Fresh start for Lyra
Implant gives 5-year-old with rare breathing disorder chance for normal future
This is shaping up to be a life-changing year for Lyra Havlin — and she only just turned 5.
In June, the Palm Coast girl underwent major surgery that will eventually allow her to breathe on her own while she is asleep without being hooked to a ventilator. And it should eliminate the need for the tracheostomy tube in her throat that connects her to the ventilator.
Lyra has congenital central hypoventilation syndrome, or CCHS, which occurs in fewer than 1,000 people worldwide. According to the American Thoracic Society, CCHS is congenital — it’s a mutation of the PHOX2B gene that is not limited to gender, race or social status. Lyra has CCHS; Liam, her fraternal twin, does not.
[READ MORE: Baby with rare breathing disorder comes home]
Diagnosed shortly after the twins were born on Sept. 5, 2014, Lyra spent her first four months in the neonatal intensive care unit of Wolfson Children’s Hospital in Jacksonville, coming home just before Christmas that year. She underwent a tracheostomy when she was a month old and was fed through a feeding tube for much of her first year.
She also had a heart pacemaker implanted during that first year, among other surgeries.
Now at 5-years-old, Lyra is an energetic, happy kid. She loves taking dance classes and attends pre-kindergarten classes weekly with Liam at United Methodist Christian School in Palm Coast.
"She goes to school five days a week. Liam goes five full days a week, she goes five days but only two of those are full days at the moment," said her mother, Cheryl Zierk. "We’re trying to build up her tolerance. She still gets really tired."
Hope on the horizon
Lyra's surgery in June to implant a diaphragm pacemaker was a big step toward increasing her eventual independence.
The operation, which took about three to four hours, was performed at Children’s Healthcare of Atlanta, one of only five hospitals in the country that offer the surgery, by a team comprised of a pediatric surgeon, pulmonologist and cardiologist, as well as an anesthesiologist and others. Lyra was in the hospital for 10 days and was the third child to have the surgery in that hospital.
"This is a surgery that is done for children that have Lyra’s condition," her doctor, Ajay Kasi, said in a phone interview. "Unlike a traditional treatment that pushes air into the lungs, the pacemaker causes air to get pulled into the lungs to help the patient breathe."
The benefit of the diaphragm pacemaker is that by next spring, Lyra shouldn’t need to be attached to the ventilator at night and she will be able to have her tracheostomy tube removed — something Lyra is hoping for.
[SEE ALSO:Baby with rare breathing disorder celebrates 1st birthday]
"She’s beyond excited," Zierk said. "She keeps saying, ‘I can go swimming? I’m gonna go swimming?’ My goal through all this too is to make her more of an independent little person and to go into kindergarten and to be more normal."
Surgical challenges
The surgery itself, Kasi said, is technically challenging. Electrodes are placed on the phrenic nerves, which are behind the heart, so her lungs had to be deflated one at a time so the phrenic nerves could be reached, the electrodes placed and tested, then each lung was re-inflated before the procedure turned to the other lung.
She also has receivers implanted under her arms, and at night, two circular antennae are taped to the receivers so she can sleep without the ventilator.
The process to wean her off the ventilator will take some time, Kasi said. "Because the diaphragm is not accustomed to being stimulated that way, we start with only using one to one-and-a-half hours at a time to increase her muscle endurance."
Zierk said it was difficult at first to convince Lyra to allow the antennae to be taped to her body, but now one of Lyra’s nurses thinks she sleeps better than she did on the ventilator. Each week Zierk increases Lyra’s time on the pacer by 30 minutes, and Lyra’s now up to 3½ hours per night. Zierk said she believes Lyra will be completely off the ventilator by spring.
"Around springtime next year we’ll go back up (to Children’s Healthcare of Atlanta) for a sleep study with the trach capped or closed off somehow, and that’s when we can really start talking about taking the trach out."
[READ MORE: Keeping up with Lyra]
'Amazing little person'
As Lyra grows older, the pacemaker will function like a lung and she won’t need repeated surgeries as she grows, Kasi said. However, because the receivers are metallic, she won’t be able to have an MRI scan should she need one. And some physical activity will be limited.
"Absolutely no contact sports, gymnastics, things like that," Zierk said. "(The receivers) fit right underneath the skin, and she’s a petite little girl, so if she were to get hit even playing, it could cause damage and we’d be back in there doing the procedure over again."
Lyra still has nurses around the clock, though once she’s completely off the ventilator, she will need only a night nurse, Zierk said.
Watching Lyra running and playing with her brother, it’s hard to believe that she has a life-threatening medical condition, let alone that she recently underwent major surgery.
"She bounces back," Zierk said. "She’s just a strong, amazing little person."
More about Lyra
Staff Writer Laurie Hahn has been following Lyra Havlin’s progress since shortly after the Palm Coast toddler was born with a rare breathing disorder in 2014. Follow the links to read Hahn’s previous stories and find out more about Lyra and her family:
-Family learning to cope with newborn's rare breathing disorder
-Lyra comes home from the hospital
-Lyra celebrates her 1st birthday