Standards of care for patients with cystic fibrosis

Standards of care define the optimal service provision necessary to deliver the best outcomes possible for patients. People with cystic fibrosis (CF) have complex care needs that demand extensive medical and allied healthcare expertise. Several guidelines have been written to assist CF caregivers in the evaluation and monitoring of patients, detection of complications and prevention of clinical deterioration. The better clinical status and improved survival of patients with CF is a result of understanding of the molecular mechanisms of CF and the development of therapeutic strategies that are based on insights into the natural course of the disease. Current CF treatments that target respiratory infections, inflammation, mucociliary clearance and nutritional status are associated with improved pulmonary function and reduced exacerbations. Patients benefit from treatment at specialised CF centres by a multidisciplinary dedicated team, with emphasis being placed on frequent visits, periodic testing and monitoring adherence to therapy. However, the current published standards of care in Europe and North America cannot be fully implemented in populations or countries with limited resources and where CF services are in the early stages of development.