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Lung Transplantation for Cystic Fibrosis

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Cystic Fibrosis

Part of the book series: Respiratory Medicine ((RM))

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Abstract

Lung transplantation provides a treatment option for many individuals with advanced lung disease due to cystic fibrosis. Since the first transplants for CF were performed in the 1980s, survival has improved, and the opportunity for transplant has expanded to include individuals who previously were not considered candidates for transplant. Criteria to be a transplant candidate vary significantly among transplant programs, highlighting that engagement of more than one transplant program may be necessary. Individuals with highly resistant CF pathogens, malnutrition, osteoporosis, CF liver disease, and other comorbidities may be suitable candidates for lung transplant or, if needed, multi-organ transplant. The transplant process involves several phases, from discussion of prognosis and referral to a transplant center, to transplant evaluation, to listing, transplant, and care after transplant. Early discussion regarding transplant as a treatment option is critical to maximizing opportunity and optimizing patient and family experience. The decision to be evaluated for transplant and to list for transplant are distinct, and early referral and evaluation may provide a treatment option that can be urgently executed if needed based on the progression to and severity of respiratory failure. Survival after transplant for CF is improving, to a median survival approaching 10 years, and most transplant survivors enjoy markedly improved quality of life.

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Authors and Affiliations

  1. University of Pittsburgh, Pittsburgh, PA, USA

    Joseph M. Pilewski

  2. Pulmonary, Allergy, and Critical Care Medicine Division, University of Pittsburgh Medical Center, Pittsburgh, PA, USA

    Joseph M. Pilewski

  3. Cystic Fibrosis Center, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA

    Joseph M. Pilewski

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  1. Joseph M. Pilewski
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  1. The University of North Carolina at Chapel Hill, Department of Pediatrics, UNC Children’s Hospital, Chapel Hill, NC, USA

    Stephanie Duggins Davis

  2. Department of Pediatrics, University of Washington School of Medicine, Division of Pulmonary and Sleep Medicine, Seattle Children’s Hospital, Seattle, WA, USA

    Margaret Rosenfeld

  3. Department of Pediatrics, Indiana University School of Medicine, Division of Pediatric Pulmonology, Allergy and Sleep Medicine, Riley Hospital for Children at IU Health, Indianapolis, IN, USA

    James Chmiel

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Pilewski, J.M. (2020). Lung Transplantation for Cystic Fibrosis. In: Davis, S., Rosenfeld, M., Chmiel, J. (eds) Cystic Fibrosis. Respiratory Medicine. Humana, Cham. https://doi.org/10.1007/978-3-030-42382-7_12

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