Bouncing back from a colorectal malformation: Reagan’s story

care for perineal fistula

It’s a situation few parents ever imagine during a healthy pregnancy. Yet there Laura and Jared Maxwell were, waiting anxiously in the Division of Genetics and Genomics at Boston Children’s Hospital as their infant daughter, Reagan, under went a barrage of tests. After their little girl had been born with a congenital anomaly just a few weeks earlier, physicians wanted to make sure that she didn’t have other related genetic syndromes that could affect her heart, kidneys and other organs. “It was one of the most terrifying days of my life,” recalls Laura.

Fortunately, Reagan didn’t have additional anomalies — but she would need surgery to correct a perineal fistula. In this type of anorectal malformation, a baby’s anus is located outside the sphincter muscle and in the incorrect location. As a result, a child may pass stool through the misplaced opening.

More than expected

At first, the Maxwells didn’t realize the gravity of Reagan’s problem. “We thought she’d have surgery, be done and move on,” says Laura. “But soon, we realized how serious it was.” After they were referred to Dr. Belinda Dickie at Boston Children’s Colorectal and Pelvic Malformation Center, the family learned that — although surgery can correct the anatomical anomaly — anorectal malformations are chronic conditions that require lifelong follow-up. “We were really heartbroken that our daughter would have to go through all this,” says Laura.

Dr. Dickie explained that she and her team would surgically relocate Reagan’s rectum so that it connected to her anus correctly. She would need to spend a week in the hospital and would require at-home dilations to ensure that the procedure remained a success. Because the surgery is best performed when a child is still young, Reagan underwent it when she was just 4 months old.

surgery to correct an anorectal malformation

Gaining perspective

The idea of their baby having surgery — and being under anesthesia — was frightening, but the Maxwells knew that they were making the right decision. While they waited in the hospital, they watched as older children and their families passed by. “We knew other families were there with even more serious problems,” says Laura. “It gave us some perspective, and we were grateful that Reagan’s issue is treatable.”

Indeed, when Dr. Dickie met with Laura and Jared after the procedure, she told them that it had gone even better than she had expected and assured them that Reagan would have a normal life. After the little girl could nurse and have bowel movements, she was able to go home. “It’s amazing how much she could bounce back after going through all that,” says Laura. “She was mostly back to normal in a few weeks.”

Reagan with her family

A challenging time

But the journey hasn’t always been easy. Now a year old, Reagan was recently hospitalized due to constipation, a problem that her parents and care team address with a high-fiber diet. “Dr. Dickie has been very responsive to our concerns,” says Laura. “She and her team help guide us to the right foods for Reagan and answer questions when we have them.”

Another challenge has been explaining to family and friends what a perineal fistula is and what treatment of this sensitive issue involves. “It’s a really weird surgery to have to explain,” Laura admits. “We initially weren’t going to tell a lot of people.”

But the couple found support from a surprising source. During the time when Reagan was still facing surgery, they discovered that the young son of the contractor renovating their home had experienced a similar, but more severe, problem as a baby. “It was comforting to hear that his son, who is now 8, is living a totally normal life,” says Laura. “It’s nice to know that other people have walked in our shoes and come through on the other side.”

Reagan on her birthday

On the road to good health

Reagan herself has also made the experience easier. An easygoing, sweet child like her older brother, she’s the couple’s “little princess.” All told, she’ll require about 20 years of clinical follow-up, but the next real hurdle will appear in a few years when she begins the potty-training process.

For now, the Maxwells are grateful that Reagan is happy and on the road to good health. “Even though it was stressful at the time, we’re glad she had the surgery when she was so young,” says Laura. “I’ll never forget it — but she won’t remember.”

Learn about the Colorectal and Pelvic Malformation Center.