By Mike Brue
GRAND FORKS – There they were, mom and daughter, together again.
In an air ambulance.
High above the Minnesota countryside, headed southeast from Grand Forks on the North Dakota border to the Twin Cities. Again.
An anxious Amanda (Proulx) Richardson looked at her little girl, feverish and in pain, a nasal gastric tube adding to her discomfort. Just like before. Except this was mid-September 2013, and Peyton was 6, not an infant born the day earlier.
“It was almost like a flashback,” Amanda recalls now. “But here it was six years later. I kept thinking, ‘What progress had we made?’”
Lots of progress, or so it once seemed.
Amanda and Kyle Richardson’s oldest child was an enthusiastic little figure skater and piano player, a fun-loving first-grader at Grand Forks Lake Agassiz Elementary School and playful older sister to two little brothers, Caleb, 1, and Rylan, 4.
“She’s really outgoing and she’s spunky,” Peyton’s mom said. “She’s always smiling, always in a good mood, always full of energy.”
Sometimes, though, Peyton also was a little girl with a tummy ache.
Once or twice a week, her abdomen would hurt, caused by chronic constipation. Usually, the Richardsons could provide Peyton some relief with a doctor-recommended laxative, but it was not a cure.
Life for Peyton since birth has included Hirschsprung’s disease, a condition that contributes to an inability to pass stool. Hirschsprung’s babies are born with missing nerve cells in the muscles of part or all of their large intestine, or colon, which prevents the colon from relaxing enough to pass stool. That leads to obstruction.
Peyton, born Jan. 26, 2007, was unable to have a bowel movement in her first few days of life in Grand Forks. That led to the first air ambulance flight for mom and daughter. They wound up at St. Paul’s Children’s Hospital, where Kyle drove to join them. A surgical team removed a portion of Peyton’s nonworking colon and performed a colostomy, creating a temporary stoma through her abdominal wall to temporary allow feces to be expelled.
At three months of age, Peyton returned with her parents to the Twin Cities, and the baby girl underwent surgery again to undo the colostomy, pull her intestine down and reconnect it. Yet, there was a new issue: Peyton was diagnosed with hypotonic or poor muscle tone that’s sometimes called floppy muscle syndrome. It can affect muscle strength, motor nerves and the brain. The Richardsons also learned that Peyton briefly would stop breathing periodically during the night.
Peyton’s sleep issue disappeared after several months. Her muscle tone was treated with physical and occupational therapy at Altru Health System’s infant development program, until she turned 3.
By then, Peyton’s childhood had gained much normalcy.
Then, about a year ago, there was a change. Peyton “would wake up in the middle of the night, screaming in pain,” Amanda recalled.
A clinical visit revealed that she had a stricture, a small intestinal narrowing when her colon had been reconnected almost five years earlier. That apparently contributed to Peyton’s constipation. A doctor put her on Miramax, a laxative.
Though Peyton had complained of stomach pain at least twice a week, it was not the scream-prompting, doubling-over type. “You would never have thought anything more was wrong,” Amanda said. “She had been doing great for such a long time.”
On Sept. 14, which was Caleb’s 1st birthday, Peyton complained “her belly hurts” toward the end of a day of activity and company. The following day, a Sunday, she still didn’t feel well. The third day, a Monday, she asked to stay home from school.
When Kyle, a BNSF engineer, returned home that day, “he took one look and said, ‘I’m bringing her in. These are not her normal stomach pains,’” Amanda recalled. Kyle took Peyton to Altru Hospital’s emergency room. After some tests, the emergency room medical staff told him what they often had told Amanda during similar visits: Keep an eye on her and give her Miramax. If she vomits and has a high fever, bring her back to her regular doctor the next day.
On Sept. 17, Peyton began vomiting around 2 a.m., and her temperature rose to about 103 degrees. Amanda called the hospital but wasn’t assured about the need to take Peyton in. The mother “felt lost,” she recalled. “I cried.”
But shortly after 8 a.m., Amanda and Peyton were at the clinic, and when their pediatrician put a hand on Peyton’s stomach, the young girl screamed. She was admitted quickly to the hospital.
“Immediately, you think Hirschsprung’s. Your mind goes right to intestinal blockage,” Amanda recalled.
When the regular on-duty surgeon declined to operate on Peyton, recommending she be airlifted to pediatric surgeons who had worked with her, “I was actually a little relieved,” Amanda recalled. Kyle, too, had been suggesting that they request going to the Twin Cities anyway.
Kyle drove to the Twin Cities to join his wife and Peyton, joining them that night. Peyton underwent exploratory surgery Sept. 18. There was no blocking obstruction. Instead, the wavelike, propulsive contractions that help pushed stool through the colon and small intestine were not functioning fully. Furthermore, Peyton’s peritoneum – the protective membrane lining her abdominal wall and covering the organs inside – was infected. As a result, Peyton was diagnosed with non-mechanical ileus – but the cause of it still wasn’t clear.
The little girl was given antibiotics to fight infection. With each day, Peyton’s oxygen levels declined. Her nasal gastric tube, removed a few days after surgery, was reinserted with 24 hours when Peyton’s condition worsened. She wasn’t speaking, either.
“It was more worrisome, because Hirschsprung’s is known to us. It just leaves you wondering, ‘So what is it?’” Peyton’s medical team still was ruling out possible causes. Kyle, “because he felt like she wasn’t getting better and we were scared that maybe it wasn’t working,” made calls to other regional medical facilities, including Mayo Clinic, in case they decided they needed to relocate their daughter, Amanda said.
Eight days after surgery, x-rays revealed that the peritoneum inflammation had begun to subside slightly. “It was just like the best news we had gotten while we were there,” Amanda said. “It was the first time we felt hopeful that she was actually getting better.”
Ultimately, the Richardsons learned, the cause of Peyton’s problems was not linked at all to Hirschsprung’s disease, but primary peritonitis – meaning it spread from the lymph nodes and blood. That’s very rare, occurs in less than 1 percent of all peritonitis cases.
Thanks to Peyton’s history of Hirschsprung’s, she was likely treated more effectively – and more promptly – than she would have been otherwise, the Richardsons were informed. And that might have saved her life.
She was released 15 days after being admitted to St. Paul Children’s Hospital.
The Richardsons, though, had at least one scare about a week after returning to Grand Forks. Peyton was brought to Altru Hospital for observation after “she was bent over in pain for about 45 minutes.” Constipation was diagnosed again, and she received a temporary higher dose of Mirilax.
In a follow-up checkup Oct. 25, the Richardsons learned they’ll have to be on guard for another possible intestinal infection, entercolitis, which is a well-known Hirschsprung’s complication.
Plans remain in the works for a new evaluation of Peyton at the Mayo Clinic in Rochester, Minn. “We want to feel good about where she is now,” Amanda said. “We want to feel like we’ve done the preventative care. Sometimes you can’t prevent this, but we want to do everything we can to prevent what could happen.”
The Richardsons also plan to bring Peyton to a geneticist.
In the meantime, the Richardsons look back at Peyton’s latest ordeal and realize the circumstances could have turned far worse.
“The biggest thing I’ve learned is to always trust your instincts,” Amanda said. Had she not brought Peyton to the hospital that early morning when mom and daughter ultimately found themselves on an air ambulance to the Twin Cities, “I don’t think she would have been here right now.”
Peyton has missed about a month of school at Lake Agassiz Elementary, but she’s in a normal routine again. And she’s definitely eager to resume piano lessons.
“I’m going to practice my piano for when we get started again,” she told her mom recently.
Peyton making music. Amanda Richardson would welcome spending more time at home with her daughter at that happy place – over and over again.
Mike Brue is communications director for NDAD.