Once a diagnosis of cystic fibrosis has been confirmed, a primary goal is mitigating future damage, since inflammation and irreversible damage may have already begun. The most important goal is to keep the airway clear, followed by preventing infection. Additionally, maintaining nutritional sufficiency is important since CF’s impact on the pancreas can lead to malabsorption, exacerbating other symptoms for the condition.[1]
Management approaches to CF often depend on the stage of the condition and the time of diagnosis. In the earlier stages, airway clearance techniques (e.g., physiotherapy; mucolytics such as dornase alfa, inhaled mannitol, and hypertonic saline) along with preventative antibiotics and influenza vaccination are often recommended to support lung health.[2][3][4] In the intermediate stages of cystic fibrosis progression, reducing the infection load (through antibiotics and antifungals) and inflammation (via ibuprofen, corticosteroids, etc.) can serve to mitigate the damage done by infection. In the end stages of CF, drainage and even lung and/or heart transplant may be warranted.[5]
There are therapies under exploration focused on correcting the mutations of the CFTR gene. Some of these gene therapies carry mixed results and side effects, though, so careful consideration of this approach, with the support of a trained medical specialist, is imperative. Adjunctive approaches, such as physiotherapy, dietary modifications, insulin, or targeted nutrient/enzyme supplementation, may still be called for with genetic therapies.[6]