“keep-calm-and-live

What a shock when you receive at fatal diagnosis.  There is a cascade of emotions and a feeling of panic.  There is anxiety and confusion and sorrow.  There are many questions and a great sense of loss.  Looking back, there are things I wish I had known at the time that would have helped.  I hope that by sharing them, it will help you.

1) Prognoses Are Not Absolute 

I was initially told that my “estimated median survival” was 7.92 years. What I heard was, “YOU WILL DIE in 7.92 years,” and I immediately began morbid calculations—What year would that be?  How old would I be?  Would I live to collect Social Security?  Would I have my mortgage paid off by then?  Would I get to know my grandchildren?  (I was sorry for myself.)

What I have learned since then is that the various prognostic scales (IPSS, DIPSS and DIPSS+) yield different results.  IPSS gave me 7.92.  DIPSS gave me 14.2.  DIPSS+ gave me 6.5.  (I decided to go with the DIPSS prediction.)

“pills

These scales are from statistical studies looking at the historical survival of groups of people with various risk factors.  “Median survival” means half the people with a certain risk score died by that point and half were still alive.  But how much longer did the other half live?  What other risk factors or treatments or illnesses or even traffic accidents were involved?  How did they adjust for the ages of the people in the study? 

It is important to remember that the prognostic scales only provide statistical truth—not individual truth.  I strongly counsel people to talk about these scales with an MPN specialist who has all the information about their health—bone marrow biopsy results, karyotype analysis, myeloid mutation panels—everything.  Your MPN specialist, who sees more MPN cases than your local oncologist, is the one who can give you the best individualized information about your prognosis. 

Do not let a number from a prognostic scale throw you into a panic.

2) Keep Doing What You Can Do

Right after my diagnosis I thought to myself, “Why am I bothering to watch my weight and exercise?  I have a fatal disease!  What’s the point?”  I bought myself pounds of dark chocolate, ate whatever I wanted, and I stopped my routine of daily exercise.  Not surprisingly, I gained more than 20 pounds.  I also started experiencing gastric reflux, and I was more affected by fatigue and brain fog than ever before.  I felt worse and worse and worried that my myelofibrosis was progressing.

“diet”

It was tempting to blame everything on myelofibrosis, but I eventually realized that I was an accomplice.  After two years, a medication helped me to feel better again, and I recognized an opportunity to make a positive change.  I dusted off my exercise equipment, got back to my early morning routine and started dieting in earnest.  Sure enough, as I lost the extra weight and got back into better physical shape I started feeling better.  Today I have more energy and less fatigue.  My acid reflux is gone.  I have more physical strength and endurance.

If anyone were to ask me why I get up at 5:00 AM to spend an hour working out, I would say, “Because I can.”  5:00 AM is early.  Exercise is work.  There is sweating and panting and grimacing involved…but for now I can do this.  I see it as fighting back against my disease and I’m glad I can still do it.

My advice is that every patient should keep doing what he or she can do as long as possible.  Consider it a war against the disease. Don’t give up territory easily.  Fight for every inch.  You might even be able to win back ground you had surrendered before. 

3) You Are Not Alone

At first I was very impressed with the rarity of myelofibrosis.  It is an “orphan disease.”  “The incidence of myelofibrosis is only about two in one hundred thousand.”  By “impressed” I mean that I felt justified in my melancholy sense of personal tragedy.

I felt sorry for myself.  I was the only person I knew who had myelofibrosis.  (I was almost the only person my hematologist knew who had this.)  I knew full well that everyone is mortal—but I had expected to live on, trouble free into ripe old age.  In my self-pity it seemed that everyone else was living on in perfect health and I alone was afflicted.  Poor me!

It did not take too long to find that while my disease is rare, my situation is as common as the human condition.  A woman in my church had been diagnosed with multiple myeloma the year before.  A man in my church had just gone through autogenic stem cell transplant for another form of leukemia.  I met a friend at a men’s retreat who had been diagnosed with CLL at about the same time as I was diagnosed with PMF.  His challenges were similar to mine. 

Eventually a friend from 20 years ago saw my Facebook posts and told me that she also had myelofibrosis.  She recommended a Facebook forum to me.  Soon I joined a couple of online forums for patients and caregivers dealing with various MPNs.  Here were hundreds or even thousands of people from around the world with myelofibrosis, essential thrombocytosis, polycythemia vera and related diseases.  Here I found understanding, experience and advice.

“pity-party”

What I realized is that many people have life-threatening diseases.  I have a fatal disease, but in my case it is presently chronic and stable.  Some have even less symptoms than I have.  Others are facing the same disease but have worse symptoms than me—or don’t respond to the medication I have responded to—or their disease has progressed and become an acute crisis.  

Self-pity suggests that I have a right to expect something better than what I’ve got.  But in my health situation, I am worse off than some but better off than others.  Should I feel sorry for myself compared to people who (so far) have no health concerns?  Maybe I should feel survivor’s guilt when I compare myself to others who are worse off—not to mention those who have died? 

Myelofibrosis has been good for me in that I am better able to identify with other people with this and other life-threatening diseases.  Instead of feeling sorry for myself I try to feel grateful.  I am not alone—I am part of a community of mortal humans.  I hope to live in the face of myelofibrosis with poise that gives other people confidence.  I hope to reach out to others with comfort and encouragement that helps them over the difficult parts of their lives. 

You are not alone either.  Be willing to help others and to receive help from others. 

Not letting an uncertain future steal my joy,

Dave Denny