As the young girl’s giggle echoed through the Southeast Decatur home, Krista Mayfield grew silent and smiled.
“There is so much for me to be thankful for this year. I am so thankful for Maddi’s new liver, her donor and the donor’s family. One day, I hope I can thank them face to face for this selfless act of life. Because of them, I still have my little girl,” Mayfield said.
On this Thanksgiving Day, Mayfield’s prayers of thanks extend to the doctors at Children’s Hospital of Vanderbilt, who oversaw her daughter’s liver transplant, and the donor, who gave 9-year-old Maddi Rae Kircher a second chance at life.
“It has been a long and difficult journey. When the day of transplant came, the doctors were giving her less than a month with the liver she had,” Mayfield said. “But now, Maddi is as healthy as she has ever been. It’s such a blessing.”
The nine-year journey through periods of frustration, fear, relief and gratitude began five weeks after Kircher’s birth in March 2010.
Looking at her newborn, Mayfield, a first-time mother, knew something was wrong — the whites of her daughter’s eyes were yellow, her skin had an orange tint and she cried nonstop.
“I was a young mom and there’s so much you don’t know. I thought she had colic at first, but the symptoms kept on and the doctors started doing tests,” Mayfield said.
After two years, dozens of tests and a surgery, doctors diagnosed Kircher with Alpha-1 Antitrypsin Deficiency, a genetic disorder that affects the lungs and liver.
“I had never heard of it. This whole journey has been a learning experience, especially with there being so little information out there. The first thing I did was Google the disease. I had to know as much about the disease as the doctors so I could advocate for my daughter,” Mayfield said.
She learned the rare disorder commonly misdiagnosed as asthma or COPD affects 1 in every 2,500 Americans. She learned of those carrying the disease, only 5% experience liver failure and 1% undergo a liver transplant. She learned that both she and Kircher’s father carried the defective Alpha-1 gene, making her daughter one of the most extreme cases.
“There was a one in 1,000 chance that we would both be carriers. It was like a double whammy for Maddi,” Mayfield said.
According to the Alpha-1 Foundation, 19 million people in the United States carry one defective alpha-1 gene.
While Kircher experienced liver failure for most of her life, she remained stable until the summer of 2018. Seeing her daughter’s slow day to day decline — her spleen was enlarged, her stomach was protruded and she was covered in bruises — Mayfield sought out a second opinion for treatment at Children’s Hospital of Vanderbilt that July.
There, the medical team told Mayfield that Kircher’s liver would not last another two years. By the time Kircher underwent a transplant on Feb. 5, the timeline had shrunk to one month. Her pediatric end-stage liver disease score had reached 39 out of a maximum of 40, the designation for complete liver failure and no hope for life. Her spleen measured 19 centimeters, almost twice the size of a normal 8-year-old’s 10-centimeter spleen.
From Kircher’s first appointment at Vanderbilt in July 2018 to the transplant, she underwent several hospital stays, a paracentesis to drain fluid from her stomach and, every six weeks, an attempt to put surgical rubber bands around esophageal veins on the verge of rupturing.
On Feb. 4, Mayfield received a call from Vanderbilt — a liver for Kircher was available. The surgery, which began at noon on Feb. 5, lasted 10 hours.
“Those were some of the hardest hours of my life, but Maddi’s always been so strong, I had no choice but to suck it up and cry later,” Mayfield said. “When she came out of surgery, her face looked completely different. She looked healthy.”
After a two-week recovery period in the hospital, Kircher and Mayfield stayed at the Ronald McDonald House in Nashville for three weeks as the medical staff regulated her medications and evaluated her progress.
Now, nine months after the surgery, the Walter Jackson Elementary fourth grader, who Mayfield described as sassy, adventurous and full of life, dreams of the future.
“I’m thankful for my liver and to be home," Kircher said. "I can’t wait to start doing jujitsu again and playing sports. I want to do soccer and football and tackle everybody."
“She’s never been able to play sports before because of her liver. We still have to be careful. She’s still small for her age, she is at a 100% risk for all types of cancers and she has to do infusions to boost her white and red blood cells. But, those are things I will worry about. She can just be a kid, finally,” Mayfield said.
To prevent any other child from going through what Kircher experienced, Mayfield hopes to raise awareness of Alpha-1 and to encourage individuals to get tested for the genetic disorder.
“You need to know what you’re passing along to your children. I wish I would’ve known. It wouldn’t have changed me having her, but it has prevented me from having more kids. I don’t want to put another child through it,” Mayfield said. “We were one of the lucky ones because of Maddi’s donor. We are more and more thankful for him each day. We will see that his memory is forever honored and celebrated.”
To find out more about alpha-1 or to receive a free test kit, visit alpha1.org.