The primary goals of cystic fibrosis (CF) treatment include maintaining lung function as near to normal as possible by controlling lung infections, clearing airways of mucus, administering nutritional therapy (i.e., enzyme supplements, multivitamin and mineral supplements) to maintain adequate growth, and managing complications. Medications play a major role in the management of CF.
- Pancreatic Enzymes
At the time of diagnosis, the majority of patients have a condition called pancreatic insufficiency, which means that the enzymes created in the pancreas that digest fat, starch, and protein aren't properly passed into the intestines. This causes problems in absorbing nutrients from foods.
By providing good overall nutrition along with extra fat and calories and prescribed enzymes, people with CF can grow healthy and strong. Enzymes are taken with each meal and snack.
Vitamins A, D, E and K are important for immune function, growth and healing. These vitamins are absorbed along with fat. And because most people with CF have trouble digesting fat, they often have low levels of fat-soluble vitamins and need to take specialized prescription supplements. Blood work done yearly helps to determine if the vitamin levels are adequate.
Antibiotics are used to fight infection-causing bacteria. Infections are common in the lungs of people with cystic fibrosis, so antibiotics are an important part of regular care.
The antibiotic drug, the dosage, and the length of time to take the drug, all vary from person to person. The infection-causing bacteria can become resistant to some drugs.
Antibiotics come in three different forms:
- Oral antibiotics – liquids, tablets or capsules that must be swallowed.
- Inhaled antibiotics – an aerosol or mist that can be nebulized (turned into a fine mist) to reach the airways directly.
- Intravenous (IV) antibiotics – liquid medicine that goes directly into the blood through an IV catheter. An IV may require a hospital stay, but can also be done at home.
- Inhaled Medications
IInhaled drugs are commonly used in cystic fibrosis care because they reach the airways quickly and easily. Inhaled treatments can be given by aerosol—a mist made from liquid medicines. The medicines go into a cup (nebulizer) that is attached to a small air compressor. The compressor blows air through the cup and makes a mist. People with cystic fibrosis breathe the mist in through a mouthpiece or mask for several minutes. Some medicines can also be given as metered dose inhalers (MDI), which deliver one dose of medicine at a time. For more information see the instruction sheet "Caring and Maintenance of your Nebulizer". This document also describes which nebulizer to use with which medication.
There are several kinds of inhaled medications used to treat CF symptoms:
- Mucolytics like Pulmozyme® to thin mucus so it can be coughed out more easily.
- Antibiotics to treat infections.
- Hypertonic Saline to draw more water into the airways and make it easier to cough out the mucus.
- Salbutamol (Ventolin) is a bronchodilator that works by opening the airways and making breathing easier. It can be given either nebulized or by a puffer (metered dose inhaler).
- Other Drugs
Azithromycin, a common antibiotic, was shown to have special benefits for some people with cystic fibrosis. In some cases, the drug helped to preserve and improve lung function. The drug also reduced the number of hospital stays for many people who participated in clinical trials. It is a treatment that is taken three times a week in pill form.