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Hirschsprung's (HURSH-sprungz) disease, or HD, is a disease of the large intestine.
The large intestine is also sometimes called the colon. The word bowel can refer to the large and small intestines. Hirschsprung's disease usually occurs in children. It causes constipation, which means that bowel movements are difficult. Some children with Hirschsprung's disease can't have bowel movements at all. The stool creates a blockage in the intestine.
If Hirschsprung's disease is not treated, stool can fill up the large intestine. This can cause serious problems like infection, bursting of the colon, and even death.
Most parents feel frightened when they learn that their child has a serious disease. This information will help you understand Hirschsprung's disease and how you and the doctor can help your child.
Normally, muscles in the intestine push stool to the anus, where stool leaves the body. Special nerve cells in the intestine, called ganglion cells, make the muscles push. A person with Hirschsprung's disease does not have these nerve cells in the last part of the large intestine.
Healthy large intestine: Nerve cells are found throughout the intestine.
Hirschsprung's disease large intestine: Nerve cells are missing from the last part of the intestine.
In a person with Hirschsprung's disease, the healthy muscles of the intestine push the stool until it reaches the part without the nerve cells. At this point, the stool stops moving. New stool then begins to stack up behind it.
Sometimes the ganglion cells are missing from the whole large intestine and even parts of the small intestine before it. When the diseased section reaches to or includes the small intestine, it is called long-segment disease. When the diseased section includes only part of the large intestine, it is called short-segment disease.
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