My Purpose

Hello, my name is Kristen. This Hirschprung Disease blog all started because of a project for my Anatomy & Physiology class. We all picked a disease or illness that someone we knew had and then interviewed them. My brother had hirschprung's disease (HD) about 20 years ago. I've always known that he's had to suffer with this disease but I never could fully understand what he went through. I'm certainly not saying that I can now because I personally didn't have to go through with this disease. However, I feel that now I know more about him and the problems he's been through, and he sure is a trooper!

I wanted to make a blog for the people who've had to deal with HD or is dealing with it right now. This blog with be fairly informative and have to stoma bag suggestions (from my mom) and stores for those of you who are looking for some. I hope that questions and stories are shared for those of you who are interested.

What is Hirschprung's Disease (HD)

Hirschsprung disease is a disease of the large intestine that creates constipation or intestinal obstruction. When a person has constipation, the stool moves slower through the intestines than normal. Some people with Hirschsprung's disease are unable to pass stool at all which means that there is a complete blockage of the intestines, which is called, intestinal obstruction. People with this disease are born with it and are typically diagnosed when they are infants. Having this disease in adulthood is very rare.
Hirschsprungs disease causes constipation because there is a lack of nerve cells in a part or sometimes even all of the large intestine. Nerve cells in the large intestine push the stool out into the anus but without this push signal, the waste product remains in the large intestines. Before birth, never cells grow along both intestines towards the direction of the anus. If someone has Hirshsprungs disease, the nerve cells stop growing too soon. This disease is due to genetics. (My Dads, cousins, son, had Hirshsprungs disease as well)
Newborns with Hirschprungs disease will most of the time be unable to have their first bowel movement within 48 hours after birth. Some symptoms that may also occur are green or brown vomit, swelling of the abdomen, a lot of gas, and bloody diarrhea. If the child is older some symptoms that may occur would be, unable to pass stools without laxatives, swelling of the abdomen, a lot of gas, bloody diarrhea, slow growth or development, and a lack of energy because of a shortage of red blood cells which is also known as anemia. Of course, see your doctor immediately if any of these signs show.

Surgery and Tests for HD

To diagnose Hirschprungs disease, a doctor will do a few tests.
1. X-rays are used to see inside of the body. The doctor might fill the large intestines with Barium liquid so that the large intestines show up clearer.

2. A manometry may also be used to test to Hirshprungs disease. Manometry is when a small balloon is inflated inside of the rectum. If the rectum is normal, the muscles will relax but if the muscles don't relax, HD may be what is causing this. This test is usually used for older children and adults.

3. The last and most accurate test to find HD is called the biopsy. A doctor will remove a little piece of the large intestine and will look at this with a microscope. If the doctor sees that there are nerve cells missing, the child or adult has Hirschprungs disease.

To treat Hirschprungs disease, surgery is the only way that it can be fixed using two methods. There is the "Pull-through Procedure" and "Ostomy surgery." Using the "Pull-through Procedure," the surgeon removes the segment of the large intestines that is lacking nerve cells and then reconnects the segment with never cells to the anus, shortening the large intestines. Ostomy surgery is usually used with a child who has be suffering from HD for a while so that they can get better and then must get the pull through procedure. During Ostomy surgery, the surgeon will first take out the segment of large intestines that has this disease, and then the end of the healthy intestine is moved to an opening in the abdominal where a "stoma" is created. Rolling the intestines back and stitching it to the abdomen creates a stoma. An Ostomy pouch is attached to the stoma and worn outside the body to collect stool. After the Ostomy surgery, during the pull through procedure, the surgeon removes the stoma and closes the abdomen with stitches.

The best thing to do

After the surgery a change in diet is important to help ease your body from the removal of part of the large intestines. It is really important for children with long-segment HD to drink a lot more fluids. The large intestines is mainly responsible for absorbing fluids. Now because the large intestine is shorter then it used to be, it is not going to be able to absorb enough fluids for the body. So, drinking more water helps make up for the smaller large intestines. Some infants may even need a feeding tube that allows food or milk to be pumped directly into the stomach or small intestines. Eating high-fiber food can also help reduce constipation and diarrhea. Fiber helps form stool, making bowel movement easier for those who have HD.

Favorite Supplies

If you live on Oahu, like myself, it can be hard to find certain things. But luckily my mom found a store that sold and still sells what she needed. My mom drove miles and miles and miles to go to this specific store to purchase specific ostomy bags. This store is DAUTERMAN HEALTHCARE & MOBILITY located at 1350 South King St, #101. This store had everything you needed for a person that went through the ostomy surgery. The ostomy bags that my mom found the best was a brand called "ConvaTec."