Individualized high-quality carbohydrate diets, supplemented by pancreatic enzyme replacement therapy and enteric tube feeding if needed, can significantly improve the nutritional status of patients with cystic fibrosis (CF), according to findings published in Nutrition.

The researchers conducted a systematic review of the literature from June 1, 2016, to March 14, 2021, to determine the most effective nutritional treatments for patients with CF, identifying 18 interventional and 22 observational studies. Outcomes focused on anthropometric measurements which indicated improvements in nutritional status.

The studies analyzed dietary management plans and frameworks, calorie and macronutrient intake and diet, supplementation (especially with probiotics and vitamins), pancreatic enzyme replacement therapy, and enteral tube feeding. Two studies investigated the efficacy of unique interventions—nebulized gene therapy and extracorporeal digestion of enteral feedings.

The literature supported pancreatic enzyme replacement therapy, individualized high-quality carbohydrate diets, and enteral tube feeding as effective nutritional interventions. Pancreatic enzyme replacement therapy enabled patients with CF to digest and absorb nutrients despite pancreatic insufficiency. High-fat, high-calorie diets focused on low-glycemic index carbohydrates significantly enhanced weight gains in pediatric patients with CF, reflecting improved nutrient absorption and status.

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Enteral tube feeding provided adequate nutrients to chronically malnourished patients when less invasive treatments failed. An immobilized lipase cartridge that enabled extracorporeal digestion of enteral feedings significantly increased anthropometric measurements, primarily in pediatric patients with CF after 1 year of treatment.

“Currently available guidelines offer direction in establishing appropriate nutritional care for CF patients, and implementation of guidelines is associated with long-term improvements in nutritional status of CF patients,” the authors stated. “However, nutritional care and its goals should be individualized and regularly reviewed, to ensure they continue to optimally fit the patient’s needs.”

The literature did not provide evidence that nebulized gene therapy or supplementation with probiotics, vitamin D, lipids, enriched multivitamins, glutathione, or docosahexaenoic acid significantly impacted the nutritional status of patients with CF.


Mielus M, Sands D, Woynarowski M. Improving nutrition in cystic fibrosis – a systematic literature review. Nutrition. Published online May 6, 2022. doi:10.1016/j.nut.2022.111725