Elise Glore, of Norco, is throwing herself into the usual summer activities that a bouncy 9-year-old enjoys, from slumber parties to sleep-away camp to her favorite pastime — diving and swimming.
But for Elise and her family, these idyllic summer days are far from typical. This is the first summer that their oldest daughter has spent without a tracheotomy, and without the noisy and cumbersome ventilator that has been a part of her life almost since her birth, said Samantha Glore, her mother.
Elise has a condition called Congenital Central Hypoventilation syndrome. When she sleeps, her brain doesn’t send a signal to the muscles that enable breathing. The rare genetic condition meant that Elise relied on ventilator around the clock when she was tiny, graduating to night-time-only use as she got older.
It also meant living with a tracheotomy, which ruled out swimming, diving and even splashing in the tub, and left the little girl vulnerable to infection. At least once a year, she would end up in the hospital, her mother said.
The Glores asked their daughter’s pulmonologist, Michael Kiernan of Tulane Medical Center, if there was another solution. Kiernan, who has only treated one other case of CCHS in more than 30 years, learned that physicians in other parts of the country were inserting diaphragm pacemakers into children like Elise.
Samantha Glore said her doctor was cautious and wanted to wait until Elise was mature enough to work with them in adapting to the devices. Initially, they thought she would have to go out-of-state for the procedure.
But last November, Rodney B. Steiner, Tulane chief pediatric surgeon, and Vincent Adolph, of Ochsner Health System, operated to place the pacemakers. It’s the first time the procedure was performed on the Gulf Coast, Kiernan said.
After sufficient time to heal, the two devices — one for each lung — were switched on the day after Christmas. Elise described the sensation as “feeling a bubbly breath.”
Kiernan said that they only expected her to sleep 30 minutes or so and thought it would take months to get her to sleep a full night with the devices. “The first night, she did it,” Kiernan said.
“She woke up and said, ‘That’s the best sleep I ever had in my life,’ ” he said.
Now, her parents say Elise has energy instead of feeling exhausted at the end of the day. Her mother hopes she’ll put on a bit more weight, too, now that she isn’t having to work so hard just to breathe.
The sound of the ventilator, which had been a constant nighttime presence, is gone, something that Samantha said her husband, Matthew, had to get used to. But then, “they noticed me snoring,” Elise said.
Her parents call it a beautiful sound.
They’re getting used to other changes, too. After years of being hyper-aware that their daughter’s life depended on a ventilator that required power, a recent nighttime power outage sent the couple scrambling. They had forgotten for a moment that they no longer have to fear losing power.
Elise’s pacemakers have two leads that end in little radio antennae, Kiernan said, and a device about the size of a cellphone that is powered by an ordinary 9-volt battery, which sends a radio signal that is turned back into electricity to stimulate the muscle.
Patients with spinal cord injuries and people who suffer from ALS are among those who can be helped by this solution, Kiernan said.
Samantha still isn’t ready for the oldest of their four daughters to sleep over at someone else’s home.
While Elise looks the picture of health and vigor, she is still medically fragile, her mother said.
She will return to Camp Pelican in Leesville, a sleep-away camp for children with pulmonary disease, again this summer. That annual trip is hard for her mother, and she spent time last week going over Elise’s new medical devices and how they work with medical personnel at the camp.
But the Glores say that they’ve worked hard as a family to make sure that they didn’t allow their daughter’s diagnosis to define her. She’s been in dance and, last year, played T-ball.
“We just roll with it,” Samantha said. “She’s a trooper.”
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