Aaron Gerds, MD, MS

Patients with myelofibrosis should reflect on their personal priorities and quality of life goals, understand the range of available JAK inhibitor treatment options, and openly communicate with their doctor to arrive at a shared decision for managing their disease.

For most JAK inhibitors dosing starts at the maximum safe dose, while ruxolitinib often requires lower initial doses and dose reductions over the first 12 weeks when cytopenias typically worsen before partially improving, warranting consideration of earlier switching to alternative JAK inhibitors instead of pushing through transfusions.

Reasons to switch JAK inhibitors include worsening blood counts and insufficient symptom or spleen response at maximum doses, with the goal of avoiding gaps in treatment by stopping one JAK inhibitor when ready to start the next to prevent symptom rebound while still allowing for potential increased efficacy from an alternative mechanism of action.

Response is assessed in patients starting a JAK inhibitor by monitoring symptoms on a severity scale, spleen size, blood counts, and overall patient impression of change, with the goal being reduction of symptoms and stabilization of blood counts to improve quality of life.

The JAK inhibitors approved for myelofibrosis have common side effects of cytopenias and gastrointestinal issues, with additional risks of weight gain and cardiac effects and diarrhea; momelotinib and pacritinib show improved tolerability compared to earlier JAK inhibitors.

An expert discusses the four FDA-approved JAK inhibitors for myelofibrosis - ruxolitinib, fedratinib, pacritinib, and momelotinib.

Christine Cooper, RN, BSN, explains anemia in myelofibrosis is managed by stimulating red blood cell production with medications, transfusions, and newer therapies that may stabilize red cell counts.

Key patient goals for managing myelofibrosis include improving quality of life by addressing low blood counts and constitutional symptoms, considering disease-modifying therapies and transplant to maximize longevity based on predicted survival, and incorporating personalized priorities through shared decision-making with their doctor when choosing treatments.

Patients should be directed to resources like the NCCN guidelines and ClinicalTrials.gov and provided with thorough discussions covering frequency of visits, required testing, medication administration, side effects to enable fully informed decisions.

To determine risk level in myelofibrosis, prognostic models are used which assign points to various clinical and pathological factors to calculate a risk score that categorizes patients as low, intermediate, or high risk; additional information like mutations and chromosomes can further refine risk stratification.

Patients with myelofibrosis commonly present with fatigue, weight loss, night sweats, bone pain, low blood counts, and concentration issues, with diagnosis requiring bone marrow biopsy.