Having a small baby or kid at home is the most wonderful thing in anybody’s life. We forget all our worries and start relaxing once we are home. A baby makes us laugh, and play, and at times we become kids while they are around. They change our whole world. But sometimes, when the baby is unwell it upsets the entire family. A baby may be suffering from a temporary illness or birth defects. Birth defects are a reason to worry for the parents. The most sorrowful part is that babies are too small to speak out their pain. One such birth defect affecting babies is – Hirschsprung’s disease (HD). To know more about the causes, symptoms, and treatment options of HD, carry on with the article.
More about Hirschsprung’s disease
Hirschsprung’s disease is a congenital disorder affecting the large intestine of the newborn. We all know that eating food daily is just as important as excreting undigested food. It is very important for digestion and excretion that all the nerves that send signals to and from the brain work without interruption. Each organ has nerve cells. Similarly, the large intestine has nerve cells known as ganglion cells. These ganglion cells act as a messenger that tells the muscles in the large intestine that they need to contract and relax wave-like so that the stool can go into the rectum and out of your body.
However, a baby born with Hirschsprung’s has no ganglion cells at all in the intestine. As a result, the stool does not move through the intestine. This leads to severe constipation in the baby. In some babies, the area near the anus does not have ganglion cells and in some babies, the entire colon is deprived of nerve cells. The accumulation of stool in the intestine can lead to bacterial growth and infection.
Causes
One of the reasons why some babies are born with Hirschsprung’s disease is that while the foetus is developing inside the uterus, the nerve cells ganglion is also developing at the same time along the intestine via the anus. However, in babies with HD, the development of the nerve cells stops too early. It is unclear why the nerve cells stop developing.
HD can also be inherited from parents. People with Down syndrome (a condition that causes mental retardation) are more likely to develop HD. HD affects about 1 in every 5,000 newborns and is more common in male babies than female babies. Certainly, HD is not a result of anything the mother does or eats during pregnancy.
Symptoms
Parents should be suspicious of HD in their babies if they are constipated and had no bowel movement for more than 48 hours. Their belly may be swollen and they may have excess gas. They may vomit brown or green colour vomit. The doctor may insert a finger into the rectum and explosively excrete the collected stool. They may have jaundice symptoms, poor appetite, poor weight, decreased growth, anaemia, and lack of energy.
In older children, symptoms include:
- Have difficulty passing stool without a laxative (medicine that loosens stool) or enema (flushing water through the anus)
- Excess gas
- Swelling of the abdomen
- Diarrhoea with blood
- Frequent infection of the colon
- Decreased growth
- Anaemia, resulting in a lack of energy
Diagnosis
Diagnosis of HD in Infants and Kids begins with questioning parents about their child’s symptoms and bowel movements. Based on this information, doctors may order tests to confirm HD.
X-rays involve injecting a barium solution into the large intestine through the anus, which shows it clearly on the x-ray. If HD is the cause, the end segment of the large intestine will be narrower than normal. Just before this narrow region, the intestine will appear bulged, which is because of the presence of stools in the large intestine.
Manometry is more commonly used in elderly children and adults. In this test, the doctor infuses a balloon into the rectum, which normally relaxes the rectal muscle, but in HD, the rectal muscle does not relax.
Finally, a biopsy is the most precise test. The doctor will take a small sample from the large intestine and examine it under a microscope. If no nerve cells are visible, then the HD is confirmed.
Treatment
Surgical treatment is the most effective treatment for HD. The surgery involves removing the segment of the large intestine that does not have nerve cells (the ganglia). The healthy part of the colon is then connected to the anus. This procedure is called the pull-through procedure and can be divided into two steps. The first step is to cut the healthy colon away from the affected one. The healthy colon is then moved into the abdominal opening where the intestine is folded into a small pouch called a stoma. This stoma is usually located outside the body and collects stool. The pouch must be emptied multiple times daily. The second step is done after a few months. The surgeon will remove the pouch from the affected one and connect the healthy portion of the colon to the anus. It can also be performed as a one-step surgery only in the case of a healthy baby and a large intestine with no stool. The surgery is performed through the anus without making any cuts in the abdomen.
After surgery
Following surgery, children tend to feel better as they can expel stool and gas more easily than before. Older children should be taught to maintain and clean their stoma pouch. Many children may feel ashamed of being different from other children, it is the responsibility of parents to explain and make them understand. Initially, the stool is loose after surgery, however, as time progresses, constipation is likely to become a major issue. It is suggested that parents consult with their dietician regarding a food plan for their children. Eating foods with high fibre content and fluid will help children with constipation. In some cases, laxatives may not be sufficient to pass stool. The most common complication after surgery for HD is enterocolitis, a severe infection of the colon that leads to severe diarrhoea. In this condition, children often require intravenous fluids and antibiotics to treat enterocolitis. Although enterocolitis is common in the initial few years after surgery, it is rarely seen after 6 years.
Outlook
Parents need to provide their children with a meal plan that is tailored to their dietary needs. Additionally, it is important for parents to be aware of the post-surgical effects of the procedure and to explain it to their child to foster a more normal lifestyle. Neglecting the emotional distress experienced by older children can be more difficult than addressing the issue directly.
