A mother and a NICU nurse each shares her account of how an infant with a rare skin condition received unique and life-saving care at Maria Fareri Children’s Hospital.
As told to Melissa F. Pheterson
As seen in the September/October 2019 issue of Advancing Care
Anna Riley, of Highland, in Ulster County, was born on September 17, 2017, with a rare genetic disorder called Harlequin Ichthyosis, in which skin cannot maintain its moisture and grows at 10 times the normal rate. Infants born with this condition have hard, thickened skin that forms diamond-shaped plates separated by cracks; because the cracking restricts blood flow, these children often lose fingers and toes.
As Anna’s mother, Jennie Wilklow-Riley, was recovering from her C-section at the hospital, doctors advised Anna’s family to bring her to Maria Fareri Children’s Hospital, a member of the Westchester Medical Center Health Network (WMCHealth) and the children’s hospital for the Hudson Valley and Fairfield County. There, she received highly specialized care at the hospital’s Level IV Regional Neonatal Intensive Care Unit (RNICU) and a surgery that saved her hands and feet.
To learn more about the Regional Neonatal Intensive Care Unit at Maria Fareri Children’s Hospital, visit mariafarerichildrens.org/RNICU.
Wilklow-Riley maintains an Instagram page with more than 45,000 followers, @harlequindiva, dedicated to sharing information and raising awareness about her daughter’s condition.
Here, Jennie Wilklow-Riley and Dee Byrnes, RN, Anna’s primary nurse in Maria Fareri Children’s Hospital’s RNICU, share their stories of Anna’s first month of life and the bond forged between nurses and families.
From Anna’s Mother, Jennie Wilklow-Riley
“From the start, the nurses did more than just nursing. They sat with my husband and had coffee. They offered reassurance. They went home at night, researched Harlequin Ichthyosis and brought back information in the morning to do the best for Anna. They’d ‘gown up’ to give her special bleach baths. When they wrapped her up, she looked like a little mummy!
“The whole RNICU went above and beyond to protect Anna from germs, help her wounds heal and help her extra skin come off. Dee stayed by my side every day, through the most stressful moments. At first, Anna couldn’t be out of the humidity-controlled isolette for more than a few minutes. Dee worked with my suggestions and tried her own ideas, like wrapping Anna in plastic, so I could hold her. She helped me find solutions to every setback and never gave up.
“I wanted to give her a real bath before she went home, so Dee helped me experiment. During that first bath, Dee noticed by Anna’s breathing that she had gotten overheated. I got anxious, but Dee kept calm. We realized we needed to keep the temperature very specific; Anna liked it between 96 and 98 degrees. Everyone was very supportive, especially the nurses, making sure I was included in Anna’s care.
“Today, Anna takes a daily, three-hour-long bath at home. She has physical therapy twice a week and occupational therapy once a week. She’s very bright and has started to speak and to walk. She loves playing with clothes, jewelry, sunglasses and makeup. She’s a ‘girly girl.’”
From Anna’s NICU Nurse, Dee Byrnes, RN
“Anna’s case was so rare that we had to formulate a unique care plan. It really took a team effort; concepts like bleach baths took us by surprise. You don’t usually put the words ‘bleach,’ ‘bath’ and ‘baby’ together!
“We focused on details, down to her diaper change. We had to moisturize around the clock, so her skin wouldn’t harden. She had so many open, exposed areas that I had to wear sterile gloves and a mask, and cover my hair because we didn’t want her to get an infection. We had to track her fluid intake and make sure she didn’t get dehydrated.
“We were always fine-tuning her baths. We were worried about her temperature: She was constantly burning calories, and we didn’t want our baths to be the source of calorie-burning. We had to solve problems as basic as how she could wear clothes. We wondered whether they would absorb too much moisturizer. It was trial and error. But the hardened skin that had built up in utero slowly came off, and that can be maintained with exfoliating baths.
“Anna didn’t really have any setbacks in our care. I attribute that to her family and the support system she continues to have. There was never a doubt in my mind that she would continue to thrive.”
Anna arrived at Maria Fareri Children’s Hospital needing surgery right away. Her hardened skin was rapidly strangling blood supply in her fingers, giving her care team just 24 hours to act. “When the nurses saw Anna’s hands were purple and dying, they called me right away,” says Elizabeth Zellner, MD, pediatric plastic surgeon at Maria Fareri Children’s Hospital. “That immediate action was key.”
Performing the surgery with local anesthesia, Dr. Zellner made two cuts down each finger and across Anna’s wrists, releasing the tightness while preserving her tendons and nerves. “Within 30 seconds, her hand totally turned around, and because she produces so much skin, she healed quickly, with very little scarring.” She was one day old.
“As a surgeon I had the opportunity to save something you can’t replace,” says Dr. Zellner. “It’s meaningful when those moments arise.”
Like the nurses, Dr. Zellner credits Anna’s family. “Jennie has been an amazing advocate for Anna. Some children with this condition can’t grow due to the metabolic demands on their body, but Anna is healthy and thriving.”
Visit us at Maria Fareri Children’s Hospital, a member of Westchester Medical Center Health Network, to learn more. Advancing Care. Here.
Photos by John Halpern