What's the best diet for cystic fibrosis?

The goal for every child with cystic fibrosis is to have a healthy weight for their height (more precisely a BMI at the 50%).  To get there, the best diet is a high calorie, high protein, high fat and high salt diet. 

In infants, extra calories and extra salt (1/8 tsp each day) is added to their formula. If a baby has required surgery, a formula with a fat (medium chain triglycerides) that can be easily absorbed is recommended. 

As the children get older, the emphasis on the diet remains the same.  Those with CF need extra protein and calories, they also need extra fat. Not only is fat a good source of calories but a lot of dietary fat is wasted, because it isn't well absorbed. Protein too can be wasted when it's not digested and absorbed completely. Extra protein is needed to provide the building blocks for the tissues needed for growth and repair. 

It's also important that patients take pancreatic enzyme replacement therapy (PERT) with their meals and snacks and that the enzymes are given in a high enough dose so that they are in balance with what they eat.

Those with endocrine pancreatic insufficiency (diabetes) as a result of CF need to rebalance their diet to adjust their calorie source. A low carbohydrate diet with insulin supplementation may be necessary.