Published on April 29, 2020
Taja Shabazz knew something was wrong. She had dizzy spells, extreme fatigue—she fell asleep at the wheel of her car while driving to school—she just wasn’t feeling well.
Her primary care doctor repeatedly diagnosed her with an infection and prescribed antibiotics, but nothing worked.
After six months of symptoms, Shabazz was referred to Dr. Apurva Mehta, a hematologist-oncologist in Cincinnati. A week later, she was diagnosed with polycythemia vera (PV), part of a group of rare, chronic blood cancers known as myeloproliferative neoplasms (MPNs). (Other MPNs include myelofibrosis (MF) and essential thrombocythemia (ET).) A bone marrow biopsy confirmed the diagnosis.
“I finally got someone to actually pay attention to what was going on,” said Shabazz, of Fairfield Township, Ohio, who was diagnosed in 2015 at the age of 23. “Outside of my blood work, I was presumed to be fine. I didn’t look sick. I wasn’t losing my hair, losing weight. It wasn’t anything that extreme.”
The rarity and the lack of awareness about MPNs make it hard for patients to find support—even from doctors who may only have medical textbook knowledge.
Shabazz said she consulted four doctors, including one at the Mayo Clinic, but found that they had little experience with MPNs and thus, were unable to individualize her treatment. (She has tried phlebotomy, an interferon, hydroxyurea [Hydrea] and enoxaparin [Lovenox].) And she has yet to meet someone her age with an MPN.
“What I have learned is you have to be your biggest advocate,” said Shabazz, who started a nonprofit, Invisible to the Eye, Inc., for individuals diagnosed with an MPN. “Doing your own research and finding the best physician for yourself, someone who is not only going to be your doctor but also advocate for you.”
Michelle Woehrle, executive director of the MPN Research Foundation, acknowledged the challenges MPN patients face: the lack of name recognition, the chronic nature of the disease and the fact that it is an invisible illness—patients may not look sick.
Based on MarketScan database estimates, reported on the MPN Research Foundation’s website, the projected prevalence for MPNs in the U.S. on December 31, 2010, was:
- MF – 12,812
- PV – 148,363
- ET – 134,534
“Patients are suffering,” Woehrle said. “People are not having their issues taken seriously by their employers, insurance companies, legislators, family members.
“Even the physician community—if you are a general practitioner or community hematologist, and you don’t see that many MPN patients you might not think this is a big deal.”
Woehrle estimates there are currently about 30 MPN clinical trials. She said while there are more treatment options for MPNs, there still isn’t a cure (aside from a transplant) nor a way to stop the disease from progressing to acute myeloid leukemia (AML).
Still, considerable strides are being made, especially with the discovery of the JAK2 mutation in 2005, she said. Studies show the mutation is present in nearly 100 percent of patients with PV and in about 50 percent of patients with ET and primary MF. Ruxolitinib (Jakafi), which targets and inhibits JAK2, was approved to treat MF in 2011, and PV in 2014.
The JAK2 mutation discovery opened the “floodgates to new drug development, research and the discovery of other mutations in MPN patients,” said Ann Brazeau, CEO and founder of MPN Advocacy & Education International. She said it also sparked more interest in MPNs—both scientifically and from a patient perspective.
“Not only did the patients need support, but the medical community wanted and needed to learn more from them,” Brazeau said. “Having some answers to these complex blood cancers was, I believe, a turning point in the relationship between patients and physicians as well as a launching pad for numerous clinical trials that continue today.”
Both Brazeau and Woehrle said support in the MPN community has grown exponentially: blogs, videos, advocacy initiatives, in-person and online support groups.
MPN Advocacy & Education International sees over 550 patients per year and meets with patients and families regularly across the country, Brazeau said. “The challenge is finding the patients who need us most.”
Summer Golden, a theater owner and performer in San Diego, had never heard of MF when she was diagnosed in 2018. Since then, she has only met one person with an MPN: Patient Power Co-Founder Andrew Schorr. Golden struggled initially with telling others about her diagnosis.
She said what has helped is comedy. She developed a routine based on her cancer. After her first performance, people came up to her and told her how inspired they were by her story.
“I guess when you get an illness like that you feel like something else is controlling you and everybody's telling you how you should do it, and you really don't have any control,” she told Schorr in a Patient Power interview. “But if you can turn it into humor you feel like, at least I felt like, I had control.”
Golden said coping with MF has a lot to do with a person’s attitude. “I think your attitude and how you’re thinking has everything in the world to do with it,” she said. “You only get one life, and you want to do as much as you can.”
Shabazz, who works as a care coordinator at a hospital, has the same mindset.
“My willpower is what keeps me going, and also my faith,” she said. “And I am very spiritual when it comes to God.
"I don’t walk around and say, ‘woe is me,’” she said. “I just take it head on and deal with it. I always say it’s a part of my journey and it makes me who I am today.”
Please remember the opinions expressed on Patient Power are not necessarily the views of our sponsors, contributors, partners or Patient Power. Our discussions are not a substitute for seeking medical advice or care from your own doctor. That’s how you’ll get care that’s most appropriate for you.
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