Hershey's disease refers to Hirschsprung disease.
Hirschsprung disease is a rare and congenital condition in which nerve cells are missing in the lower part of a baby's large intestine.
And without these nerves in Hirschsprung disease, poop cannot move normally through the baby's bowel, which causes severe constipation and requires surgical correction.
The cause of Hirschsprung disease is when nerve cells called ganglion cells fail to develop properly and migrate to the end of the bowel before the baby is born.
The affected section of the colon in Hirschsprung disease lacks the ability to relax and acts like a blockage and causes waste to back up, which results in a swollen abdomen and discomfort.
Hirschsprung disease is also often diagnosed in newborns just shortly after their birth and often when a baby fails to pass their first poop, although very mild cases of Hirschsprung disease may not be noticed until later in their childhood.
The primary and required treatment for Hirschsprung disease is surgery, which is done to remove the non functioning part of the intestine and restore normal bowel movements.
The long-term effects of Hirschsprung disease primarily involve chronic bowel dysfunction.
Even after successful surgical removal of the aganglionic (nerve-missing) bowel segment, many people experience lingering gastrointestinal challenges.
While the vast majority of people achieve good social continence, long-term monitoring is often required.
Most Common Long-Term Effects of Hirschsprung's disease include.
Fecal Incontinence or Soiling: Up to 50% of people experience fecal soiling or difficulty holding back poop, often because the rectum—which usually acts as a storage reservoir—is absent.
Chronic Constipation: Paradoxically, persistent or recurring constipation can occur after surgery for Hirschsprung's disease and often requires long-term management with dietary changes, stool softeners, or laxatives.
Hirschsprung-Associated Enterocolitis (HAEC): This is an inflammation and infection of the intestines that can recur even after surgery, causing watery diarrhea, fever, and abdominal pain.
Urological & Sexual Health: Some adults experience lower urinary tract symptoms, and in rare cases, impaired sexual function or fertility challenges.