Nutritional Management of Cystic Fibrosis - Hardcoverby Maria R. Mascarenhas (Guest Editor), Jessica Alvarez (Guest Editor) Cystic Fibrosis is an inherited disease that starts in utero. It is caused by a defective protein called the CF transmembrane conductance regulator (CFTR) and while the effects are seen throughout the body, changes in the lungs, gastrointestinal tract, pancreas and liver primarily impact nutritional status. Survival and pulmonary function have been linked to nutritional status.