Cystic fibrosis (CF) is a genetic disorder, caused by mutations in the CFTR gene. The CFTR protein, for which this gene encodes, channels salts in and out of cells. Mutations in the CFTR gene cause the CFTR protein to be made incorrectly or not at all, leading to a buildup of thick mucus in various organs and tissues. In the lungs, this mucus makes breathing difficult, narrowing the airways and making patients more susceptible to infections.
There currently is no cure for CF, but there are treatments to thin the mucus and make it easier to cough out. However, frequent coughing can induce bronchospasms (a sudden constriction of the airways). To prevent bronchospasms, and to ensure that patients can get a full dose of any inhaled medications they may be using, bronchodilators are often prescribed.
What are bronchodilators?
Bronchodilators are medications (usually inhaled), which cause the smooth muscle surrounding the airways to dilate or relax, allowing the lungs to fill more easily with air. Bronchodilators are used to treat people who experience bronchospasms as a result of asthma or conditions such as CF.
Albuterol (brand names Ventolin and Proventalin), Xopenex (levalbuterol), and Combivent, a combination of ipratropium bromide and albuterol, are examples of bronchodilators used in treating bronchospasms caused by CF. Albuterol and levalbuterol are small molecules that bind to a protein receptor called the beta-2-adrenergic receptor found in smooth muscles and activate it, causing a cascade of signals that relax the muscle, opening the airway.
CF patients generally take bronchodilators using a metered dose inhaler before taking any other inhaled medications. This ensures that they are able to breathe in a full dose of their medications.
Bronchodilators research for CF
Although bronchodilators are frequently prescribed to CF patients, there are few clinical trials that have looked at the safety and efficacy of the treatment specifically in CF patients. There have also been very few studies on the long-term consequences of bronchodilator use in CF patients.
Bronchodilators can cause side effects, including tremors, rapid heartbeat, nausea, and nervousness.
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