Cystic Fibrosis 101

There is no cure for CF, but significant progress in treatment has been made in recent years. In the 1950s, children with CF rarely lived long enough to attend school. But today, it’s a much different story. There are steps you can take to control CF, including nutritional and respiratory therapies, medicines and specialized exercises. The Cystic Fibrosis Foundation recommends patients CF complete a combination of the following therapies every day:

• Airway Clearance to help loosen and get rid of the thick mucus that can build up in the lungs. Some airway clearance techniques require help from family members, friends or respiratory therapists. Many CF patients use an inflatable vest that vibrates the chest at a high frequency to help loosen and thin the mucus.
• Inhaled Medicines to open the airways or thin the mucus. These liquid medicines are made into a mist and then inhaled through a nebulizer.
• Pancreatic Enzyme Supplement capsules to improve the absorption of vital nutrients. These supplements are taken with every meal and most snacks. CF patients also typically take multivitamins.

There is no one way to treat CF, so it’s important to talk to your specialist about which treatments are right for your child. Earlier this year, the US Food and Drug Administration (FDA) approved a drug to treat the root cause of CF: a gene mutation called F508del. This medication makes the body produce a protein that affects how water is transported into the body. This is the first drug designed specifically to target the root of CF.