Treatment:

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.

Support Groups:

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

Expectations (prognosis):

Most children with cystic fibrosis are fairly healthy until they reach adulthood. They are able to participate in most activities and should be able to attend school. Many young adults with cystic fibrosis finish college or find employment.

Lung disease eventually worsens to the point where the person is disabled. Today, the average life span for people with CF who live to adulthood is approximately 37 years, a dramatic increase over the last three decades.

Death is usually caused by lung complications.

Complications:

The most common complication is chronic respiratory infection.

• Bowel problems, such as gallstones, intestinal obstruction, and rectal prolapse
• Coughing up blood
• Chronic respiratory failure
• Diabetes
• Infertility
• Liver disease or liver failure, pancreatitis, biliary cirrhosis
• Malnutrition
• Nasal polyps and sinusitis
• Osteoporosis and arthritis
• Pneumonia, recurrent
• Pneumothorax
• Right-sided heart failure (cor pulmonale)

Calling your health care provider:

Call your health care provider if an infant or child has symptoms of cystic fibrosis.

Call your health care provider if a person with cystic fibrosis develops new symptoms or if symptoms get worse, particularly severe breathing difficulty or coughing up blood.

Call your health care provider if you or your child experiences:

• Fever, increased coughing, changes in sputum or blood in sputum, loss of appetite, or other signs of pneumonia
• Increased weight loss
• More frequent bowel movements or stools that are foul-smelling or have more mucus
• Swollen belly or increased bloating