What is Hirschsprung's Disease?
Hirschsprung's Disease also known as megacolon is a rare condition of the large intestine (colon) which affects 1 in 5000 new born babies.
It is more common found in males than females and is commonly found in children with downs syndrome.
If Hirschsprungs is not treated it can cause serious problems such as infection, and bursting of the colon
What causes Hirschsprung's Disease?
Hirschsprung's develops while the baby is the womb. Muscles in the large intestine push stool to the anus where it is then released from the body. Nerve cells called ganglion cells control the pushing for these muscles. Normally these cells grow in the large intestine and continue to the anus. When a child has Hirscsprung's Disease these cell's are missing from a part of the large intestine and so is unable to function, the healthy muscle's push stool to the part without these cell's, the stool stops moving, new stool begin to store behind it and cause's a blockage.
No one knows why the ganglion cells stop moving. But ...
It isn't caused by anything the mother did while pregnant.
Although symptoms usually begin within the first few days after birth, some people don't develop them until childhood or even adulthood.
The main symptom of Hirschsprungs Disease is constipation babies may not pass meconium in their first 24 - 48 hours.
Reluctance to eat, have bile stained vomiting, and abdominal distension.
Symptoms in older children and adult's include diarrhoea and a lack of appetite.
How is Hirschsprungs Disease Diagnosed?
To diagnose Hirschsprung's Disease the child's doctor will do one or both of the following
An x - ray to identify if a segment of the intestine is constricted causing an obstruction. If a child younger than 3 months old, or in any child with a very short or very long collapsed portion of the intestine, the x - ray may appear normal despite the presence of hirschsprung's disease.
The most accurate is a rectal biopsy - this is done by taking a small piece of tissue from the intestine to examine under a microscope.
If the piece of tissue doesn't have the ganglion cells then Hirschsprung's disease has been diagnosed.
Hirschsprung's Disease will always have to be treated by having an operation called a pull - through. This is done by removing the part of the large intestine without the ganglion cell's, pulling the healthy part through and attaching it to the anus.
However the child's doctor may suggest the following treatments as well as the pull through operation.
Rectal wash outs. These are done by placing a thin tube in the child's anus, which is then filled with saline. This softens the stool and flushes from the large intestine. If this is an option the nurses who are caring for the child will teach the parent / guardian how to do them before the child is discharged from hospital.
A colostomy is when the doctor pulls the end of the large intestine out onto the abdominal wall through a small hole called a stoma. The intestine is then folded outwards onto its self and then attached to the abdomen with stitches Stool leaves the body through the stoma where it is collected into a bag which is placed around the skin of the stoma. The specialist stoma nurse will teach the parent / guardian how to care for the stoma.
Hirschsprung's awareness is for information only, it is not meant to diagnose Hirschsprung's disease. And may have to be updated from time to time