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Health > Conditions > H > Hirschsprung's disease                           
 Hirschsprung's diseaseHirschsprung’s disease is a rare life-long condition that affects the intestines. It occurs at birth but may not be diagnosed until later. Your digestive system is made up of your food pipe, stomach, small and large intestines, rectum and anus. When you eat something, the food travels down your food pipe and into your stomach, where it’s turned into a mushy liquid. From there, it travels through the small and large intestines, where all the goodness and excess water is taken out. Finally it travels to the rectum and comes out through your anus when you go to the toilet. The intestines contain lots of nerve cells, called ganglion cells, which control how the intestine squeezes food along. But, if you have Hirschsprung’s disease you lack the ganglion cells in part of your intestine, usually the large intestine. This means that poo can’t be squeezed through it. How is it diagnosed?Hirschsprung’s disease may be first noticed when a baby doesn’t pass meconium (the dark poo that usually appears in the first few days of life), or it may be diagnosed later if a child has severe periods of constipation. After a while, the baby or child becomes unwell because he or she may not able to poo properly and may also go off food and be sick. TreatmentThe first step is to wash out the intestine to get rid of all the poo. This is followed by an operation to remove the affected part of the intestine. Sometimes, a series of operations may be needed. The first operation creates an artificial opening (stoma), so that the poo is passed into a bag rather than through the intestines. This is called a colostomy. After a few months, a second operation to remove the affected part of the intestine is usually needed. Hirschsprung’s disease is a life-long condition, so if you have it you will need to have regular check ups until you reach adulthood. The operations are usually successful, but treatment for periods of constipation may be needed occasionally as well as needing to learn how to poo regularly.
Last reviewed by Great Ormond Street Hospital: 21 January 2008  Produced and written for Children First for Health IMPORTANT NOTICE: All content within Children First for Health is provided for general information only to help answer the most common questions. It does not replace the need to consult with a health professional or GP. Children First for Health is not responsible or liable for any diagnosis made by a user based on the content of this site. Personal details will not be stored for any purpose other than administering responses and feedback.
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If you would like to know more about Hirschsprung’s disease, please ask your doctor or contact the Gut Motility Disorders Support Network. You can ring them on 01398 351173.