ACCCBuzz spoke with Dr. Gabriela Hobbs, MD, at Massachusetts General Hospital Cancer Center to learn how to best manage patients' care for those with myelofibrosis.
Read the first blog from this ACCC education program on treating the psychosocial and financial needs of patients with myelofibrosis.
Myelofibrosis is a rare type of chronic leukemia, defined by the development of scarring in the bone marrow that may make it difficult for normal, healthy cells to grow. People with myelofibrosis may experience symptoms, while others may not. Symptoms can include anemia, fatigue, itching, fever, shortness of breath, and frequent infections, among others. Treatment goals for patients with myelofibrosis often focus on managing symptoms and issues that develop, such as anemia and an enlarged spleen.
It is important that the cancer care team speaks with patients regarding any potential side effects from their medication. Treatment-related side effects will depend on many factors like treatment, dosage, patient age, as well as any pre-existing conditions. While most side effects are temporary and resolve upon treatment completion, providers should address patients’ concerns at every visit.
Recently, ACCCBuzz spoke with Gabriela Hobbs, MD, clinical director, Leukemia Service at Massachusetts General Hospital Cancer Center in Boston, Mass.
ACCCBuzz: Myelofibrosis is a slow developing cancer. What are some of the first symptoms patients will notice?
Dr. Hobbs: Often patients will present with no symptoms. Myelofibrosis can be diagnosed by abnormalities found in blood work from routine care by a primary care provider. However, some patients can have symptoms from an enlarged spleen, such as pain on left side of the abdomen, decreased appetite, decreased weight, or more general symptoms, such as itching anywhere, night sweats, or fevers.
ACCCBuzz: The curative treatment for myelofibrosis is an allogeneic stem cell transplant, but there are many risks with this treatment. Can you speak to this?
Dr. Hobbs: For patients with myelofibrosis, an allogeneic stem cell transplant has the potential for a cure. Unfortunately, many patients are either not eligible for this treatment or are never referred. In addition, a transplant can have serious consequences, including infections and graft versus host disease. Currently only about 10 percent to 15 percent of patients receive allogeneic stem cell transplants. Providers should be aware that outcomes have improved significantly with transplantation and, thus, should screen and refer patients who have intermediate or high-risk disease for transplant. Risk can be determined using several risk scores, including DIPSS+ [Dynamic International Prognostic Scoring System plus] and MIPSS70 [Mutation-Enhanced International Prognostic Scoring System].
Eligibility requirements have been updated to include more patients—individuals over the age of 70, who meet specific requirements. There are also improved supportive care options after treatment, which is why more patients are able to successfully undergo this procedure.
ACCCBuzz: What medications are used to treat myelofibrosis and are there side effects with using these? How are patients’ side effects managed?
Dr. Hobbs: Many medications have side effects. The most often used drugs for myelofibrosis are JAK [Janus kinase] inhibitors, each with its own side effects, such as weight gain and shingles. However, there are side effects that are common with other medications, such as low blood counts.
ACCCBuzz: How should patients discuss side effects and symptoms with their healthcare provider(s)?
Dr. Hobbs: It is important to encourage open communication with patients and to specifically ask about medication side effects. In addition, each patient will receive the MPN [myeloproliferative neoplasms] symptom assessment form to complete at each visit. This assessment allows the patient to ask questions or bring up any concerns and help track a response to their current medication. The provider also asks the patient about any concerns with their treatment. It is important for patients to understand that there are side effects attributable to their medication.
ACCCBuzz: How do you assess for nutritional needs among patients?
Dr. Hobbs: When treating patients with any MPNs and, specifically, myelofibrosis, providers and patients need to be concerned with blood clots. We know that chronic health conditions (e.g., cardiovascular, diabetes, obesity, and smoking) increases the risk for blood clots andlifestyle modification can help with this risk. That is why we work with patients to diet and exercise to enable a healthy weight and active lifestyle to better prevent complications.
However, there are also patients with myelofibrosis that have lost a lot of weight due to their disease and an enlarged spleen, and we tackle this by giving them medications to shrink their spleen and allow them to eat more freely. In addition, we recommend frequent, small meals with calorie dense foods for those that continue to have an enlarged spleen.
ACCCBuzz: Is there anything else you would like to add?
Dr. Hobbs: Yes, I wanted to note that patients with myelofibrosis have unique needs. Symptoms are an important part of life for patients with myelofibrosis. It is important for providers to review blood counts with patients, but there also needs to be a continuous discussion about medication, side effects, and nutritional needs. All are important in having better health outcomes for patients.
For more, listen to the latest CANCER BUZZ episode, where Dr. Hobbs discusses symptom scoring, risk stratification, and effective practices in building a strong myelofibrosis program.
The ACCC Advancements in Treating Patients with Myelofibrosis and Strategies to Improve Care education program is supported by Abbvie and GSK.
