Treatment of Cystic Fibrosis

 


Specific treatment for cystic fibrosis (CF) will be determined by your child's doctor based on:

  • Your child's age, overall health and medical history
  • Extent of the disease (body systems affected)
  • Expectations for the course of the disease
  • Your child's tolerance for specific medications, procedures or therapies
  • Your opinion or preference

Currently, there is no cure for CF; however, basic and clinical research is being performed in laboratories worldwide. The gene that causes CF has been identified, and this has led to a better understanding of the disease. Different drug regimens also are being researched to help slow the progression of CF. Treatment goals, such as easing the severity of symptoms and slowing the disease include:

  • Management of problems that cause lung obstruction, including:
    • Chest physical therapy (to help loosen and clear lung secretions; this may include postural drainage and the use of devices such as a percussor or flutter, which vibrate the chest wall and loosen secretions.)
    • Physical activity (to loosen mucus, stimulate coughing and improve overall conditioning)
    • Medicines (to loosen secretions and help breathing, such as bronchodilators and anti-inflammatory medicines)
    • Antibiotics (to treat infections)
    • Management of digestive problems, including:
      • Appropriate diet
      • Pancreatic enzymes to aid digestion
      • Vitamin supplements
      • Treatment for intestinal obstruction
      • Psychosocial support (dealing with issues such as independence, sterility and sexuality, depression, financial issues and relationships)

      For severe end-stage disease, lung transplantation is an option. A double lung transplant is the typical procedure. Not everyone is a candidate for a lung transplant. Consult your child's doctor for more information.

      With medical and psychosocial support, children and adolescents with CF can cope well and lead a productive life.

      The Cystic Fibrosis Foundation has established a CF Care Center Network to ensure that optimal care is provided to children with CF. CF care centers that are accredited by the CF Foundation are expected to maintain a multidisciplinary team that includes physicians, nurses, social workers, nutritionists, respiratory therapists and other professionals specifically trained in CF care. These centers also work to ensure the optimal performance of all laboratory testing necessary to care for CF, and are also engaged in teaching and research. In addition, as more and more CF patients reach adulthood, adult CF care centers that maintain equally high standards are available to care for them. It is strongly recommended that all CF patients obtain care from an accredited CF care center.

      Feeding Your Child With Cystic Fibrosis

      How many calories does my child need?
      Children with cystic fibrosis (CF) often have trouble consuming enough calories to grow or gain weight. Infections, breathing problems and malabsorption all can contribute to increased calorie needs. The diet for children with CF should be high in calories. If your child consumes extra calories, then growth and development should occur normally. Children should eat three meals and two to three snacks a day to meet their calorie needs. Adding extra fat to meals and snacks can help to meet your child's calorie needs. Pancreatic enzymes usually will be needed to help digest this diet.

      Does my child need extra vitamins?
      Vitamins A, D, E and K are fat-soluble vitamins. They are important for proper growth and development. Many children with CF do not get enough of these vitamins from the foods they eat because they have difficulty digesting and absorbing fat. When this happens, your child may need to take extra vitamins. Your child's doctor can recommend the kind and amount of vitamins you should give to your child. These are specifically made for children with CF. Consult your child's doctor for more information.

      Does my child need extra salt?
      Children with CF need extra salt in their diets because they lose more salt in their sweat than other children. Add extra salt by using table salt at meals and including salty foods and snacks in their diet (e.g., chips, pretzels, fries, pickles, nuts and lunch meats). Extra salt intake is important in hot weather when children are playing outside and sweating more.

      What if my child is not gaining weight?
      Sometimes, children with CF do not grow or gain weight despite a high-calorie diet. This can occur if your child's appetite is poor, if he has frequent infections or if he has malabsorption. If your child is unable to eat enough food to grow and develop appropriately, then supplemental oral or tube feedings may be needed, as directed by your child's doctor. Tube feedings involve placing a small tube through the nose and into the stomach. This is referred to as a nasogastric tube (NG tube).

      Parents can give a high-calorie formula or supplement to their children through this tube to help promote appropriate growth and development. Some patients with CF require extra calories delivered for a long period. The can be accomplished by placing a feeding tube (gastrostomy or "G-tube") through the stomach wall, or by giving intravenous fat solutions (intralipids). Consult your child's doctor for more information.