Nutrition and cystic fibrosis

Malnutrition and growth arrest, mainly due to malabsorption, are common problems in patients affected by Cystic Fibrosis (CF), especially in childhood Therefore, adequate nutritional care and support should be part of the disease management, to improve the patient's life expectancy.

In a recent Italian study, published in the famous journal "Nutrients"  by Mariotti et al., the nutritional management of CF patients was analyzed, highlighting the relationships among growth, nutritional status, and use of CFTR (Cystic Fibrosis transmembrane conductance regulator) modulators, acting to this poorly functioning chloride channel.

The results obtained showed that nutritional support in CF should start as soon as possible after diagnosis in order to support growth in children and puberty achievement, as well as maintain a good quality of life even in adult life. Moreover, respiratory function and survival in children and adults are inversely related to the degree of malnutrition, short stature, low weight, and body mass index. Lung function in adulthood would also be associated with nutritional status in children and childhood.

The cornerstone of nutrition in CF patients is a high-calories and high-fat diet, combined with pancreatic replacement enzyme therapy to reduce malabsorption and adequate fat-soluble vitamins supplementation. In case of poor oral caloric intake, not enough to achieve the anthropometric nutritional goals, additional enteral supplementation should be quickly started to improve the patient's growth and nutritional status.

In the last decade, the CF therapeutic approaches have increased significantly: in several studies, CFTR modulators would have increased weight and body mass index, both in children and adults, as well as improved lung function and reduced acute CF flair-ups.

In conclusion, several challenges have yet to be overcome, but new drug development is also underway that could contribute to the improvement of nutritional status and lung function and, consequently, the life expectancy of patients, transforming cystic fibrosis from a deadly disease to a treatable chronic disease.

Bibliography

Ashkenazi, M.; Nathan, N.; Sarouk, I.; Bar Aluma, B.E.; Dagan, A.; Bezalel, Y.; Keler, S.; Vilozni, D.; Efrati, O. Nutritional Status in Childhood as a Prognostic Factor in Patients with Cystic Fibrosis. Lung 2019, 197, 371–376
Mariotti Zani, E.; Grandinetti, R.; Cunico, D.; Torelli, L.; Fainardi, V.; Pisi, G.; Esposito, S. Nutritional Care in Children with Cystic Fibrosis. Nutrients 2023, 15, 479
Meoli, A.; Eickmeier, O.; Pisi, G.; Fainardi, V.; Zielen, S.; Esposito, S. Impact of CFTR Modulators on the Impaired Function of Phagocytes in Cystic Fibrosis Lung Disease. Int. J. Mol. Sci. 2022, 23, 12421