Cameron’s Story: Surgical Care for Extremely Complex Presentation of Hirschsprung’s Disease
Cameron’s Story: Surgical Care for Extremely Complex Presentation of Hirschsprung’s Disease
When Cameron was just 4 days old, his colon ruptured as a result of Hirschsprung’s disease. He was brought to Children’s Hospital of Philadelphia (CHOP), where surgeons performed a series of incredible procedures on his bowel over the course of his first few years of life. Their efforts and expertise helped Cameron transform from a very sick infant near death, to a high-energy and hilarious 4-year-old.
Four-year-old Cameron is a happy-go-lucky kid with a bright personality. He loves to dance, play and laugh, which is amazing considering how sick he was when he was a baby.
“When you meet Cameron, you would never recognize the vibrant little boy that he is now compared to that very fragile, sick baby,” says Emily Partridge, MD, PhD, MHS, a surgeon at CHOP.
When he was 4 days old, Cameron suddenly became lethargic, stopped eating, was breathing strangely and looked blue. His parents, Patty and Roger, frantically called 911. By the time the ambulance arrived, Cameron was barely responsive. He was rushed to the nearest hospital where the care team found that he had free air in his abdomen, which can be life-threatening. They placed an IV line and a breathing tube to stabilize Cameron and had him transferred by helicopter to CHOP.
Dr. Partridge was the on-call surgeon when Cameron arrived at CHOP. She and her team quickly assessed him and found a hole (perforation) in the middle of his large intestine (colon). As a result of that hole, Cameron’s abdomen had filled with poop (stool), which had caused a rapid progression of septic shock and multi-organ failure.
Within about 20 minutes of his arrival at CHOP, Cameron was undergoing emergency surgery to save his life.
Finding the cause of the problem
Cameron’s medical team discovered that the cause of the perforation was Hirschsprung's disease. This is a condition in which a child doesn’t develop ganglion cells (nerve cells) in the bowel wall.
“Your whole intestine is a living organ, and it's supposed to contract and relax,” explains Dr. Partridge. “When a segment of bowel lacks the nerve cells, there are no waves of movement to move food from your stomach through your small and large intestine. Things are basically static in a rigid, inflexible tube. The stool can't exit the large bowel, and that essentially causes an obstruction or a blockage.”
Cameron's case was extraordinary. A blockage of stool had caused a bowel perforation that essentially ripped a hole in the middle of his large intestine. It is very unusual for this to be the first sign of Hirschsprung’s in a child, says Dr. Partridge. The part of the colon that was destroyed originally had good nerves, but the perforation cut off blood supply to it, so half of Cameron’s colon needed to be removed.
Additional surgeries and care from the unique Neonatal Surgical Team
By the time he was 9 days old, Cameron had undergone three surgeries. The first few were to control the infection and help his body recover from the septic shock. Then, Dr. Partridge had to create a way for him to be able to poop without using the diseased part of the colon. She performed an ostomy surgery, which creates an opening (stoma) in the belly to allow poop to exit the body into a colostomy bag. This bypasses the affected part of the intestines, which would allow Cameron’s intestines to heal while the surgical team planned the rest of his care.
Roger and Patty were touched by the little things the care team did to show how much they cared about their baby boy. Like how Dr. Partridge would cut Cameron’s surgical bandages into the shapes of different animals.
“It's just one more step that she takes to just be so loving and caring,” says Patty. “She probably doesn't even realize how much that means to families.”
During this time, Cameron was cared for by a team within CHOP’s Harriet and Ronald Lassin Newborn/Infant Intensive Care Unit (N/IICU) that’s devoted specifically to patients who need surgeries. It’s called the Neonatal Surgical Team.
“We work in an extremely close partnership together when we have a baby who requires surgery,” says Dr. Partridge of the unit within a unit. “A lot of hospitals don't have that very close working relationship between neonatology and pediatric surgery.”
Thanks to this close partnership, fragile babies actually undergo surgery at their bedside in the N/IICU, which helps keep them stable.
Surgical collaboration for bowel reconstruction
As Cameron healed in the N/IICU, Dr. Partridge collaborated with Myron Allukian, III, MD, a CHOP surgeon with special expertise in colorectal surgery, to plan the surgeries to reconstruct his bowel. All the while, the care team kept close watch on Cameron’s overall health.
The surgeons hoped to be able to perform a pull-through procedure, in which the healthy part of the colon with functioning nerve cells is surgically connected to a child’s rectum (bottom) so they can poop normally. They found Cameron only had about 10 to 15 centimeters of healthy colon remaining. It wasn’t a lot to work with.
The care team prepared extensively for the pull-through procedure. It was a challenging surgery that lasted eight hours, in large part due to the amount of scar tissue in Cameron’s abdomen from all the operations he’d already undergone. But it went very well.
One more surgery was required after the pull-through procedure: The doctors reversed Cameron's ostomy. After three and a half years of colostomy bag changes, Patty and Roger were thrilled that Cameron was finally able to go to the bathroom normally!
Pediatric Colorectal Program
CHOP’s Pediatric Colorectal Program provides comprehensive treatment for children with long-term bowel management needs.
Remarkable resilience
Cameron’s family credits the whole team at CHOP for their son’s transformation from a very sick infant near death, to a healthy, high-energy kid. Especially Dr. Partridge, who has been a calming source of support from day one.
“If it wasn't for her, he wouldn't be here and doing as well,” says Patty. “She really has taken every step to give him the best chance possible. And he's doing so well because of her and because of their team. It's unbelievable.”
“This really is the greatest privilege of what we do,” says Dr. Partridge. “It's the reason why I went into pediatric surgery, because it's not just the care of the patient, it's the care of the whole family, and it's the potential to impact a lifetime of future health.
“He is a very special kid,” she adds. “Even from the time when he was a small baby in the N/IICU, he had just a remarkable resilience. He constantly surprised us with how quickly he recovered from his surgeries, and nothing ever really slowed Cameron down. He has been this special little light. It's like he knows how amazing it is that he's here and he's healthy.”
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