Care of Patients with Cystic Fibrosis

Abstract

Cystic fibrosis (CF), the most common autosomal hereditary pediatric disease, is no longer restricted to childhood as modern therapy has given patients both increased longevity and better quality of life. When the gene was identified more than 16 years ago and the most common mutation, ΔF508, found, general hope of a rapid cure for the disease was expressed. The discovery entailed a marked increase in research on pathophysiology, but to date the improvements in clinical therapy are entirely due to developments in traditional treatment. Improvements in the patient’s condition are related to better pancreatic enzyme supplements and more intense nutritional treatment, including the attention paid to abnormal lipid metabolism. Pulmonary treatment has resulted in better preserved lung function into adulthood with access to a battery of efficient antibiotics and the development of new strategies; mucus-dissolving agents in combination with encouragement of physical activity from an early age together with modifications in physiotherapy, and improved results in lung transplantations as the ultimate treatment. Liver disease is recognized more, and often treated long-term with ursodeoxycholic acid, although its effect in preventing progression of liver disease has not been fully explored. Neonatal screening is increasing, challenging short-term problems such as those of a psychological nature when carriers or very mild diseases are discovered, and exploring long-term results to prove that clinical status improves and survival further increases. The limitations in survival still present seem mainly to be due to the mystery behind the chronic colonization of the airways by microorganisms.

Supplementation With Fatty Acids Influences the Airway Nitric Oxide and Inflammatory Markers in Patients With Cystic Fibrosis

“…Patients with CF in Sweden are generally recommended 1 to 2 tablespoons of extra fat, rich in FA, given as sunflower or corn oil per day. This could be one of the explanations why patients with CF in our population have good clinical status well into adulthood (17).…”