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
