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Cystic Fibrosis Nutrition: High‑Calorie, High‑Fat, Low‑Fiber Dietary Principles

Cystic fibrosis (CF) causes pancreatic insufficiency → fat/protein malabsorption and increased energy expenditure. Nutritional management is a cornerstone of survival and quality of life.

Core Dietary Pillars

Principle	Rationale	Practical Targets
High Calorie	Compensate for malabsorption + ↑ REE (110–200% predicted)	120–150% RDA for age; often 3000–5000 kcal/day adults
High Fat	Fat = 9 kcal/g; essential for fat‑soluble vitamins (A, D, E, K)	40–45% total calories; MCT oil if severe malabsorption
High Protein	Prevent catabolism; support growth, lung repair	1.5–2.0 g/kg/day
Low Fiber (selective)	Prevent distal intestinal obstruction syndrome (DIOS); manage constipation	Moderate soluble; limit insoluble if DIOS‑prone
High Salt	Excessive sweat NaCl loss	Liberal salting; salt tablets in heat/illness

Pancreatic Enzyme Replacement Therapy (PERT): Mandatory with all fat‑containing meals/snacks. Dose by fat content (lipase units).
Fat‑Soluble Vitamin Supplementation: A, D, E, K (water‑miscible forms) — levels monitored quarterly.
Hydration: 3–4 L/day; prevents inspissated secretions.

Food Examples

Calorie boosters: olive oil, butter, cream, cheese, nut butters, avocado, whole milk, ice cream.
Protein: eggs, meat, fish, Greek yogurt, protein powders.
Low‑residue carbs: white rice, pasta, potatoes, refined cereals (during DIOS risk).

Individualization

Every CF patient needs a CF‑registered dietitian for personalized prescription based on genotype, pancreatic status, lung function, growth trajectory, and comorbidities (CFRD, liver disease).

Nutritional status correlates directly with lung function (FEV₁) and survival in CF.

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