Treatment of Cystic Fibrosis

An early diagnosis of CF and a comprehensive treatment plan can improve both survival and quality of life. Follow-up and monitoring are very important. If possible, patients should be cared for at cystic fibrosis specialty clinics.

Treatment for lung problems may include:

  • Antibiotics to prevent and treat lung and sinus infections
  • Inhaled medicines to help open the airways
  • DNAse enzyme replacement therapy to thin mucus and make it easier to cough up
  • Flu vaccine and pneumococcal vaccines
  • Lung transplant is an option in some cases
  • Oxygen therapy may be needed as lung disease gets worse

Treatment for bowel and nutritional problems may include:

  • A special diet high in protein and calories
  • Pancreatic enzymes to help absorb fats and proteins
  • Vitamin supplements; especially vitamins A,D,E, and K

Airway clearance techniques which can include:

  • Coughing or huffing (a gentle cough)
  • Chest physical therapy (CPT) which involves lying in various positions and clapping on the chest
  • Using a positive expiratory pressure (PEP) device, which is often a mask or a mouthpiece attached to a special machine.
  • Breathing out through a flutter device
  • Wearing a special vest that is attached to a machine that vibrates at a high frequency
  • Albuterol and Pulmozyme may be given during this type of treatment, but no inhaled antibiotics.
  • Autogenic drainage, or drainage controlled by the person with CF
  • This requires good technique and control and the technique must be evaluated by a respiratory therapist frequently

Self care considerations:

  • See your Cystic Fibrosis Care Team every three months as recommended by the Cystic Fibrosis Foundation
  • Avoid cigarette smoke
  • Exercise three to five times per week; walking, swimming, cycling, jogging are good options but be careful not to over-exert yourself
  • Drink plenty of fluids
  • Keep your distance from others who are sick
  • Use good hand washing techniques and call your doctor when you think you may be getting sick