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Transcript of Hirschsprung's Disease
An example of meconium. Causes Currently, the cause of Hirschsprung's Disease is unknown. Doctors have assumed that it is a genetic dissorder, passed down from carrier parents, but no defined cause has been discovered as of yet. Research is still undergoing to determine the cause. A quick fact: as a sufferer of Hirschsprung's Disease, I was not diagnosed until the age of two because when I was born, I passed my meconium within the alotted time. Doctors assumed constipation until I was finally diagnosed and treated. Cases in Canada Treatment According to Statistics Canada, 14 people died of Hirschsprung's Disease between the year 2000 and 2006. Due to the current treatments and relatively accurate diagnosis of the disease, many people survive and even live normal lives after treatment. According to studies done at the University of Ottawa, there are three main theories for the appearance of the disease.
"The first and most popular theory holds that there is a defect in the vagal neural crest resulting in disordered neuronal migration and population of the distal gut."
"The second theory holds that the enteric neuronal precursors colonize the gut normally, but subsequently, there is a region specific neuronal cell death as a result of some toxic event."
"The third theory suggests that the enteric microenvironment is abnormal and as such, unable to support or else locally excludes enteric precusors." In the past, Hirschsprung's Disease was easily mistaken for simple constipation in the patient. Docters provided patients with laxatives that produced temporary relief of the symptoms, but in order to maintain regular bowel movements, laxatives were needed to be taken regularly in order to allow stool to pass. If the person is suspected of having Hirschsprung's, there are multiple ways for the doctor to make a diagnosis.
The first is a barium enema. Barium is inserted in to the colon and an x-ray is taken. Someone with Hirschsprung's will have an area of their colon that appears thinner than usual.
Manometry is another, less accurate test. A balloon is inflated in the rectum. Normally, the anal muscles will relax, but if they don't Hirschsprung's might be the problem.
The most accurate test is a biopsy. The doctor removes a small portion of the colon and manually checks for the ganglion cells. If they aren't there, Hirschsprung's is the problem. Pull-Through Surgery Treatment of Hirschsprung's today depends on the severity of the disease and the age of the patient. Originally, the process began with a colostomy. Doctors cut open the colon and created an artificial path for stool to exit into a small bag on the outside of the body. When the colon was clean, pull-through surgery was performed. Pull-through surgery is now the most common solution to Hirschsprung's and more often than not, a colostomy is no longer needed.
Here we see a diagram of the infected colon. The first step is to remove the diseased portion of the colon. This leaves the rectum and functional portion of the colon. The colon and rectum are reattached and allowed to heal. Around 85% of patients that have a pull-through surgery perfomed lead normal lives after surgery. The remaining 15% will have either constipation or a lack of control in which laxatives or a stoma must be used respectively. A stoma is similar to the previously mentioned colostomy. What we've learned... Hirschsprung's Disease has helped physicians understand major issues with constipation of the colon. The study involved is still ongoing, but initial understanding of ganglion cells helped educate doctors about peristalsis and the movement of fecal matter through the digestive system. That being said, there is still a lot of research to be done surrounding the cause of Hirschsprung's. Infants with the disease are commonly found to have Down Syndrome and other genetic problems. Personally, I'm one hell of a lucky patient. Works Cited
Box, Checking This. "Digestive System Disease :: Congenital Megacolon -- Britannica Online Encyclopedia." Encyclopedia - Britannica Online Encyclopedia. [Online] Available <http://www.britannica.com/EBchecked/topic/163199/digestive-system-disease/45426/Congenital-megacolon>. 09 June 2010.
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“Digestion and Digestive Systems: Aganglionic Megacolon.” Encyclopedia Britannica. 1989 ed.
"File:Meconium.jpg." Wikipedia, the Free Encyclopedia. [Online] Available <http://en.wikipedia.org/wiki/File:Meconium.jpg>. 09 June 2010.
"Hirschsprung's Defined." The Hirschsprung's & Motility Support Network (HMDSN). [Online] Available <http://www.hirschsprungs.info/hd.html>. 09 June 2010.
"HIRSCHSPRUNG'S DISEASE." Université D'Ottawa - University of Ottawa. [Online] Available <http://www.medicine.uottawa.ca/nsc/eng/hirc.html>. 09 June 2010.
"Hirschsprung's Disease." [Online] Available <http://18.104.22.168/library/healthguide/en-us/support/topic.asp?hwid=tp12696>. 09 June 2010.
"Hirschsprung's Disease." Wikipedia, the Free Encyclopedia. [Online] Available <http://en.wikipedia.org/wiki/Hirschsprung's_disease>. 09 June 2010.
"Meconium | Define Meconium at Dictionary.com." Dictionary.com | Find the Meanings and Definitions of Words at Dictionary.com. [Online] Available <http://dictionary.reference.com/browse/meconium>. 09 June 2010.
"Statistics Canada: Canada's National Statistical Agency." Statistics Canada: Canada's National Statistical Agency / Statistique Canada : Organisme Statistique National Du Canada. [Online] Available <http://www.statcan.gc.ca/start-debut-eng.html>. 09 June 2010. Thanks for watching!