Cystic fibrosis is an inherited disease characterized by the thickening and accumulation of mucus in the respiratory and digestive tracts. The great efforts that patients have to make to breathe as well as digestive malabsorption due to pancreatic insufficiency caused by the disease often lead to undernutrition. The bronchial congestion requiring daily respiratory physiotherapy exercises, very tiring for the body, it consumes a lot of energy.
“This is why nutritional monitoring is set up for each patient as soon as the diagnosis of cystic fibrosis is made” explains Hélène Bernard, dietician-nutritionist at the Lille CHRU. “It consists of evaluating regularly (every two months during infancy then two to four times a year) the nutritional status of the person with cystic fibrosis. The objective is to monitor growth during childhood. and to identify a possible deterioration in nutritional status in adulthood “.
What to do in case of undernutrition?
The enrichment of the diet with fat (these are the ones that provide the most calories even in small quantities) and the splitting of meals (six small rather than three large) are the measures taken in first intention.
“But at the same time, pancreatic enzymes must be increased to aid digestion. Indeed, in most people with cystic fibrosis, the ducts of the pancreas are blocked by the same type of mucus found in the lungs. foods are then not properly absorbed “insists the nutritionist.
If this fat enrichment is not sufficient, oral nutritional supplementation may be considered. This supplement is in the form of drinkable yogurts, flavored milks, fruit juice or small cakes, providing calories and proteins. But these oral nutritional supplements should definitely not replace meals. They should also be taken two hours before or two hours after a meal, so as not to cause satiety.
“When you have cystic fibrosis, the nutritional status is essential in the course of the disease. And it starts in early childhood since the screening for the disease has been done at birth since 2002. Moreover, in addition to the evaluations On a regular basis, a complete check-up must be carried out once a year in a day hospital “concludes Hélène Bernard.