Grace's story: Beating the odds after multiple surgeries to treat rare genetic disorder

Earlier this year, Michelle Rogers and her 9-year-old daughter, Grace Andrea, visited Texas Children’s Hospital West Campus for a doctor’s appointment when Grace noticed a very familiar face passing by.

“Hello there. Do you remember me?” she asked.

“What is your name?” he asked. She replied, “My name is Grace Rogers.”

“Hi Grace! Of course I remember you!”

This memorable but brief exchange between Grace and her pediatric surgeon, Dr. Paul Minifee, brought back a flood of memories for Michelle and her daughter. Nine years ago, Dr. Minifee performed Grace’s first surgery when she was 3-days-old. He was also her doctor for subsequent surgeries.

“We’ve kept in touch with Dr. Minifee over the years and it was great to see him again in person,” Michelle said. “My husband and I met Dr. Minifee shortly after our daughter was born on Jan. 13, 2012. From the moment I laid eyes on her, I knew something was wrong. Dr. Minifee told us Grace was gravely ill and needed surgery. I remember him telling us, ‘I will do everything I possibly can to help your daughter, but remember, I am not God. I know my limitations.’ Despite not knowing what our baby’s outcome would be, I admired Dr. Minifee’s honesty, compassion and transparency with us during this very difficult time.”

When Michelle was pregnant with Grace, concerns developed early on in her pregnancy. In addition to Michelle’s unusually low amniotic fluid levels, Grace wasn’t reaching certain fetal markers – she couldn’t suck her thumb or swallow. Weeks went by and when she still wasn’t able to reach those markers at 37-weeks gestation, Michelle’s physicians decided it was time to deliver Grace via emergency C-section.

“My husband left work and rushed to the hospital to be there for our daughter’s delivery,” Michelle said. When Grace was born, she looked very ill. She had a high fever, her skin had a green tinge to it and her belly was very swollen. When I tried feeding her, she couldn’t keep anything down. She never expelled her first stool, which is what newborns typically pass in the first 12 to 24 hours after birth. I was told her intestines had erupted in her belly and she had sepsis, a potentially fatal blood infection. As a new mom, I was emotionally torn apart. My husband and I navigated this journey together, not really sure what to expect as each day passed. We relied on our faith and our care team to help us get through it. We were desperate to find out what was wrong so our baby could receive the prompt care she needed.”

To establish a diagnosis for Grace, Dr. Minifee and his team performed a laparotomy on Jan. 16, 2012 when Grace was 3-days-old. They found extensive inflammation in her large intestine (colon) and the lower third of her small intestine (ileum). Since her colon was perforated, some of the stool was present in her abdominal cavity. Biopsy results confirmed Grace had enterocolitis-induced Hirschsprung disease. This occurs when the baby’s intestinal nerve cells (ganglion cells) in the colon are missing, which delays the progression of stool through the intestine causing intestinal obstruction and deadly infections, like sepsis.

“You can judge Hirschsprung disease by the extent of intestinal involvement,” Dr. Minifee said. “In 80% of cases, this disease involves the rectum and part of the colon. In Grace’s case, she had a rare variant of this disease where nerve cells were missing from her entire colon and part of her small intestine. I created an end jejunostomy and multiple intestinal fistulas to bypass her nonfunctional ileum and colon. She was able to receive some food and medicines by mouth.”

Two months later, Grace had a second major abdominal intestinal surgery where Dr. Minifee performed a gastrostomy. He inserted a G-tube through Grace’s abdomen and into her stomach. The G-tube is used to provide a route for tube feeding for a longer period of time and to vent the stomach for air or drainage. During this surgery, Dr. Minifee removed the fistulas and the entire colon and ilium that did not function.

“Within the first two months of her life, Grace went through two major intestinal surgeries,” Michelle said. “The hardest part for us was not being able to hold our daughter as much as we wanted to. It was very difficult for me and my husband to see Grace in this condition as Hirschprung disease is very rare among African-American girls. We prayed our daughter would pull through her surgeries like a trooper and get stronger each day. Dr. Minifee always was there to explain and answer our questions – before and after surgery – and gave us timely updates on Grace. Our baby spent four months in the NICU before we were able to finally take her home. That was the greatest feeling a mother and father could ever experience!”

When Grace was 2-years-old, she had another surgery to reconnect and re-establish her gastrointestinal system so she could achieve normalcy in her life. During this procedure, Dr. Minifee took the remaining rectum out and brought the jejunum (middle part of the small intestine) down to the anus so whatever went into her mouth or into her small intestine via the G-tube, would pass through her body normally.

Today, 9-year-old Grace is doing considerably well. She is a third grader who loves to eat and has a huge palate. Her diet consists of high fats to keep her weight up. But, her parents say no matter what she eats, whether it’s a big plate of sushi or filet mignon, she gets hungry very quickly. Even though she eats solid foods, her intestine can’t absorb nutrients. To supplement her nutrition and keep her hydrated, Grace receives total parenteral nutrition (TPN), or IV nutrition feeding, to control her potassium, magnesium and electrolytes. Her doctors say if her remaining intestine can reprogram itself to absorb nutrients, Grace will not have to depend on TPN down the road. Since her last surgery, she no longer needs a colostomy bag.

“Our daughter has come a long way in her journey,” Michelle said. “She’s faced her challenges head on with a positive attitude. She really is our superhero. As her parents, this journey has been difficult for us, too. Despite the ups and downs, and the unknowns with Grace’s rare condition, I did not walk this journey alone. My husband, who is an incredible dad and provider for our family, walked this journey with me. He was there when doctors told me things I didn’t want to hear. He has been my rock throughout all of this. Without him, our faith, physicians like Dr. Minifee, and others involved in Grace’s care, I don’t know how we could have dealt with this on our own. Despite caring for a child with special needs, we treat Grace like any one of our eight children. We want Grace to know she can do things like any other normal child.”

Grace meets regularly with her physicians for follow-up care including her gastroenterology and nutrition team who manage her diet and the interventional radiology and vascular access team for TPN care. As for Grace’s pediatric surgeon, Dr. Minifee says Grace’s remarkable story is really about “saving lifetime.”

“The motto for the American Pediatric Surgical Association is ‘dedicated to saving lifetimes,’” Minifee said. “Grace’s outcome is truly a manifestation of this. Without the support she received within the first few hours of her life, she wouldn’t have survived as an infant with this GI condition. We are creating and saving a lifetime with the resources Texas Children’s offers in improving patient care and outcomes.”

Click here to learn more about pediatric surgery at Texas Children’s. Click here for more information about Hirschprung disease and how our multidisciplinary team provides care to these patients.