What is Hirschprung’s Disease? It is condition in which the nerve cells that are normally present in the large intestine are absent. When these nerve cells are missing, the intestine cannot move stool along normally and your child will have severe constipation or obstruction of the large intestine.
How do I know my child has Hirschprung’s? Most children have their first bowel movement within a day after they are born, and then have several soft stools a day. If your child goes more than 24 hours before having a BM, or goes several days without a BM at all, your child could have Hirschprung’s. If your child has these symptoms your doctor may order a barium enema or a rectal biopsy. If the rectal biopsy shows no nerve cells in the rectal tissue, your child probably has Hirschprung’s.
How is Hirschprung’s treated? Surgery is the only way to treat this disease. If a child remains severely constipated or obstructed, they can become very ill and it can be life- threatening. In order to prevent this, and to achieve more normal bowel function, we remove the part of the large intestine that is affected. This can involve removing only a short piece of intestine, or it can involve the entire large intestine (called long-segment disease). Once the diseased portion is removed, the remaining intestine will be used to create a new rectum for your child.
Will my child need a colostomy? If your child’s surgery is performed in two separate steps your child will have a colostomy, temporarily, until the final procedure (called a pull-through) is completed. The second step of the procedure is usually to close the colostomy and to reconnect the remaining intestine to the anus. If your procedure is done in one operation, then your child will not need a colostomy. Your surgeon will decide which method to use based on the extent of involvement of the intestine.
What can I expect from surgery? After your child wakes up from surgery, he or she will receive IV fluids and pain medicine in the hospital. There will probably be a tube in the child’s nose that removes air from the stomach. When your child begins to pass gas, the tube is removed and your child is allowed to start drinking some fluids. They can then advance to formula or regular food if there is no vomiting. When the child is eating well and passing stool either from the bottom, or the colostomy, they may go home.
What should I expect at home? You will have to put special ointments on your child’s bottom and keep it very clean in order for the wounds to heal. Some children will also need the anus dilated daily with a special dilator that your surgeon will give you. Children with Hirschprung’s will occasionally still have problems with constipation and require laxatives. Others may never have full control of their bowels and will have occasional accidents in their underpants. Your child will also be prone to an intestinal infection called Hirschprung-Associated Enterocolitis (HAEC), so it is VERY IMPORTANT to call your surgeon if there is ANY explosive, watery diarrhea. Your child should otherwise grow and develop normally.
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