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'an old person's disease': how did stomach upset turn out to be a rare blood cancer?

carol cameron was 39 when she was diagnosed with essential thrombocythemia, a rare blood cancer that typically affects people over the age of 60.

by robin roberts

sep 9 2022
read time 5 minute read
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you could have essential thrombocythemia and not know it
myelofibrosis is a chronic blood cancer in which the bone marrow is replaced with fibrous scar tissue, according to cleveland clinic. getty

carol cameron was diagnosed with the blood cancer essential thrombocythemia (et) the way many people are: through a blood test for a completely unrelated issue. she had been suffering with stomach issues so her family doctor referred her to a gastroenterologist. as part of getting to a diagnosis, he ordered routine blood work, which showed that her platelet count was more than twice the normal range, which is a common indication of cancer. cameron then saw a hematologist who did a bone marrow biopsy — a procedure in which a thin needle is inserted into the pelvic bone (sometimes the breastbone) to extract a sample of bone marrow for examination.

the diagnosis was confirmed: cameron had et, one of a group of three progressive blood cancers called myeloproliferative neoplasms, or mpns. the other two include primary myelofibrosis (mf) and polycythemia vera (pv). while b oth et and pv are generally considered less aggressive, they both may progress to the more serious myelofibrosis. and all mpns have the risk of progressing to other cancers, particularly acute myeloid leukemia.

et mostly affects women over 50. cameron was just 39.
“i was surprised,” says the lac du bonnet, manitoba, resident. “i had no family history, no idea what it was or what to expect.”

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john clark, who lives with polycythemia vera, a myeloproliferative neoplasm (mpn), says one of the biggest challenges for people with an mpn is access to medication. his treatment costs $60k per year.
john clark (left, with his wife, bea) was diagnosed with polycythemia vera in 1995, kicking off his involvement in patient advocacy. supplied

leukemia: types, symptoms and treatment
leukemia is a broad term for multiple different forms of blood cancer. it primarily affects the stem cells that grow into blood cells.
leukemia is the most common cancer in children under 15.

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she reached remission in just three-and-a-half months with the help of oral chemotherapy, but after 20 years of living with the disease, cameron began to experience sore muscles and extreme fatigue.
“i would lay down and sleep for two hours after walking,” she says. “it took my doctor and my hematologist almost a year to figure out that it had progressed to myelofibrosis.”

this type of mpn, according to the cleveland clinic , is a chronic blood cancer in which the bone marrow is replaced with fibrous scar tissue.

“sometimes patients, because of the scarring, might have low red cell counts, [causing] anemia,” says dr. shireen sirhan, hematologist and oncologist, division of hematology at the jewish general hospital in montreal, where she co-founded canada’s first specialized, dedicated mpn clinic. “sometimes their white blood cells can be high or low. [as a result] patients tend to have symptoms of fatigue, weight loss, night sweats, in many cases an enlarged spleen [which makes them] unable to eat a full meal.”
although sirhan and other specialists cannot pinpoint the cause of this group of blood cancers, there is plenty of ongoing research trying to find out.

cheryl petruk, executive director of the canadian mpn research foundation and the canadian mpn network, has intimate knowledge of the disease after caring for her late husband, eugene, who had both et and mf.

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the leukemia & lymphoma society of canada (llsc)

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“eugene was diagnosed in 1992 and lived for 20-plus years with et before it progressed to mf,” she says, adding that while many mpns are related to a genetic mutation, sometimes exposure to environmental toxins may play role. “ eugene lived on a farm where he was exposed to benzene and toluene — chemicals in diesel fuel.”

“an old person’s disease”

like cameron, eugene did not have any signs or symptoms until he was sent for blood work after experiencing chest pains.
“that’s when they diagnosed him with et — they also did a bone marrow biopsy to confirm it.” petruk says. “you should only have up to a million platelets in your blood; he had something like 20 million. typically, this type of cancer is diagnosed later in life, but eugene was 30 years old. even the hematologist said that it was an ‘old person’s disease’.”
eugene may have had et for many years, she says, since he didn’t see his doctor regularly for check-ups. but with more comprehensive blood tests today, petruk suggests that people are being diagnosed earlier.
sirhan agrees, adding that earlier diagnosis can prevent negative outcomes.
“i think we do blood tests more often so we are diagnosing people earlier and that’s good because you can prevent someone from having a blood clot,” she says. clots can occur in people with any mpn because their blood is thicker and their blood cells are stickier than normal. clots can in turn cause a stroke or a heart attack.

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and while patients who are asymptomatic may require no treatment in the early stages of the disease, once they start experiencing symptoms, such as vision problems, numbness, tingling, bruising or unexplained pain, they will require a therapy that’s dependent on their age, general health, and their risk for blood clots. these can include platelet-lowering drugs, interferon for reducing spleen size and other side effects, or low-dose aspirin to reduce clumps or clots.
carol cameron had et, one of a group of three progressive blood cancers called myeloproliferative neoplasms, or mpns. supplied
carol cameron had et, one of a group of three progressive blood cancers called myeloproliferative neoplasms, or mpns. supplied

medications that target quality of life

petruk says while there is no cure for mpns, there are effective medications to help manage quality of life, including one that’s fully approved and awaiting the reimbursement process, and three more in the final stages of clinical trials. there are also more treatments available in the u.s., adds sirhan, which canada is working on getting access to.

when asked how many people are affected by mpns, sirhan says it’s a challenge to pin down the exact numbers of those diagnosed in canada, but it’s generally measured by dividing u.s. statistics by 10. according to the canadian mpn research foundation , the projected prevalence of mpns in the u.s. in 2010 was 12,812 people diagnosed with myelofibrosis, 148,363 with polycythemia vera and 134,534 people with essential thrombocythemia.

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life expectancy varies, but outcomes are better than ever

sirhan says that while life expectancy varies among the cancers, outcomes for patients are better than ever, particularly if they receive care from someone who specializes in mpns.
“if we look back 10 years ago, we didn’t have the options we have now, so there’s always hope,” she says. “research is always advancing. patients should always seek an opinion at a specialized centre at least once in order to assess what’s available.”
and don’t underestimate the value of speaking up.
“mpn patients are very well-educated and that makes a huge difference because they advocate for themselves, they advocate for newer treatments,” says sirhan.
cameron is doing just that. after being told that she would have to wait two years to see an mpn specialist in canada, she is heading to the mayo clinic in phoenix to meet with one there for a second opinion.
“i’m fortunately in a position where i can do that,” she says. “i feel extremely blessed because some people’s journeys are not good and other people can live with this for years, even decades. i try not to think about it. the anxiety is always there, wondering whether it’s going to progress into something more. i take it one day at a time, because i could get hit by a bus tomorrow. there’s no point in worrying about what’s going to happen in six months from now, because no one’s guaranteed tomorrow.”

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robin roberts is a vancouver-based writer.
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