Crouzon Syndrome with Acanthosis Nigricans
Chandra and John Holland of Athens will never forget that day in March when doctors told them their youngest daughter had been born with Crouzon syndrome with acanthosis nigricans (CAN)—a very rare genetic condition occurring in just one per million births. She also was diagnosed with hydrocephalus and Chiari malformation. “Crouzon can cause disfigurement, breathing problems and even mental impairment,” Chandra explains. “We were told that Gracie might never walk or talk or hear. Shortly after that, our journey with Children’s of Alabama began.” Gracie’s first surgery at Children’s took place when she was just 1 month old. Her parents were told there was a 90 percent chance she would not survive the procedure. “That first surgery was a tracheotomy to help her breathe,” Chandra said. “At the same time, she also had a central line put in to prepare for her first major surgery, which took place a month later. During that surgery, doctors cut her from ear to ear, pulled her face down and removed 80 percent of the back of her skull.” Today, 22 years and 35 surgeries later, all you can say about Gracie is that she is living up to her nickname – Amazing Grace! With her faith, tenacity, courage and zest for life, Gracie has surpassed her parents’ and doctors’ most hopeful expectations – and life is truly great for the Judson College student. Gracie’s 35th surgery, performed by her plastic surgeon since age 2 (John H. Grant, III, MD, director of the UAB Cleft and Craniofacial Center at Children’s), took place in the summer of 2015. “I wasn’t nervous at all about this major surgery,” Gracie said. “I guess that has to do with maturity. I also remember thinking that this would likely be the last time I would be anesthetized with gas that smells like bubble gum! They always gave us a choice at Children’s – and I always chose bubble gum!” Gracie has literally grown up with Children’s. When she turned 21 in 2016, Gracie began seeing adult neurosurgeons and specialists. “She has been a patient of more than a dozen surgeons,” Chandra said. “She also has been seen at North Alabama Children’s Specialists, Children’s South and Children’s Russell campus – all buildings, old and new – as an inpatient and an outpatient!” Gracie said she went through five years of major orthodontic and dental work over a span of 10 years without ever visiting Dr. Grant. “But I knew that when I had that last critical surgery on my nose, I wanted him to do it,” she says. “I wanted him to finish the work he had done on me through the years.” She remembers being at home in her bathroom when the bandages came off. “I looked in the mirror and there were joyful tears,” she said. “Dr. Grant had given me a bridge to my nose where I had never had one before, and he also corrected my lips and mouth to better cover the amount of gum that showed. For the first time in a long time I could tell a big change in my appearance – and I could finally actually breathe through my nose!” Throughout two decades of surgeries, Gracie has had more than one brush with death. “I know our faith and the prayers of family and friends were responsible for getting us through all the really difficult times,” she said. “I know God is not done with me here.” Now it’s back to enjoying college life and preparing to pursue her dream of a career as a social worker in a medical setting. “I love the fact that I am at a small, faith-based college with professors who are so approachable,” Gracie said. “I really never pictured myself as being able to graduate from high school and go on to college and live a semi-normal life. “I sometimes feel I am the only person around who has been cared for by such a ridiculous number of medical specialists,” she added. “But I am very grateful to each and every one of them. I know it was a team effort and that each person played a part in helping me present my best self. That’s really the reason I chose to major in social work. I want to be able to give back to an area that has helped me all these years.”
