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Treatment for 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)

  • 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 done. The gene that causes CF has been identified, and this may lead to an increased understanding of the disease. Gene therapy research hopes to eventually cure, prevent, or slow progression of the disease. 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. This is to help loosen and clear lung secretions, which may include postural drainage, and devices, such as a percussor or flutter, which vibrate the chest wall and loosen secretions. Chest physical therapy can be done manually or mechanically, with a vest.

    • Exercise. This can help loosen mucus, stimulate coughing, and improve overall physical condition.

    • Medications, such as bronchodilators and anti-inflammatories. These are used to decrease inflammation, reduce mucus, draw water into the lungs to loosen mucus, and help breathing.

    • Antibiotics. These are used 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. Provides help in dealing with issues such as coping, independence, sterility, and sexuality, financial issues, and relationships.

As lung disease progresses to end stage, other medical options include lung transplantation. The type of transplant done is usually a double lung transplant, because leaving the other, sicker lung in place puts it at risk for infection and damage. Not everyone is a candidate for a lung transplant. Consult your child's doctor for more information.

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

Online Medical Reviewer: Bass, Pat F. III, MD, MPH
Online Medical Reviewer: Grantham, Paula, RN, BSN
Last Review Date: 7/30/2012
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