Featured Cases: State-of-the-Art Treatment for CF Lung Disease

Volume 2, Issue 10.

Clinical practice guidelines have become a regular component of health care. The Cystic Fibrosis Foundation has supported the development of several recent clinical practice guidelines to review the evidence that endorses many of the therapies currently in use.

Although numerous clinical manifestations of cystic fibrosis (CF) exist, the principal causes of disease-related morbidity and mortality are due to pancreatic insufficiency and chronic airway infection. Recommendations are now available on the assessment and treatment of nutritional disease, use of airway clearance therapies, and use of chronic medications to maintain lung health. Acute complications of CF lung disease include pulmonary exacerbations, hemoptysis, and pneumothorax; guidelines on the management of these complications are currently available as well. Finally, as newborn screening for CF has become standard in all states, we can expect that most patients will be diagnosed in the absence of symptoms. Infant care guidelines should provide primary care physicians with the information needed for appropriate assessment and treatment of newly diagnosed patients.

In this podcast recording, Dr. Patrick Flume utilizes patient cases to summarize the clinical practice guidelines for individuals with CF, to aid clinicians in improving the quality and consistency of the care they provide.