Co-morbidities and ET

Now what? Navigating heart valve issues

with ET.

In addition to juggling ET, I am also being monitored for a wonky mitral (heart) valve. Just had my annual echocardiogram to check on its progress, so this is a good time to talk a little about how ET complicates the health picture for those of us with additional health issues.

My mitral valve prolapse was officially diagnosed when I was about 30. (I think I've recounted the story about how one of my college friends in vet school actually detected the murmur when we were in our 20s and playing around with her dog stethoscope. Yes, people doctors could learn a lot from veterinarians ...) 

My Day: Twelve years into ET, what's it like?

Warning, warning, warning: This post is more or less a reality check about what it's like to live with ET if you're me 12 years after the first onset of the disease. It's not necessarily a typical day for most ET patients, or even ET patients in their 60s. We are all different, and our diseases progress at different rates based on lots of different factors. So I'm not touting what I think is "normal," here, just what's been my experience.

I will also say that I feel better than I did before I started on hydroxyurea (HU) and when I was working and my time was not my own. On those days, I was exhausted by 3 p.m., and I would often go to bed as soon as I got home from work. The HU helped with fatigue, but so did learning to pace myself and find my own daily rhythm after I retired. 

Anyhow, here's my typical day: 

Night time: I try to get 7-8 hours of good sleep each night. This is easier said than done. I have always been an anxious and light sleeper, and this has become worse over the years. I also have night sweats sometimes and very dry mouth that wakes me up. On a good night, I wake up once and go back to sleep immediately. On a bad night, I wake up every two hours, as if an alarm had been set. Fatigue is always worse after one of the bad nights. 

MPN Foundation studying MPN disease progression

Understanding how ET and other MPNs progress to more serious illnesses--myelofibrosis or acute myeloid leukemia--will be a focus of the MPN Foundation in the coming years, Dr. Raajit Rampal (Memorial Sloan Kettering), MPN expert and advisor to the foundation, said in a Zoom program February 24.

The program was also sponsored by MPN Advocacy and Education International.

Dr. Rampal outlined the many questions that still surround progression in MPN patients: Why do some patients progress and not others? Can progression be stopped? Will patients continue to need treatment if disease progression can be halted? And at what point in disease progression should any new medications be introduced to avoid what Dr. Rampal called "clinical or financial toxicity," that is the patient's ability to tolerate and afford a med that halts disease progression should one become available?

ET and covid booster vaccines

NOTE: Vaccination is a personal decision. I chose to be vaccinated, and I am not going to argue this decision with others. Comments of a non-factual nature that spread fear and misinformation about the vaccines will not be published. 

Good news from the docs this week! Like everyone else in the U.S., I have been hearing that cancer patients should be getting a booster shot as soon as possible because of their compromised immunity. But even as an ET patient, you may not need to get a booster right away. I was happy to learn from my hematologist that I'm not as decrepit as I thought, and I can wait the full eight months before getting a booster, just like a "normal" person! 

HOWEVER: Hydroxyurea can affect immunity, as the info sheet reminds you every time you get your prescription refilled. So don't assume that the advice I got will be the same for everyone. Let your hematologist make that call for you. 

I emailed my doc about the booster. One thing the pandemic has made much easier is communication with the hematology office. I usually get an answer within 24 hours for a non-urgent question, and the hematology oncology office has laid on a new nurse practioner to deal with just this type of issue. So the same day I asked the question, the nurse looked up my last blood draw results and decided that I should wait for the booster based on several immunity markers, including white blood count. 

My platelets swim around in the 400-450 range and none of my other blood counts are in the high or low range. I take a relatively low dose of hydroxyurea--500 mg per day plus an extra 500 mg on Mondays, Wednesdays, and Fridays. Those taking higher doses of HU or with blood counts that run high or low may be at different immunity levels, so let the hematologist make the call about a booster for you. This isn't a decision you want to freelance. 

Also, as my hematologist's office reminded me, wear a mask when you are indoors with people whose vaccination status you don't know whether you are vaccinated or not. I know it's a drag, but delta variants are up in my area, and there are more reports of vaccinated people getting sick. 

And while we are on the general topic of wellness, seasons are turning, but there is still plenty of good outdoor weather in both the Northern and Southern hemispheres. Please remember that some ET medications, including HU, can increase your chances for skin cancer. So carry that sunscreen around with your mask! And remember to drink lots of water. Be well! 

Related: Vaccination roulette 

Related: Practical tips for ET patients and COVID19 

Related: Thinking about skin and ET 

Home

ET patients deserve better!

My husband and I celebrate a recent

wedding anniversary.

My husband had a heart attack last week. Technically, it was an acute myocardial infarction, which means he was stricken with chest pain in the parking lot of the Home Depot while loading up some sheets of plywood at 11:45 a.m. He drove himself to the nearest ER, and by 4:30 p.m., he had been taken on an exciting ambulance drive to the big regional hospital, had a stent inserted to clear an artery that was 95 percent blocked, and was eating a pudding cup and feeling no pain.

All of this came out of the blue, and of course we are deeply grateful to the quick thinking of emergency personnel, cardiac catheter lab staff, and the doctors and nurses in cardiac care. 

But we couldn't help comparing the care he got to the care ET patients are offered. It certainly revealed holes in the care most of us ET patients receive. For example:

Vaccination roulette

Health department nurses distribute oral        
polio vaccine ca. 1960.

I have an appointment with a chain pharmacy here in Michigan to receive my first dose of one of the COVID-19 vaccines on March 9. I don't know whether I will get the Moderna or Pfizer vaccine. There isn't any way to choose; you take whatever vaccine the vaccination site has.

Having an appointment for a vaccine, of course, is not the same as actually getting the vaccine. Lags in vaccine production, shipping delays due to weather, difficulty ensuring that there are enough people trained to administer the shots--all of this has created problems in vaccine distribution here in the United States. 

In addition, state and local health departments had to create a network of vaccine distribution sites for people in each locale, and this took time.  Like a lot of people over age 65, I remember the 1960 distribution of the oral polio vaccine and couldn't help comparing that effort to the one going on now. In our town, people were directed to their local polling stations on a given day. There, the public health nurses gave us sugar cubes with the vaccine on it. I remember teasing my younger brother that he would have to get his via an eye-dropper because, at age 4, he was still a baby. 

Seven wishes about ET

In the six years since I started writing this blog, I've talked to hundreds of ET patients online here and through my Facebook page. 

Besides sharing our ET diagnosis, we all seem to share some common wishes as ET patients. So I thought I'd start out 20201 by offering the seven things I wish about ET that I think I share with most of you: 

Wish 1: I wish that health care providers understood that lack of information is the worst "side effect" of having ET. I hear something like this from a lot of my fellow patients: "I was just diagnosed with ET, and my doctor says he's not worried about it. But then I read online that I have cancer! I am really scared now!" Please, health care providers: Five minutes of clear, brisk info and a fact sheet would go a long way toward  helping us better understand our disease. 

Practical tips for ET patients and COVID19

ET patients hoping to stay health should continue to practice social distancing , get fresh air and exercise, have a mask and hand sanitizer when out,  maybe take a multivitamin, and not worry excessively. ET patients who do get sick should get tested for COVID19 and contact their hematologists.

That was the bottom line advice for MPN patients Drs. Ruben Mesa and Aaron Gerds at a Webinar session Tuesday. The session was sponsored by MPN Advocacy & Education International.

Coronavirus and cancer patients

Clean hands are safe hands! Wash hands frequently
to help prevent the spread of infectious diseases!

UPDATE, April 1: More specific info on C19 and MPNs from Drs. Ruben Mesa and Robyn Scherber here.

ET patients MAY be at higher risk for coronavirus than the general population. What does that mean exactly? It means that we may get the virus more easily or become more seriously ill if we do. That's because those on oral chemo are considered immune suppressed, and that makes it generally harder for us to fight off infections.

The Leukemia and Lymphoma Society has some brief guidelines and info specifically for blood cancer patients about corona virus.

But there's a lot we don't know about coronavirus, and the most important thing you can do for yourself and loved ones is to keep a clear head and not panic. The next most important thing is to stay informed. Here are some tips:

New book for MPN patients a mixed bag

Living Well with an Myeloproliferative Neoplasm by Dr. Krisstina Gowin is a new book--it may be the ONLY book--aimed at helping MPN patients understand and "live well" with our uncommon cancers. Is sells on Kindle for about $10. It's probably a very good resource for people who are just diagnosed. It can help you get your thoughts together and provides some good medical explanations that you probably won't get from your hematologist. But the book has some down sides. Here's my review:

My CBD disappointment

CBD oil: It's sold everywhere. But not all products are of
equal quality, and they may not help some people. CBD
can also create unwanted problems. Proceed with CAUTION.

The short version of my experiment with CBD oil is that it did not help me sleep, did not reduce restless leg syndrome, and did not reduce anxiety, common complaints among ET patients. CBD oil also seemed to worsen my acid reflux

Here's the longer version:

I was interested straight CBD, that is cannabidiol without THC. THC, as you may recall from your college days if you are of a Certain Age, is the substance that makes you giggly, red-eyed, and want to eat Cheetohs.

Signing up for Medicare

It's Elder Me getting ready to
sign up for Medicare!

I'll be 65 in September, and that means it's time to deal with Medicare, the government health insurance program for the elderly here in the U.S.  And much as I cringe to think of myself as "elderly," I have to remember the alternative.

Anyhow, here's my "first dive into Medicare" report. This is just a record of my experiences, and NOT MEDICARE ADVICE!!

The government knows your birthday, your income, whether you've been naughty or nice, and the folks at Medicare will automatically send you your Medicare insurance card before your 65th birthday. Everyone has to be enrolled in Part A, and you are automatically enrolled in Parts A and B (which together make basic Medicare) without having to do anything. If you're dropping out of Part B because you have retirement health care coverage from an employer, union, or professional association, there's info on the card that tells you what to do.

Thinking about skin and ET

Ummm. Balmy and sunny (all night) in Reykjavik,
Iceland. ET patients: Don't forget to take your
sun screen!

My summer here at north latitude 45 around the Great Lakes has so far been unseasonably rainy and cold. In fact, right now it's 55 degrees with a cold drizzle. That means the weather is actually better in Reykjavik, Iceland, where it is 59 and partly sunny (and will be for the next 24 hours because of the Midnight Sun). It's also 63 in Fairbanks, Alaska, and a sweltering 67 in Yellow Knife, Northwest Territories of Canada.

Thanks a lot, Gods of Weather. I hate it when it's hot and humid, but no need to be sarcastic.

But never mind my digressions and resentments of people getting better weather far to the north of me. Today's topic is ET and skin cancer. This comes up fairly frequently among people who read the info that comes with their hydroxyurea, which warns that it may cause skin cancer, and become worried about sun exposure. So I did some digging, and I hope this info will encourage you to talk to your doc about this. Here's what I found:

How to bird-dog your doctor

Bird dogging is a communication technique people
are using with weasel-y politicians. It could help
you communicate better with your doctor!

This week, I heard a radio bit about people learning to "bird dog" politicians in order to flush out their true opinions about issues instead of just letting them ramble on with a lot of canned and meaningless statements. I instantly realized that this was a technique we might use with our hematologists, who are often stymied by ET and give us frustrating non-answers.

Anyhow, good bird-dogging requires four things:

1. Be non-confrontational. You may think your doctor isn't doing a great job or ignoring your questions because he or she is not very well informed about ET. If that's so, getting a second opinion should be on your to-do list. The MPN Research Foundation has a good resource page to get you started if you're looking for a new doc. You won't get anywhere with your current doctor if you have a chip on your shoulder.

ET and pregnancy

Women with ET can have healthy babies, but they should be
aware of complications, risks, and whom to contact in the event
of an unplanned pregnancy.

While the typical ET patient is 50-70 years old, about 20 percent of women with ET are younger than 40, that is, within child-bearing age. Women with ET can have healthy babies, but ET does pose elevated risks for both mothers and fetuses that fertile women should know about and discuss with both their hematologists and gynecologists.

A link between ET and early miscarriage has been known for many years. A 2007 report in the Orphanet Journal of Rare Diseases, and a 2017 article specifically on ET and pregnancy in the Obstetrical and Gynecological Report offers this info:

Happy International Women's Day!

Happy International Women's Day! Enjoy
yourself. But drink that wine in moderation!

As you know, there are 1.5 female ET patients to every male--that is, about 65 percent of patients with ET are women. So, on this International Women's Day, let's take a look at some of the challenges women ET patients face.

Women diagnosed with ET in their childbearing years need to be aware that taking chemo can cause serious birth defects. However, interferon-based drugs such as Pegasys, may be taken without harming fetal life. Discuss this with your doctor! Anecdotally, younger women with ET who are not on medication may run a higher risk of miscarriage or experience more bleeding during their menstrual cycles.

Younger ET patients who hope to become parents should also understand that the ET is caused by somatic mutation, that is, one that is not passed along genetically.  But because ET (and other MPNs) seems to run in many families, researchers theorize that the children of parents with ET may inherit a sensitivity to developing ET later in life if exposed to unknown carcinogens in the environment.

Shingrix: Mild side effects are common, as I discovered

NOTE: If you are generally opposed to vaccines, this is your choice, you are free to opt out of treatments you don't want, and I support your right to do that. If you are interested in the Shingrix vaccine, read on. I won't entertain debates here about vaccines in general.

As you know, Shingrix is a killed-virus vaccine that helps prevent shingles. According to the American Journal of Managed Care, about a third of all people will get shingles if they have had chicken pox, and the risk seems to be climbing.  If you're on hydroxyurea like me, you're considered immune compromised, and that adds another shingles risk factor. 

Fortunately, Shingrix, unlike the old vaccine, Zostavax, is a killed-virus vaccine and so safer for ET patients. It's also much more effective (about 90 percent) in preventing shingles. If you're over 50, your family doc or hematologist has probably urged you to get the Shingrix vaccine.

In the past year, Shingrix was scarce because production did not keep up with demand. My family doctor explained with some exasperation that everyone who got the old shingles vaccine rushed to get the new one, which is more effective, and that created a run on the supply. So those of us who a) had not had the old vaccine and b) who are immune compromised and can't take Zostavax often had to get on a waiting list at the local pharmacy or clinic.

You may want to let your local pharmacist know that you have an immune compromising condition. Mine moved me to the "priority" list for when the vaccine became available. Recently, Shingrix makers ramped up production, and I got a call from my local pharmacist telling me that my number was up. I got my first shot Sunday. 

MDS and MPNs

MDS--myelodysplastic syndromes--are a group of disorders that cause "bone marrow failure." Basically, the bone marrow stops producing enough usable blood cells. I bring it up here because it is kinda, sorta related to myeloproliferative neoplasms, the family of cancers that includes ET, and ET MIGHT be a risk factor for developing MDS.

Before you freak out, read on ...

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.