What 35 Years with Polycythemia Vera Reveal About Diagnosis and Hope

Nona Baker, 40, was raising three children in West Sussex, England, when a hematologist told her she had an incurable blood disorder and 15 years to live. The diagnosis was essential thrombocythemia (ET), one of a group of rare blood cancers now known as myeloproliferative neoplasms (MPNs).

Polycythemia vera (PV) followed roughly a decade later. She has both ET and PV, though the PV symptoms have been the more prominent of the two. When she was diagnosed, the illness was classified as a blood disorder. The World Health Organization reclassified it as a neoplasm in 2011.

Baker is 35 years past that conversation, and she credits new drugs coming down the line for much of her hope about what comes next.

She spent her career in counseling and psychotherapy and still sees clients, though advocacy is now her main focus. She also co-chairs MPN Voice, a charity affiliated with Guy's and St Thomas' Hospital in London that grew out of the eight-patient support group she was invited to join.

"Although I've lived with [an MPN] for a long time, I think it gives hope to other people," she said. "But also, I have the privilege of meeting so many people who struggle with a little-known cancer."

What symptoms lead to a myeloproliferative neoplasm diagnosis?

Baker's first sign of illness was in her feet. She developed ulcers and pain that turned out to be thick blood moving through small vessels.

"In our deep freeze we had lots of frozen peas, and they were known as mum's mushy peas, because I would strap them to my feet to try and relieve [the pain]," she said. "The children used to go and get mum's mushy peas out of the deep freeze."

She also had floaters in her eyes and experienced chronic fatigue. With three children, her fatigue would be dismissed as tiredness.

"It was more than tiredness," Baker said. "It's really, really difficult when people don't believe what you're saying to them."

She suspects the illness had been with her far longer than the 1991 diagnosis suggests. She was sent home from school five times as a child with what was called glandular fever, known in the United States as mononucleosis, and its main symptom is the chronic fatigue she carried into adulthood.

Why do patients with a myeloproliferative neoplasm face delays before diagnosis?

Baker described going what she calls “round the houses”, a British expression for a roundabout route, from one specialist to the next. An orthopedic surgeon put insoles in her shoes. When that failed, he sent her to a rheumatologist, who told her she had an alcohol problem while her husband sat beside her. Baker does not drink.

The same physician then suggested the equipment might be at fault and ordered another blood test on a Thursday. She was home alone that Saturday morning when he called to say something was seriously wrong with her blood and that a hematologist would see her Monday.

What is it like to be diagnosed with an incurable blood cancer at 40?

Baker spent months being told nothing was wrong, so finally having a name for what she was experiencing, essential thrombocythemia, came as a relief, layered with the fear of everything it had taken to get there.

Then came the question she regretted the moment she asked it.

"How long have I got to live?" she asked her hematologist.

Fifteen years, he said. Baker was 40, with three children at home.

"I felt I'd been run over by a bus, because everything was coming at me in one go," she explained.

Where can patients with polycythemia vera find accurate information?

In the early 1990s, Baker turned to an internet that was barely a few years old and found unreliable material. Years later, when her PV diagnosis came with a high hematocrit, a measure of how much of the blood is made up of red blood cells, her general practitioner suggested a second opinion, which took her to St Thomas' Hospital and to Claire Harrison, then a doctor and now a professor. Harrison told her a support group was forming precisely because so much incorrect information was circulating, and asked whether she wanted to join.

"There were eight of us in a room, and it was like a light bulb moment," Baker said.

That group became MPN Voice, which now runs a buddy program pairing newly diagnosed patients with people who have lived with the illness for at least two years. Buddies are screened against set criteria, including knowing the name of their own hematologist, and the program has expanded internationally, with volunteers in the UK supporting patients in Hong Kong and the United States.

"There's a huge amount of strength in being part of a professional patient advocacy group," she noted. "And I say professional. We're volunteers."

How is fatigue from a myeloproliferative neoplasm different from ordinary tiredness?

The distinction between fatigue from a myeloproliferative neoplasm and ordinary tiredness is poorly understood, according to Baker. Fatigue can arrive without warning, and she has a shorthand for the moment it does.

"I say I've hit a wall, and my family knows what I mean," she said.

The social cost was the hardest part for Baker. She looked fine, so when she asked for time off work for hospital appointments, she was left feeling that people doubted she was ill at all.

"I simply couldn't do things. If there was a party or an event going on I always felt that people thought that I was making an excuse," she said. "I wasn't, but it was hard to actually tell people, because [an MPN] was so rare."

What does a phlebotomy appointment involve for a patient with polycythemia vera?

Venesection, the term used in the UK, removes blood to bring down elevated counts.

"There was not such a heavy load of blood going through my system. It wasn't so sluggish," she said, adding that the veins in her feet improved considerably once she was diagnosed and on treatment.

Baker felt the benefits immediately, but the appointment takes a full day: Travel into London, blood drawn, waiting for results, the consultant, the day unit, the procedure, 25 minutes of observation, then the journey home.

She goes alone, takes a humorous novel and practices mindfulness in the waiting room. The routine has become normal for her in a way it may not be for someone newly diagnosed.

"Our health service here is incredibly stretched," Baker said. "I am taking up clinic time because I have to go up there to have my blood count done to see if I need a phlebotomy."

How does treatment for polycythemia vera change over time?

Baker has cycled through several approaches, with the options remaining limited. One early medication was a chemotherapy drug, which she discovered by reading the patient information leaflet.

"I remember going into my [general practitioner] and saying, 'Nobody's told me I've got cancer. Why am I on this drug?'" she said. He explained that she had a proliferation of cells and the drug reduced them, which she credits with finally making the illness clear to her.

A second drug worked well for years, until the skin damage accumulated. Interferons were tried next, but her liver did not tolerate one and her gut did not tolerate the other. She is now back on the earlier drug at a reduced dose, with more frequent venesections to compensate.

"It's a chicken and egg situation," she said. "I'm just so hopeful that one day something will happen that I won't have to think about having to have these phlebotomies."

What skin side effects can polycythemia vera treatment cause?

Treatment left Baker with widespread actinic keratosis, rough scaly patches caused by sun damage that can turn into skin cancer if untreated, along with basal cell carcinomas, a common and slow-growing form of skin cancer that has required removal. She has a full skin check twice a year, where the actinic keratosis can be frozen off with liquid nitrogen, a quick in-office procedure, while the carcinomas need more involved treatment. Anyone on treatment for an MPN, she said, should be careful in the sun, and she noted that high-factor sunscreens have made that far easier than they once were.

Besides fatigue, itch is the other symptom patients with PV struggle with most, she said, and she knows a woman whose itch is severe enough that she cannot go in water.

How can patients with polycythemia vera advocate for themselves?

Baker's approach came down to two commitments: gathering accurate knowledge, and following what her clinicians advised.

"I don't underestimate how getting accurate knowledge is really, really empowering for a patient," she said. "But it's making sure you source the accurate knowledge."

She encouraged patients still stuck in the diagnostic process not to give up, and to extend trust once they reach someone who knows the illness.

"Don't despair if you have a difficult path to being heard in a diagnosis, because it's very common for people to go round the houses," she said. "When you get in the right place with somebody who understands an MPN and the treatment options, commit to it and go with the flow."

How does living with a myeloproliferative neoplasm affect family members?

Baker is direct about what relatives take on when they live alongside someone with a chronic illness, navigating fatigue and uncertainty and adjusting to a schedule of regular hospital appointments while never being the ones receiving care.

"It's having that confidence to say, 'Look, I'm really sorry. I may look well, but I'm feeling [awful],'" she said. Recognizing what a chronic illness asks of caregivers, she added, matters as much as supporting the patient.

At home, that has meant her husband picking up the day-to-day work she cannot always manage, including learning to run the dishwasher and the washing machine.

Family support matters most once a patient enrolls in a clinical trial, since a difficult stretch or a new side effect rarely stays between the patient and the study team, and a worried relative may push them to stop.

"If they have a bit of a blip on the trial and they get back to the family member, the family member will say, 'Stop taking it, stop taking it,'" Baker said. "It's really important to embrace supporting the families as well."

What has changed for patients with myeloproliferative neoplasms since the early 1990s?

Diagnoses are being made earlier now, including in children and young adults, which Baker credits to how far science has progressed. Patients also have a hand in the research itself, through patient insight councils that weigh in as studies and trial protocols are written.

"It feels good that we are listened to," she said. "It feels like together we will make a change."

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