At two days old, Elise Glore of Norco, La., was diagnosed with a rare condition called Congenital Central Hypoventilation Syndrome (CCHS). When awake, Elise would breathe like any other child, but when asleep her brain wouldn’t send the signal or impulse through the nerves to her breathing muscles.
To breathe properly, 9-year-old Elise Glore uses an implanted pacemaker system that runs on a 9-volt battery. She says she feels more rested and has more energy throughout the day. (Photo by Jennifer Zdon)
Dr. Michael Kiernan, Tulane pediatric pulmonologist, had only seen two other cases of CCHS in more than 30 years.
“Once the diagnosis was made, she had a tracheostomy performed, was put on a ventilator and started on our home ventilator system,” says Kiernan, professor of clinical pediatrics.
Every night, Elise’s parents, Samantha and Matt Glore, hooked Elise up to her ventilator.
The Glores approached Kiernan to ask if there was another option. The large machine is bulky, dependent upon electricity, requires special water to run and makes transportation difficult. There are also negative side effects to dependence on the ventilator such as increased respiratory infections and chronic bronchitis.
Kiernan consulted physicians across the country on alternative breathing modalities and learned physicians were inserting diaphragm pacemakers in children with CCHS.
The lifelong device works by stimulating the nerves that move the diaphragm muscle. The procedure had never been performed on the Gulf Coast.
Local surgeons Dr. Rodney B. Steiner, Tulane chief pediatric surgeon, and Dr. Vincent Adolph of Ocshner Health System placed her diaphragm pacemakers at Tulane Medical Center
Most patients find it hard to adjust to the pacemaker and start off gradually with just a few hours of use at a time. But Elise surprised everyone with her easy adaption to the pacemakers.
“It shocked us,” says Samantha Glore. “When we turned on the pacers she slept through the whole night. We couldn’t believe how well she did.”