Nutritional Status and Caregiver Knowledge in Pediatric Cystic Fibrosis Management

Cystic fibrosis (CF) is a rare, life-limiting genetic disorder characterized by abnormally thick mucus secretions that obstruct the respiratory, gastrointestinal, and reproductive tracts. Globally, CF affects an estimated 70,000 to 100,000 individuals. This study investigated nutritional awareness and dietary management among caregivers of pediatric CF patients. Specifically, it evaluated parental nutritional knowledge, caregiving behaviors, and patients' actual nutrient intake and diet quality relative to established clinical guidelines.

A cross-sectional study was conducted with a representative sample of 50 parents and their children with CF (aged 4-18 years) receiving specialized care at Caritas Baby Hospital in Bethlehem, Palestine. Data collection utilized a comprehensive parental questionnaire alongside a 3-day food diary completed by participants. Objective clinical status and biochemical markers were extracted directly from patients' medical records. CF-specific recommendations were used where available, and general pediatric reference values were used otherwise.

A significant disparity (p ≤ 0.05) was observed between patients' actual dietary intake and the recommended daily allowances for protein, carbohydrates, zinc, and calcium; however, intakes of sodium and iron did not differ significantly from guidelines. Higher patient adherence to dietary recommendations was significantly associated with superior lung function (measured via FEV1) and optimal serum albumin levels (p ≤ 0.05). Notably, 38% of the study sample were classified as underweight, and 76.9% of children with available 25(OH)D measurements (30/39) exhibited concentrations below 30 ng/mL. Clinical and biochemical measurements were not available for all participants. Caregiver nutritional knowledge was not significantly associated with the measured clinical or biochemical outcomes, whereas better patient dietary adherence was associated with higher FEV1% predicted and serum albumin concentrations.

The high prevalence of underweight status and micronutrient deficiencies highlights a critical need for targeted nutritional education and clinical counseling programs. Enhancing awareness among patients and parents is essential to correct dietary deficits, optimize growth, and improve long-term health outcomes.