Born in China with imperforate anus, in which the anus does not fully develop in utero, Sean Hoolan underwent corrective surgery two days after birth. The good news is the operation, in which surgeons opened the area where the anus should be, went well. The not-so-good news? Sean did not receive sufficient follow-up care, including daily dilation of the rectum to prevent scar tissue from contracting, essential for a good long-term outcome. Consequently, Sean suffered damage to the nerves of his bowel that required another surgery when he arrived at the Johns Hopkins Children’s Center at age 3 with his adoptive parents, Mary Eileen Aldana and Paraic Hoolan.
“Dr. Hackam said Sean’s surgeons in China did a wonderful job, but they didn’t continue doing dilations for the next several months, so his anus closed up and backed up his digestive system,” says Aldana. “Had they done that follow-up, we wouldn’t be here.”
The here Aldana referred to is the Johns Hopkins Children’s Center’s new colorectal surgical center, one of six surgical centers of excellence David Hackam planned to develop after taking the helm as Johns Hopkins’ new pediatric surgeon-in-chief in the fall of 2015. The other centers include fetal medicine, short gut/intestinal care, trauma/burn, oncology and vascular anomalies.
For the colorectal center, Hackam recruited pediatric surgeons Alejandro Garcia and Isam Nasr, who specialize in correcting colorectal conditions like anorectal malformations, constipation, Hirschsprung’s disease, and inflammatory bowel disease. But in addition to an evidence-based center staffed by surgeon-scientists who could develop innovative, minimally invasive surgical approaches for colorectal problems, Hackam wanted a bowel management clinic fully staffed with all the pre- and post-surgical services needed by patients like Sean Hoolan.
“Our goal is to work with gastroenterologists, nutritionists, nurse practitioners, psychologists, radiologists and urologists so we can not only provide a consultative surgical opinion but also the best follow-up care, helping families who struggle with bowel management,” says Garcia. “It’s important to have a one-stop shop where all the important issues are being discussed by one group, and also to involve the knowledge of multiple teams, especially for the more complex patients.”
Maria Oliva-Hemker, director of the Division of Pediatric Gastroenterology, Hepatology and Nutrition, agrees. “This surgical center allows patients here to have access to a wide variety of expertise in intestinal surgeries for children,” Oliva-Hemker says. “And it will certainly benefit children who come to Johns Hopkins for evaluation and treatment of inflammatory bowel diseases like Crohn’s disease and ulcerative colitis.”
While the experience and skill of the surgeon in correcting such complex colorectal conditions are vitally important for these patients and their families, says Nasr, an expert in treating patients with Hirschsprung’s disease, managing these patients following surgery is just as important. “You can’t just operate and that’s it—the key for these kids is long-term follow-up in our bowel management clinic,” says Nasr, who co-directs the clinic.
Indeed, after Nasr performed a posterior sagittal anorectoplasty to create a functional anus and rectum for Sean, his parents regularly attended the bowel management clinic. There they met with Nasr and pediatric nurse practitioner Margie Birdsong to learn how to use an instrument to dilate Sean’s rectum at home each day to prevent scar tissue from contracting.
Birdsong, a certified wound ostomy and continence nurse, coordinates the care of all patients in the clinic and guides parents on how to perform enemas and titrate laxatives for their child at home to achieve continence. Birdsong also conducts biofeedback testing to measure patients’ level of anal rectal muscle control and then prescribes at-home exercises to strengthen the muscle. Many patients tend to have little or no control over their sphincter muscle, which can be extremely embarrassing in school, sleepovers and other settings outside the home.
“The feedback tells me where the muscles are most weak, which allows me to home in on where they need to concentrate their exercises to strengthen their muscle,” says Birdsong. “I call patients and families on a regular basis to make sure the regimen we gave them is working and, if not, tweak it to get to the happy medium where every patient needs to be. A lot of the kids like Sean are really doing well but it takes a good four to six months of exercises.”
So, how is Sean, now almost 4, doing?
“He has no fear, no fear at all—he makes it all fun, jumps on the table for his X-rays, calls all the nurses by their first name,” says Aldana of Sean’s visits to the bowel management clinic. “His diet has been great, and his transition has been very good.”
For more information or to refer a patient, call 443-997-5437.