PRESS RELEASE

FAIRFAX—The October issue of the AANA Journal—the official scholarly journal of the American Association of Nurse Anesthetists—features a letter by rare disease specialist and DC area plastic surgeon, Dr Craig R Dufresne, highlighting nuances of anesthesia in Freeman-Burian syndrome (FBS), a very rare birth defect. The letter is in response to a previously published article in the Journal Dufresne felt contained inaccuracies that could potentially endanger patient care.

Dufresne’s comments highlighted the avoidance of long-acting opiates in patients with FBS. Opiates can depress respirations, and patients with FBS patients are already at higher risk for respiratory complications following anesthesia. He discussed using ibuprofen for managing very high fever during or after anesthesia in certain patients with FBS, as little evidence has ever materialized that malignant hyperthermia—a rare metabolic reaction to certain anesthesia drugs that includes very high fevers—is associated with FBS. Finally, he gave a brief overview of the syndrome.

Dufresne has written extensively about FBS and is considered to be one of the leading experts in the world on this exceptionally rare condition, having cared for affected patients since the mid-1980s. He has authored the only clinical practice recommendations for anesthesia care of patients with FBS which have seen international dissemination with the help of OrphanAnesthesia and Orphanet. OrphanAnesthesia, is a project of the German Society of Anesthesiology and Intensive Care Medicine, and in France, Orphanet is part of the Institute National de la Santé et de la Recherche Médicale (INSERM). Two of Dufresne’s other anesthesia recommendations recently have been translated into Spanish and Czech to facilitate adoption.

An exceptionally rare and difficult to treat condition, FBS is primarily a condition of facial and skull muscles that frequently involves muscles in the arms, legs, and elsewhere. For Dufresne, writing about rare conditions is all about educating people—scientists, physicians, care teams, family members, and patients—in order to improve patients’ chances for a healthy, normal, and productive life. An ever-humble gentleman, publishing medical articles is his way to help many more patients than he ever could directly.

For more information and to arrange interviews with Dr Dufresne and a patient who has this very rare syndrome, please contact the office.