The facts

Hi! My name is Megan and I have a 10 month old boy with long segment Hirschsprung's. I have decided to start this blog to give people somewhere to learn more about it. When he was first diagnosed I looked around on the web and I couldn't really find something that answered my questions. So I read medical research papers. Now I need other parents who have been through what I have (or something like it) to post their stories. My hope is that parents will come here to figure out how to help their child through Hirschsprung's and debate what to do or not to do. I would LOVE your thoughts and input! How is YOUR child doing?

First I would like to explain more about the small intestines and colon.


1. The esophagus is a long muscular tube, which moves food from the mouth to the stomach.

2. The abdomen contains all of the digestive organs.

3. The stomach, situated at the top of the abdomen, normally holds just over 3 pints (about 1500 ml) of food from a single meal. Here the food is mixed with an acid that is produced to assist in digestion. In the stomach, acid and other digestive juices are added to the ingested food to facilitate breakdown of complex proteins, fats and carbohydrates into small, more absorb able units.

4. A valve at the entrance to the stomach from the esophagus allows the food to enter while keeping the acid-laden food from "refluxing" back into the esophagus, causing damage and pain.

5. The pylorus is a small round muscle located at the outlet of the stomach and the entrance to the duodenum (the first section of the small intestine). It closes the stomach outlet while food is being digested into a smaller, more easily absorbed form. When food is properly digested, the pylorus opens and allows the contents of the stomach into the duodenum.

6.The small intestine is about 15 to 20 feet long (4.5 to 6 meters) and is where the majority of the absorption of the nutrients from food takes place. The small intestine is made up of three sections: the duodenum, the jejunum and the ileum.

7. The duodenum is the first section of the small intestine and is where the food is mixed with bile produced by the liver and with other juices from the pancreas. This is where much of the iron and calcium is absorbed.

8. The jejunum is the middle part of the small intestine extending from the duodenum to the ileum; it is responsible for digestion.

9. The last segment of the intestine, the ileum, is where the absorption of fat-soluble vitamins A, D, E and K and other nutrients are absorbed.

10. Another valve separates the small and large intestines to keep bacteria-laden colon contents from coming back into the small intestine.

11. In the large intestines, excess fluids are absorbed and a firm stool is formed. The colon may absorb protein, when necessary.


Deacon has no colon. He is missing #11. "Can I get a number 11?!!"

He is also missing # 10, and a part of #9. He also has no appendix. So in medical terms he has total colonic aganglionosis. Which means, total loss of colon. Or something like that...



Just for some facts Ive grabbed a research article following Deacon's type of Hirschsprung's:



A 32-year retrospective review from 1972 to 2004 analyzed complications and long-term outcomes in children with total colonic aganglionosis (TCA) as they relate to the procedure performed.






Results
Thirty-six patients (27 boys, 9 girls) had TCA. The level of aganglionosis was distal ileum(the point of connection between the small and large intestines.) (26), mid–small bowel (8), midjejunum (1), and entire bowel (1).

Enterostomy was performed in 35 of 36. Eight developed short bowel syndrome. Twenty-nine (81%) had a pull-through at 15 ± 6 months (modified Duhamel 20, Martin long Duhamel 4, and Soave 5). Six had a Kimura patch.

Postoperative complications (including enterocolitis) were more common after long Duhamel and Soave procedures.

Seven (19%; 2 with Down's syndrome) died (3 early, 4 late) from pulmonary emboli (1), sepsis (1), fluid overload (1), viral illness (1), liver failure (1), arrhythmia (1), and total bowel aganglionosis (1).

Mean follow-up was 11 ± 9 years (range, 6 months-29 years). Twenty-four (83%) of 29 patients exhibited growth by weight of 25% or more, 21 (91%) of 23 older than toddler age had 4 to 6 bowel movements per day, and 17 (81%) of 21 were continent.

In 5 of 6, the Kimura patch provided functional benefit with proximal disease.

Conclusion
Long-term survival was 81%. The highest morbidity occurred with long Duhamel or Soave procedures. The modified Duhamel is our procedure of choice in TCA. Bowel transplantation is an option for TCA with unadapted short bowel syndrome.

I know that's alot of information. But it has helped me KNOW what is going on inside my son. I wanted to start with some facts, so now I will tell you a little about Deacon. :)