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The Reuters news staff had no role in the production of this content.
It was created by Reuters Plus, the brand marketing studio of Reuters.
Myelofibrosis:
Knowledge is power in this journey
A complex blood cancer, myelofibrosis is challenging to diagnose and treat, as patients can experience a wide variety of symptoms. The good news is that with the right roadmap, it’s possible to knowledgeably navigate the different phases of this cancer journey.
or most, a visit to the furniture store is nothing out of the ordinary, but for Thomas Bierfeld, it was a trip that changed everything. While the 35-year-old professional musician was shopping, his phone rang. On the other end was his doctor, with unthinkable news: Thomas had blood cancer.
F
“I just went numb,” remembers Thomas, now 39, who lives in the town of Ulm, near Munich, in southern Germany. “I was shocked, I was upset, and I was angry. I went home and cried, then I wiped my face and started reading everything I possibly could about the condition.”
The diagnosis was myelofibrosis (MF): a complex blood cancer that Thomas had never heard of before that call. This news had come unexpectedly—despite having been diagnosed years prior with a blood disorder called essential thrombocythemia (ET) that can progress to MF. Now, he threw himself into researching the illness—on the internet, by reading medical journals and by speaking to anyone he could.
1
3 types of MPNs
There are different types of myeloproliferative neoplasms (MPNs). Each has unique symptoms and different effects on the body. Three common types are:
1
Essential
thrombocythemia (ET)
ET happens when your body makes too many platelets. Having high amounts of platelets in your blood can lead to blood clots.
2
Polycythemia
vera (PV)
PV is a type of MPN that happens when your body makes too many red blood cells. In PV, your body may also make too many white blood cells or platelets.
3
Myelofibrosis
In people with myelofibrosis, blood cells don't function normally and build up inside the bone marrow. This can lead to inflammation and scarring, which is called fibrosis. Fibrosis can cause your body to make too few blood cells.
Cancer Support Community;2023;1-5;Myeloproliferative Neoplasms,, https://www.cancersupportcommunity.org/myeloproliferative-neoplasms#myelofibrosis
“I just went numb,” remembers Thomas, now 39, who lives in the town of Ulm, near Munich, in southern Germany. “I was shocked, I was upset, and I was angry. I went home and cried, then I wiped my face and started reading everything I possibly could about the condition.”
I had never heard of myelofibrosis before.
The first time I heard of it was when I was diagnosed.
Thomas quickly became frustrated about the lack of awareness around the disease, which is part of a larger group of blood cancers called myeloproliferative neoplasms, or MPNs.
He saw multiple doctors in an effort to find clarity on the condition and treatment approach, but through this process, discovered that few doctors have sufficient experience to offer informed guidance.
“I went with my wife to see one doctor who told us I was reading too many things I didn’t understand,” Thomas says. “Not only did he fail to give us any answers, he sent us home with even more questions.”
Finally, his exhaustive research brought him to an MPN specialist, who had the expertise and knowledge to help him.
“For me, it was crucial to identify the correct medical professional to work with,” Thomas says. “To find an expert who will answer all of your questions fully and guide you towards the right decisions for your life.”
The good news for Thomas was that he was young, otherwise healthy and displayed minimal symptoms. Instead of undergoing the intensive stem cell transplant that others had recommended, Thomas’s MPN specialist worked with him to co-create a personalized plan to best manage his disease, which included a regimen of physical activity, rest and controlled diet, coupled with regular medical checkups, to help Thomas maintain his overall health.
Often, people struggle to find the right doctor and the right diagnosis.
While still concerned, Thomas was encouraged: Within four months of diagnosis, he was under expert care, well informed and had a clear roadmap to navigate the journey ahead of him. Other MF patients are less fortunate, as delayed diagnoses and misdiagnoses are still frustratingly common.
Myelofibrosis is a complex cancer characterized by the build-up of scar tissue in the bone marrow and excessive inflammation. The chronic condition is both rare (affecting 25,000 people in the United States and 1 in 500,000 people worldwide ) and difficult to diagnose, as signs and symptoms can differ widely for each person. Key symptoms may include fatigue, bone aches, night sweats, weight loss and pain beneath your left ribs due to an enlarged spleen (splenomegaly).
One of the hallmarks of MF is anemia—characterized as a low red blood cell count. Approximately 40% of patients have moderate to severe anemia when diagnosed with MF and another 20% develop anemia within a year after diagnosis. Nearly all patients will develop anemia during their MF journey—especially since some MF treatment options exacerbate anemia—resulting in profound fatigue.
2
2,3
4
2
5,6,7,8
Each person experiences myelofibrosis differently
The symptoms of myelofibrosis can be different for each person, which means you may not experience all of them. Some of the key signs and symptoms are anemia, enlarged spleen, low platelets and other symptoms.
Anemia
Having a low red blood cell count is called anemia. Your doctor may call it low hemoglobin.
Anemia can cause symptoms like:
Tiredness/fatigue
Weakness
Shortness of breath
Migraines
Clevelandclinic;2023;1-13;Myelofibrosis: Symptoms, Types, https://my.clevelandclinic.org/health/diseases/15672-myelofibrosis
Enlarged spleen
Your spleen acts like a filter for your blood. Having an enlarged spleen is called splenomegaly.
Splenomegaly can cause symptoms like:
Feeling full too fast
Pain under the left ribs
Severe abdominal discomfort
Low platelet count
Platelets are important to help your blood clot. Having a low platelet count is called thrombocytopenia.
Thrombocytopenia can cause symptoms like:
Bleeding easily
Bruising easily
Bleeding for a long time when cut
Other symptoms
Myelofibrosis can cause different symptoms because it affects the body in many ways.
Other symptoms of myelofibrosis may include:
Tiredness/fatigue Night sweats
Abdominal pain Bone pain
Itchy skin Weight loss
heryl Petruk, originally from Edmonton, Canada, who cared for her late husband, Eugene, during his battle with MF, now realizes they overlooked early key symptoms, including extreme fatigue caused by anemia, rapid weight loss, and “eggplant-purple bruises” on his body.
Frustrated by the lack of information and local support during her husband’s illness, Cheryl started a local patient support group, and later established a national research foundation dedicated to stimulating research on new treatment options and ultimately finding a cure for MPNs like MF. She currently runs a nonprofit organization called Heal Canada, helping educate people to be better patient advocates.
C
There's a community that has a diagnosis of an incurable rare blood cancer, and they need to be given every opportunity to live a normal life.
“When he [Eugene] was first diagnosed in 2010, I would search for others who had the disease, because we felt so alone when we left that doctor’s office,” she says. “We really felt it was important to connect, to have a community. We got together with some other folks in Canada and established the network. And today, [there are] support groups across Canada.”
While people diagnosed with this complex blood cancer face numerous obstacles and uncertainties throughout their MF journey, Cheryl believes “they need to be given every opportunity to live a normal life. They should be able to see their children through school, walk them down the aisle and see their grandchildren born. That’s what drives me: a passion to help these people live better, longer lives.”
The good news is that medical science has made significant strides in the treatment of MF over the past decade. There are a variety of treatments that can help address the symptoms of MF, including lifestyle measures, over-the-counter medicines, targeted therapies as well as blood transfusions, which can be required as supportive care for those with anemia, among others. Additionally, few patients may qualify for an allogeneic stem cell transplant, which is the only potential cure for MF, but carries significant risk. It’s critical to speak to a healthcare provider who can map out a treatment plan.
With the right information, support and attention, there is hope, which is something Thomas discovered after he met his MPN specialist, who not only helped him adopt a holistic master plan, but also encouraged him to pursue his two main life goals: building his dream home and starting a family. Four years after his diagnosis, Thomas is now living in that dream house with his wife and 2-year-old daughter.
“I’m very lucky to be able to have a normal life at the moment,” says Thomas, who continues to play the French horn with some of the most prestigious orchestras in Germany. While Thomas understands he will likely need more intensive treatment in the future, “For now, I have my plan and will continue following it as long as I can. There’s a lot happening in this field, and I try to keep myself educated on everything: a lot of research and a lot of possibilities. Because the more informed you are, the more empowered you are.”
If you need medical advice, please consult a healthcare provider.
My advice is to look for patient groups, be responsible for your own condition and find the best doctor that can help you.
To learn more about myelofibrosis and hear from members of the myelofibrosis community, check out the
Mapping Myelofibrosis podcast series on YouTube.
REFERENCES:
1. “What Is Primary Myelofibrosis (MF)?” MPN Research Foundation. https://www.mpnresearchfoundation.org/primary-myelofibrosis-pmf/
Thomas and his daughter
— Thomas Bierfeld
— Thomas Bierfeld
Thomas still enjoys white-water kayaking...
Cheryl Petruk
— Cheryl Petruk
4
— Thomas Bierfield
2. “Primary myelofibrosis” U.S. Department of Health and Human Services National Institutes of Health. https://medlineplus.gov/genetics/condition/primary-myelofibrosis/
3. Data on file. Sierra Oncology. 2021
4. “Myelofibrosis: Symptoms, Types” Cleveland Clinic. https://my.clevelandclinic.org/health/diseases/15672-myelofibrosis
5. Tefferi A, Lasho TL, Jimma T, et al. One thousand patients with primary myelofibrosis: the mayo clinic experience. Mayo Clin Proc. 2012;87(1):25-33. doi:10.1016/j.mayocp.2011.11.001
6. Bose P, et al. Curr Hematol Malign Rep. 2018;13:164-172. doi: https://doi.org/10.3109/10428194.2013.813500
7. Scherber, RM, Mesa, R. Management of challenging myelofibrosis after JAK inhibitor failure and/or progression. Blood Rev. 2020;42:100716. https://doi.org/10.1016/j.blre.2020.100716
8. Bassiony S, Harrison CN, McLornan DP. Evaluating the Safety, Efficacy, and Therapeutic Potential of Momelotinib in the Treatment of Intermediate/High-Risk Myelofibrosis: Evidence to Date. Ther Clin Risk Manag. 2020;16:889-901. Published 2020 Sep 25. doi:10.2147/TCRM.S258704
... as well as mountain climbing.