Children with Hirschsprung’s disease lack nerve cells in a part of the intestine. This means that the child can not poop at all without help. The treatment consists of removing the diseased part of the intestine.
Hirschsprung’s disease is often detected already when the baby is newborn and cannot poop properly. But the disease can also be detected during the baby’s first months when the baby starts eating regular food. The vast majority are diagnosed before the age of one.
Symptoms of Hirschsprung’s disease
A child who has Hirschsprung’s disease is very difficult to poop and haggle. Most people cannot bark at all. They need help to get the bag out, for example with the help of enema and a thin tube. Enema is a liquid drug or saline that is introduced into the rectum.
When the baby is unable to poop, they get a very tense and swollen stomach and stomach pain. The child may also vomit.
Another sign of the disease is that the child does not grow properly either.
When and where to seek care?
Contact BB if your baby is newborn and has not pooped during the first two days of life.
If it’s in a hurry
Immediately contact a health care center or an on-call clinic if the child has difficulty swallowing and has one or more of the following symptoms of Hirschsprung’s disease:
- The baby has a lot of stomach aches.
- The baby is sweating cold and has a swollen and tense stomach.
- The child vomits.
- The child has blood and mucus in the pouch and does not feel well.
If closed, seek care at an emergency room.
When you and the child meet with the doctor, you start by telling them about the child’s problems and how long they have lasted. Then the doctor examines the child. Often, the child may have blood and urine tests. If the doctor suspects Hirschsprung’s disease, the baby’s intestine is examined with x-rays and sampling of the cells of the rectum.
Depending on the child’s inconvenience, the child may undergo the examinations directly, or the child will be referred to them.
X-ray of the rectum and colon
Before X-ray, the child receives a fluid called contrast medium in the rectum. The fluid makes it easier to see the rectum and colon in the X-rays.
Sampling from the rectum
A doctor examines the child’s gut by taking a tissue sample from the rectum, called a biopsy. Sometimes the doctor takes a biopsy with a small suction instrument and then the baby can be awake. The child then gets a sugar solution in his mouth. The sugar solution has a calming effect. Sometimes children may need to be anesthetized prior to such sampling.
The tissue sample is then examined under a microscope. It is then possible to see if there are nerve cells in the intestinal wall and what they look like.
Treatment of Hirschsprung’s disease
The child needs to have the diseased part of the gut removed if they have Hirschsprung’s disease. How quickly the child needs surgery depends on how the child is feeling. Sometimes it is not so urgent, but the doctor plans an operation a few weeks ahead in time.
The child needs to empty the bowel until the surgery
Until the child is operated on, the child needs help emptying the bowel. The contents of the intestine are rinsed out by giving the child enema via a thin tube in the rectum. After a few rinses, the baby will quickly recover.
The child needs to receive enema once or several times a day until the operation is performed. You will learn how to help the baby clear the gut.
The diseased part of the intestine is removed
During surgery, the surgeon removes the diseased part of the intestine. The healthy part of the intestine is then sewn together with the rectum opening. The child is asleep during the operation.
After the operation, the child needs to remain in the hospital for a few days.
The child may need to have an ostomy temporarily
The child may need surgery immediately if they are very ill. The surgeon then removes the diseased part of the intestine. But instead of sewing the intestine into the rectum, the doctor pulls the healthy intestine through a hole in the stomach, called an ostomy. The child then needs an ostomy bag on the stomach that captures the intestinal contents.
The child usually quickly becomes better after such an operation. You and the child may remain in the hospital until you have learned how the stoma works and how it is to be managed.
When the child is feeling well, they are operated on again. Then the surgeon stitches the healthy bowel into the rectum, and the baby no longer needs an ostomy bag.
The first months after surgery
After the operation, the baby pauses several times a day. You may need to change diapers up to 20 times a day, and the skin around the rectum becomes easily irritated. Lubricate the skin around the rectum frequently so that no ulcers are formed.
There are different types of ointments to be used in different ways. The healthcare staff will help you choose and show you how to do it.
A few months after the operation, the child does not poop as often. But up to school age, many children who are operated on for Hirschsprung’s disease need to poop more often than other children.
Use a pot or toilet as early as possible
Let the child start using the potty or go to the toilet as early as possible. It will be easier for the child to poop when sitting down. Often, the entire bowel function improves when the child learns to poop on the pot or the toilet.
There are special toilet seats the child can use in the toilet. Place a stool in front of the toilet seat on which the child can rest his feet. It is both safer and more comfortable, and facilitates when the baby is going to cruise.
Living with Hirschsprung’s disease
The child will grow and develop properly after surgery.
As the child grows, they may have periods when they have trouble pooping. It can also leak poop into the underwear. This is often because the sensation in the rectum is poorer, and the muscles of the rectum have less control.
The bowel usually works better when the baby is in his teens. Most people with Hirschsprung’s disease have fewer problems as adults than they had as children.
What happens in the body?
The work of the intestines in transporting the intestinal contents forward is controlled, among other things, by nerve cells. It is these nerve cells that are missing from Hirschsprung’s disease. This means that there is no movement in the intestine that drives the intestinal contents forward.
Hirschsprung’s disease is also called aganglionosis.
The intestinal portion lacking nerve cells can be different in length
It is usually a 15 to a 20-centimeter portion of the rectum and colon that lacks nerve cells. But in some children, they are missing in the entire large intestine. It is uncommon, but sometimes the nerve cells are also missing in the small intestine and throughout the gastrointestinal tract.
The larger the intestine that lacks nerve cells, the more difficult it becomes to get a gut function that works properly after surgery. The bowel movements in the part of the intestine that remain are often rapid, and the control of the rectum’s work can be weak. That is why it can sometimes be difficult to keep tight and that there can be problems with it leaking and pooping.
If the child grows worse, it may be because the intestines cannot absorb nutrition properly. Then the child gets extra nutrition and vitamins.
What is Hirschsprung’s disease?
Hirschsprung’s disease is a congenital disease that has occurred during fetal development. The disease is to some extent hereditary.
Having an untreated disease and not being able to empty the gut on contents can lead to gut bacteria accumulating in the gut. The bacteria are usually present in the intestine, but if it becomes too much the intestinal wall can become inflamed. Such inflammation is called enterocolitis. The bacteria also form toxic substances, which go into the blood.
In enterocolitis the child has the following symptoms:
- Stomach upset.
- The buoy smells very bad.
The treatment of Hirschsprung’s disease consists of rinsing the contents of the intestine several times daily. The child also receives antibiotics.
Affect and participate in care
In order to be involved in the care and treatment, it is important that you understand the information you receive from the healthcare staff. Ask questions if you don’t understand. For example, you should get information about the treatment of Hirschsprung’s disease options and how long your child may have to wait for care and treatment.
Children should also be involved in their care and receive information from care staff that is adapted to the child’s age.
You have the opportunity to get help from an interpreter. You also have the opportunity to get help from an interpreter if you have a hearing loss.