Kathleen is a vibrant 5-year-old — a “girly girl” who takes ballet and plays t-ball wearing a pink batting helmet. Even her parents, Jim and Tegwen, say it’s sometimes hard to believe that Kathleen spent a combined two of her first four years of life at The Heart Center at Children’s, where she ultimately underwent the heart transplant that transformed her life.
Kathleen’s mom, Tegwen, was 23 weeks pregnant when an ultrasound technician became concerned when she couldn’t detect the lower left chamber of Kathleen’s heart during a routine exam. She referred Tegwen and her husband, Jim, to Heart Center cardiologist Nina Gotteiner, MD, who has special expertise in fetal heart conditions. Last year, more than 12,000 children received care at Children's.
That same day, Dr. Gotteiner confirmed the technician’s suspicions: Kathleen had a rare form of single ventricle heart defect. Because she had only one chamber pumping blood from her heart to her body, Kathleen would need a series of three open-heart surgeries spaced out over several years to reconfigure her heart.
“We were told that without immediate surgery her chances of surviving more than a week after birth were slim,” says Tegwen, who had previously given birth to two healthy boys.
Just hours after Kathleen was born at Prentice Women’s Hospital at Northwestern Memorial Hospital, she was transported by ambulance to Children’s. Four days later, a surgical team led by Carl L. Backer, MD, Head of the Division of Cardiovascular-Thoracic Surgery, successfully performed the first surgery. Dr. Backer, Surgical Director of the Heart Transplant Program, would ultimately perform all of Kathleen’s operations.
After Kathleen’s third surgery, which took place shortly before her fourth birthday, she seemed to heal slowly. Her mom says she had trouble keeping up with other kids and, in general, felt “crummy.” She also developed a cough that sometimes brought up thick strands of mucus.
Tests determined that Kathleen had acquired an extremely rare condition called plastic bronchitis, a potentially devastating form of lung obstruction caused by root-like casts of mucus that form in the bronchial branches. For a year she underwent chest physical therapy, using an inflatable vest to loosen up the casts.
The treatments left Kathleen exhausted, and Jeffrey Gossett, MD, Kathleen’s primary cardiologist at Children’s, consulted with colleagues across the country. While there wasn’t much in medical literature about treatments for her unusual combination of conditions, what he learned convinced him that Kathleen’s best — and only — chance was a heart transplant.
“I explained to Kathleen’s parents that if we did nothing she would not survive,” says Dr. Gossett. “While I couldn’t guarantee that a heart transplant would reverse the lung problems, I was encouraged by what I had learned about similar patients who had undergone transplants.”
Four months after Kathleen was placed on the transplant list a suitable donor heart became available. The 13-hour surgery under the direction of Dr. Backer went smoothly, and Kathleen spent two months recovering at the hospital. After the first month, her lung problems had virtually disappeared. Today, a year after her transplant, Kathleen has normal lung function.
“We feel like we have a new child,” says Tegwen, who raves about Kathleen’s medical team. “The other day she was talking about how her ‘old’ heart kept her from doing certain things, and then she said, ‘This new heart is great. It‘s a keeper!’ Children’s really is a place that gives parents their children back. Just spend five minutes at Children’s and you can’t help but think, ‘This is a truly amazing place!’”
Inspired by Kathleen’s case, Dr. Gossett initiated a research project through the Pediatric Heart Transplant Study group to gather data on the fewer than 20 children with plastic bronchitis who have received heart transplants. Dr. Gossett says their goal is to provide more information and increased hope for parents of children like Kathleen.
Story originally appeared in the May 2011 issue of Heroes Update.