Abstract
Cystic fibrosis (CF) is the indication for transplantation in approximately 15% of recipients worldwide, and Cystic Fibrosis Lung Transplant Recipients (CFLTRs) have excellent long-term outcomes. Yet, CFLTRs have unique comorbidities that require specialized care. The objective of this document is to provide recommendations to CF and lung transplant clinicians for the management of perioperative and underlying comorbidities of CFLTRs and the impact of transplantation on these comorbidities. The Cystic Fibrosis Foundation (CFF) organized a multidisciplinary committee to develop CF Lung Transplant Clinical Care Recommendations. Three workgroups were formed to develop focused questions. Following a literature search, consensus recommendations were developed by the committee members based on literature review, committee experience and iterative revisions, and in response to public comment. The committee formulated 32 recommendation statements in the topics related to infectious disease, endocrine, gastroenterology, pharmacology, mental health and family planning. Broadly, the committee recommends close coordination of care between the lung transplant team, the cystic fibrosis care center, and specialists in other disciplines with experience in the care of CF and lung transplant recipients. These consensus statements will help lung transplant providers care for CFLTRs in order to improve post-transplant outcomes in this population.
Highlights
The Cystic Fibrosis Foundation (CFF) invited a multidisciplinary team of adult and pediatric transplant pulmonologists, infectious diseases, gastroenterology, endocrinology physicians, transplant coordinator, dietitian, pharmacist, psychologist, 2 adult Cystic Fibrosis Lung Transplant Recipients (CFLTRs), and a caregiver to participate in the development of these consensus statements
The Cystic fibrosis (CF) Foundation recommends that CF Lung Transplant Recipients have mental health screening and consultation for depression, anxiety, and post-traumatic stress disorder (PTSD) within 6 months of transplant, resume annual screening per the International Committee on Mental Health (ICMH)
Despite improvement in the overall outcomes of individuals with CF, lung transplantation remains an important therapy in the spectrum of advanced CF lung disease
Summary
The CFF invited a multidisciplinary team of adult and pediatric transplant pulmonologists, infectious diseases, gastroenterology, endocrinology physicians, transplant coordinator, dietitian, pharmacist, psychologist, 2 adult CFLTRs, and a caregiver to participate in the development of these consensus statements. 1) The CFF recommends that CFLTRs follow up with a multidisciplinary CF care team within 6-12 months of transplant to resume extra-pulmonary CF care. Communication between the transplant and CF care teams is essential for coordination of care. No studies have examined the impact of multidisciplinary CF care after lung transplantation, extra-pulmonary manifestations of CF persist after transplant and require expertise in CF care.[5,6,7,8] Individuals with CF should resume CF care at either their referring or transplant institution’s CF care center and continue follow-up at minimum every 12 months, or more frequently depending on the individual’s clinical course. Close communication between the individual with CF, lung transplant and the CF care team is essential to ensure coordination of care.[6,8]
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