Not all heroes wear capes. And not every medical condition can be seen. This week we are highlighting a rare “invisible illness”. The condition is Hirschrpung’s diseases named after the person who first described it, Harald Hirschsprung.
What is the condition?
Hirschsprung’s affects roughly 1 in every 5000 children. With the condition, children are unable to pass stools. The reason for this is that as a child develops the nerve cells on their colon (also called ganglion cells) develop down the length of the intestines. In a child with the illness, the cells don’t develop down the entire length. Because these nerve cells are needed to make the bowel contract, faeces stay in one spot. The result of this is that food becomes stuck and a build up can cause the bowel to perforate, which can be dangerous as effectively you have faecal matter in places where it shouldn’t be. If not caught quickly a perforated bowel is fatal.
So what can be done about it?
The standard for the procedure for Hirschsprung’s is a condition called a pull-through surgery. This is a procedure where they ascertain how much of the colon is aganglionic (doesn’t have any nerve cells) and cut it out and drag what is remaining of the bowel down to the anus and sew it up.
Bag it up
If a child suffers from a perforated bowel they may end up having to have what is referred to as a stoma. This is where the colon or ileus is pulled out throw an incision in the stomach. A bag commonly referred to as a colostomy bag (although they are used for ileostomy too) is attached to the stomach. These bags are used to hold bowel movements and need to be emptied and changed regularly.
In most cases, the ostomy bag will only need to be a part-time solution and eventually, the child is scheduled for the pull-through surgery. Unfortunately for parents, this surgery doesn’t just “fix the condition” nearly all kids suffer as a result of this, sometimes in a pronounced, sometimes in minor, if inconvenient ways.
Sometimes in kids with Hirschsprung’s their anal sphincter doesn’t function properly. This can lead to chronic constipation. Sometimes this is because the muscle hasn’t been used because the baby’s poo has been coming out via the stoma. Steroid injections are used to combat this.
Sometimes there will be an obstruction near to the sphincter, or one can develop as the scars from the surgery heal. This can cause issues in Hirshprungs kiddies and so has to be avoided. This is achieved by having a large rod inserted into the child’s bum twice a day. The equipment used is scary looking.
Perhaps the biggest post-surgery issue they have is because of their lack of colon. Kids with Hirschsprung’s don’t absorb water so readily. Because of this, they dehydrate easier than a person with a healthy colon. This may not seem like a big deal but kids with the condition are prone to enterocolitis. Enterocolitis can see them rapidly dehydrate and can be potentially fatal. Signs are fever, vomiting, loose stools and commonly a distended stomach which means there is a blockage. As previously mentioned blockages can lead to a perforation so another skill parents of Hirschsprung’s kids need to master is the rectal washout. This involves passing sterilised water into their bowel via a catheter. It is not a fun procedure to carry out.
The list goes on
In addition to all these HD kiddies are prone to severe nappy rash. They also need to have their diet monitored as some kids will really struggle with some foods. And toilet training can be a lot more difficult for them. If there are surgical complications people with Hirschsprung can become incontinent.