The Changing Face of Cystic Fibrosis: An Update for Anesthesiologists

Abstract

Cystic fibrosis (CF) is the most common fatal genetic disease in North America. While CF is more common among Whites, it is increasingly being recognized in other races and ethnicities. Although there is no cure, life expectancy has steadily improved, with the median survival exceeding 46 years in the United States. There are now more adults than children with CF in the United States. CF is caused by mutations in a gene that encodes the cystic fibrosis transmembrane conductance regulator (CFTR) protein, expressed in many epithelial cells. More than 2100 CFTR mutations have been linked to CF, and newer CFTR modulator drugs are being used to improve the production, intracellular processing, and function of the defective CFTR protein. CF is a multisystem disease that affects primarily the lungs, pancreas, hepatobiliary system, and reproductive organs. Anesthesiologists routinely encounter CF patients for various surgical and medical procedures, depending on the age group. This review article focuses on the changing epidemiology of CF, advances in the classification of CFTR mutations, the latest innovations in CFTR modulator therapies, the impact of the coronavirus disease pandemic, and perioperative considerations that anesthesiologists must know while caring for patients with CF.

In Response

“…We appreciate the comments from Nause-Osthoff et al 1 regarding our recent article on the perioperative management of patients with cystic fibrosis that was published in Anesthesia and Analgesia . 2 We agree with them that propofol is superior to volatile anesthetics at blunting airway reflexes, and therefore, propofol is commonly used in patients with cystic fibrosis for induction, maintenance of general anesthesia, or to supplement a general anesthetic with volatile agents for short procedures, such as bronchoscopy, central venous access, sinus surgery, etc. We also agree that desflurane would be a poor choice as it has been shown to cause bronchoconstriction and increase airway resistance in children with bronchial hyperreactivity, such as asthma, recent upper respiratory infections, and cystic fibrosis.…”

“…As mentioned in our article, we recommend humidification of airway gases, chest physiotherapy, lung recruitment maneuvers, use of bronchodilators if necessary, and suctioning of the endotracheal tube to mobilize secretions and avoid atelectasis. 2…”

Anesthesia and Mucociliary Clearance: Implications for Cystic Fibrosis

“…A prospective randomized study by Ledowski et al 3 examined the impacts of anesthetics on bronchial mucus transport velocity in 22 patients undergoing elective general anesthesia and found that patients receiving propofol and remifentanil had significantly less impairment of bronchociliary clearance compared to those receiving sevoflurane and remifentanil. Given that the cornerstone of treatment of CF-associated lung disease is mobilization of secretions and aggressive pulmonary clearance, 1 impairment of bronchociliary clearance may be more important for the patient’s overall health than any immediate intraoperative effect, though this remains unstudied. Of note, ketamine and remifentanil may also reduce mucociliary clearance, while morphine does not.…”

“…We were pleased to read the excellent article by Lee et al 1 describing the advances in epidemiology, diagnosis, and management of cystic fibrosis (CF). While there were many important recommendations regarding the perioperative optimization of these patients, there was one that requires further clarification.…”