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Practice Guideline
. 2021 Jan;24(1):18-30.
doi: 10.1089/jpm.2020.0311. Epub 2020 Sep 16.

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

Dio Kavalieratos  1 , Anna M Georgiopoulos  2 , Lara Dhingra  3   4 , Melissa J Basile  5 , Elliot Rabinowitz  6 , Sarah E Hempstead  7 , Albert Faro  7 , Elisabeth P Dellon  8
Affiliations
Practice Guideline

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

Dio Kavalieratos et al. J Palliat Med. 2021 Jan.

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.

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Conflict of interest statement

Dr. Dellon reports grants from Cystic Fibrosis Foundation, during the conduct of the study; Dr. Georgiopoulos reports nonfinancial support from Cystic Fibrosis Foundation, during the conduct of the study; grants, personal fees, and nonfinancial support from Cystic Fibrosis Foundation; and grants and personal fees from Vertex Pharmaceuticals, Inc., outside the submitted work. Dr. Goss reports grants from Cystic Fibrosis Foundation, grants from European Commission, grants from NIH (NHLBI), grants from NIH (NIDDK and NCRR), during the conduct of the study; personal fees from Gilead Sciences, personal fees from Novartis, grants from NIH, grants from FDA, other from Boehringer Ingelheim, and personal fees and nonfinancial support from Vertex, outside the submitted work. Mr. Hovater reports having received honoraria for other work with the Cystic Fibrosis Foundation unrelated to this article, outside the submitted work; Dr. McKinzie reports personal fees from Vertex Pharmaceuticals, Inc., outside the submitted work; Dr. Rabinowitz reports grants from Cystic Fibrosis Foundation First and Second Year Clinical Fellowship Training Grant Institutional Training Grant, RABI NO 1680, outside the submitted work. None of the work presented in this opinion piece was influenced by the funding sources noted above. The funding sources that support other ongoing research played no role in writing this article, or in the decision to submit for publication.

Figures

FIG. 1.
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.
FIG. 2.
Palliative care needs assessment strategy for people with CF and their caregivers. Color image is available online.
See this image and copyright information in PMC

References

    1. Cystic Fibrosis Foundation: Cystic Fibrosis Foundation Patient Registry 2018 Annual Data Report. Bethesda, MD, 2019
    1. Marmor M, Jonas A, Mirza A, et al. : Opportunities to improve utilization of palliative care among adults with cystic fibrosis: A systematic review. J Pain Symptom Manage 2019;58:1100–1112 e1101. - PubMed
    1. Trandel ET, Pilewski JM, Dellon EP, et al. : Prevalence of unmet palliative care needs in adults with cystic fibrosis. J Cyst Fibros 2020;19:394–401 - PMC - PubMed
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    1. Quill TE, Abernethy AP: Generalist plus specialist palliative care—Creating a more sustainable model. N Engl J Med 2013;368:1173–1175 - PubMed

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