pass oral chemo cost bill; Alaska could be next

Michigan may be the 40th state to approve a so-called chemotherapy parity bill that would bring the cost of oral chemotherapy drugs--the type most of us with ET rely on--more in line with IV chemo meds. The measure was passed May 10 in the state Senate, and should be considered by the House soon.

Without such laws, oral chemo tends to cost patients more than IV chemotherapies, partly because manufacturers charge more for them and partly because patients pay a higher co-pay for oral chemo vs. those given IV.

The bill has wide bipartisan support.

Read more at the Detroit Free Press site.

In April, the Alaska state Senate passed a similar bill. Read more here.

If you live in either of these states, please write your state House representatives explaining how much your oral chemo costs, particularly if you take Jakafi, which can run up to $7,000 per month.

If you live in Idaho, Montana, North Dakota, Pennsylvania, New Hampshire, Arkansas, Mississippi, Tennesse, North Carolina, or South Carolina, you may want to check with your state representatives to find out whether a chemotherapy parity law is in the works for you.

Be well!

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Remembering absent friends in May

May is National Cancer Research Month. May is also the birthday month of two dear people lost to blood cancer, and this seems like a good time to honor them:

Ronnie

In 2009, my close friend Ronnie died of a rare form of leukemia. Ronnie and I were college roommates (there she is left in those good old days), and she was my matron of honor. She was a hilarious and dear friend. As we got into our sixth decade, we envisioned a cable access show for middle-aged women called "Don't Get Me Started," in which we'd sit in ratty recliners wearing our bathrobes, drinking coffee, calling up elected legislators and bureaucrats, and exploding myths about anti-aging and beauty products. We never got it on the air, but we had some great times planning our "episodes." She died far too soon, and I miss her every day.

Dad and our son on his first birthday

In 2010, I lost my dad to complications from COPD. Dad, like me, had ET, though his was diagnosed later in life. While Dad didn't die from ET, he did have unpleasant side effects from chemotherapy as well as blood clots and numerous complications from blood thinners. Dad was always high energy and in charge. He loved practical jokes and arguing about politics. Although he was nearing 70 when our son was born, and was in failing health, he was the greatest grandpa to my son (there they are on his first birthday).

It was an honor to know Ronnie and Dad, and I invite you to remember those you've lost to blood cancers in the comments.

I also want to honor all the people who read this blog and follow its sister page on Facebook. Thank you for making me feel less alone with the mysteries of ET.

Be well!

Related: All in the family: My dad's history with ET

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Mukherjee's article on oncological treatment is worth a read!

This chart shows how certain genetic mutations found 

in cancers in one part of the body are linked to cancers

found in other body parts. Glad that's all cleared up ... 

Siddartha Mukherjee seems to be everywhere these days, first with his book, "The Emperor of Diseases," about cancer; then with his latest offering, "The Gene: An Intimate History," and now in the pages of the New York Times with a fascinating article about what he calls "improvisational oncology."

Mukherjee's article is worth a read for anybody with ET or another MPN because it delves into the very complicated link between genetic mutation and cancer, and underscores what we and our doctors have known for years: Not all forms of ET respond to the same treatment.

In the "olden days," before the genetic factors of cancer began to be understood, Mukherjee explains that cancers were classified by the parts of the body in which they occurred--lymph, breast, prostate, colon, blood, etc.--and chemotherapies were developed to address cancers in those areas as if they would all respond the same way. If they didn't, Mukherjee writes:

Why aren't you knitting?

Here's Madame DeFarge and her friends knitting while watching 

the beheadings during the French Revolution. I just knit while 

watchingTV.

Awhile back, I posted 10 tips for dealing with fatigue by MPN expert Ruben Mesa. Along with the tips that Dr. Mesa mentioned, add knitting. And yes, men, this means you, too.

Why knitting? Earlier this year, New York Times writer Jane Brody outlined the many physical and mental benefits for knitters. It does everything from lower blood pressure, to reducing cravings of smoking and eating disorders, to keeping arthritic fingers nimble.

I can testify, as someone who's been knitting for more than 50 years, that it reduces stress and helps you maintain a positive attitude. Brody points out that it's hard to get too downhearted when you see a mitten or a sock or hat or scarf emerging before your eyes. And there's always some new knitting skill you can master to impress yourself with.

Philadelphia chromosome?

This is the Philadelphia steak sandwich, not to be confused
with the abr/bcl rearrangement in the Philadelphia chromosome.
The sandwich is, however, quite messy to eat.

Researchers have used the Philadelphia chromosome to help nail down the diagnosis for ET for many years, though I just heard about it last week. The short story is that those who appear to have all the symptoms of ET but are carrying the Philadelphia chromosome with a certain abnormality should be diagnosed with chronic myeloid leukemia instead. Here's more info.

The Philadelpha chromosome abnormality is called "the abr/bcl rearrangement." And, yes, like so many things related to essential thrombocytosis (including its acronym, ET), the ABR/BCL Rearrangement sounds like a science fiction story about teleportation gone horribly wrong.

MPN Research Foundation/Incyte survey of MPN patients

OK, let me say up front that I am leery of giving info to Big Pharma like Incyte, the manufacturer of Jakafi. However, with Jakafi poised to be approved for use with some forms of ET at a price tag of $7,000 per month, I think the company needs to know that a lot of us won't be able to afford it. 

So I did take the survey co-sponsored by Incyte and the MPN Research Foundation as advertised on Facebook:

A research study (sponsored by Incyte Corporation) is being conducted to learn more about the impact of MPNs on patients’ lives. Incyte is seeking patients aged 18 to 70 with MF, PV or ET to participate in a one-time, 30-minute online survey about how your condition has affected your work, finances, and quality of life. If you are eligible and complete the survey, you will be compensated. For more information, please visit: https://www.surveymonkey.com/r/VX27NG2

The survey takes about 10 minutes to complete, and collects info about your household income and how ET has affected your work and lifestyle. This seems to be for patients in the U.S. only (the first question requires that you confirm you live in the U.S.).

Related: New York Times editorializes on drug costs
Related: Jakafi, oral chemo, and drug costs

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Nix on blood and organ donation

Even if you can't donate organs,
you can get a cup, pin, or shirt to
raise awareness about donor needs
at the Donate Life America site.

April is National Donate Life Month, and that might be a good time to remind those of us with ET that we should probably NOT be signing up for organ donations or giving blood, though, as always, check with your doc first.

The American Cancer Society offers cancer patients interested in donation some general donation guidelines, and the rule of thumb seems to be that you are not a good candidate for organ donation if you have an "active" cancer. ET and other MPNs are always "active" because they're chronic and there is no cure.

ET can also affect the liver, spleen, and bone marrow, so those tissues might not be healthy enough for donation.

While it's rare, cancers from donors have shown up in recipients. Researchers think that this could be because many recipients have to take anti-rejection meds, which lower their immunity and perhaps make it easier for the transmission to occur.

So, to keep everyone healthy, blood and organ donations are probably off the table for us.

The good news is that you can still support donation and medical research in other ways:

1. Volunteer to work at an an organ donation sign-up event. My college has a yearly bone marrow donation registration drive, and I'm happy to use class time to let the students sign up. They fill out a short form, the tech takes a cheek swab. They're back in class in five minutes.

2. Volunteer to help at or publicize a local blood drive.

3. Make financial contributions to the American Red Cross or your state/local organ donation organization.

4. Wear a Donate Life pin if you've got one. A co-worker whose husband had a heart and kidney transplant gave me one years ago, and I still wear it throughout April. Many people ask me about it and several have talked to their families and signed their donor cards. (Michigan makes it easy to indicate your willingness to be a donor by signing the back of your drivers license.)

5. Donate your body to medical science. The Huffington Post has a great informative essay on the how-to's of body donation. This idea used to give me the creeps, but, dammit, I've got a really rare disease, and I ought to be interesting as hell to some young medical student who wants to "look into it" in depth. Plus, whatever school you donate to (you do have a choice) will cremate you and return your remains to your family at no expense. So, if you have cheapskate tendencies like me, you can save your family a bundle "when the time comes."

Be well!

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Up in smoke? Medical marijuana and ET

I live in a state that approved medical marijuana, and, within weeks of legalization, clinics sprang up where you could pay a doctor $100 up front, get a "diagnosis," and smoke your dope legally. It was something of a debacle. People were getting authorization cards for everything from migraines to back pain to PMS. For a term or two, I had students wafting enough dope smoke into my classroom to give everybody a contact high. I had no idea there were so many suffering college students ...

It's been 30 years since I had a toke, but I honestly see nothing wrong with legalizing recreational use of marijuana and taxing it like liquor. But I'm also pretty leery of using marijuana as a treatment for ET, especially after doing some looking around on the Internet.

Google "essential thrombocytosis" and "marijuana," and you'll be inundated with lots of hits (no pun intended) for sites that make a lot of wild claims about cures and symptom management that look like they're designed to prey on desperate people. But there is some reliable info, and here's what I found:

A cuppa cuppa cuppa cuppa cup

Happy Drogo's Day, patron saint of coffee, coming up April 16.

As far as I know, St. Drogo never drank coffee, but he was reported to have been able to bilocate, which means he could zip around in two places at once to multi-task, Hence the connection with the effects of caffeine if not the actual drink.

Anyhow, whether you're into Catholic saints or not, make this Saturday a day to treat yourself to an extra special blend or brew. I'll have one with you. It could be a thing! A Coffee-Drink-In shared with ET patients worldwide!

And don't forget to thank whatever Higher Power you believe in for that friendly cuppa joe that refreshes and enlivens.

Prefer to offer your thanks in song? Here's "The Java Jive" by the Ink Spots. Is there anything that these guys can't make better with their extra smooth latte harmonies? No. There is not.

Be well and pass the percolator!

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What patients say about Hydrea

No, it's not Hydrea, it's a hydrangea,
which is prettier, though Hydrea capsules
are also sometimes pink and blue.

Hydrea (hydroxyurea) is the go-to drug used with ET if platelet counts go too high, and many patients fear it because it usually comes with some side effects AND because it may be linked to an increased risk for developing leukemia, though research isn't conclusive.

The Mayo Clinic lists side effects of Hydrea here. But a Web site called Ask a Patient collects comments from patients themselves about how they've responded to various drugs, including Hydrea, and I'm sharing what I got from that site in this post.

To put info together, I looked only at ET patients (not those with other MPNs or sickle cell anemia), since people with other diseases might respond differently to Hydrea. I also looked only at patients who have been taking Hydrea for more than three months, about the time it takes for the drug to work and for side effects to settle down.

And here are a bunch of qualifiers, caveats, and blah blah to think about.

First is that some of the side effects that patients reported are actually symptoms of ET, such as dizziness and fatigue. So disease or drug? Not clear.

Second is that Hydrea doesn't affect everybody the same way or with the same intensity; length of time on the drug and dosage may make a difference.

Third, women on Hydrea outnumbered men by about five to one, and most people were middle-aged (between age 40 and 67). So hair thinning and skin problems? Maybe a function of age as much as meds. (Ladies, once we get to a Certain Age, waking up one morning to find you suddenly look like your mother's older sister is not that uncommon now, is it?)

So, all that said, here's what I found out:

My self-improvement kick #3: Reading my way out of lethargy

Awhile back, I posted Dr. Ruben Mesa's 10 tips for fighting fatigue. One of them is spending less time on your computer and more time reading. I'm not sure that reading actually fights fatigue, but it does fight lethargy by improving concentration.

My recommended reading dose for lethargy is one or two chapters of something interesting. You'll either feel energized enough to get something done. Or you'll spend the rest of the day reading. Either way, you will feel less guilty for "wasting time," a feeling that plagues a lot of us ET orphans.

So in this installment of my self improvement kick, let me share some recent books to inspire you to get your own reading list going:

Carsick by John Waters. John Waters, whose compassion and admiration for the for the truly weird and grotty is legendary, decides to hitchhike from Baltimore to San Francisco. It's not something most of us with ET would have the energy to do (and probably not with John Waters, who, admittedly, is not for all tastes). But Waters sure knows how to write a travelogue. In fact he writes three: In the first part of his book he imagines his proposed trip's best and worst outcomes. Which, if you're familiar with Waters, aren't really that different; his imaginary stint with a carnie named Polka Dottie is memorable, but I can't remember if it was part of the best or worst imaginary trip. The real trip turns out to be less weird and a quite touching homage to the kindness of strangers. Added bonus: Waters offers his list of travelin' songs so you can make your own mix tape for your own road trip.

Mentos and Diet Coke: What really causes ET?

No, Mentos plus Diet Coke does not cause ET (probably),
but the way Mentos and Diet Coke cause a chain reaction
offers an analogy for how ET mutations might occur.
Click to see a cool Mentos/Diet Coke powered car!

Most ET research focuses on treatment and cure instead of what causes the mutations that lead to ET and other MPNs in the first place. In fact, I only found one such study in the International Journal of Research and Public Health. It was done on a relatively small group of mostly PV patients in Pennsylvania in 2015. You can read the whole thing here if you want.

The gist of the study is this: Researchers theorize there may be both a genetic and an environmental component to ET and other MPNs. Let's call it the Mentos and Diet Coke Theory to make things simple:

Researcher offers more info about CALR as a factor in ET

Dr. Kim Dao of the Knight Cancer Institute talks about the CALR (say it "cal R") factor in some ET patients. She gets a little technical at times, but the bottom line is that the discovery of CALR indicates that there are several "pathways" to ET, and researchers are trying to determine how those pathways affect ET progression, symptoms, and treatment differently.

The up side here is that the more pathways that are discovered, the more treatments can be targeted to individual ET patients for more successful treatment. The down side is that ET and other MPNs are complicated diseases, and that means that the one-size-fits-all treatments  now available (oral chemo and JAK2 inhibitors) may not work for everyone and lots more research is needed.

Hang in there and be well!

Related: JAK-2 or CALR?

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Getting on with life, one damn term paper at a time

College English teachers, even the young idealistic ones ones, will tell you that end-of-term grading is THE most grueling part of their jobs.

It's not that there aren't hidden delights in there. This morning I read one of the most entertaining papers ever about the invention of the flush toilet by a student interested in a public health career. It was chock full of facts about how toilets and sewers improved public health, as well as those fascinating tidbits that never fail to tickle the imagination.

Whom do you tell? And how? And when? And why?

Having an incurable rare disease that affects your energy levels and desire/ability to socialize inevitably leads to questions about whether to level with people about the fact that your life has changed.

Most of us with ET don't consider ourselves terminally ill. But if you've told people you are sick and have used the word "cancer" to explain your disease, some of the responses from people in the article,  "How To tell Someone You're Terminally Ill," from the New York Times Magazine will sound familiar. 

Interestingly, that same NYT magazine issue has a question in The Ethicist's column from a woman troubled by a promise she made to her sister, who was dying of breast cancer, that she would not reveal the news to their elderly mother. (Scroll past the question about the adopted sister to see the cancer question.) 

Here's my riff on the issues raised about whom to tell what to and when in these two articles as they pertain to those of use with chronic, incurable cancers like ET:

Essential thrombo ... whatsis?

A fellow "orphan" with another rare disease, CPS (carbamoyl phosphate synthetase), recently mentioned that she carries around a fact sheet about her condition because not every health care provider outside of her specialist has heard of it. If you don't educate them, she noted, they can't treat you properly.

I thought the fact sheet was a great idea, and promptly decided to swipe it and recommend it to you!

If you want something ready-made, you can download the info from any of the helpful links at right. You could personalize the sheet by jotting down your last platelet count and other blood numbers that are "off," circle the symptoms you have and medication you're taking, indicating dosage and frequency.

The only downside I can see to that plan is that some of these pages may be a little out of date because ET researchers are finding new info all the time. For example the CALR genetic mutation was just discovered a couple of years ago, and that isn't mentioned in some of the info. Plus most of the resources are multiple pages, and most health care providers aren't going to read something that extensive.

So, using my obsessive organizational control freak powers, I've developed a one-page template you can crib from and personalize:

Kudos and thanks to fellow OI "orphan"

National Public Radio announced that Gaelynn Lea, Duluth, has won its annual Tiny Desk Contest. The singer/violinist has osteogenesis imperfecta (OI), aka brittle bone disease. Only about 50,000 people in the U.S. have OI, making it an "orphan disease" that's about three times more rare than ET.

But being a fellow "orphan" isn't the nearly the most interesting thing about Gaelynn Lea. You can read the story from the link. But for my money, playing the video will tell you everything you need to know.

Thank you, Gaelynn, for bringing five minutes of beauty into a wintry, cold afternoon.

Be well!

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Share info for Rare Disease Day

It's Rare Disease Day! Time to write your legislators and educate your friends and neighbors about rare diseases generally and essential thrombocytosis specifically.

Helping people understand ET is difficult because we just don't look or act that sick. That doesn't mean that we don't feel diminished, crappy, or worried. We run higher than normal risks for serious blood clots, ischemia in the extremities, and debilitating headaches and fatigue. ET can affect other organs, such as the spleen and liver. It can become more dangerous when platelet counts rise and other blood cells get off balance. In addition, ET can turn into more severe diseases like myelofibrosis and rare and deadly forms of leukemia.

Fortunately, those of us with ET know we're in this for life, and we're a pretty tough bunch.

I offered some points in my previous post that you might want to include if you're writing to your elected representatives today. But don't stop there.