The question of how long I’ve had polycythemia vera (PV) is an interesting one. I guess you could say I’ve had it since 2019, though I’ve only been treated since 2022.

First, a Misdiagnosis

In 2019, I became quite ill with severe pain in my left side. An ultrasound indicated I had swollen lymph nodes in my abdomen and splenomegaly (a swollen spleen). I was given a colonoscopy and an endoscopy and told I had irritable bowel syndrome (IBS), though there was nothing specific to justify that diagnosis. When I pointed out that IBS didn’t explain my swollen spleen, I was told it wasn’t important. I now know that I had PV and should have been receiving treatment.

Some people have asked why I didn’t go to other specialists. Frankly, I tried. I had received recommendations from friends who are medical professionals and was hoping to see a hepatologist and/or a hematologist. However, I was told that since I had a diagnosis that wasn’t in their area of expertise, they wouldn’t see me.

Symptoms and the Stress of Grief

Shortly after all those tests that ended in misdiagnosis, my spouse of 40 years was diagnosed with late-stage, metastatic cancer. I spent the next 18 months providing home care during treatments. Throughout that time, I was not well but was determined to provide quality home care. I believed my fatigue was from caregiving and anticipatory grief. Once I became a widower, I continued to believe my fatigue and other ailments were due to the stress of grief.

It wasn’t until I became extremely ill and went to the doctor that it became clear something more significant was wrong. I was given blood tests, with the assumption that we were looking for an infection. When the results showed rather extreme levels, I was called back for an additional blood test. Unbeknownst to me, the second set of tests included a JAK2 mutation test. My hematocrit and hemoglobin were extremely high, and I tested positive for JAK2 mutation. I began taking aspirin immediately and started a formal PV treatment under the care of a hematologist two weeks later.

Starting Treatment for Polycythemia Vera

My initial treatment was phlebotomy and hydroxyurea. Phlebotomies were weekly for several months, and hydroxyurea was started at a basic dose and gradually increased.

During that time, I was advised by friends in a myeloproliferative neoplasm (MPN) support group to go to a major cancer center and find an MPN specialist, which I did. I went to Dana-Farber Cancer Institute in Boston. I had a number of additional blood tests performed, met with an MPN specialist who was exceedingly helpful, and subsequently had a bone marrow extraction and biopsy. (If you haven’t had a bone marrow biopsy for your MPN, I encourage you to do so. While they aren’t all that fun, the key point is: How will you know if your condition progresses if you don’t have a baseline from which to measure?)

Medical Care Challenges

I confess that medical care has been a challenge. As you might expect, I have no interest in going back to the primary care provider (PCP) who misdiagnosed me. I wouldn’t have had a problem with him not knowing what was wrong with me, but insisting that his diagnosis was correct and dismissing symptoms like my splenomegaly did not inspire confidence.

The challenge is that it’s very difficult these days to find a new PCP, so I haven’t had a physical or seen a PCP in years. The blood tests for my initial diagnosis were ordered by a nurse practitioner. In any event, the point is that medical care has been a challenge.

Finding the Right Specialist

As I was starting treatment and trying to learn about PV, I would ask my hematologist questions. When I asked about what appeared to be an increasing iron deficiency, he told me “not to look at the lab results” and that “he would tell me what I needed to know.”

Two weeks after that, I met with the MPN specialist in Boston, who asked me if my doctor had told me I was extremely iron deficient. Hmmm … Needless to say, I have a new hematologist.

After my second visit with the new hematologist, we discussed some changes in my care plan. He asked if it was OK if he consulted with my MPN specialist in Boston. It was then that I knew I had found the right doctor.

Worsening Symptoms Require a Change in Treatment

For a while I was getting weekly, and then biweekly, blood tests. When my iron was dropping, iron and iron retention were added to regular tests. The numbers showed that the standard treatment of hydroxyurea and phlebotomies wasn’t working for me. I was not recovering well, I was sick and unable to function, and my numbers weren’t coming down.

Some of my fatigue and inability to function could probably be attributed to the lack of iron (and also vitamin B12). My quality of life was getting so bad that I had decided that if they couldn’t do anything more for me, I would take my chances with Mother Nature. My thinking was that it was better to have two or three good years than several bad ones.

I have since started using Besremi (generic name: ropeginterferon-alfa-2b-njft), an interferon that’s self-injected. While the drug isn’t doing its job yet, it’s too early to tell if it’s going to work. Time will tell. One thing is for sure, however — not being on hydroxyurea has made a huge difference in how I feel and subsequently my overall quality of life.

Fighting for Insurance Coverage

Speaking of Besremi, I had quite the adventure getting insurance approval to cover the medication. Once my doctor had determined that hydroxyurea and phlebotomies weren’t going to work for me, we decided I should start Besremi. My doctor submitted my prescription, and a week later I received a denial from the insurance company. He then followed up with a call for a peer-to-peer consult. They didn’t even take his call, and they then sent me another denial letter.

What this meant at this point was that I, not the doctor, would be forced to submit an appeal. Well, as my niece said to me, they screwed with the wrong guy.

I subsequently learned more than I really wanted to know about the underbelly of our health care system. It’s a mess! To give you a sense of what I mean, in 2021, HealthCare.gov users appealed less than two-tenths of 1 percent of denied claims from in-network providers, and insurers upheld 59 percent of the denials that were appealed. That means it’s in the health insurer’s financial interest to deny your claim, since the average person will not appeal. I did, however. My appeal was considered by an external review board and the denial overturned.

Build a Medical Team You Feel Confident About

One thing I’ve done for myself is to be vigilant in getting the care I need. Vision can be affected by blood cancers, as well as by treatments, so annual eye appointments are a good idea. I spoke with my ophthalmologist about my cancer. In response, he took a number of images of my eyes and examined factors that he might not normally do in a regular exam, so he’d have a baseline in case things begin to change.

Since PV treatments can cause gum disease and tooth loss, many resources recommend seeing your dentist more often than the regular biannual appointment. I spoke with my hygienist and discovered my dental insurance has a program for people with medical issues. I was asked a few questions about my cancer and treatment, my hygienist completed an application, and I was approved by my insurer for four hygienist appointments annually instead of the usual two.

Ultimately, my advice is to take care in assembling your team. I now have on my team dental specialists, an ophthalmologist, a physical therapist, a psychologist, a spiritual director, a hematologist, and a hematology MPN specialist. I’m in the process of finding a new PCP and plan to eventually add an acupuncturist to my team. I encourage everyone to establish a strong team. Confidence in the quality of your care and knowing you have access to great resources can be very reassuring when you’re struggling to get out of bed in the morning.

Listen … and Then Determine Your Own Path

I’ve asked people for advice, and I’ve been asked for advice myself. My advice is to listen to people’s stories and then determine your own path. I’ve found that none of us seem to be the same. We have similarities in our experiences and have learned things that are important to others dealing with relatively similar health challenges, but our reactions to medications, our doctors’ ideas about what’s best for us, and much more are all quite different. Some of us do well on certain medications while others don’t.

Since we all respond differently and have different symptoms, our treatments will, understandably, also vary. We can’t expect that what worked for someone else with PV will work for us. We can only use their experience as additional information as we forge ahead in developing and managing our own care.

Member Perspective articles discuss myeloproliferative neoplasms from a specific point of view. We understand that everyone with MPNs, or caring for someone with one of these conditions, has a different experience. We aim to share as many of those viewpoints as we can.

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