Abstract
Cystic fibrosis (CF) is now more common in adults than children in countries with
well-developed health care systems. The number of adults continues to increase and
will further increase if the new cystic fibrosis transmembrane conductance regulator
(CFTR) modulators are disease modifying. Most of the complex morbidity and almost
all the mortality of CF occur in adults and will increasingly follow this pattern
even with new effective modulator therapies. Maintaining good quality of life including
social functioning and maximizing survival for adults are the key priorities. This
requires a highly knowledgeable and adaptable multidisciplinary team, which, though
focused on maintaining lung health, requires an increasing range of other disciplines
and specialties to maximize well-being. Changes in health care systems will require
current models of care to adapt to provide care for the large number of adult patients.
With increasing survival and age, many are likely to have both CF morbidities and
additional diseases of aging. New models are needed for health care delivery for this
expanding population with complex medical conditions.
Keywords
cystic fibrosis - adults - multidisciplinary team - CFTR dysfunction - comorbidities
