Drugs that block the production of stomach acid can be used to:

• Treat gastroesophageal reflux disorder (GERD), in which excess acid enters the esophagus, leading to irritation and heartburn symptoms.

• Help pancreatic enzymes to work better. Pancreatic enzymes are packaged in tiny enteric-coated beads designed to protect them from stomach acid. Decreasing the amount of acid in the stomach usually allows more enzymes to reach the small intestine where they can digest food.

Antibiotics
Antibiotics, or drugs that kill bacteria,
are frequently administered to CF patients as a:

• Treatment for newly acquired  bacteria such as P. aeruginosa.
• Treatment for increased pulmonary symptoms or an “exacerbation.”
• Chronic therapy for patients who have persistent or recurrent symptoms.

Antibiotics may be given orally, by inhalation or intravenously. There are several classes of antibiotics, which work by different mechanisms. Penicillins and cephalosporins, for example, affect the bacterial cell wall. Fluoroquinolones, such as ciprofloxacin and gatifloxacin, block an enzyme required for DNA synthesis and bacterial growth. The combination antibiotic trimethoprim-sulfamethoxazole blocks folic acid production needed for bacterial growth.

Bronchodilators
Bronchodilators relax the muscle in the airways, enlarging them and aiding in airway clearance. These medications are used to treat wheezing that may occur in CF or asthma. Short-acting bronchodilators, such as albuterol or levalbuterol (Xopenex®), act rapidly to improve symptoms. Long-acting bronchodilators, such as salmeterol (Serevent® or Advair®) are maintenance medications used to prevent the airways from narrowing, and should not be used to relieve acute symptoms of shortness of breath or wheezing.

Mucolytics
Mucolytics are medications that thin the mucus within the lungs. Pulmozyme® is the brand name for recombinant human DNase (rhDNase) which is an enzyme that breaks down the DNA in the sputum of CF patients. This therapy is typically given once daily before airway clearance is performed.

Hypertonic saline, a solution with a high concentration of salt, is typically nebulized twice daily. Hypertonic saline also causes people to cough, thereby, increasing the amount of mucus cleared from the lungs.

Pancreatic Enzymes
Pancreatic enzyme supplements help reverse CF pancreatic dysfunction. These enzymes contain amylase, lipase and protease, which help to digest starches, fats and proteins. Enteric-coated enzyme capsules help prevent destruction of the enzymes in the acidic stomach environment.

Patients usually need 500 to 4000 units of lipase per gram of fat per day. Dosages are also based on body weight, with approximately 2000 units of lipase needed per kilogram of body weight for every meal (and half of that amount for snacks). This may be adjusted based on symptoms or results of a 72-hour stool fat collection. DO NOT USE GENERIC BRANDS because the amount of enzymes in these preparations may vary. If a correct dosage is ineffective, this may be due to the acidity of the GI environment.

Steroids
Steroids, or corticosteroids, are potent anti-inflammatory medications produced naturally in the adrenal gland. Steroids can be inhaled, given orally or intravenously. Typically they are used to treat people who wheeze. Steroids are also the best therapy for allergic bronchopulmonary aspergillosis (ABPA).

Steroids can have several side effects, including slower growth, glucose intolerance, high blood pressure, reduced bone density, and the formation of cataracts. These problems are more likely to occur when larger doses are used, especially for prolonged periods. The risk of side effects is also decreased by the use of inhaled, rather than systemic, steroids.

Alternative Medicine
There are many unproven nutrition therapies that promise to cure, help, or lessen CF symptoms and ailments. Although it might be tempting to try these products, discuss all therapies with your CF team first. Herbal supplements, or other nutrition supplements, can be dangerous to your health. This dietary supplement brochure by the National Consumers League, provides a thorough review of dietary supplements, their regulation and the potential risks.