HD FAQ's

Most people have never heard of Hirschprung's disease and that means that they often have lots of questions.  Here's a few of the questions we get asked most often (some of this may be a bit of a repeat from other posts).

1.  What is it?
Hirschprung's disease is a condition that a person is born with that effects the function of the intestines in that the ganglion cells that move food and waste through the body failed to completely develop.  I recently learned that it can be described as a disease or a birth defect-- when it happens randomly and cannot be connected to genetics in any way it is considered a disease, when it can be linked to genetics, i.e. it is hereditary or occurs because of another defect or disability such as Down's Syndrome, it is a birth defect.  Baby J's Hirschprung's could be either (there is a small family history).

2. How much intestine was effected in Baby J?
A lot of other parents I have talked to know how many centimeters or inches of their child's intestines were removed, I don't.  I do know that he lost his rectum, all of the sigmoid colon, and about an inch of his descending colon.

3.  Will he have to have more surgeries?
The hope is that he will not need surgery again, but there is always a chance that something could happen that could make more surgery necessary.  Among the possible scenarios in which he might need surgery are severe enterocolitis, severe constipation, or a build up of scar tissue that causes a stricture.  As of right now, though, things look really good.

4.  How is he doing?
He is doing really well.  We started solids a few months ago and have learned that we have to introduce new foods slower than we did with our older kids and there are a lot of foods we need to avoid so that he doesn't get constipated or sick (rice, pears, peaches...).  He is still nursing and seems to prefer nursing to solids, though he eats those well too.  Despite our best efforts Baby J does occasionally suffer from some tummy discomforts such as gas pains and occasional constipation, but we work through these as they come.  He has been a little delayed on some physical milestones, but when you spend a total of three weeks in the hospital basically immobilized that will happen.  With a little extra work he is catching up on these milestones.  He is overall very happy and healthy and has two, very sharp, teeth.

5. What about the diaper rash?
His diaper rash is under control as long as I don't run out of my magic diaper duty supplies.

6. Is he all better now, will this effect him the rest of his life?
The answer to this one is a little complicated in that he is better in many ways, but this will probably effect him in some way for the rest of his life.  When you lose a major organ (or part of one) there are always complications.  Many times there are scars, sometimes there are dietary restrictions, risks of infections, or other complications.  Baby J faces most of these.  He has the scars on his tummy from the surgeries, he has scar tissue where things were reconnected that could build up and cause issues, he will most likely have food allergies/intolerances that will stay with him his whole life, he may never have "normal" stools, and he is always at risk for enterocolitis.  When he is older, probably about 3.5 or 4, and ready to potty train we will work with a group of doctor's to help him learn muscle control and possibly put him on some special diets to help his digestion and pooping.  There's a good chance that he will have to be on some kind of fiber supplement or laxative for his whole life to avoid issues like constipation.  For now, he is doing very well and, as long as we are careful about what he eats, he has few problems doing his duty.  Occasionally he will get backed up and we have to give him an irrigation (enema), but that is the exception, not the rule.  So the simple answer is, no, he is not all better, and yes, this will effect him the rest of his life.

I hope this can help answer any questions you, our readers, may have.  If you ever have questions about Baby J and his HD please ask.  I may not have all the answers, but I am always learning more about HD and will find the answers as best I can.