Medical Q&As

Hirschsprung's disease - cancer related?

I am a seventeen-year-old male and I was born in England. When I was eleven months old I was diagnosed with a rare cancer called Hirschsprung’s disease. I had to wear a bag for a while and at twelve months of age I had to have a further bowel operation. I know that I have a shadow hanging over me for the rest of my life because of the bowel cancer. I soil myself everyday and I sometimes get pains in my abdomen. Could you give me some information about my condition? Will I need further surgery?

There has clearly been some confusion along the way with regard to informing you about your condition. Hirschsprung’s disease is not a rare form of cancer. It is a genetic disorder of the bowel in which nerve ganglia are missing. Ganglia are collections of nerves and the specific ganglia that are absent in Hirschsprung’s disease are those responsible for relaxing the bowel wall. Normally this relaxation permits the stool to pass on towards the anus where it is expelled from the body. In the case of a Hirschprung’s sufferer this relaxation is absent and the stool cannot pass on which gives rise to obstruction of the bowel. In some cases the bowel obstruction presents soon after birth but in others the pattern may be one of chronic constipation and abdominal distension during early childhood. Hirschsprung’s disease is treated surgically and very often the first step is to fashion a colostomy in order to relieve the pressure within the obstructed bowel. That is the reason why you had a colostomy bag for a short while when you were an infant. The second operation you underwent was to remove the segment of bowel where the ganglia were absent. Most children with Hirschsprung’s disease do very well so it is unfortunate that you have been left with the problem of frequent faecal soiling. It may be that the abnormal segment of your bowel extended far down into your rectum and consequently a long segment of bowel had to be removed. I am not aware if this can be repaired but a bowel specialist would be able to advise you of what options might be available to you. You can learn more about Hirschsprung’s disease by referring to another Ask the Doctor question that I answered some time ago: http://www.irishhealth.com/askdoc03.html?f_qid=1141. Finally there is no association between Hirschsprung’s disease and cancer. The information you have been given previously is mistaken.