Cystic Fibrosis

Cystic fibrosis (CF) is a disease where both parents contribute an abnormal gene to cause a problem with the salt that is in most of the fluids that the body produces.  This results in a build-up of mucus in the lungs, pancreas, and other areas of the body (including the intestines and sweat glands).

The Symptoms of Cystic Fibrosis

  • Chronic cough, wheezing or difficulty breathing (can look like asthma)
  • Fatty, bulky bowel movements (the stools may float in the toilet)
  • Difficulty gaining weight and growing
  • Constipation in some infants of older children
  • Salty sweat (sometimes, can detect by kissing the skin)
  • Male infertility

Fortunately, screening tests are performed when every baby is born in the US and those tests usually detect the most severe cases.  If not, tests of the sweat and bowel movements can help to make the diagnosis.

Nutrition in Cystic Fibrosis

Good nutrition is very important

  • to achieve optimal growth
  • to improve lung function
  • to improve quality of life
  • to prolong health

 

To help patients with Cystic Fibrosis achieve the best results, they are usually taught to add

  • Increased calories
  • Increased protein 
  • Unlimited fat
  • Extra salt
  • Additional vitamins (particularly, A, D, E, K)
  • High calorie drinks
  • Tube feedings (if they are unable to drink enough)
  • Pancreatic enzymes with each meal to replace what their bodies can't produce
  • Fiber to prevent constipation
  • Antacids or acid blockers to help the pancreatic enzymes work better