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, which can be found in many communities. When children reach adulthood, they should transfer to a cystic fibrosis specialty center for adults.

Treatment for lung problems includes:

• Antibiotics to prevent and treat lung and sinus infections. They may be taken by mouth, or given in the veins or by breathing treatments. People with cystic fibrosis may take antibiotics only when needed, or all the time. Doses are usually higher than normal.
• Inhaled medicines to help open the airways
• DNAse enzyme therapy to thin mucus and make it easier to cough up
• High concentration of salt solutions (hypertonic saline)
• Flu vaccine and pneumococcal polysaccharide vaccine (PPV) yearly (ask your health care provider)
• Lung transplant is an option in some cases
• Oxygen therapy may be needed as lung disease gets worse

Lung problems are also treated with aerobic exercise or other therapies to thin the mucous and make it easier to cough up out of the lungs. These include a Percussion Vest, manual chest percussion, A-capella, or TheraPEP device.

Treatment for bowel and nutritional problems (see: Cystic fibrosis - nutritional considerations) may include:

• A special diet high in protein and calories for older children and adults (see: Cystic fibrosis nutritional considerations)
• Pancreatic enzymes to help absorb fats and protein
• Vitamin supplements, especially vitamins A, D, E, and K
• Your doctor can suggest other treatments if you have very hard stools

Care and monitoring at home should include:

• Avoiding smoke, dust, dirt, fumes, household chemicals, fireplace smoke, and mold or mildew
• Clearing or bringing up mucus or secretions from the airways. This must be done one to fours times each day. Patients, families, and caregivers must learn about doing chest percussion and postural drainage to help keep the airways clear
• Drinking plenty of fluids. This is particularly true for infants, children, in hot weather, when there is diarrhea or loose stools, or during extra physical activity
• Exercising two or three times each week. Swimming, jogging, and cycling are good options.

For additional information and resources, see: Cystic fibrosis support group

Images

Clubbing

Clubbed fingers

Postural drainage

Cystic fibrosis

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Review Date: 05/01/2011
Reviewed By: David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc., and Denis Hadjiliadis, MD, Assistant Professor of Medicine, Division of Pulmonary, Allergy and Critical Care, University of Pennsylvania, Philadelphia, PA.

A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org)