Treatment of Cystic Fibrosis (CF)

Cystic Fibrosis Center

Treatment of Cystic Fibrosis (CF)

The University of Wisconsin Cystic Fibrosis Center in Madison, Wisconsin, is dedicated to providing state-of-the-art care to patients with cystic fibrosis (CF). Cystic fibrosis treatments may vary from one person to another, depending on the systems affected and the phase of the disease.

Overall, the goals for treatment are to:

  • Educate family members about the child's care
  • Promote the health of the child
  • Prevent the disease from progressing
  • Help the child and his/her family lead normal and fulfilling lives

Airway Clearance Techniques (ACT)

Airway clearance techniques (ACT) are methods used to assist with secretion removal. ACT should be viewed as part of your daily routine cares. The key to success is to choose the one that is most effective for you, as well as one you will faithfully follow.

  • Manual chest physiotherapy (CPT)
  • Positive expiratory pressure (PEP)
  • Active cycle breathing (ACBT) 
  • Forced expiration technique (FET): Forced expiration technique consists of one or two forced expirations (huffs) followed by relaxation and comfortable breathing. 
  • Autogenic drainage (AD): Autogenic drainage consists of three phases: (1) The "unstick" phase, where normal breathing helps move the secretions up the bronchial tree, (2) The "collect" phase, where small volume breathing is increased to a higher level, allowing for secretion collection in the central airways, (3) The "evacuate" phase, where breathing is normal to high volume breathing and secretions can be removed from the upper airways. 
  • Exercise: Some types of exercise that others have found beneficial are walking, swimming, trampoline jumping and bicycling.

Nutritional Care

To compensate for problems with digesting foods, people with cystic fibrosis generally take enzyme pills, eat foods high in calories, take extra vitamins and add extra salt to foods.

Most people with cystic fibrosis take capsules with each meal; these capsules contain enzymes. Parents of infants and young children can open the capsules and sprinkle the enzymes into applesauce to be spoon-fed or given through a nipple with an enlarged hole. Mothers of infants may continue breast feeding if they wish.

Families work closely with dietitians, who offer parents ideas about how to meet the child's nutritional needs at various stages of the child's development. The dietitian may suggest ways to boost the child's calories to between 120 percent and 150 percent of the recommended daily allowance. The physician may prescribe fat-soluble vitamin A, E and K supplements.

Parents are also taught how to recognize signs of digestive problems. The child's weight and height are measured on a regular basis to be sure that s/he is growing well and receiving adequate nutrition.

Open Letter From One CF Parent to Another (pdf)

Infertility Treatment

Several options are available for people with cystic fibrosis who wish to have children but have fertility problems. These options may include but are not limited to artificial insemination or adoption. For further information about those options, consultation with cystic fibrosis experts is recommended.