cmml: treating a difficult rare blood cancer
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chronic myelomonocytic leukemia can be asymptomatic, but also cause symptoms like bone and joint pain, a decrease in appetite and weight loss. getty
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cmml, or chronic myelomonocytic leukemia, is a rare type of blood cancer that accounts for about three in a million cancer diagnoses in canada, according to the leukemia and lymphoma society of canada (llsc). the majority of people diagnosed with cmml are between 65 and 75, and it’s twice as common in men as in women.
“it’s not terribly common,” says dr. jeff lipton, an oncologist at princess margaret cancer centre in toronto who specializes in leukemia and stem cell transplants – the course of treatment if a donor match is found for patients with the acute and aggressive form of cmml.
“it’s what we call a myeloproliferative disorder, meaning it’s an abnormal development of white cells or parts of the bone marrow to generate initially a chronic form of leukemia that has the potential to transform into something more acute,” he says.
the abnormal cell development interferes with the production of oxygen-carrying red blood cells and platelets that stop bleeding. and while most gps will go their entire careers without seeing a patient with cmml, lipton’s clinic is where patients are referred for treatment.
for people who have the disease at its earliest stage cmml-0, it may never progress or require any treatment. cmml-0 means that less than two per cent of the white cells in the blood and less than five per cent of the cells in the bone marrow is made up of blast cells. there are two other stages as well, cmml-1, where blast cells make up less than five per cent of white cells in the blood and less than 10 per cent of the cells in the bone marrow, and cmml-2, where blast cells make up five to 20 per cent of the white cells in the blood, or they make up 10 to 20 per cent of the cells in the bone marrow.
cmml symptoms
so how would you know if something is wrong? lipton says there’s a whole spectrum of symptoms — or none at all. some patients are asymptomatic patients, but diagnosed after randomly having a blood test that comes back abnormal. others may get symptoms related to an overproduction of white cells, such as an enlarged spleen which can cause abdominal discomfort, and create a sense of fullness in the stomach even when a small amount is eaten. bone and joint pain, a decrease in appetite and weight loss, or bruising are other signs.
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also, because of the proliferation of white blood cells, other cells like red blood cells and platelets decrease.
“so you may have some people present with fatigue, fevers or bleeding or things like that, or to the point where if it’s transformed into something more acute, they may actually present with, sepsis [a life-threatening response to an infection] or fevers,” says lipton. “so it’s not just one set of symptoms.”
lindsay thompson, marked 15-years post-transplant this past march. supplied
treatment, too, is all over the map, he adds, depending on the stage, but cmml can transform into an acute leukemia within a matter of months. drug therapy is the main form of treatment, followed by allogeneic stem cell transplantation when it turns acute — where a healthy person’s donated stem cells are transplanted to the patient following chemotherapy. the high-risk procedure can slow the growth of the disease.
statistics from llsc note that about 50 per cent of people with cmml will live for one to two years after the start of treatment. a successful transplant, however, can extend life expectancy for many years when patients survive the first two years after the transplant.
“the bottom line is with any kind of leukemia, your ability to treat it successfully is very age-related,” says lipton. “so as we get older, your ability to withstand treatment is much lower.”
the first step in treating his young patients, says lipton, is identifying what stage they are in and how aggressive it is.
“i follow them initially, or if it looks like it’s starting to get aggressive, give some kind of treatment to try and get it under control and then do a transplant on them as quickly as possible,” he says.
cmml “changed my life dramatically”
a stem cell transplant gave lindsay thompson a chance at a normal life after she was diagnosed with cmml-2.
she was on duty as a nurse clinician at the alberta children’s hospital in calgary in 2007 when she accidentally whacked her elbow on a table. the injury quickly led to an enormous goose egg and over the next couple days, the bruise became black stretching all the way to her wrist. by the following week, her elbow joint felt hot, so she went to an urgent care clinic.
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“i thought i possibly had an infected joint, so that meant i’d start on intravenous antibiotics,” thompson says, adding that the clinic called shortly afterward asking if she’d known she was severely anemic.
“i was 51, i was menopausal,” she says, so alarm bells weren’t sounding for her until the next morning. she had been referred to an antibiotic program in hospital for further treatment where she asked the nurse what was going on with her blood.
“she obviously had never dealt with this before,” says thompson. “she handed me my lab result and i could see that at the very bottom there was an urgent message that i had blasts in my blood, and the only time you had blasts in your blood, as far as i knew, was when you had leukemia.”
when more blood work and a bone marrow biopsy confirmed cmml, thompson was devastated. she had become a patient, and now fully understood what the parents she worked with felt like when they were told their child actually didn’t have an infection, but instead, would need to be treated in the oncology clinic.
“the world drops out from under them because it’s the last thing they were expecting,” she says.
when a course of drugs didn’t work, she needed a stem cell transplant. with the search for a donor in process, thompson was able to return to work for a few months longer. she started losing weight, having terrible night sweats, loss of appetite and exhaustion. her coworkers celebrated the team christmas party the third week of november so that she could be a part of it.
a match was found and she had daily appointments for the month prior to surgery — which is the normal process to make sure people have a “reasonably good chance of survival,” she says. but when her cmml turned acute, the transplant was postponed until she had chemotherapy and two months in hospital to get stabilized before going ahead with the transplant in the spring of 2008.
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people don’t want statistics, they want to hear personal stories
she then developed graft-versus-host disease (gvhd) in the first 100 days post-transplant — a common systemic disorder that happens when the graft’s (donor’s) cells recognize the host (your cells) as foreign and attack them. then, a year after the transplant, thompson had a viral infection that damaged her lungs and required steroids for seven years. she has been on and off oxygen therapy ever since, and has at least one hospital admission a year for respiratory issues. she also deals with ongoing skin irritations on her arms and legs, and gets an immunoglobulin infusion every month after a 2016 diagnosis of b-cell immunodeficiency — which increases infection risk.
“[cmml] changed my life dramatically,” she says. “i was never able to get back to work. so we struggle financially, and i struggle with depression on and off.”
sharing lived experience is critical
while she couldn’t return to nursing, she has joined llsc to help with advocacy, education of health professionals and patient outreach.
“when i speak to other patients, and they ask, ‘what was it like for you?’ i say, ‘this is what it was like for me. but you will be different,’” says thompson. “i ask them if they know that saying, ‘you can’t see the forest for the trees’, and then i tell them that they don’t want to deal with the forest because the forest is overwhelming. you want to deal with the one in front of you and figure out how to get around that tree today. then, tomorrow you deal with a new tree. then it is all manageable.”
that kind of mentoring and support — from someone with lived experience — is critical for people with cancer, says nadine prevost, business unit director of research and community support with llsc.
“people don’t want to hear about statistics, they want to hear someone’s personal story,” she says. “being connected through our peer support program, which matches people with someone in their age group with often the same type of cancer, can change their perspective. after all, here’s someone who is alive with this and has a good quality of life.”
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prevost says that this meaningful connection has also been proven to change health outcomes, especially at a time when even your closest loved ones can’t fully understand what you’re going through or how they can support you. while the organization raises funds and contributes to research in blood cancer treatment, it considers patient empowerment and connectedness as top mandates.
thompson, who marked 15-years post-transplant this past march, says all she ever wanted to be was a nurse — cmml took away that identity.
“when i couldn’t go back to work, i didn’t know what to do with myself,” she says. “[volunteering] gives me a purpose. i still am valid, i still have a use in life. i can still contribute to help others. that keeps me going.”
karen hawthorne is a toronto-based writer.
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