Addressing pain in people living with cystic fibrosis: Cystic fibrosis foundation evidence-informed guidelines

E P Dellon¹, G Allada², S J Allgood³, A M Georgiopoulos⁴, J L Goggin⁵, D Hadjiliadis⁶, J D Lowman⁷, S Madge⁸, B Middour-Oxler⁹, C Muirhead¹⁰, M Noel¹¹, P Wilson¹², S E Hempstead¹³, A Faro¹³, D Kavalieratos¹⁴; Committee on Pain in CF.

Collaborators, Affiliations

Collaborators

Committee on Pain in CF.:
Steven D Freedman¹⁵, Elpis Hatziagorou¹⁶, Cade Hovater¹⁷, Joann B Hunsberger¹⁸, Sigrid Ladores¹⁹, Emily Lawson²⁰, Marcin Madej²¹, Jessica Millard¹⁷, Kasey Raffensperger¹⁷, Erin Tallarico²²

Affiliations

¹ Department of Pediatrics, Division of Pulmonology, University of North Carolina School of Medicine, Chapel Hill, NC, USA.
² Department of Medicine, Division of Pulmonary and Critical Care Medicine, Oregon Health and Science University, Portland, Oregon, USA.
³ Johns Hopkins University School of Nursing, Baltimore, MD, USA.
⁴ Department of Psychiatry, Massachusetts General Hospital, Boston, MA, USA.
⁵ Pulmonary Service Line, UC San Diego Health, San Diego, CA, USA.
⁶ Department of Medicine, Division of Pulmonary, Allergy, and Critical Care Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.
⁷ Department of Physical Therapy and Adult Cystic Fibrosis Center, University of Alabama at Birmingham, Birmingham, AL, USA.
⁸ Adult Cystic Fibrosis Centre, Royal Brompton Hospital, London, UK.
⁹ Department of Pediatrics, Emory University, and Children's Healthcare of Atlanta and Emory University Cystic Fibrosis Care Center, Atlanta, GA, USA.
¹⁰ Pediatric Cystic Fibrosis Center, Doernbecher Children's Hospital, Portland, Oregon, USA.
¹¹ Department of Psychology, University of Calgary, and Alberta Children's Hospital Research Institute, Calgary, AB, Canada.
¹² Pharmacy Department, Nottingham University Hospitals NHS Trust, Nottingham, UK.
¹³ Cystic Fibrosis Foundation, Bethesda, MD USA.
¹⁴ Department of Family and Preventive Medicine, Division of Palliative Medicine, Emory University, Atlanta, GA, USA.
¹⁵ Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, USA.
¹⁶ Paediatric Pulmonology and Cystic Fibrosis Unit, Hippokration Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece.
¹⁷ Community Representative to the CF Foundation.
¹⁸ Department of Anesthesiology and Critical Care Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
¹⁹ University of Nevada Las Vegas School of Nursing, Las Vegas, NV, USA.
²⁰ RAND Corporation, Santa Monica, CA, USA.
²¹ Department of Rehabilitation Medicine, New York-Presbyterian Hospital, New York, NY, USA.
²² Cystic Fibrosis Foundation, Bethesda, MD, USA.

PMID: 39645476
DOI: 10.1016/j.jcf.2024.11.012

Free article

Review

Addressing pain in people living with cystic fibrosis: Cystic fibrosis foundation evidence-informed guidelines

E P Dellon et al. J Cyst Fibros. 2025 Mar.

Free article

. 2025 Mar;24(2):224-235.

doi: 10.1016/j.jcf.2024.11.012. Epub 2024 Dec 6.

Authors

Collaborators

Committee on Pain in CF.:
Steven D Freedman¹⁵, Elpis Hatziagorou¹⁶, Cade Hovater¹⁷, Joann B Hunsberger¹⁸, Sigrid Ladores¹⁹, Emily Lawson²⁰, Marcin Madej²¹, Jessica Millard¹⁷, Kasey Raffensperger¹⁷, Erin Tallarico²²

Affiliations

¹ Department of Pediatrics, Division of Pulmonology, University of North Carolina School of Medicine, Chapel Hill, NC, USA.
² Department of Medicine, Division of Pulmonary and Critical Care Medicine, Oregon Health and Science University, Portland, Oregon, USA.
³ Johns Hopkins University School of Nursing, Baltimore, MD, USA.
⁴ Department of Psychiatry, Massachusetts General Hospital, Boston, MA, USA.
⁵ Pulmonary Service Line, UC San Diego Health, San Diego, CA, USA.
⁶ Department of Medicine, Division of Pulmonary, Allergy, and Critical Care Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.
⁷ Department of Physical Therapy and Adult Cystic Fibrosis Center, University of Alabama at Birmingham, Birmingham, AL, USA.
⁸ Adult Cystic Fibrosis Centre, Royal Brompton Hospital, London, UK.
⁹ Department of Pediatrics, Emory University, and Children's Healthcare of Atlanta and Emory University Cystic Fibrosis Care Center, Atlanta, GA, USA.
¹⁰ Pediatric Cystic Fibrosis Center, Doernbecher Children's Hospital, Portland, Oregon, USA.
¹¹ Department of Psychology, University of Calgary, and Alberta Children's Hospital Research Institute, Calgary, AB, Canada.
¹² Pharmacy Department, Nottingham University Hospitals NHS Trust, Nottingham, UK.
¹³ Cystic Fibrosis Foundation, Bethesda, MD USA.
¹⁴ Department of Family and Preventive Medicine, Division of Palliative Medicine, Emory University, Atlanta, GA, USA.
¹⁵ Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, USA.
¹⁶ Paediatric Pulmonology and Cystic Fibrosis Unit, Hippokration Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece.
¹⁷ Community Representative to the CF Foundation.
¹⁸ Department of Anesthesiology and Critical Care Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
¹⁹ University of Nevada Las Vegas School of Nursing, Las Vegas, NV, USA.
²⁰ RAND Corporation, Santa Monica, CA, USA.
²¹ Department of Rehabilitation Medicine, New York-Presbyterian Hospital, New York, NY, USA.
²² Cystic Fibrosis Foundation, Bethesda, MD, USA.

PMID: 39645476
DOI: 10.1016/j.jcf.2024.11.012

Abstract

Even as many outcomes for people living with cystic fibrosis (PLwCF) improve, individuals still experience extensive symptom burdens. From birth, many PLwCF experience both pain as a symptom of their CF disease and procedural pain, posing detriments to health, functioning, and quality of life. Despite its prevalence and impact, there is no CF-specific guidance for the assessment and management of pain. Similarly, no guidance exists regarding communication with PLwCF about their pain experiences or its impact on their lives. Therefore, the Cystic Fibrosis Foundation (CFF) assembled an expert panel of clinicians, researchers, PLwCF, and caregivers to develop consensus recommendations for pain management in CF. We utilized literature review and expert opinion to develop 13 recommendations addressing pain assessment, management, and communication. Recommendations are centered on guiding principles of utilizing a multimodal approach to pain management, offering age and developmentally appropriate assessment and interventions, concurrently treating underlying conditions causing, contributing to, and/or exacerbated by pain, considering societal stigma of the pain experience, particularly for minoritized and marginalized people, and sensitivity to issues of access and cost. These recommendations are intended to guide clinicians in managing pain and improving quality of life for PLwCF with pain at all stages of illness and development.

Keywords: Cystic fibrosis; Lung transplant; Pain; Palliative care.

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Conflict of interest statement

Declaration of competing interest Elisabeth Dellon: Cystic Fibrosis Foundation and National Heart, Lung, and Blood Institute (research support) Gopal Allada: No disclosures Sarah Allgood: No disclosures Anna Georgiopoulos: Belgian Cystic Fibrosis Foundation/King Baudouin Foundation (personal fees), Cystic Fibrosis Foundation (research support, personal fees, travel reimbursement), Italian Cystic Fibrosis Research Roundation (personal fees), National Heart, Lung, and Blood Institute (research support), Vertex Pharmaceuticals (research support and personal fees), University of Kansas Medical Center (personal fees), Virginia Commonwealth University (personal fees). Jessica Goggin: No disclosures Denis Hadjiliadis: Cystic Fibrosis Foundation (research support, honoraria for committee memberships, travel reimbursement), Iridium (honorarium for CME program development) John Lowman: No disclosures Susan Madge: No disclosures Brandi Middour-Oxler: Cystic Fibrosis Foundation (research support, consultant fees), Vertex Pharmaceuticals (honoraria) Corinne Muirhead: No disclosures Melanie Noel: No disclosures Patrick Wilson: No disclosures Sarah Hempstead: Cystic Fibrosis Foundation employee Albert Faro: No personal disclosures, however, to advance drug development and a search for a cure, his employer the Cystic Fibrosis Foundation (CFF) has contracts with several companies to help fund the development of potential treatments and/or cures for cystic fibrosis. Pursuant to these contracts, CFF may receive milestone-based payments, equity interests, royalties on the net sales of therapies, and/or other forms of consideration. Resulting revenue received by CFF is used in support of its mission. Additionally, CFF may license CFF Patient Registry data to some companies to monitor drug safety as part of the U.S. Food and Drug Administration's required Phase 4 clinical trials process and to encourage research aimed at improving the care of people with CF, while maintaining our obligation and commitment to protect the privacy of Registry participants. In connection with these licenses, and upon request, CFF may also assist company researchers in interpreting CFF Patient Registry data to aid in designing, analyzing, and interpreting real world studies in CF. Dio Kavalieratos: Cystic Fibrosis Foundation and National Heart, Lung, and Blood Institute (research support). Additionally, Dr. Kavalieratos serves on the Board of Directors of the American Academy of Hospice and Palliative Medicine. The views presented herein were done so independently and do not necessarily reflect those of the American Academy of Hospice and Palliative Medicine or its members.

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Addressing pain in people living with cystic fibrosis: Cystic fibrosis foundation evidence-informed guidelines

Collaborators

Affiliations

Addressing pain in people living with cystic fibrosis: Cystic fibrosis foundation evidence-informed guidelines

Authors

Collaborators

Affiliations

Abstract

Conflict of interest statement

Publication types

MeSH terms

LinkOut - more resources

Full Text Sources

Medical