Setting Norah Free

August 31, 2017

Norah and her parents, Corrie and Curtis, with her care team, CAMP director Dr. Debra Weese-Mayer (far left) and pediatric surgeons Dr. Anthony Chin and Dr. Rashmi Kabre.


Two-year-old Norah Calvert is unable to breathe on her own. Born with a rare, life-threatening autonomic nervous system disorder, she has spent her entire young life tethered by a 5-foot tube to the mechanical ventilator that keeps her alive.

That will change in November 2017, when the diaphragm pacer she had implanted by surgeons at Lurie Children's in August will be activated, enabling her to breathe without her ventilator during her waking hours, and providing her with increased mobility, more confidence and an overall better quality of life.

"Right now one of us is trailing behind Norah everywhere she goes, carrying her 17-pound ventilator in a backpack," says her mom, Corrie. "She has no independence, which makes her more timid, and that’s not her personality."

Diaphragm pacing was pioneered at Lurie Children's and is available at only a handful of pediatric medical centers worldwide. The surgery is a component of a unique multidisciplinary program at the hospital's Center for Autonomic Medicine in Pediatrics (CAMP).

Norah was born with the genetic disorder congenital central hypoventilation syndrome (CCHS), a condition that affects fewer than 1,300 individuals worldwide. CCHS can vary in severity and can affect a variety of autonomic nervous system processes, most commonly the ability to breathe adequately to sustain proper oxygenation and ventilation in activities of daily living. 

The Calvert family lives in Kansas, and it took stays at three area hospitals before a neonatologist at a children's hospital in Kansas City, MO, ordered genetic tests on a hunch, which indicated Norah had CCHS.

"We were both relieved and scared by the diagnosis," says Corrie. "Relieved to finally have an answer to why Norah couldn't breathe on her own but scared to learn it was a lifelong issue that isn't curable."

At four months of age, Norah finally went home. Corrie and Norah's dad, Curtis, began searching the web for hospitals with expertise in treating CCHS. They ultimately contacted Lurie Children's CAMP program, which had also been mentioned by Norah's pulmonologist in Kansas City.

Led by Debra E. Weese-Mayer, MD, CAMP is the first and only program of its kind in the world, one that integrates the expertise of specialists from across the hospital. CAMP is also a leader in research into the genetic basis of CCHS and related disorders, including sudden infant death syndrome and rapid-onset obesity with hypothalamic dysfunction.

In June 2016, the Calvert family came to Chicago, where Dr. Weese-Mayer, respiratory therapists, sleep medicine specialists and other members of the CAMP team gave them a crash course on all aspects of CCHS.

The team spent five days performing extensive tests on Norah, monitoring her closely and adjusting her ventilator settings.

"Developmentally, Norah is exactly where she should be," says Corrie. "She can speak in complete sentences and vocalizes clearly. We attribute a lot of that to the CAMP team, who made sure she has the optimal ventilator settings to provide oxygen to her brain."

One of the CAMP team specialists they met with was Anthony Chin, MD, one of only a few pediatric surgeons in the U.S. with expertise in performing an advanced minimally invasive thorascopic surgical procedure that offers pediatric CCHS patients meeting specific criteria an alternative to mechanical ventilation. Norah met the requirements for the procedure but would need to wait until she was age 2 to undergo the surgery.

In August 2017, Dr. Chin, assisted by surgeon Rashmi Kabre, MD, implanted a quarter-sized receiver in Norah's chest that receives electric impulses from an external transmitter. These impulses travel from the receiver to electrodes attached to the phrenic nerve, the nerve that moves the diaphragm, which is the main muscle needed for breathing. The stimulation of the nerve causes a diaphragmatic contraction that generates breathing.

In November, Norah and her family will return to Lurie Children's to have her pacer activated and programmed. During the daytime, Norah will be freed from being physically connected to her ventilator and will wear a small backpack that will hold the pacer's battery-powered transmitter. At night she will continue to use her mechanical ventilator.

"I think having more independence and being able to go down a slide by herself or run around on her own like other kids will help Norah blossom into the person she’s meant to be," says Corrie.

You can help physician-scientists improve the lives of children like Norah by supporting Lurie Children’s CAMP team’s research efforts. Contact Sharon Hurwitz at 312.227.7248 or shurwitz@luriechildrens.org.