By Maureen Gilmer, IU Health senior journalist, email@example.com
Adah Maust doesn’t understand all the fuss being made about her.
At 8 months old, she doesn’t know what a G-tube is or why her digestive system doesn’t work or why she is in line to get a transplant.
So she stares wide-eyed at the doctors who are watching her, examining her, talking to her as she is propped up on an exam table by her mom, Jordan Maust, at the Riley Outpatient Clinic.
Adah was born last summer with a birth defect called omphalocele, which affects the wall of the abdomen. The infant’s intestines were protruding outside the belly. She also suffered a congenital anomaly to the intestines where there were multiple areas of stricture or narrowing and incomplete formation.
“They told us at first it would be an easy fix,” Jordan Maust said of the team at the hospital where she delivered. “But they then realized she had a lot more issues.”
The first two months of Adah’s life were difficult for her and her parents, Jordan and Ethan of Carmel. At times, they wondered if there was any hope. She underwent two surgeries, but neither was successful at repairing the damage, and she was left with a very small portion of intestines, not enough to do their job of digesting food and regulating the body’s water balance.
SPECIALIZED CARE AT RILEY
The young parents, who also have a 2-year-old daughter, transferred Adah to Riley Hospital for Children at IU Health, where she is under the care of Drs. Alan Ladd and Charles Vanderpool, co-directors of the intestinal rehabilitation center at Riley.
Their team assists with the complex care surrounding children with intestinal disabilities. In addition to surgery and gastroenterology, the clinic also incorporates specialized pharmacists, nutritionists and social work to help families with whatever they need.
“We aim to optimize the functioning of their intestine so that they may hopefully lead independent lives, but some will require ultimate intestinal transplantation,” Dr. Ladd said.
Dr. Ladd leads the surgical half of the team, responsible for central lines, G-tubes and intestinal procedures, while Dr. Vanderpool leads the pediatric GI side of care.
Both physicians have been working to manage Adah’s care, ensuring that she gets proper nutrition through the tube that goes into her stomach. While originally hopeful that Adah’s digestive system could be salvaged, a “heroic” surgery at Riley last fall was not successful, Dr. Ladd said.
“It’s damaged beyond repair,” he said, though they continue managing her care as they await the next step in her treatment.
ON TRANSPLANT LIST
Together with the physicians and with IU Health transplant surgeon Dr. Richard (Shane) Mangus, the family has decided to pursue a multivisceral transplant – involving stomach, liver, pancreas, small and large intestine – believing that will give her the best quality of life long term.
So now, Adah’s parents wait for the call that could change their baby’s future. After the first few tumultuous months of her life, they have fallen into a comfortable rhythm caring for their baby girl, despite the challenges she faces.
“Right now we’re doing pretty well because it feels normal at this point,” Jordan said. “The first two months of constant discovery of bad news was less than encouraging, but now that she’s home, I feel we have a different outlook on life and what it means to be a parent.”
Wearing a shirt with the message Faith over Fear, Jordan said she and her husband believe children are a gift from God, so they accept each day as a precious gift.
Meanwhile, Adah is growing and developing as she should, her mom said. She’s finally on the growth curve – she weighs a little over 16 pounds – evidenced by her chunky thighs, Jordan said.
That’s thanks to the full nutritional support provided her by way of IV nutrition, Dr. Ladd said, explaining that the Riley clinic treats kids from all over the state and surrounding states with minor to severe digestive and feeding issues.
“We see the entire spectrum of cases,” he said.
Not all require IV nutrition like Adah, but the intestinal rehabilitation center team works together to optimize feeding plans for these kids, oversee the nutrition they get at home and follow them in clinic to make sure they are growing appropriately.
“There are plenty of kids that might be on IV nutrition their whole life, but they’re eating enough and doing well enough that it’s OK,” Dr. Ladd said. “She’s one of the neat ones that we’ve been able to support perfectly, but we know for her lifetime she needs a transplant. For our kids, that’s not a planned pathway; it’s only for the ones where we can’t intervene.”
UNDER ADAH’S SPELL
During a clinic visit earlier this month, Adah earned a good report from both doctors, who were pleased with her liver and kidney function, as well as her iron levels, urine output and overall growth.
When they walked into the exam room, they were charmed by Adah’s watchful gaze.
“She’s doing so well,” Dr. Vanderpool exclaimed, noting how much hair she has. “Hi sweetie!”
Adah lay patiently, enjoying her pacifier, while he reviewed her progress with Jordan and Dr. Ladd.
The crying began a few minutes later after Jordan asked Dr. Ladd to look at her baby’s G-tube.
“She’s having trouble with vomiting, and it clogs up a lot,” she told the doctor.
Dr. Ladd opted to upsize her G-tube a bit to drain her stomach better, thus reducing the episodes of vomiting.
“I’m sorry,” he told Adah, finishing the brief procedure in a matter of seconds. “That should help.”
As Jordan finished dressing Adah and put a white bow back in her baby’s dark hair, she thanked the doctors for their care and left for home, knowing that the call for transplant surgery could come at any time.
This is the calm before yet another storm, but her faith grounds her.
“We’re not promised our kids for any period of time, or that they’re going to get married and have their own kids,” she said. “We got more than we thought we’d get with Adah. Just looking at life like that has been a huge weight off of us. So now we’re just living and enjoying it and having a good time.”
Photos by Mike Dickbernd, IU Health visual journalist, firstname.lastname@example.org