“I was always tired and couldn't make it through the day. Then I started on a home ventilator, and it just made a world of difference.”
Using noninvasive treatment to rest her breathing muscles every night gives Jacquie Eubanks the energy to keep up with her demanding job as an NICU nurse every day.
When Jacquie Eubanks was born, her doctors didn't give her much of a chance. She was very small and had several congenital conditions, including a cleft palate and severe scoliosis. Jacquie's father was in the Air Force, so the family moved a lot, living all around the world. When he retired, they settled in a small town near Dover, Delaware.
Jacquie lived in a close-knit neighborhood with plenty of friends who still stay in touch today. She wore a brace because of her scoliosis, a curve in her spine that affected her movement and breathing. "I am very small anyway — I only weigh like 60 pounds — and I'm about the size of a 10-year-old," she said. "And I had a really bad curvature. It was almost like a candy cane." Between the curve in her spine from scoliosis and her small body frame, "everything is just compacted in my chest."
When Jacquie was a junior in high school, doctors recommended spinal fusion surgery to correct the scoliosis. She was in traction for several weeks, then in a full-body cast for ten months.
Confusion and Frustration on the Way to a Diagnosis
Doctors didn't think Jacquie would live into her 20s, but she wanted to prove them wrong. She attended college, got married, and gave birth to two beautiful, healthy children.
She became a neonatal intensive care unit (NICU) nurse at ChristianaCare Health System in Delaware, a position she's now held for 25 years. It's a physically demanding job that keeps Jacquie on her feet much of the day. "Sometimes I work 80 hours in a week, running around the hospital. I'm not one to call out or take a lot of sick days," she said.
Jacquie started to feel really out of breath one day on her way into work. It was a long walk from the parking lot to the NICU, but usually not a problem. She scheduled an appointment to see a specialist about it.
The doctor thought she might have pulmonary hypertension. "I was confused because I've worked with patients who have pulmonary hypertension. They are on ventilators, on nitric oxide, and I definitely didn't need that," said Jacquie. "But he was a doctor, so I just trusted what he said."
He scheduled her for a cardiac catheterization procedure, which resulted in complications that sent Jacquie looking for a new doctor.
Finding the Right Treatment Team for Chronic Respiratory Insufficiency
Jacquie started doing some research to find a new specialist. She lived in Delaware but had treatment at Children's Hospital of Philadelphia (CHOP) as a child, so she looked into Penn Medicine. "I found out they had a pulmonary hypertension clinic, so I started there," Jacquie said.
She took a copy of her echocardiogram and cardiac catheterization results to see Kerri Akaya Smith, MD in the Penn Medicine Pulmonary Hypertension Program. Dr. Smith took one look at the test results and saw that her shortness of breath was not the result of lung disease. It was from another condition that caused blood pressure to build up behind her lungs. Dr. Smith referred Jacquie to John Hansen-Flaschen, MD in the Fishman Program for Home-Assisted Ventilation. "I'm very thankful that Dr. Smith got me connected to this program," said Jacquie.
A Surprise Diagnosis
At Jacquie's first appointment, Dr. Hansen-Flaschen ordered respiratory tests to check pulmonary function and lung capacity. Her breathing muscles were very weak and there was a lot of carbon dioxide in her blood. That was a sign that her severe scoliosis was causing early chronic respiratory failure. During a sleep study, Jacquie's blood oxygen fell dangerously low at night. Dr. Hansen-Flaschen thought she would benefit from home-assisted ventilation.
Dr. Hansen-Flaschen and the Fishman Program team started Jacquie on noninvasive ventilation with a mask. It allows her breathing muscles to rest while she sleeps at night.
"I feel totally, 100 percent different than when I first went in," said Jacquie. "I was always tired and couldn't make it through the day. Then I started on a home ventilator, and it just made a world of difference."
She sleeps with the ventilator every night and gets a full seven to eight hours of sleep. "Without it, I probably wouldn't be able to work so many hours a week and do all the things I love," said Jacquie.
Living a Healthy, Rewarding Life
Dr. Hansen-Flaschen believes that early diagnosis likely prevented a cascade of events that would have seriously affected Jacquie's quality of life: without support, her breathing muscles would have gotten progressively weaker. Without strong lungs, carbon dioxide would have continued to build up in her body. Eventually, Jacquie may have been hospitalized urgently for invasive ventilation and sent home with a tracheostomy.
That kind of extreme treatment is usually not necessary when someone gets proper breathing support with a home ventilator. "What happens too often is that people just start to get sick. They're not sleeping at night, they get more and more tired, and then get symptoms like headaches and foggy brain," said Dr. Hansen-Flaschen. "People think they're suffering from dementia, or doctors think they have heart failure."
Many doctors don't think about how other conditions — such as scoliosis or a misshapen chest — can contribute to respiratory failure. Jacquie feels lucky to be at Penn Medicine in the care of a team of pulmonology specialists with extensive expertise in home breathing support.
"I'm so happy to be involved with Penn Medicine; I owe them my health," said Jacque. "They keep me on track, and I'm doing great, thanks to Dr. Hansen-Flaschen and everyone in the Fishman Program."