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.