When Kirstin and Sam Paquin found out they were having a baby, they were full of the hopes and dreams for the future that many new parents share. They had no idea of the long road that lay ahead of them.
When Kirstin was 32 weeks pregnant, she noticed that her normally active unborn baby had stopped moving and kicking. She went to her OB/GYN, just to be safe. It’s a good thing she did. Scans showed an intestinal blockage in her unborn baby that required immediate action. Linley Brice Paquin was delivered by emergency C-section on that early February day, eight weeks before her due date.
Linley was transferred to University of Minnesota Masonic Children’s Hospital when she was just a few hours old for exploratory surgery. The pediatric surgical team discovered that a large portion of Linley’s small intestine had died and needed to be removed. During the surgery, the team found evidence that the intestinal blockage may have been caused by cystic fibrosis (CF), a serious, progressive disease that affects the lungs and gastrointestinal system. This proved to be the case: Linley was diagnosed with CF at just a few days old.
As Linley recovered from surgery over the next four weeks, her care team became concerned by her continued weight loss. Her digestive tract wasn’t working as well as it should be. To boost her recovery, Linley was enrolled in the hospital’s intestinal rehabilitation program. This multidisciplinary program supports patients who require nutritional support to grow. This support may include intravenous (IV) nutrition as well as various tube feeding mechanisms.
“After the surgery, Linley had 55 centimeters of small intestine left, whereas an average child of her age would have 170 centimeters,” said Pediatric Gastroenterologist Catherine Larson-Nath, MD, CNSC. “Our bodies absorb most of the nutrients we need through our intestines, so losing over half of her small intestine was a big setback.”
The key to the intestinal rehabilitation program’s success, according to Larson-Nath, is that it brings together team members from many different specialties—all of whom keep in close contact with each other and the patient’s family during every step of the patient’s healthcare journey. Because the program is based at University of Minnesota Masonic Children’s Hospital, the team has access to leading-edge therapies. These therapies include specialized lipid formulations, such as SMOF® lipids and Omegaven®, that have proven very helpful in getting Linley—and other children in the program—caught up with growth milestones.
When Linley’s weight didn’t rebound after her transfer to a hospital near her home, she came back to University of Minnesota Masonic Children’s Hospital for additional support. Larson-Nath and the intestinal rehabilitation team worked closely with Linley’s cystic fibrosis care team to help Linley gain weight. The team fed Linley fortified breast milk with specially formulated fats (lipids) to complete her nutrition. Linley’s intestinal rehabilitation team also included a dedicated dietician and a nurse care coordinator. These care providers helped while Linley was in the hospital—as well as preparing Linley and her family for the transition home with the equipment, supplies, and support they would need.
“It was a constant balancing act, trying to give Linley as many calories as possible without upsetting her digestive system,” Kirstin Paquin said. “The intestinal rehabilitation team worked with us to find a happy medium that would allow her to gain weight without causing an intestinal blockage.”
Over time, Linley began to gain weight and meet growth milestones for children with CF. Then, she began meeting the larger growth milestones for children without CF. After a few months, Linley was growing well enough to be weaned off her feeding tubes. She began receiving all of her nutrition by mouth. Once her weight caught up, there was no stopping her.
Today, Linley is a bright and energetic two-and-a-half-year-old girl who has been off tube feedings for over a year and is meeting all of her growth milestones. She is above the 50th percentile for both height and weight, which is especially important for children with CF.
“Linley is a very active little girl—has been since pregnancy,” Kirstin said. “She’s always on the go, and she loves to run, jump, swim, and play games. We’re always chasing after her to give her more calories, because she burns so many running around, but we wouldn’t have it any other way.”