Effects of nutritional status and dietetic interventions on survival in Cystic Fibrosis patients before and after lung transplantation

BackgroundThis study retrospectively investigated nutritional status, dietetic intervention and intake in Cystic Fibrosis (CF) patients before and after lung transplantation (LTX).MethodsBody Mass Index (BMI), Fat Free Mass Index (FFMI) and nutritional intake were retrieved from 75 out-patients aged 15-53 years. Patients were seen every 3-4 months during the waiting list time (range 0-81 months) and up to 116 months after LTX. Survival was measured in months.ResultsThe median BMI at baseline was 19.2 kg/m2 (range: 15.3 to 28.4 kg/m2) with 29 patients (39%) below ≤ 18.5 kg/m2. FFMI (measured in 65 patients) had a median of 15.2 kg/m2 (range: 11.1 to 22.4 kg/m2) with 39 patients (60%) ≤ 16.7 kg/m2 (men) or ≤ 14.6 kg/m2 (women). Median energy intake was 2800 kcal, 239 kcal higher than the estimated energy requirement. However, 8 patients consumed ≥ 500 kcal less than recommended. Protein intake was 104 (range 60-187) g or 1.9 g/kg per day. Despite dietetic intervention with oral nutritional supplements (ONS) (36 patients), tube feeding (12 patients), or both (13 patients), BMI and FFMI hardly improved pre-LTX. LTX was performed in 51 patients (68%); 10 patients died during follow-up, median survival time was 41 months. A BMI ≤ 18.5 kg/m2 was more prevalent in patients who died before LTX (6/9) or who died after LTX (4/10) than in patients who were still alive on the waiting list (5/15) or who survived LTX (14/41). Results for FFMI were comparable. From 6-12 months post-LTX, BMI and FFMI markedly improved, especially in underweight patients.ConclusionA BMI ≤ 18.5 kg/m2 and an FFMI ≤ 16.7 kg/m2 (men) or ≤ 14.6 kg/m2 (women) appears to impair survival in LTX candidates with CF. Patients maintained a low body weight before LTX. After LTX weight gain is achieved.