The challenges of living with myelofibrosis: Jenny Wilson – anaemia and fatigue
Jenny Wilson, who is 66 years of age, has lived with myelofibrosis for 5 years. Here she describes how anaemia and its treatment with transfusions has been a major challenge for her. She shares how she has kept active and positive, living in hope that further therapies will be forthcoming
Fatigue and anaemia
In 2017, Jenny Wilson, at the age of 61, went for her routine annual check with her GP, and she mentioned that she was feeling tired all of the time. “I used to do cardio circuit training, and I’d noticed it was getting harder rather than easier, which didn’t make any sense to me.”
The doctor reassured Jenny that this would be easy to check and took some blood. “The surgery phoned me the day after to say they needed to repeat the blood test to check something. And then the day after that, I was told that I was being referred to haematology because I was anaemic: my haemoglobin was too low.”
Despite a lack of specific symptoms, Jenny strongly believed there was something seriously amiss with her health: “It’s hard to explain, but I felt something was wrong. And I did not think it was something simple.” When the haematology department sent her for a bone marrow biopsy, Jenny immediately feared she might have cancer.
“When the diagnosis of myelofibrosis came, I felt numb. They told me my prognosis was 10 to 14 years. And I foolishly thought, ‘Oh 10 to 14 years, that’s not really so bad for cancer. Of course, I wasn’t thinking, ‘Gosh, 10 to 14 years of failing health and feeling rubbish at times!’”
One of the medics involved in Jenny’s care described living with myelofibrosis as “much more of a marathon than a sprint”. This makes more sense to Jenny now than it did at the time. “The fact is that most people who get a cancer diagnosis don’t have a great deal of time to get their head around it. I think that’s what that advice gave me: you’ve got time to hope. Time also gives you an awful lot of time to worry—if that’s what you do. I try not to because it’s not actually very productive.”
Weighing up treatment options
Following her diagnosis, Jenny was faced with a dilemma about her treatment: “Around 50% of people who have myelofibrosis have involvement of a faulty JAK2 gene, and I don’t. So, that meant some treatments were not available to me, and I was concerned about the side effects of the other treatments that were available. One of the therapies can cause neuropathy to extremities, the hands and feet. I do crafting and stuff like that, so that was not attractive.
“I know people take steroids on a long-term basis, but the side effects are quite considerable. With long-term use, you end up with a very distinct moon face and you gain weight, for example.”
Jenny was so concerned about side effects that she decided she would take her chances with waiting for a clinical trial which might enrol her on a novel therapy. She was adamant that she wouldn’t start any existing treatment because the need for a “wash-out” period might preclude her from joining a trial later on. Jenny feels fortunate that she was finally enrolled in a clinical trial, and more fortunate still that her symptoms have stabilised with that therapy.
Anaemia and the burden of repeated transfusions
When she decided not to pursue the standard treatments offered to her, Jenny’s only remaining option at that time was transfusion therapy, but her haemoglobin levels were too high for the first five months after diagnosis. When they fell, Jenny started having blood transfusions eight weeks apart.
With her haemoglobin levels progressively falling, each successive transfusion was needed a week earlier, and ultimately Jenny had to go through the ordeal of a blood transfusion every four weeks. She describes that experience as a physical and emotional “rollercoaster”.
Living with anaemia was a tremendous physical burden for Jenny: “It was unbelievably and unremittingly hideous to be that low in energy. When my haemoglobin was under 85, it was treatable, but they liked it to be closer to 80. When I was down that low, I could scarcely walk across the car park! My best friend, a nurse, told me later that she thought I was dying because of the way I looked.”
“I would go in for a transfusion with a face the colour of putty, and I would come out pink, so it was an instant difference. But even after two units of blood, my haemoglobin still didn’t make it into the low–normal range. I’d have a couple of weeks of feeling better and then a couple of weeks of going down again, sliding down into the treatable zone again.”
The emotional impact of this was enormous too, Jenny says: “It was depressing. Thinking about it now, I do wonder how I got through it, but sometimes you just get through things because you have no choice. At times I got through it by simply not thinking about it or not focusing on it.”
Jenny notes the practical challenges with transfusion therapy. She says patients need to constantly monitor how they’re feeling and whether they think they need treatment. “There were no tests I could do myself. So, it was very much a question of ‘how do I feel?’. But when anaemia is mixed with fatigue—one of the main symptoms of myelofibrosis—it’s hard to tell which is which.”
Jenny explains that transfusions also take up a lot of time. “If the transfusions were eight weeks apart, they would review me at six weeks to check my level, and then check it again, two weeks later. And if I felt like I was low, I would have to phone up haematology and ask them to order a blood test.
“It would be a whole day to go in, get a blood test, check the haemoglobin. Then, they’d have to do a cross match for the transfusions, they’d have to order the blood, I’d have to wait around or disappear for an hour or so and then go back. And then it’s three or four hours of transfusion.”
The logistical challenges associated with transfusions mean that patients must make major changes to their lifestyle, Jenny says: “You do cut down massively on what you plan to do. I didn’t plan to go anywhere or do anything in the time I was needing blood transfusions.”
“It was a necessity to have the transfusions, but it wasn’t lovely. And it’s not a curative treatment at all—it’s just fixing the symptom that you have at the time. I’ve also still got all that iron in my system because you don’t secrete it.”Jenny
Jenny’s approach to maintaining physical and mental well-being
Between treatments, Jenny still has days when she feels less well. Her most recent haemoglobin level was in the low–normal range, but Jenny still found herself struggling to do her daily walk, and she can no longer do the cardio circuits or Pilates classes she used to enjoy. With her prognosis being so uncertain, she is reluctant to share her diagnosis with people she meets; people tend to assume that her life expectancy is short when they hear she has a form of cancer.
Support groups are often a useful resource for people affected by a disease, but it is important to be prepared for the differences between regions. When Jenny was first diagnosed, she found a support group that was based in the United States, but she was quite distressed by how patients had access to treatments not yet approved in her own country.
To help her cope psychologically with living with myelofibrosis, Jenny has found it helpful not to concern herself too much with the details of the disease. She has watched some of the seminars produced by MPN Voice, but generally she prefers to concentrate on her own lived experience rather than studying the disease or looking for other people with myelofibrosis.
Today, Jenny takes care of her physical fitness through walking and looks after herself mentally through mindfulness activities. She jokes that the two are perhaps best not done together for fear of tripping, but is earnest about their benefits.
She says her daily walk is along the same route whatever the weather: “I know that sounds boring, but it’s a clifftop walk by the sea. It’s beautiful, and you can you never get tired of it. I know every bench along the way, and I can gauge how I’m feeling simply by how far I can walk, how fast and how often I need to stop—if I need to stop at all.
“I know that when my haemoglobin level is okay and I’m not too fatigued, I’ll do half the walk without stopping. When my haemoglobin is lower, I’ll know the difference. So, it’s a little bit of a dipstick. Even if I don’t feel like doing it, I do it. I would suggest walking to anybody, especially when you really can’t exercise as you used to.”
From her diagnosis onwards, Jenny has found mindfulness enormously helpful in dealing with the stress of living with myelofibrosis. She says that the frequent medical interventions she has needed, which have often been physically invasive, have felt emotionally challenging, and she suspects other patients may feel this way too. She wholeheartedly recommends mindfulness to them.
“After I’d been diagnosed, I did a mindfulness course. It was something I wanted to try, to cope with the stress of the diagnosis, blood transfusions and not knowing where it was all going. I needed to find the off-switch and turn my brain off so I could live in ‘day-tight compartments’.
“Mindfulness teaches you not to mind that your brain is constantly thinking and to ignore some of its constant chatter—that cocktail party going on in your head. It is basically about finding a quiet time to just sit and mellow out a bit.”
“One of my care team in London has said that she thinks attitude is really important for patients like me. And I think she’s right. It is much easier to deal with if you remain as optimistic as you can.
“There’s no profit in my worrying and thinking about how bad it might be, it won’t do me any good. And it won’t save me from any of it. So, I don’t do that. I try hard not to anyway.”
Jenny’s attitude is to approach every day as it comes: “I much prefer to deal with what’s in front of me. Tomorrow’s problems will stay in tomorrow, until they’re today’s problems.” She prefers to look forward with hope rather than with fear and expresses gratitude for the hope that clinical trials bring: “They are the only thing that can help people like me, and they give you hope.”
NP-GBL-MML-WCNT-220003 / Date of preparation: November 2022 Funded by GSK
RARE Revolution and GSK are not responsible for the content of external sites linked to within this article.