Nutritional management of people living with cystic fibrosis throughout life and disease continuum: Changing times, new challenges

Cystic fibrosis (CF) is a genetic disease caused by mutations in the gene encoding for the ion channel cystic fibrosis transmembrane conductance regulator (CFTR). The management of CF disease has evolved in recent decades from treating downstream disease manifestations affecting the airways, the lun...

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Bibliographic Details
Published inJournal of human nutrition and dietetics Vol. 36; no. 5; pp. 1675 - 1691
Main Authors Mailhot, Geneviève, Denis, Marie-Hélène, Beauchamp-Parent, Caroline, Jomphe, Valérie
Format Journal Article
LanguageEnglish
Published England Blackwell Publishing Ltd 01.10.2023
Subjects
Body weight
Conductance
Cystic fibrosis
Cystic fibrosis transmembrane conductance regulator
Demographics
Demography
Gastrointestinal system
Genetic disorders
Genotypes
Ion channels
Malnutrition
Medical treatment
Metabolism
Modulators
Nutrient deficiency
Nutrition
Prognosis
Undernutrition
Underweight
nutrition
life stages
CFTR modulators
cystic fibrosis
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Summary:Cystic fibrosis (CF) is a genetic disease caused by mutations in the gene encoding for the ion channel cystic fibrosis transmembrane conductance regulator (CFTR). The management of CF disease has evolved in recent decades from treating downstream disease manifestations affecting the airways, the lungs and the gastrointestinal system to addressing the CFTR gene defect. The advent of CFTR modulators, which correct the functionality of the defective CFTR, contributes to reshaping the landscape of CF demographics, prognosis and therapies, including nutritional management. A spectrum of clinical manifestations is emerging within the same patient population where undernutrition and nutritional deficiencies coexist with excessive weight gain and metabolic derangements. Such contrasting presentations challenge current practices, require adjustments to traditional approaches, and involve more individualised interventions. This narrative review examines the current state of knowledge on the nutritional management of people living with cystic fibrosis from early life to adulthood in the era of CFTR modulation.
Bibliography:ObjectType-Article-2
SourceType-Scholarly Journals-1
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ObjectType-Review-1
ISSN:0952-3871
1365-277X
DOI:10.1111/jhn.13214