Currently, there is no cure for cystic fibrosis (CF). However, advancements in medicine allow many individuals with CF to live well into adulthood. The treatment for cystic fibrosis is to prevent infections, decrease and remove mucus from the lungs, increase airflow in the lungs, and maintain proper nutrition.
Pancreatic Enzymes (Digestive Enzymes)
Normally in healthy people, the pancreas produces digestive enzymes (proteins that help break down and absorb food) which are secreted through small ducts in the small intestine. However, the abnormally thick mucus in CF patients clogs these ducts in the pancreas, resulting in an insufficient amount of digestive enzymes. As a result, food is not digested correctly and intestinal problems such as diarrhea and greasy stools occur.
Pancreatic enzymes are medications that contain digestive enzymes. They are taken to help the body break down food and absorb nutrients. The majority of patients with CF take supplemental digestive enzymes. Most people with CF must take digestive enzymes with all snacks and meals. Common digestive enzymes include:
- Pancreatin
- Pancrelipase
- Pancrelipase Powder
Bronchodilators
Inhaled bronchodilators are commonly used to help maintain adequate airflow and decrease the breathing difficulties that cystic fibrosis (CF) patients can experience. When the lining of the bronchi become swollen, the muscles surrounding the bronchi contract and make it difficult to breathe. Bronchodilators are prescription medications used to relax the muscles surrounding the bronchi. Albuterol is a common bronchodilator used in CF patients.
Mucus-Thinning Drugs
Since cystic fibrosis causes normally thin mucus to become thick and sticky, drugs that thin the mucus are often prescribed to patients with CF. Thinning thick mucus can allow patients with CF to breathe easier and it can reduce the risk of lung infections associated with cystic fibrosis. The aerosolized drug Pulmozyme is an enzyme that that makes mucus in the airways thinner and easier to cough up.
Antibiotics
Antibiotics are used to fight infections that are caused from bacteria. Infections are common in the lungs of people with cystic fibrosis, so antibiotics are an important part of daily care. The particular antibiotic will vary from person to person.
Lung infections are difficult to manage, especially in people with CF. With CF patients, the bacteria that cause these lung infections are able to grow in the thick and sticky mucus. The thick mucus can prevent antibiotics from reaching the infection, making it even more difficult to manage. Therefore, new inhaled and oral antibiotics have been developed especially for cystic fibrosis patients. Among these are aerosolized antibiotics that send medication directly to the airways rather than absorbing the medication through the bloodstream.
Common antibiotics include:
- Inhaled TOBI (tobramycin) is a common antibiotic treatment for CF. TOBI can be effective against the most common source of chronic lung infections, a bacterium called Pseudomonas aeruginosa.
- Azithromycin (zithromax) is also a common antibiotic treatment for CF. The Cystic Fibrosis Foundation conducted a study on azithromycin, and found that, in some cases, the antibiotic helped to increase lung function.
Ibuprofen
Ibuprofen is used to decrease inflammation in the lungs and help slow the rate of lung damage in people with CF. People with CF often experience inflammation in the lungs, and inflammation can lead to lung damage. Problems with the respiratory system associated with lung damage account for 95% of the sickness and deaths from cystic fibrosis. It does not prevent lung infections or increase lung function.
Exercise
Exercise has many benefits for people with CF. Regular exercise increases strength, endurance and helps keep the heart strong. It is no different for people living with CF; the better their endurance, the easier their bodies will react to normal activity.
Nutrition
Thickened mucus obstructs small tubes in the pancreas of people with CF. The blocked tubes prevent pancreatic enzymes from reaching the intestines. The enzymes are responsible for food digestion and nutritional absorption. Without adequate nutrition, the body is not absorbing the proper amount of proteins, fats, vitamin A, vitamin D, vitamin E and vitamin K. Since CF patients have difficulties absorbing nutrients, it is recommended that they follow a high-calorie and high-fat diet.
Airway Clearance Techniques (ACT’S)
Airway clearance techniques (ACT’S) are treatments that help individuals with cystic fibrosis (CF) breathe better. ACT’s help remove thickened mucus from the lungs. Removing thickened mucus from the lungs reduces lung infections and increases the air flow through air tubes in the lungs.
Airway clearance techniques are used with other cystic fibrosis treatments. Bronchodilators are often used before ACT’s and inhaled antibiotics are often used after ACT’s are done.
Lung Transplantation
Lung Transplantation is a surgery to replace the diseased lungs with healthy lungs from a donor. A lung transplant is usually considered when the lungs are so severely damaged that other treatment options no longer work.
