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Advances in Myelofibrosis Treatment and Enhancing Patient Quality of Life

Diagnosing Myelofibrosis

Christine Cooper, RN, BSN, and Aaron Gerds, MD, MS,discuss optimizing outcomes and quality-of-life in patients with myelofibrosis, the current treatment options and promising advances, and side-effect management.

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.

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.

Assessing Risk Level of Patients With Myelofibrosis

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.

Educational Resources for Patients Diagnosed With Myelofibrosis

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.

Key Patient Goals for Myelofibrosis Management

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.

Management of Anemia in Patients With Myelofibrosis

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

Clinical Data on the Approved JAK Inhibitors for Treatment of Myelofibrosis

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.

Adverse Events Seen With JAK Inhibitors for Myelofibrosis

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.

Assessing Treatment Response in Patients With Myelofibrosis

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.

Best Approaches to Switching Therapies for a Patient With Myelofibrosis

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.

Dosing JAK Inhibitors in Patients With Myelofibrosis

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.

Key Takeaways for Patients With Myelofibrosis

CURE Expert Connections® is a video series designed to help patients and their loved ones along their cancer care journey by offering information and expert advice from leaders in oncology care. Expert medical professionals share their knowledge and insights on an array of topics to allow for a better understanding of what to expect following a cancer diagnosis. Expert Connections hopes to bridge the gap between patients and oncology professionals to ensure no question is left unanswered.

Diagnosing Myelofibrosis

EP: 1.Diagnosing Myelofibrosis

EP: 2.Assessing Risk Level of Patients With Myelofibrosis

EP: 3.Educational Resources for Patients Diagnosed With Myelofibrosis

EP: 4.Key Patient Goals for Myelofibrosis Management

EP: 5.Management of Anemia in Patients With Myelofibrosis

EP: 6.Clinical Data on the Approved JAK Inhibitors for Treatment of Myelofibrosis

EP: 7.Adverse Events Seen With JAK Inhibitors for Myelofibrosis

EP: 8.Assessing Treatment Response in Patients With Myelofibrosis

EP: 9.Best Approaches to Switching Therapies for a Patient With Myelofibrosis

EP: 10.Dosing JAK Inhibitors in Patients With Myelofibrosis

EP: 11.Key Takeaways for Patients With Myelofibrosis