Total Colonic Aganglionosis (Hirschsprung's Disease)
No parent ever expects their child to experience health issues, especially when the entire pregnancy went smoothly and according to plan. But that was exactly the case for Jessie and Michael Anthony and baby Harper. The night before her very first pediatrician checkup, Harper began exhibiting signs that something just wasn’t right. “She began spitting up, and it was a bright green, almost neon color,” Jessie explains. “By the time we went to the pediatrician appointment the next day, she started getting worse and vomiting much more.”
The Anthonys were immediately sent to the emergency department at Children’s of Alabama, where Harper was soon admitted. For two weeks, test after test was performed, and doctors diagnosed her with total colonic aganglionosis, also known as Hirschsprung’s disease. This condition occurs when the wall of the intestine does not have needed nerve cells, which compromises normal motility and prevents stool from passing through the intestine. “When it’s your first child and you’ve never heard of the disease before, a scary situation is made even harder,” Jessie says. “But everyone at Children’s was great. They talked through everything with us and made sure we understood what was going on and that Harper was going to be ok.”
Surgery was Harper’s only option, and it came just a few days after diagnosis. The surgery required doctors to remove all of her large intestine and most of her small intestine, and then insert a colostomy bag. She also had a G-tube inserted into her stomach. “Since her body cannot absorb nutrition, she’s on PN (parenteral nutrition) and SMOFlipid through a central line catheter 15 hours a day,” Jessie says. “She also has a variety of G-tube feedings throughout the day as well.”
After a successful surgery, Harper spent upwards of four months in the NICU at Children’s. Now she only requires a visit to Children’s Infusion Clinic every six weeks for an iron infusion and checkups with the GI Intestinal Rehabilitation Clinic several times a year. “She never had any complications from her surgeries, and she’s done just great,” Jessie adds. “They keep a close eye on her at the GI Clinic and make sure she’s getting all of the nutrition that she needs.”
Harper continues to receive a large part of her nutrition through fluids every night, as well as part of the day while she is at school. “She takes it all in a little backpack with her to school, and she does great,” Jessie says. “When she’s not hooked up to the pump, she runs around and plays just like any other child her age.”
That running around includes playing soccer, which Harper has done the past couple of years and has become one of her favorite pastimes. As Harper has shown time and again, there is no slowing her down. “We’re so grateful to the doctors and nurses at Children’s,” Jessie says. “They have always provided the very best care for Harper, and they have been so supportive of our family. They truly are the best.”
