Drug interactions and treatment burden as survival improves

With our growing understanding of the pathophysiology of cystic fibrosis, the pace of drug discovery is accelerating. Newer agents and therapies have traditionally been added to available medications, given the urgency in treating the disease. As the cystic fibrosis population ages, the number of associated comorbidities increases, requiring additional therapeutic approaches. Thus, while current management strategies have dramatically extended projected life expectancy, the treatment burden of the disease in adulthood has become onerous, and there is increasing concern over unintended effects and drug-drug interactions of new and existing therapies.

A number of recent studies have sought to quantify the treatment burden of cystic fibrosis care, and to identify ways to reduce this burden. Mechanistic studies have identified the potential for a number of cystic fibrosis medications to impair the host response, or to interfere with the efficacy of other agents.

As the cystic fibrosis formulary grows, a primary emphasis will be for providers to develop personalized treatment plans, with a goal to reduce unnecessary treatment burden and an awareness of potential unanticipated effects of medications.