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

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.

Keywords: clinical guidelines; cystic fibrosis; healthcare delivery; palliative care; patient-reported outcomes.

FIG. 1.

Integrating primary and specialty palliative care to address palliative needs throughout the CF experience. Palliative care is delivered continuously throughout the illness experience, from diagnosis through bereavement. The intensity of palliative care should match the patient and family's palliative needs, including but not limited to: assessment and management of physical and emotional symptoms; existential and spiritual suffering; provision of information regarding prognosis and treatment options, elicitation of goals of care, and enactment of advance care planning; and support during transplant evaluation and post-transplantation. Palliative care is ideally a partnership, whereby CF care team members provide continuous first-line monitoring and support for palliative needs (“primary palliative care”), while palliative care specialists support the CF care team with expert management of complex or severe concerns. Palliative care needs are illustrated as the variable white band throughout the center of the figure; the width and opacity of the band indicate the fluctuating severity of palliative needs. Importantly, this figure is a crude depiction of palliative needs, which will vary by patient/family; as such, CF care teams should continuously monitor suffering and needs to customize palliative care for each patient. Furthermore, this illustration does not specifically depict certain milestones, such as the transplantation process, which may also affect palliative needs. Color image is available online.

FIG. 2.

Palliative care needs assessment strategy for people with CF and their caregivers. Color image is available online.