Addressing Gaps in Knowledge of the Optimal Approach to Managing Myelofibrosis

Myeloproliferative neoplasms, including myelofibrosis, polycythemia vera, and essential thrombocytopenia, affect approximately 13,000, 134,000, and 148,000 individuals in the United States, respectively. Due to difficulties in identifying the disease, low disease prevalence, a complicated symptom profile, differences in patient and provider perspectives regarding symptom burden and treatment goals, and a lack of effective and tolerable therapies, there is confusion over the optimal approach to treating patients with myeloproliferative neoplasms.

The knowledge gap in how to provide the best care for patients with myeloproliferative neoplasms was identified in baseline data collected from i3 Health’s nursing continuing professional development (NCPD)–approved visiting faculty meeting series titled Nursing Management of Myelofibrosis: Controlling Symptoms and Optimizing Patient Quality of Life, led by Alice Lynn, APN, and Dawn C. Urbanovsky, RN, BSN, OCN®, both of the MD Anderson Cancer Center.

The activity was available online from March 11, 2020 until March 10, 2021; 491 learners began the activity, and 451 learners saw it through to completion. The majority (95%) of learners were registered nurses, followed by nurse practitioners (2%), advanced practice nurses (2%), and individuals with other professions (1%). Participants had a wide range of practice experience, from zero to five years (19%) to 20 or more years (23%). Participants saw an average of nine patients with myelofibrosis per month. Learners were given a pretest prior to beginning the activity and a posttest consisting of the same questions following the activity’s conclusion.

The baseline assessment revealed a significant need for improvement in the knowledge of the optimal therapeutic approach for individual patients with myeloproliferative neoplasms. Only 4.7% of participants correctly identified myelofibrosis symptoms; only 27.7% correctly identified the efficacy of ruxoltinib; only 32.6% recognized the efficacy of lenalidomide; and only 43.2% selected the correct risk category for a patient. Slightly more participants, 58.7%, correctly identified the safety profile of hydroxyurea.

Significant learning took place during the activity, based on the posttest scores. Ninety-six percent of participants, an addition of 91.3%, recalled that joint pain is not a symptom of myelofibrosis. Almost 95% of participants, an addition of 66.8%, correctly identified ruxoltinib as superior to momelotinib for reducing the spleen volume and total symptom score of a patient with intermediate-2 post-essential thrombocythemia myelofibrosis and palpable splenomegaly who complains of night sweats, early satiety, and fatigue. About 94.3% of participants selected lenalidomide as the appropriate therapy for a patient with anemia associated with intermediate-1 post-polycythemia vera myelofibrosis, a 61.7% learning gain. In addition, 92.9% of participants, a learning gain of 49.7%, were able to select intermediate-2 as the correct Dynamic International Prognostic Scoring System risk category for an 81-year-old patient with primary myelofibrosis, who has a white blood cell count of 30×10 g/L, a hemoglobin level of 11 g/dL, 2% peripheral blood blasts, palpable splenomegaly, as well as complaints of fatigue, early satiety, fever, abdominal pain, and easy bruising. Finally, 91.2% of participants, a gain of 32.5%, identified hydroxyurea as the treatment least likely to further elevate liver enzymes in a patient with intermediate-2 primary myelofibrosis and elevated liver enzymes.

Learners’ performance on the pre- and post-activity assessment suggested that their competence improved regarding symptom identification and management, treatment selection, and risk stratification for patients with myelofibrosis. Therefore, i3 Health has determined that the multidisciplinary team may benefit from future NCPD–approved activities that provide further and updated education on myelofibrosis.

Upon completion of the activity, 83% of participants felt more confident in treating their patients with myelofibrosis, and 83% felt that the material presented would be used to improve the outcomes of their patients with myelofibrosis.


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