LAUSR

Obstructive sleep apnea (OSA) is a common sleep disorder affecting up to 80% of people with Down Syndrome (DS). Tonsillectomy and adenoidectomy (T&A) are commonly utilized as the first line of treatment of OSA in patients with DS. However, even after T&A, OSA can persist in up to 60% of patients. Positive airway pressure (PAP) compliance is low in patients with DS, limiting treatment efficacy. Hypoglossal nerve stimulation (HNS) is FDA-approved for the treatment of select adult patients with OSA. Limited research is available on HNS in people with DS; however, the limited data suggests that HNS may be a safe and effective treatment in this patient population. We describe two cases of patients with DS and HNS with unique challenges with treatment. Case 1: A 36-year-old male with a history of DS presented for follow-up after activation of the HNS. The patient had been living part-time in a group home. While the patient’s mother thought he was using HNS every night, the download from the remote showed 41% usage over a three-week time frame. With further investigation, it was determined that the device was turned on the nights when the patient was at home with his parents, but not when he was at the group home despite detailed caregiver education. Case 2: A 19-year-old male with OSA and DS returned for a follow-up of his HNS. Upon exam, the patient’s HNS was already “on” in clinic, which the mother was unaware of. Download from the remote showed a usage time of 13+ hours, and the HNS was commonly “on” into the afternoon well after the patient had left for school in the morning, indicating that the HNS was commonly left on while the patient was awake. Despite significant education, the caregiver was not correctly turning the HNS off. HNS offers a therapeutic alternative in patients with DS, who are refractory to T&A and PAP. The success of the treatment is dependent on multiple factors, including therapy mechanics, patient tolerance, and caregiver education/compliance. Additional caregiver instruction may be necessary to ensure optimal use.