For those with cystic fibrosis who have certain gene mutations, doctors may recommend cystic fibrosis transmembrane conductance regulator (CFTR) modulators. These newer medications help improve the function of the faulty CFTR protein. They may improve lung function and weight, and reduce the amount of salt in sweat.
The FDA has approved these medications for treating CF in people with one or more mutations in the CFTR gene:
•The newest combination medication containing elexacaftor, ivacaftor and tezacaftor (Trikafta) is approved for people age 12 years and older and considered a breakthrough by many experts.
• The combination medication containing tezacaftor and ivacaftor (Symdeko) is approved for people age 6 years and older.
• The combination medication containing lumacaftor and ivacaftor (Orkambi) is approved for people who are age 2 years and older.
• Ivacaftor (Kalydeco) has been approved for people who are 6 months and older.
Your doctor may recommend a long-term program that may improve your lung function and overall well-being. Pulmonary rehabilitation is usually done on an outpatient basis and may include:
• Physical exercise that may improve your condition
• Breathing techniques that may help loosen mucus and improve breathing
• Nutritional counseling
• Counseling and support
• Education about your condition
Courtesy: Mayo Clinic