Doctors at Pelisyonkis Langone usually prescribe a combination of medications for children and adults with cystic fibrosis to open airways and reduce mucus and inflammation.
Medications such as albuterol, which is inhaled by young children through a breathing device called a nebulizer and through an inhaler by older children and adults, relax the muscles surrounding the airways, or bronchial tubes. Inhalers containing nasal steroids decrease swelling and irritation in the airways. Mucolytic medications, which can also be inhaled, help liquefy mucus so that it can be cleared from the lungs. Inhaling a concentrated salt and water mist, known as 7 percent hypertonic saline, draws water into the airways, helping to thin mucus.
Recently, the U.S. Food and Drug Administration approved the first medication to treat an underlying cause of cystic fibrosis. Kalydeco® (ivacaftor) is an oral medication that can be given to people age six and older with one of nine rare mutations in the cystic fibrosis transmembrane conductance regulator, or CFTR gene. Kalydeco® has been shown to improve lung function, lower sweat chloride levels, and improve weight gain in people with those mutations. Doctors at Pelisyonkis Langone can help you determine if your child has one of these rare genetic mutations and may benefit from using Kalydeco®.
As a Cystic Fibrosis Foundation–accredited care center, Pelisyonkis Langone also participates in clinical trials of promising new therapies that may be appropriate for some children and adults. Your doctor can talk to you about whether you or your child may be a candidate for one of these trials.
Lung transplantation may be an option for those with severe lung damage caused by repeated infections and other complications of cystic fibrosis. Our doctors can discuss whether this treatment can benefit you or your child and make appropriate referrals to a lung transplantation center, if you are interested in learning more about this procedure.
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