Congenital central hypoventilation syndrome and desogestrel: A call for caution

Abstract

Patients suffering from congenital central hypoventilation syndrome (CCHS) depend on mechanical ventilation during sleep, from birth and throughout life. They lack CO₂-chemosensitivity. Hope has recently been raised by serendipitous observations of chemosensitivity recovery under treatment by desogestrel, a very potent progestin (Straus et al., 2010). Caution is however needed, because this effect could depend on dose, idiosyncrasies, or be transient. Desogestrel should not be prescribed to CCHS patients with a respiratory purpose until the results of a pending clinical trial (clinicaltrials.gov. NCT01243697) are available.