ET? Tell your kids and siblings

Inherting a predisposition to ET doesn't
work in a predictable way, like
the yellow/gray genes in cats. At this time
there is no way to predict whether close
relatives have inherited a predisposition
to ET. And inheriting the predisposition
does not mean someone will develop
ET.

  

Rare Disease Report covered a study in August of this year that showed immediate relatives of ET patients have a 7 percent higher chance of getting an MPN than the general population. Those with a parent who was diagnosed with an MPN at a young age might be at a higher risk.

I wrote about the "diet coke and mentos" theory of ET genetics a couple of years ago, so the study shows more statistical evidence for that theory.

If you spend much time in MPN support groups, you will learn that many patients report they had a grandparent, parent, or sibling with an MPN.  Not all family members had the same MPN; remember that the mutations that cause ET can also cause myelofibrosis or polycythemia vera.

Hydroxyurea Adventure: The first nine months

There's me earlier this
summer, feeling pretty good,
all things considered.

I had my yearly hematologist's visit at the end of October, and Dr. Blood was happy to see a steady downward trend in platelet levels (down from about 800 to 550), and that I have no anemia or any other levels that are of concern.

My MCV/MCH were slightly elevated, which the doc says is a function of being on hydroxyurea, and this isn't something to worry about. She said to think of it as a marker that simply indicates I'm taking HU.

Since starting on the HU, I have had no digestive upsets or hair loss (though you can see in the photo at left that I was cutting my hair short just in case).

So all of that is good news.

The not-as-good news is that Doc told me to take two extra HU capsules twice a week to more quickly nudge the platelets into the normal range.

Thoughts for this Veterans Day

Today is Veterans Day (Armistice Day or Remembrance Day if you live outside the U.S.). This year, it marks the 100th anniversary of the end of World War I.

Once in awhile, I like to post something that's not about ET to remind us that, even though we have a chronic illness, we are still alive and still have the capacity to engage with the human condition, past and present. Maybe that's what we were put on this earth to do. 

So here are some thoughts on this special Veterans Day.

That's my grandfather, Corporal Clinton Foster, in the photo  at left. My grandmother, whom he would marry a few years later, dressed in her best summer whites, is standing at far left with his sisters. 

Grampa enlisted in 1917 and served Stateside during the war in the mailroom. 

His brother-in-law, my great-uncle Martin Keehn (his future wife, my Aunt Mary, is in the photo in the black hat at right), would be sent to France and serve out the war in a trench. Uncle Martin received a Purple Heart for wounds he received in battle. He would never speak of what he had seen.

Jakafi/lymphoma link?

Lymphoma News Today has a more about the Jakafi-
lymphoma link. 

Many of you have been hearing about a possible link between Jakafi (ruxolitinib) and an aggressive form of lymphoma. In two European studies of MPN patients, a significantly higher number of those those receiving Jakafi developed an aggressive form of lymphoma than those receiving another drug. This means that doctors should proceed with caution when prescribing this drug. 

Jakafi was first approved by the U.S. Food and Drug Administration for myelofibrosis patients in 2011. Jakafi was approved for polycythemia vera patients a few years later. It is not yet officially approved for use in ET patients, but some doctors prescribe it "off-list" if ET patients aren't responding to hydroxyurea or anagrelide (trade names Hydrea and Agrylin). 

Probiotics! Are they good for you?

I have lived through a variety of health fads, so color me skeptical of the hot new thing. One of the latest is probiotics, a group of substances that are supposed to be helpful for all kinds of bowel ailments as well as allergies, tooth decay, colds, etc.

Some people also take probiotics following a course of antibiotics, in hopes of restoring "good" bacteria to the gut.

According to the National Center for Complementary and Integrative Health (a division of the U.S. Department of Health and Human Services), more research needs to be done on probiotics before anything definitive can be said about their efficacy. Their page is really helpful.

I eat yogurt a few times a week because I prefer to get my calcium from food rather than pills and because I am mildly lactose intolerant. (Yogurt in small amounts doesn't bother me.) Yogurt contains lactobacillis, one type of probiotic. (Bifidobacterium is the other.)

I have never had ill-effects from lactobacillis in yogurt. That's because eating yogurt or some other fermented food that naturally contains probiotics is not the same as frontloading these substances through supplements, which usually give you a megadose.

If you are thinking about taking probiotics supplements, be aware that they may cause unwanted side effects such as infections in people with weakened immune systems--and that includes any of us taking chemo like hydroxyurea.

Bottom line: I'm not trying to create a scare here, only urging you to talk to your doctor before you start taking any supplements. Be ready to explain why you think the probiotics would help you and discuss dosing.

Meantime, enjoy your yogurt and be well!

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Related: Natural treatments and ET

MPN "specialists" and support groups

Things you hear about MPN "specialists" and other ET-related
issues on social media (including this blog) are usually things
you need to learn more about and discuss with your doctor. 

If you follow online support groups for people with ET or MPNs generally, you'll eventually encounter people who urge everyone to see an MPN specialist. Often, when people complain about their doctors, the first question others will ask is, "Is he/she an MPN specialist?"

I've been following these conversations for over a year, and I think we need to understand that there is no such thing as an MPN "specialist," and that things we hear in support groups (or on this blog that you're reading right now) are things we need to learn more about.

Chronic cancer in the workplace

ET fatigue and other symptoms can create problems for
workers with chronic diseases.

How many people reading here have quit working, taken early retirement, changed to less stressful jobs, or cut back work hours due to ET? I've done All of The Above. And I'm not aone.

The MPN Research Foundation shared a story from the Minneapolis Star Tribune about the plight of acute cancer patients for whom Americans with Disabilities Act protections are inadequate. The story touches on the complex problems cancer treatment poses for workers and employers: Some workers come out of cancer treatment unable to do their jobs, some workers need more than the 12 weeks of unpaid leave the ADA offers, and some patients are fired at the end of their leave period, losing their health care insurance. This can be financially disastrous for patients who want to work, but it's also difficult for employers who have obligations to meet, and have to hire interim help or train new employees.

Working is even more complicated for chronic cancer patients like those of us who have ET, especially younger ones. Federal work leave requirements allows us to take time off to get used to a new chemo or medication. (I took a week off work when I started hydroxyurea. Even though I was only working part-time by then, my employer was very supportive.)

Charting fatigue levels

How is a gear stick related to your ET
fatigue? Read on to think about
developing your own way to gauge
and chart symptoms.

Has your hematologist ever asked how you've been feeling and you haven't been able to give a good answer? Or you tell her how you are feeling that particular day, but you have an idea that that's not really typical for you. Too many hematologists already think that ET patients don't have symptoms, and if you start hemming and hawing when asked about them, it can confirm this belief.

Charting symptoms like fatigue levels can be a good way to help you gauge what's average for you so that you can give the doctor better info. It can also help you spot patterns--times or triggers that make your symptoms worse.

To help me figure out a consistent way to gauge my fatigue, I devised a five-point scale based on the old "four on the floor" stick shifts I used to drive:

Park (Neutral). I spend most of the day parked in bed or on the sofa reading or watching TV. I don't get dressed, and even washing up or making a meal seems like too much. I don't do any exercise. I have brain fog most of the day and feel disorganized. Sometimes I feel like I'm getting sick.

ET and stress

Having comic relief in stressful times can

help you cope.

 There's a lot of talk about the role of stress in cancer. Teasing out how stress might trigger or worsen cancer involves a lot of Complicated Science, and no one really knows the extent to which stress affects patients like us.

Certainly we all feel worse when we're stressed out. And science has discovered a link between stress and some ailments such as psoriasis and migraine headaches. Stress also plays a role in platelet levels. My GP tested my platelets for a few months because I had been stressed out about a heart problem. But when they stayed high and went higher, she sent me to the hematologist, and that's when I got my ET diagnosis.

About six weeks ago, I saw firsthand how stress can increase ET symptoms when my mother died. I also officially retired a couple of weeks after that. And Mom named me as executor of her will. So for the past month and a half, I was busy getting her house ready for out-of-state family members, planning a funeral and making arrangements, talking to a lawyer, and trying to make sense out of Mom's estate.

Yes, ET is cancer. Why is that so important?

Do you ever feel like this when you're trying to come to
terms with your ET diagnosis? Yes, I thought so.

I belong to and participate in some of the private Facebook support groups for ET. When new people come on line, one of the first questions is often this: "What's wrong with me? Is ET cancer or not?"

Often the confusion arises when doctors tell patients that they have a "disorder" or a "pre-cancer" or "cancer but not really cancer," or (weirdly) a "benign cancer." Then they look up their disease on line, and find out that ET is a "rare, chronic blood cancer."

And they freak out.

It's beyond me why doctors don't realize that, when they give patients vague info, those patients are going to go online. And that's not going to make the doctor's life easier. But that's a doctor problem.

When new people ask the "is it cancer?" question, support group members often jump in and very vehemently affirm that ET IS cancer.  I wanted to learn more about why patients feel this is so important.

Golden retrievers may hold cancer keys

A study of 3,000 golden retrievers in the U.S. may eventually help researchers understand the role that environmental factors play in spontaneous mutations like the ones that cause ET. (And, yes, dogs can get ET, though instead of coming with clot risks, canine ET more often results in inflammatory disease.)

Dogs and humans share as many as 500 diseases in common, and dog studies have already been parlayed into treatments for some cancers in humans. NBC news reported  that Emily Brown, who suffered from a terminal type of bone cancer, was treated with an immunotherapy designed for dogs.  She has lived for 20 years since receiving that therapy.

According to the Washington Post, cancer is the leading cause of death in all dogs after age 2. Sixty percent of golden retrievers, according to a CBS report, get some form of cancer in their lifetimes. (Here's a healthy but somewhat hyper goldie in the video below!)

Family viewing , triaging, and MPN tracking

I found a really good video that you may want to view with your family that explains a lot about MPNs, why we're tired, and how it affects our lives. The speaker is Dr. Robyn Scherber, Mayo Clinic MPN expert. Even if some of the science is over your head (and I admit it's often over mine, which is why I have a doctor ...), the 30-minute video covers a lot of ground. It will help your family understand that, even though we don't look sick, we are often jealous of our time and energy.

As always, many thanks to MPN Advocacy & Education International for making these conference videos available! They are linked in the Resources list at right.

Hydroxyurea adventure: The first six weeks

Yes, those are almonds in my pill holder. It's not a good idea to
put the HU in with your other meds in case of capsule breakage,
so the almond reminds me to take the HU. I got some paper pill
cups in a package of 500 to help avoid handling the caps. I just
shake one from the bottle into the cup and take with lots of water.
I also keep the HU in a separate sealed bag. That way, the pills
can't escape if I forget to put the bottle cap on tight.

Readers who follow Got Essential Thrombocytosis on the FB page (link at right) may recall that I started hydroxyurea (HU) February 2. I'm doing just fine.

But because there is sometimes a lot of fear about "chemo," and HU is still the first line of treatment for most ET patients around the world, I thought it might be helpful to capture my own experiences on it in real time.

Remember that your experiences may not be like mine. If there's anything this disease teaches us, it's that nothing is "typical."

January 12: My cardiology nurse (I have a heart valve problem not related to ET), asked how I was doing on the hydroxyurea. What? I thought Dr. Blood wanted me on hydroxyurea only if my spleen showed enlargement back in November. It didn't, so I assumed that even though my platelets had moved to about 750, I was still stable. Emailing Dr. Blood.

January 19: Dr. Blood called today and said, yes, she wants me on HU and to await instructions from Oncolology Nurse S. "We don't just call that in to your local pharmacy any more," Doc said. She said Nurse S would answer all my questions.

It's Rare Disease Day

It's Rare Disease Day, which falls on February 29 (a rare day), but on February 28 in non-Leap Years. I want to mark the day with a little info about where we've been and where we need to go as ET patients.

While ET is recognized by the National Organization of Rare Diseases, ET does not fit the legal definition of "rare." A "rare" disease is one that affects one in 200,000 people or more. ET affects about one in 135,000 people.

Why does that matter?

Thinking about shingles

I've noted before that ET complicates everything, and that includes protection against the shingles virus.

Shingles affects about one in three people over age 65. Those who had chicken pox as children already have the shingles virus (though this is not a guarantee you will get shingles). Shingles is not pleasant, and a more severe strain of the virus can cause painful blistering for many months. Blisters can also leave faint scars.

Those of us with ET, especially those on chemo, are considered immuno suppressed and may run a higher risk of shingles and for getting a more severe case.

Compression stockings: "natural treatment" for ET complications?

As someone who was on my feet a lot as a teacher, or sitting in one place for long periods as a writer, I found compression stockings made my legs feel less tired. As an ET patient, I've since moved on to medical grade compression socks to help prevent prevent deep vein thrombosis (DVT), which can be a complication for some of us with ET.

The Centers for Disease Control has lots of information on DVT along with general guidelines about compression stockings. And here are some Canadian guys who explain compression stockings specifically!

But before you head off to your medical supply store, read the rest of this post!

Why are my drugs so expensive? Dr. Prasad explains

Cancer patients are too often paying exorbitant prices for medications that offer no real benefits in quality or length of life, according to Dr. Vinay Prasad, hematology oncologist at the Oregon Health and Sciences University.

Prasad spoke Wednesday in a Webinar offered by Breast Cancer Action, a California-based group that advocates for less toxic cancer treatments and a less toxic environment that contributes to cancer incidence. The group takes no money from drug companies. See the Webinar. 

Some highlights and what this all means for you:

The cost of cancer drugs has risen far above the cost of inflation. Prasad noted that the cost of cancer treatment in 1975 was $129 per month. That would be about $600 today, adjusted for inflation. However, the average price of new cancer drugs today is about $10,000 per month. That is in line with the cost of Jakafi (ruxolitinib), which many ET patients take.

Top five stories in 2017

Taking a moment to identify the five stories you were most interested in last year. If you missed them, they are linked below:

Number 5: Diagnosing ET and scoring your ET risks. Related to this story: IPSET helps you figure your thrombosis risk

Number 4: Taking chemo? Use a condom

Number 3: Incremental care and ET

Number 2: Why your doctor doesn't think you have symptoms

Number 1: The debate: Should some ET cases be reclassified as pre-MF

Anything on the horizon you want to know about for 2018? Leave a comment here or on our FB page, link at right.

Be well!

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ET and mental health

Jimmie Holland probably isn't a name you've heard, but her work was well known to oncologists and patients with acute cancers.

Holland, who died recently, pioneered onco-psychology and mental health care for cancer patients. Her obit in the New York Times tells a bit about her work:

"It's bad enough to have cancer," she told the web site Medscape.com in 2015, but when all your family and friends are saying you have to be positive and you have to fight this thing, and the patient is exhausted and beaten up by the treatments--it seemed to me that adding that burden to be positive was just ridiculous." 

Our ET is chronic rather than acute. Our treatments and disease are less intensely exhausting, at least initially, but they last a lifetime.

Those of us with ET are often told our cancer won’t kill us and won’t shorten our lives. That isn't entirely true. And we often are not told about how to improve quality of life or deal with med side effects.

I talk to ET patients every day who think that their symptoms must be in their heads because support groups are thin on the ground, and they don't know anyone with ET.

How do you deal with the mental aspects of ET? Discuss here or on our Facebook page, link at right.

Be well! 

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ET in younger patients

This little video featuring Dr. Naveen Pemmaraju from Anderson Cancer Center is getting a lot of buzz over on our Facebook page, so I am cross-posting it here. Based on the hits, I'm guessing there is a real need to talk about treatment for children and young people in child-bearing years with ET.

Please take a listen (about three minutes), and discuss here or on our FB page (link at right).

Some things younger patients should discuss: Can youngsters take daily aspirin therapy? What are the long-term effects of chemo? How will ET affect fertility? How will ET affect pregnancy? What ET medications are safe to take during pregnancy?

Be well!

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How many MET minutes are in your week?

As many of you know from reading this blog and my FB page, my go-to exercise is my "bike to nowhere." I get 30 minutes of moderate exercise a day on it while watching the news. I also do about 15 or 20 minutes of physical therapy/yoga exercises a three times a week.

It doesn't seem like much. Is any of this helping my cardiovascular health and lowering overall stroke and heart attack risks? Yes, it is. It has also helped with overall fatigue and stamina.