Massachusetts General Hospital for Children and Massachusetts Eye and Ear have begun a new FDA-approved clinical trial recently. Researchers will test and review the use of a hypoglossal nerve stimulator in a group of adolescents with Down syndrome and obstructive sleep apnea (OSA). A hypoglossal nerve stimulator is currently a technology available to adults who suffer from severe OSA. The technology stimulates the upper airway to help facilitate breathing during sleep.
The first patient in the trial has had a case report done, which was reported in the May issue of the journal, Pediatrics. This patient had the stimulator implanted on April 8, 2015, and this marks the first time in which the technology has been used in a patient under the age of 18 in the United States. Now, the trial is going to be expanded to four more sites around the country.
Dr. Christopher J. Hartnick, lead investigator of the study and the director of Pediatric Otolaryngology at Massachusetts Eye and Ear states that there is still a long way to go to demonstrate the efficacy and safety of this technique in children; however, the case study is a significant first step. Most compelling is that this research could potentially help a group of patients with a condition that is very difficult to treat.
Approximately 60% of young patients with Down syndrome also suffer with severe obstructive sleep apnea, so this issue presents a unique constellation of challenges for treatment. For example, one of the key anatomical problems with OSA is that tongues will fall back into the throat during sleep and obstruct the airway, and many people with Down syndrome have larger tongues, making this an even greater problem. Additionally, many patients are sensitive to air blowing on their faces, which would make wearing a cpap mask, such as that used in a CPAP or BiPAP machine, very difficult and likely intolerable.
With the threat of heart conditions like hypertension and heart disease caused by untreated sleep apnea, and without favorable treatment options for those with Down syndrome, patients’ families and physicians are often left with the difficult decision of resorting to the invasive procedure of a tracheostomy should obstructive apnea prove to be severe and unsafe.
The hypoglossal nerve stimulator works by activating nerves in the upper airway and causing the tongue to move forward and out of the airway while sleeping. The stimulator is implanted surgically by placing a cuff around branches of the nerve and then connecting it to a receiver in the chest with wires, as well as to a sensor that is located below the ribs. The sensor works by detecting breathing interruptions and then sending signals to the cuff, which sets the entire process into motion. The technology can be programmed by physicians and caregivers to turn on at a certain time each night, usually about a half hour after the child lays down for sleep.
The first patient was a 13-year-old young man who needed a tracheostomy tube in order to breathe because the CPAP machine was intolerable. This child has responded positively to the therapy thus far. A follow-up sleep study three months after the surgery showed a decrease in obstructive sleep apnea symptoms using an index score to below 10 from above 40. He has been able to have the tube removed with excellent results.
Since this first surgery was performed, four additional patients have had the device surgically implanted. The patients in the clinical trial are followed closely with sleep studies and through regular office visits in order to completely evaluate the safety of the technology and treatment.
Co-author of the study, Brian Skotko, M.D., M.P.P., who is co-director of the Down syndrome program at Massachusetts General, states that the stimulator is potentially a game changer for the Down syndrome community because the patients who were part of the study have already experienced life-changing results.
Author: Rachael Herman is a professional writer with an extensive background in medical writing, research, and language development. Her hobbies include hiking in the Rockies, cooking, and reading.
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