Category Archives: Myelofibrosis

Tenuous

I am happy to report that I have a strong, healthy heart.

Which is good, because my Kryptonian blood wants to make it beat really fast, all the time now, so I’m glad I’m built to handle the extra work.

My increased pulse is a symptom of myelofibrosis, and it frustrates me, as I have faithfully banked 4-6 cardio workouts per week for the past 15 years. My resting heart rate in recent months has been an acceptable (though rapid) 100 – 115 beats per minute, but at my appointment last week it was 130. My doctor sent me to a cardiologist, just to ensure that we don’t have anything new to worry about. He set me up for some tests, all the while reassuring me that it was all precautionary; not to worry, blah blah blah (“You haven’t satisfied your 2011 deductible yet, so that will be $1,078.41, please. Will you be writing a check?”)

This morning, I went in for an EKO (see price tag, above) without really knowing what to expect, until I saw the ultrasound machine. I smiled, remembering several screens similar to this one, that, back in the late ’90’s, revealed first glimpses of babies sucking their thumbs and kicking tiny feet back at the ultrasound waves. Back then, my only medical visits were related to my chronic status as a baby factory. When Richard, my tech, turned up the sound of my own heartbeat this morning, I fought back tears as I tried to process the stark contrast between my current medical situation and the visceral memory of burgeoning maternal joy that came along with that same audio track, first heard clearly at Week 11 of Pregnancy #1. That moment, 16 years ago, was surreal and overwhelming. That tiny, steady gallop provided the first undeniable evidence that Sam was really in there.

Today, my heartbeat made virtually the same “woosh, gallop, woosh,” that I remember from prenatal ultrasounds. I realized that over three pregnancies I must have developed a kind of Pavlovian response to that sound; one of gratitude for life, health and unlimited potential. I was unprepared for how much the same pattern, heard in such a different context, would jar me emotionally. What a difference a diagnosis makes.

Richard was kind, but didn’t say much as he moved the transducer around, assessing and measuring chambers and valves and ventricles. While I don’t know the specifics of which structure is what, I couldn’t help but wonder how it is that we depend so completely on the proper function of something that delicate, with its impossibly thin walls and fluttery valves. I thought about how hard I have pushed my heart in the gym over the years, never once worrying about its ability to rise to the challenge. But there it was in front of me, revealing itself for the mortal structure that it is, capable of so much despite its impossibly fragile appearance.

The line between health and illness is fuzzy and tenuous, far more so than I ever realized. I’m grateful for the fitness I had going into this disease, because I’m sure that all of those years of sweat and effort will make the difference in my ability to come out healthy on the other side.

My heart is fine, but I’m going caffeine-free to help bring my pulse down, a prescription that would have left me whimpering in a closet a few months back. But it seems a small price to pay to help keep things running at full strength.

A Shift In the Wind

So, it turns out that there was a reason my body started craving more sleep.

I never in a million years thought this would turn into a cancer blog, although I suppose nobody really sees that one coming.

Despite my 5-6 days per week workout habit, my belly started expanding last summer, even though I was losing jeans sizes at the same time. I also noticed some strange changes including a decreased energy level during my workouts and throughout the day, and sheet-soaking night sweats. I vaguely thought it was a combination of some kind of gastrointestinal problem and post-40 aging issues, so I ignored it for a while.

Two x-rays, one CT scan, 16 vials of blood and a bone marrow biopsy later, I learned that I have a mixed myeloproliferative disorder, or post-polycythemia vera myelofibrosis. That combination of large words I had never heard before means that I have a rare blood disease that is trashing my bone marrow. The growing belly turned out to be the result of, as articulated in my medical chart by my big shot doctor, an “amazingly large spleen.” Turns out that the spleen tries to take over the job of manufacturing blood cells when the marrow doesn’t want to anymore, and eats up all the extra cells made as a result of the disease, enlarging in the process. So while I’ve lost weight, I also look a little bit pregnant.

So, here’s the thing. There is no real treatment for this disease, aside from the possibility of bone marrow transplant. But, BMT is risky, so not generally recommended unless it is clear that things are not going well. I am currently being treated with a mild dose of hydroxyurea, a chemo drug that keeps my high blood levels in check enough to alleviate some of the symptoms. This drug has blessedly few side effects… I even get to keep my hair. There is a new drug, one made by Incyte, with the very catchy name INCB018424. This drug has caused significant buzz in the hematology/oncology world in that it seems to offer hope as the first potential treatment for this disease. Both of my doctors feel strongly that this is the right treatment for me, but it is still in clinical trials and not yet available.

I am very fortunate to live only three hours away from one of the finest cancer centers in the world; one that contains a pre-eminent expert on my particular disease. He is the lead researcher on the drug I need, and has offered hope that there may be an appropriate trial available for me this spring.

So, we wait.

I keep telling friends and family that this is the longest learning curve I have ever been on. The issue that challenges me the most is the question of how to explain it all to my kids. That is the main reason it has taken me this long to write about it. I truly believe that the best explanation always involves the truth, no matter how difficult, but this gets more complicated in a situation where even the experts don’t really know how this will go for me. I think that kids can tell when their parents are protecting them, and that their fears stemming from that silence will always be worse than the truth. I want them to understand what’s happening, but also don’t want to scare them needlessly. Sam keeps asking me to tell him that I’m “at a low risk for dying.” I believe that I am, based on my age, my history as a medical outlier (if there’s a category that involves 5% of all patients, I’m usually in it, a bizarre fact that is oddly common in my family.) However, I also need to stare realistically into the face of odds that I don’t like very much. I think that’s an important part of conquering this sucker.

For now, I’ll get my blood counts checked monthly with my local hematologist/oncologist, travel a few hours quarterly to see Dr. Big Shot, get out and listen to live music as frequently as possible, celebrate our kids’ successes and appreciate amazing support from friends and family that stuns me in its scope and seems to grow daily. I wish my kids would fight a little less and clean their rooms a little more, but in a way, it’s comforting that life goes on as usual.

The yellow bracelet on my left wrist inspires me to literally Livestrong, and I am determined to continue that throughout all of this. As Lance’s foundation manifesto says, “Unity is strength, knowledge is power and attitude is everything.”

And now, I’m off to fetch socks for Sam, conditioner for Abby and a library book for Hannah. Our regularly scheduled life continues.