Monday, February 2, 2009

Hirschsprung Disease

Hirschsprung disease is a disease seen in neonates where congenital absence of special nerve cells in the rectum called ganglion cells resulting in the failure of the that segment of large intestine fails to function. Newborns fail to pass meconeum, and they are diagnosed and operated by paediatric surgeon as soon as possible to save them. A very small percentage of children born with this disease might survive with chronic constipation. This is usually when the affected intestine is very short; short segment Hirschsprung disease. Rarely they live beyond that and into adulthood untreated. This is what I am highlighting today.

In the last 20 years of my practise, I have seen many such patients, 16 to be exact. These people was never brought to seek treatment by their parents when they were small kids. Some was forced by their teachers and friends to seek help because they were smelly with overflow incontinence - cursed busuk, tak tau basuh punggung ke?

This is an intra-operative picture of a segment of a large intestine (rectum) in a 33 year old policeman with Hirschsprung disease. (I once joked to a colleague; I removed a cat that accidentally went into the man's rectum while he pleasured himself with the animal ! )
He had a laparotomy and colostomy done in a nearby hospital when his colon perforated. He was then referred to me for the definitive surgery called Duhamel operation, and later closure of the colostomy.
The point is - our peoples wait till the last minute to seek treatment. If they had done so earlier; they would have led a normal life. But they rather wait and suffer the consequences; in this disease - chronic constipation, abdominal distention, overflow incontinence (ke mana pergi busuk bau taik) and if unlucky enterocolitis and death.
Ini sebut je operation, tak payah lah. Tunggu besar lah; bila besar yang senang dah jadi susah. Apa nak jadi !!!

1 comment:

Anonymous said...

hey doctor, i have a question. i'm a 21 years old female. when i was born, i've been diagnosed with hirschsprung's disease. i'm not sure what kind of treatment/operation yang dah dibuat masa saya lahir but till now i'm still having the chronic constipation problem and i couldn't control the overflow. after spm, i went to HUKM for further treatment. sadly, sampai sekarang i can't live like a normal person. the doctors said, yang spinter/spinchter (i forgot what did it called)which something like they said,'the force yang membolehkan saya untuk buang air besar' doesnt function and there's no treatment for that. i'm not sure if it was because of the technology or memang takde any opreations/treatments yang saya boleh undergo in order for me to remove the stool like a normal person. they suggested me to undergo colostomy which i was totally disagree sebab you know how the process is which i'm gonna look completely abnormal. so, all they gave me was the ubat2 like laxatives in order for me to avoid constipation. however, even i've taken the laxative, i still couldn't control bila nak passed motion. do you have any idea or suggestion di mana saya boleh dapatkan treatment/operation so that i can live my life like a normal person regardless how much it will cost me?