Congenital Nephrotic Syndrome and Kidney Transplant: Molly’s Story
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Four-year-old Molly was born with a kidney condition that required hours of dialysis every day, numerous medications and constant restrictions on her playful activities. After she received a new kidney in March, her life was transformed. “She went from being so tired and yucky feeling to having endless energy and freedom,” her mother, Stephanie, reports happily.
Prior to birth, Molly was diagnosed with congenital nephrotic syndrome, a condition that typically leads to irreversible kidney failure. She needed 12-plus hours of dialysis through the night to filter her blood, which “wiped her out — she had no energy,” says Stephanie. Molly would vomit up anything she ate, so she received nearly all her nutrition through a tube into her belly. “She was so small, we spent a lot of time just trying to get her to gain weight.” She had to be vigilant about avoiding infections, and so couldn’t do things like go in a pool or play where she might get dirty. Her life was filled with appointments with Children's Hospital of Philadelphia’s (CHOP) Pediatric Kidney Transplant and Dialysis Program.
At 2 ½ years old, she qualified for a kidney transplant, and the family’s hopes were raised when a family friend offered his kidney. Unfortunately, the friend’s kidney was not a good match for Molly. But Molly and the family friend still had an option, thanks to CHOP’s involvement with the National Kidney Registry (NKR) through a partnership with Penn Medicine.
How the National Kidney Registry works
The NKR matches and facilitates living donor kidney transplants all over the country. A willing donor who isn’t a good match gets listed on the registry on behalf of the patient, and a better matched donor on the registry gets identified for the patient. Sometimes this process involves a direct donor exchange between two families, but often it works more like a domino effect. The family friend donated his kidney on behalf of Molly, and the NKR used its network to track down a compatible kidney for Molly. The whole process took about a year.
When the family was informed that Molly had a donor, “It was unbelievable — just surreal,” says Stephanie. “You hear about the hope of a transplant and life getting better, but, while you wait, you’re not sure that will ever happen.”
What happened is that Molly recovered from the transplant surgery quickly and now can just be a kid like her friends. Dialysis and tube feedings are things of the past.
“She was never strong enough or energetic enough to run and now I watch her run down the sidewalk for hugs when I get home. She can go to the park, slide down the slide, and go to the pool. She eats three meals a day, plus snacks, and she likes to bake and cook.”
CHOP’s partnership with Penn Medicine and the NKR was crucial to making this transformation happen. “The NKR means hope to our family,” says Stephanie. When they discovered that their friend wasn’t a direct match, “It felt like a door just closed for her. When he accepted to go into the NKR, we had restored hope. To know there are two people that are willing to sacrifice to save lives of people they may never know is humbling. Molly often fondly thinks about both of those people. She considers both of them her donors.”
