What are the primary considerations for managing a patient with a myeloproliferative disorder (MPD) in hospice care?

Managing Myeloproliferative Disorders in Hospice Care

For patients with myeloproliferative disorders (MPD) transitioning to hospice, prioritize comprehensive symptom management focusing on quality of life domains (physical, functional, emotional, spiritual, social), blood product support for symptomatic cytopenias, and aggressive management of constitutional symptoms while discontinuing disease-modifying therapies. 1

Primary Goals of Hospice Care in MPD

The fundamental shift in hospice is from disease modification to symptom-oriented palliation and quality of life optimization. 1 When prolongation of survival or cure is no longer possible, management centers on:

• Relief of physical suffering through targeted symptom control 1
• Psychosocial and spiritual support addressing all quality-of-life domains 1
• Prevention and management of complications including bleeding and thrombosis 2
• Coordination of care with interdisciplinary hospice teams 3

Critical Symptom Management Priorities

Constitutional Symptoms

Profound constitutional symptoms are a hallmark of advanced MPD and require aggressive management. 1 These include:

• Fatigue, weight loss, and cachexia - the most debilitating symptoms requiring multimodal support 1
• Night sweats and low-grade fever - may respond to low-dose corticosteroids 1
• Pruritus - particularly common in polycythemia vera, managed with antihistamines and cooling measures 1
• Bone and joint pain - requires systematic pain management using WHO Pain Ladder approach 4

Hematologic Support

Continue supportive transfusion therapy for symptomatic relief, not target hemoglobin levels. 1

• RBC transfusions for symptomatic anemia (generally leukocyte-reduced) when causing functional impairment 1
• Platelet transfusions for severe thrombocytopenia or active bleeding 1
• Irradiated blood products for all transfusions in hospice patients 1
• CMV-negative products when possible for CMV-negative recipients 1

Splenomegaly-Related Symptoms

Marked hepatosplenomegaly causes severe abdominal discomfort, early satiety, and compromised mobility. 1

• Pain management with opioids titrated to effect for splenic pain and infarcts 1, 4
• Small, frequent meals to address early satiety 1
• Consideration of palliative hydroxyurea only if life expectancy exceeds several weeks and symptoms are refractory 1, 5
• Avoid splenectomy or radiation in hospice patients due to poor risk-benefit ratio 1

Bleeding and Thrombosis Management

Bleeding Complications

Bleeding is frequent in advanced MPD, particularly with myelofibrosis (31.8 events per 100 patient-years). 6

• Discontinue aspirin immediately if active bleeding occurs 2
• Platelet transfusions for thrombocytopenic bleeding 1
• Aminocaproic acid or antifibrinolytic agents for refractory bleeding or profound thrombocytopenia 1
• Local measures for skin and mucosal bleeding (90% of episodes) 6

Thrombosis Risk

Fatal thrombosis is the leading cause of death in MPD (5 of 24 deaths in one cohort), more lethal than bleeding. 6

• Continue low-dose aspirin (81-100 mg daily) unless active bleeding or extreme thrombocytosis >1,500 × 10⁹/L 1, 2
• Therapeutic anticoagulation for active thrombosis using appropriate agents based on bleeding risk 2
• Discontinue cytoreductive therapy (hydroxyurea) in hospice unless needed for symptom control 1, 5

Infection Management in Neutropenic Patients

Neutropenia with recurrent infections requires judicious antibiotic use balanced against goals of care. 1, 7

• Obtain cultures before antibiotics for febrile neutropenia 7
• Broad-spectrum antibiotics for symptomatic infections causing distress 7
• Avoid prophylactic G-CSF or GM-CSF in hospice setting 1, 7
• Discontinue prophylactic antimicrobials to prevent resistance and medication burden 7

Medication Management

Discontinue Disease-Modifying Therapies

Stop all cytoreductive and disease-modifying agents when transitioning to hospice. 1, 5

• Hydroxyurea - discontinue due to myelosuppression risk and lack of symptom benefit 5
• Interferon therapy - stop due to poor tolerability and no palliative benefit 1
• JAK inhibitors - discontinue as they target disease modification, not symptom relief 1
• Erythropoiesis-stimulating agents - stop as transfusions provide more immediate relief 1

Continue Symptom-Directed Medications

• Opioids for pain management, titrated systematically 4
• Antiemetics for nausea from disease or medications 4
• Corticosteroids (prednisone 15-30 mg daily) for constitutional symptoms and appetite 1
• Antihistamines for pruritus 1

Psychosocial and Spiritual Care

Address all quality-of-life domains beyond physical symptoms. 1

• Psychological support for anxiety and depression, which are common 8
• Spiritual care addressing existential concerns and meaning-making 1, 3
• Family support and education about disease trajectory and what to expect 3
• Grief and bereavement services for patient and family 3

Communication and Care Coordination

Early and ongoing discussions about prognosis and goals of care are essential. 1, 3

• Clarify code status and document DNR/DNI preferences 3
• Discuss hospice philosophy emphasizing comfort over life prolongation 3
• Coordinate with interdisciplinary hospice team including nurses, social workers, chaplains 3
• Regular reassessment of symptom burden and treatment effectiveness 1

Common Pitfalls to Avoid

• Don't continue cytoreductive therapy (hydroxyurea, interferon) in hospice unless specifically for symptom control of extreme leukocytosis or thrombocytosis 1, 5
• Don't transfuse to target hemoglobin levels - transfuse only for symptomatic relief 1
• Don't use G-CSF prophylactically in hospice patients with neutropenia 1, 7
• Don't pursue aggressive interventions like splenectomy or radiation for splenomegaly 1
• Don't delay opioid initiation for pain - use WHO Pain Ladder systematically 4
• Don't overlook non-physical suffering - address psychological, social, and spiritual dimensions 1, 3

Prognosis Communication

Median survival varies significantly by MPD subtype: ET approaches 20+ years, PV 10-20 years, and primary myelofibrosis 5-10 years, but hospice patients represent end-stage disease. 1, 6 In hospice-appropriate patients, prognosis is typically weeks to months, with death most commonly from thrombosis, transformation to acute leukemia (20% risk over 10 years), or complications of cytopenias. 1, 6, 9