Respiratory Disorders

Treatment of Cystic Fibrosis

Treatment for cystic fibrosis (CF):

Specific treatment for cystic fibrosis (CF) will be determined by your child's physician 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

At the present time, there is no cure for CF. However, research in gene therapy is being performed. Learn more about gene therapy research at Lucile Packard Children's Hospital.  The gene that causes CF has been identified and there are hopes that this will lead to an increased understanding of the disease. Also being researched are different drug regimens to help stop CF. Goals of treatment are to ease severity of symptoms and slow the progress of the disease. Treatment may include:

• management of problems that cause lung obstruction, which may involve:
  
  
  • chest physical therapy (to help loosen and clear lung secretions; this may include postural drainage, and devices such as a percussor or flutter, which vibrate the chest wall and loosen secretions)
    
    
  • exercise (to loosen mucus, stimulate coughing, and improve overall physical condition)
    
    
  • medications (to reduce mucus and help breathing, such as bronchodilators and antiinflammatory medications)
    
    
  • antibiotics (to treat infections)
    
    
• management of digestive problems, which may involve:
  
  
  • appropriate diet
  • pancreatic enzymes to aid digestion
  • vitamin supplements
  • treatments for intestinal obstructions
    
    
• psychosocial support (dealing with issues such as independence, sterility, and sexuality, financial issues, and relationships)

Newer therapies include lung transplantation for patients with end-stage lung disease. The type of transplant done is usually a double lung transplant or a heart-lung transplant. Not everyone is a candidate for a lung transplant. Consult your child's physician for more information. Please visit the Pediatric Lung Transplant program to learn more.

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