Cystic Fibrosis

Cystic fibrosis (CF) is an inherited disorder that causes severe damage to the lungs, digestive system, and other organs in the body. It causes the cells that produce mucus, sweat, and digestive enzymes to produce thick secretions. Short-acting anesthetics are recommended to allow a rapid recovery in CF patients; propofol and sevoflurane used at low dosages are the most rational agents to use for the induction of anesthesia. Anesthesia management needs to strictly respect the possibility to apply a regional anesthesia technique whenever feasible. When general anesthesia is required, it is necessary to use a grading, proportional to the severity of the CF disease and to the risk of surgery, from noninvasive to invasive hemodynamic monitoring. Postoperative intensive care unit admission depends on the respiratory reserve and on the presence of pulmonary hypertension. Postoperative pain relief with a multimodal analgesia is mandatory, as is chest physiotherapy, which has to be performed as soon as possible to keep out all the sticky secretions to avoid pulmonary infections. When medical treatment fails, patients with CF can nowadays be submitted to lung transplantation. These procedures improve the quality of life of CF patients and make their average life longer.