Nutritional challenges in adult patients with cystic fibrosis pre and post lung transplantation

This thesis aims to increase the knowledge on measuring body composition, caloric demands, and effectiveness of nutritional interventions in people with cystic fibrosis (CF). This contributes to optimal nutritional care for improvement of survival and quality of life at various stages and complications of CF throughout life.

Data were derived from annual check-ups of patients at the outpatient clinic and from outpatient measurements performed in patients with end-stage lung disease before and after lung transplantation.

We measured body composition in fasting and non-fasting state with bioelectric impedance analysis and found no clinically relevant differences in lean body mass and fat mass, and their associations with lung function remained the same. In addition, another study showed that resting energy expenditure estimated by prediction formulas was underestimated in 75% of patients with CF before lung transplantation. This underestimation was only 33% in another group of patients with CF after lung transplantation. Up to 1 year after lung transplantation, this underestimation was confirmed in a group of patients measured both before and after lung transplantation. We recommend using a 20% surcharge on predicted values for calculating energy requirements if measuring is no option.
The effectiveness of enteral tube feeding was investigated. In 6 months, an average weight gain of 3.5 kg (1.3 kg/m2) was achieved. That is important for patients with CF because mortality before and after lung transplantation was more common in patients who were underweight and had a low lean body mass.