Hirschsprung's disease

What causes Hirschsprung's disease?
Scientists are not sure why the intestinal nerve cells do not form completely.

• Nothing has been shown to cause this problem, including medications a mother takes while pregnant or what a mother eats during pregnancy.
• Genetic factors likely play a role in Hirschsprung's Disease, especially when longer lengths of intestine are involved.
  • If a family has a child with Hirschsprung's disease, there is a 3 to 12 percent chance that another baby from the same parents will also have the disease.
  • There is an increased chance that a couple will have a child with Hirschsprung's disease if one of the parents has the disease.
  • The chances are higher if the mother is the parent with Hirschsprung's disease.

How often does Hirschsprung's disease occur?

Hirschsprung's disease occurs in one out of every 5,000 babies.

Who is at risk for Hirschsprung's disease?

Hirschsprung's disease occurs five times more frequently in boys than in girls.

• Children with Down syndrome have a higher risk.

What kind of problems might my child have?

Because a segment of the intestine lacks normal nerve cells, digested food and stool cannot move forward through that portion of the digestive tract.

The intestine becomes blocked with stool, and your baby will be constipated (unable to have normal bowel movements). The obstruction causes pressure on the inside of the intestine, causing part of the intestinal wall to wear thin. Eventually, a bacterial infection can develop in the digestive tract, causing serious problems.

What are the symptoms of Hirschsprung's disease?

Eighty percent of children with Hirschsprung's disease show symptoms in the first six weeks of life.

• Children who only have a short segment of intestine that lacks normal nerve cells may not show symptoms for several months or years.

Each child may experience symptoms differently, but here’s a list of the most common ones:

• failure to have bowel movement in the first 48 hours of life
• gradual bloating of the abdomen
• gradual onset of vomiting
• fever

Children who don’t have early symptoms may also have the following symptoms:

• constipation that becomes worse with time
• loss of appetite
• delayed growth
• passing small, watery stools