Cystic Fibrosis

Cystic fibrosis is most commonly considered a disease that affects the lungs, but this disease involving body secretions also affects the pancreas and digestive system. Ensuring that patients with cystic fibrosis get adequate nutrition is essential for their quality of life.

Cystic Fibrosis (CF) is a genetic disorder that affects the respiratory, digestive and reproductive systems, and involves the production of abnormally thick mucus linings in the lungs and other organs. An individual must inherit two defective cystic fibrosis genes, one from each parent, to have the disease. Each time two carriers of the disease conceive, there is a 25 percent chance of passing cystic fibrosis to their children; a 50 percent chance that the child will be a carrier of the cystic fibrosis gene; and a 25 percent chance that the child will be a non-carrier. This disease is most common in North America and Europe, where it affects between one in every 2,000 to 3,500 babies.¹

There are varied symptoms of CF. The respiratory system is largely affected where mucus build-up can cause persistent coughing, wheezing and shortness of breath , combined with an increased risk of respiratory infections. Mucus can also affect the digestive system, causing a deficit of enzymes released by the pancreas that aid in digestion. The result is reduced absorption of nutrients, greasy, bulky stools, intestinal blockage, as well as poor weight gain and growth in infants. People with CF are also at higher risk of diabetes because of their condition. Other symptoms include reproductive problems in both men and women, excessive salt content of a person’s sweat and lower bone density.²*

People with CF need to consider important lifestyle changes to protect their health, including keeping vaccines that help prevent respiratory infections up to date, avoiding smoke, frequent hand washing, and increased calorie and nutritional intake. As mucus buildup can prevent proper absorption of nutrients, eating a high-calorie, high-fat and high-protein diet can help prevent decreased weight gain and slow growth in infants. Staying well hydrated and getting sufficient levels of salt are both important as well, as the changes in the secretory function cause more salt to be excreted in the sweat. Since adequate nutrition may help patients manage CF, at Nestlé Health Science, we are committed to research in the nutritional management of CF and other genetic diseases which may help improve their quality of life.³