Hirschsprung’s disease is an illness that children are born with that prevents them from having a normal bowel movement.
Squeezing of the muscles in the gut helps move digested food through the intestines. The squeezing of the muscles is triggered by nerves. Hirschsprung's disease is a problem that happens when some of the nerves are missing. Without the nerves, the muscles cannot move digested food through the intestine, so the intestine gets blocked. This can make it hard for your child to pass bowel movements (constipation).
Hirschsprung’s disease may affect only the area near your child’s rectum, or it may affect a larger section of the large intestine.
Before birth, a child’s nerve cells normally grow along the intestines toward the anus. When a child has Hirschsprung’s disease, the nerve cells stop growing too soon. Why the nerve cells stop growing is not known.
Sometimes Hirschsprung’s is inherited, which means that it’s passed from parents to children through their genes. Genes are inside each cell of your body. They contain the information that tells your body how to develop and work. This disease affects boys more often than girls.
The symptoms depend on how much of the intestine is involved. Symptoms may include:
If your child has a mild case of Hirschsprung’s, symptoms may not start until your child is older. In addition to some of the symptoms listed above, older children may have constipation that gets worse over time.
Your child’s healthcare provider will ask about your child’s symptoms and medical history and examine your child. Tests may include:
Hirschsprung’s disease is treated with surgery to remove the part of the intestine that has no nerve cells. The healthy part of the intestine is then connected to your child’s anus. (The anus is the opening of the rectum where bowel movements leave the body.)
If your child is very sick at the time of diagnosis, your child may need a procedure called an ileostomy or colostomy for a few months to a year. These procedures are done to connect the healthy part of your child’s intestine to a new opening in the belly called a stoma. Your child’s bowel movements will empty through the opening and collect in a disposable bag outside the body. After your child heals, a second procedure may be done to connect the healthy intestine to the anus. The stoma will be closed during this procedure.
Your child’s surgeon and nurses will teach you how to care for your child. After surgery, you will need to care for your child’s incision area. If your child has stoma, you will also learn how to care for it. To help prevent infection and other problems, follow your healthcare provider’s directions carefully.
Follow the full course of treatment prescribed by your child’s provider. Ask your provider:
Make sure you know when your child should come back for a checkup. Keep all appointments for provider visits or tests.