Hirschsprung's Disease: Khloe's Story

Khalilah Felder’s fifth pregnancy was unlike anything she had ever experienced. Due to low amniotic fluid levels, she was placed on modified bed rest at 30 weeks and delivered her daughter, Khloe, by C-section five weeks later.

Though premature, Khloe initially seemed healthy. But on her second day of life, doctors grew concerned. Khloe was having trouble eating, hadn’t yet had a bowel movement and threw up bile.

“That was very scary,” Khalilah recalls.

Luckily, the Hospital of the University of Pennsylvania, where Khloe was born, is literally connected to The Children’s Hospital of Philadelphia (CHOP), the nation’s top-ranked pediatric hospital.

Khloe was transferred to CHOP’s Newborn/Infant Intensive Care Unit (N/IICU), which has ranked among the nation’s best in newborn care by U.S. News & World Report. There, a specialized team of neonatologists stabilized her and started her on a special nutrition plan.

Diagnosed with Hirschsprung's disease

khloeAs Khloe grew healthier and stronger, the team ran diagnostic tests and found she had Hirschsprung’s disease, a condition that affects the bowel and can cause severe constipation. The muscles in a portion of Khloe’s colon were missing the usual nerve cells (ganglia). These nerves allow the bowel to relax. In their absence, which is the hallmark of the disease, the bowel acts as if it’s obstructed.

CHOP has a particular expertise in the treatment of Hirschsprung’s disease, seeing a large volume of children with the condition.

Khloe spent a few more weeks growing in the N/IICU before CHOP surgeon Michael Nance, MD, successfully performed surgery. During the operation, experienced CHOP pathologists evaluated samples of colon tissue to help surgeons determine exactly how much to remove.

The part of Khloe’s colon without ganglion cells was removed and the remaining normal colon was reconnected. The surgery used to be done in three stages and was very invasive, but Nance was able to repair Khloe’s colon in a single stage, through the anal opening, leaving no scars.

After surgery, Khloe’s recovery was nearly instantaneous. She started eating the next day and went home just a week after the operation, surprising even her physicians, who expected her recovery would take weeks.

“She’s not sick anymore,” Khalilah says. “It’s a miracle.”

Care in the N/IICU

Over the course of Khloe’s seven-week stay in the N/IICU, her mom grew not only to know but also trust her daughter’s care team. She says from day one they made her feel at ease in the midst of an overwhelming situation.

“The staff is incredible,” Khalilah says. “I trusted them and they followed through with everything they said. They never let us down. Not once. Their attitude kept us in high spirits every day. If the staff had not been that incredible, I don’t know that I could have made it through.”

Khloe is now approaching her first birthday and is healthy and growing normally. She’s happy, energetic and smiles often, and is a ray of sunshine to her family, says her mom. 

She’ll continue to follow up with Nance as she grows, but her long-term outlook is bright.

“Surgery all but cures this potentially life-threatening condition,” Nance says. “There are some life-long concerns that accompany the disease, but they are relatively uncommon. Khloe did quite well. We expect great things from her in the future!”
 

Originally posted: May 2012