Models of Palliative Care Delivery for Individuals with Cystic Fibrosis: Cystic Fibrosis Foundation Evidence-Informed Consensus Guidelines.

Anna M. Georgiopoulos,Albert Faro,Lara Dhingra,Sarah E. Hempstead,Elisabeth P. Dellon,Dio Kavalieratos,Elliot Rabinowitz,Melissa J. Basile

doi:10.1089/jpm.2020.0311

Abstract

Cystic fibrosis (CF) affects more than 70,000 individuals and their families worldwide. Although outcomes for individuals with CF continue to improve, it remains a life-limiting condition with no cure. Individuals with CF manage extensive symptom and treatment burdens and face complex medical decisions throughout the illness course. Although palliative care has been shown to reduce suffering by alleviating illness-related burdens for people with serious illness and their families, little is known regarding the components and structure of various delivery models of palliative care needed to improve outcomes for people affected by CF. The Cystic Fibrosis Foundation (CFF) assembled an expert panel of clinicians, researchers, individuals with CF, and family caregivers, to develop consensus recommendations for models of best practices for palliative care in CF. Eleven statements were developed based on a systematic literature review and expert opinion, and address primary palliative care, specialty palliative care, and screening for palliative needs. These recommendations are intended to comprehensively address palliative care needs and improve quality of life for individuals with CF at all stages of illness and development, and their caregivers.

Highlights

Cystic fibrosis (CF) is a life-limiting genetic disorder affecting more than 70,000 individuals and their families worldwide
Palliative care has been shown to reduce suffering by alleviating illness-related burdens for people with serious illness and their families, little is known regarding the components and structure of various delivery models of palliative care needed to improve outcomes for people affected by CF
Eleven statements were developed based on a systematic literature review and expert opinion, and address primary palliative care, specialty palliative care, and screening for palliative needs

Summary

Introduction

Cystic fibrosis (CF) is a life-limiting genetic disorder affecting more than 70,000 individuals and their families worldwide. CF is progressive and fatal, therapeutic advances have dramatically enhanced life expectancy, with median predicted survival for individuals with CF in the United States increasing from the late 20s in 1986 to 44 years in 2018.1 Yet, the multifactorial burdens of the disease continue to profoundly affect the lives of individuals with CF and their families.[2] CF-related suffering encompasses physical symptoms (e.g., pain, dyspnea, fatigue), emotional distress (e.g., anxiety, depression), impaired function, social isolation, role limitations, treatment-related burden, and existential distress.[2,3]. Recommended for individuals with lung disease,[6] integration of palliative care within CF care is rare

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