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Background: Individuals with cystic fibrosis (CF) experience symptoms affecting quality of life and may benefit from palliative care (PC). Objectives: To present results of content analysis from open-ended survey questions assessing knowledge and experiences with PC among CF stakeholders. Design, Setting, Subjects: Online surveys were sent to CF stakeholders through CF-specific listservs predominantly in the United States. Measurements: Responses to five open-ended questions about CF PC—delivery, health care provider training, and lung transplant—underwent content analysis. Responses were coded using NVivo12 Software™. Results: Forty-eight CF adults, 59 caregivers, and 229 providers responded to the open-ended survey questions. Analysis showed 5 primary categories related to CF PC: (1) stakeholder perceptions of PC for CF, (2) delivering PC to people with CF, (3) conversations about PC for CF, (4) perceptions that PC services are underutilized for people with CF, and (5) beliefs that PC services are critical for people with CF considering or pursuing lung transplant. Analysis showed variation among and within groups in defining PC for CF, when, and how to deliver it. Many respondents felt PC was underutilized in CF. Most saw PC as particularly important when considering lung transplant, managing anxiety around transplant, and for goals of care discussions. Some believed PC and lung transplant were mutually exclusive. Conclusion: Respondents felt PC is underutilized for CF, and that people with CF may miss out on the benefits of PC. Among stakeholders, respondents felt people with CF would benefit from access to primary and secondary PC services. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background: Lung transplantation is a common therapeutic option for individuals with cystic fibrosis (CF) and advanced lung disease, yet many individuals with CF are not appropriately referred for evaluation. The present study sought to enhance CF transplant referral guidelines by integrating patient‐centered input to identify possible psychosocial barriers contributing to suboptimal referral for appropriate CF transplant candidates. Methods: As a component of developing the Cystic Fibrosis Foundation (CFF) Lung Transplant Referral Consensus Guidelines, we convened a focus group of lung transplant recipients with CF and two spouses of CF recipients. Each session involved standardized approaches to elicit qualitative, thematic content. Results: CF patients and caregivers characterized five areas for improvement, which were integrated into formal CFF referral guidelines. These included (a) timing of transplant discussion with CF providers, (b) accuracy of transplant‐related knowledge and expectations, (c) stigma associated with the need for transplantation, (d) treatment team transition issues, and (e) social support and mental health concerns. Earlier introduction of transplant, greater details regarding manageable aspects of treatment, and greater provision of social support were all associated with better psychosocial experiences. Conclusions: Integrating patient‐centered input into guideline development yielded important and previously unknown psychosocial barriers contributing to suboptimal transplant referral. [ABSTRACT FROM AUTHOR]

Background: Studies suggest palliative care may be different in cystic fibrosis (CF) than in other conditions. To provide quality palliative care to individuals with CF, unique needs must be understood. Objective: To examine perceptions of how palliative care may be different in CF, top palliative needs of individuals with CF, and barriers to palliative care in CF. Methods: Online surveys with closed- and open-ended questions about palliative care needs were administered to multiple stakeholders in CF care, including adults with CF, caregivers of individuals with CF, and CF care team members from U.S. centers. We used descriptive statistics to report survey findings. Results: A total of 70 adults with CF, 100 caregivers, and 350 care team members completed surveys. While care team members reported they introduce palliative care to patients a majority of the time, adults with CF and caregivers rarely recalled learning about it. Very few reported having seen a palliative care specialist. A majority of participants reported that palliative care is valuable in CF care. Over 80% of participants felt palliative care is different in CF, most often citing the unpredictable disease course. Top palliative care needs identified include emotional support, emotional symptom management, and communication about treatment decisions. Major barriers to palliative care identified include perception that it is for dying people and lack of CF care team knowledge and training in palliative care. Conclusions: Participants felt palliative care is valuable in CF and identified many palliative care needs, and also barriers that can be addressed with education and training. Our findings will be used to develop interventions targeting specific needs and inform guidelines to enhance provision of palliative care in CF. [ABSTRACT FROM AUTHOR]

Background & Aims Improved therapy has substantially increased survival of persons with cystic fibrosis (CF). But the risk of colorectal cancer (CRC) in adults with CF is 5−10 times greater compared to the general population, and 25−30 times greater in CF patients after an organ transplantation. To address this risk, the CF Foundation convened a multi-stakeholder task force to develop CRC screening recommendations. Methods The 18-member task force consisted of experts including pulmonologists, gastroenterologists, a social worker, nurse coordinator, surgeon, epidemiologist, statistician, CF adult, and a parent. The committee comprised 3 workgroups: Cancer Risk, Transplant, and Procedure and Preparation. A guidelines specialist at the CF Foundation conducted an evidence synthesis February−March 2016 based on PubMed literature searches. Task force members conducted additional independent searches. A total of 1159 articles were retrieved. After initial screening, the committee read 198 articles in full and analyzed 123 articles to develop recommendation statements. An independent decision analysis evaluating the benefits of screening relative to harms and resources required was conducted by the Department of Public Health at Erasmus Medical Center, Netherlands using the Microsimulation Screening Analysis model from the Cancer Innervation and Surveillance Modeling Network. The task force included recommendation statements in the final guideline only if they reached an 80% acceptance threshold. Results The task force makes 10 CRC screening recommendations that emphasize shared, individualized decision-making and familiarity with CF-specific gastrointestinal challenges. We recommend colonoscopy as the preferred screening method, initiation of screening at age 40 years, 5-year re-screening and 3-year surveillance intervals (unless shorter interval is indicated by individual findings), and a CF-specific intensive bowel preparation. Organ transplant recipients with CF should initiate CRC screening at age 30 years within 2 years of the transplantation because of the additional risk for colon cancer associated with immunosuppression. Conclusions These recommendations aim to help CF adults, families, primary care physicians, gastroenterologists, and CF and transplantation centers address the issue of CRC screening. They differ from guidelines developed for the general population with respect to the recommended age of screening initiation, screening method, preparation, and the interval for repeat screening and surveillance. [ABSTRACT FROM AUTHOR]

Cystic fibrosis (CF) may cause a spectrum of hepatobiliary complications, including portal hypertension, multilobular cirrhosis, and liver failure. Current guidelines on the detection and monitoring of hepatobiliary complications in CF were published in 1999. The CF Foundation assembled a committee to evaluate research advances and formulate revised guidelines for CF-associated liver disease. A committee of hepatologists, gastroenterologists, pulmonologists, pharmacists, nurses, dietitians, individuals with CF, and the parents of a child with CF devised "population, intervention, comparison, and outcome" questions regarding hepatobiliary disease in CF. PubMed literature searches were performed for each population, intervention, comparison, and outcome question. Recommendations were voted on with 80% agreement required to approve a recommendation. Public comment on initial recommendations was solicited prior to the formulation of final recommendations. Thirty-one population, intervention, comparison, and outcome questions were assembled, 6401 manuscripts were title screened for relevance, with 1053 manuscripts undergoing detailed full-text review. Seven recommendations were approved for screening, 13 for monitoring of existing disease, and 14 for treatment of CF-associated hepatobiliary involvement or advanced liver disease. One recommendation on liver biopsy did not meet the 80% threshold. One recommendation on screening ultrasound was revised and re-voted on. Through a multidisciplinary committee and public engagement, we have assembled updated recommendations and guidance on screening, monitoring, and treatment of CF-associated hepatobiliary involvement and advanced liver disease. While research gaps remain, we anticipate that these recommendations will lead to improvements in CF outcomes through earlier detection and increased evidence-based approaches to monitoring and treatment., (Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc.)

A multidisciplinary committee developed evidence-based guidelines for the management of cystic fibrosis transmembrane conductance regulator-related metabolic syndrome/cystic fibrosis screen-positive, inconclusive diagnosis (CRMS/CFSPID). A total of 24 patient, intervention, comparison, and outcome questions were generated based on surveys sent to people with CRMS/CFSPID and clinicians caring for these individuals, previous recommendations, and expert committee input. Four a priori working groups (genetic testing, monitoring, treatment, and psychosocial/communication issues) were used to provide structure to the committee. A systematic review of the evidence was conducted, and found numerous case series and cohort studies, but no randomized clinical trials. A total of 30 recommendations were graded using the US Preventive Services Task Force methodology. Recommendations that received ≥80% consensus among the entire committee were approved. The resulting recommendations were of moderate to low certainty for the majority of the statements because of the low quality of the evidence. Highlights of the recommendations include thorough evaluation with genetic sequencing, deletion/duplication analysis if <2 disease-causing variants were noted in newborn screening; repeat sweat testing until at least age 8 but limiting further laboratory testing, including microbiology, radiology, and pulmonary function testing; minimal use of medications, which when suggested, should lead to shared decision-making with families; and providing communication with emphasis on social determinants of health and shared decision-making to minimize barriers which may affect processing and understanding of this complex designation. Future research will be needed regarding medication use, antibiotic therapy, and the use of chest imaging for monitoring the development of lung disease.

Patient and family participation in guideline development is neither standardized nor uniformly accepted in the guideline development community, despite the 2011 Institute of Medicine's Guidelines We Can Trust and the Guideline International Network's GIN-Public Toolkit recommendations. The Cystic Fibrosis Foundation has included patients and/or family members directly in guideline development since 2004. Over time, various strategies for increasing patient and family member participation have been implemented. Surveys of recent patient/family and clinical guidelines committee members have shown that inclusion of individuals with cystic fibrosis and their family members on guidelines committees has provided insight otherwise invisible to clinicians., (©Sarah E Hempstead, Kelsey Fredkin, Cade Hovater, Edward T Naureckas. Originally published in Journal of Participatory Medicine (http://jopm.jmir.org), 13.07.2020.)

Cystic fibrosis (CF) clinical care guidelines exist for the care of infants up to age 2 years and for individuals ≥6 years of age. An important gap exists for preschool children between the ages of 2 and 5 years. This period marks a time of growth and development that is critical to achieve optimal nutritional status and maintain lung health. Given that disease often progresses in a clinically silent manner, objective and sensitive tools that detect and track early disease are important in this age group. Several challenges exist that may impede the delivery of care for these children, including adherence to therapies. A multidisciplinary committee was convened by the CF Foundation to develop comprehensive evidence-based and consensus recommendations for the care of preschool children, ages 2 to 5 years, with CF. This document includes recommendations in the following areas: routine surveillance for pulmonary disease, therapeutics, and nutritional and gastrointestinal care., (Copyright © 2016 by the American Academy of Pediatrics.)