It's normal to feel anxious about the little things as well as the joy of bringing a newborn home, isn't it? Parents are especially concerned about their baby's eating, drinking, bathing, and defecating (we call it ' peeing '). So, if your baby hasn't pooped in two days, or about 48 hours, or if his belly is unusually swollen and hard, this is something that will be very important to you. Today we're talking about a condition that some babies are born with called Hirschsprung's Disease.
What is Hirschsprung's Disease?
Simply put, Hirschsprung's Disease is a condition that your baby is born with (i.e., a congenital condition). It causes the passage of stool in a part of your baby's large intestine (colon) to slow down or stop completely. Sometimes doctors call it ``congenital megacolon.''
Think about it, when we eat, we need to digest the food we eat and pass it out as waste. For this process to happen properly, the muscles in our intestines need to contract and relax. This is signaled by nerve cells. In a baby with Hirschsprung's Disease , the nerve cells at the end of the large intestine, near the anus, are not properly developed. These special nerve cells are called ``neural crest cells.''
So, when the stool travels through the baby's intestines and reaches the area where the nerve cells are not functioning properly, it becomes blocked and cannot pass through. This is when constipation occurs. If this condition is not treated immediately, it can lead to serious complications . Therefore, it is very important to be aware of this.
How common is this disease?
Hirschsprung's Disease is not a very common disease. It affects about one in 5,000 births . It is also three to four times more common in boys than in girls.
What are the symptoms of this disease?
Some babies can have a bowel obstruction at birth due to Hirschsprung's Disease. If your baby hasn't had a bowel movement within 48 hours of birth , it could be a major sign of this condition. So it's important to be aware of it.
Other symptoms that may be seen in newborn babies include:
- Abdominal bloating: The baby's abdomen may feel as if it is full of gas and may feel hard to the touch.
- Constipation: Defecation is very difficult to pass, or it does not pass for several days.
- Vomiting: Sometimes the vomit may be yellow or green.
- Diarrhea: Some babies may have diarrhea instead of constipation.
- Loss of appetite and weight loss: The baby may not want to drink milk, so he or she may not gain weight properly.
- Growth retardation.
If your baby has one or more of these symptoms, it is best to seek medical advice immediately.
Why does Hirschsprung's Disease occur?
When a fetus is in the womb, those neural crest cells we talked about earlier grow from the top of the baby's small intestine all the way down to the anus. However, in babies with Hirschsprung's Disease, these nerve cells stop growing somewhere in the large intestine. Doctors still don't know exactly why this happens.
However, in less than 20% of cases, this disease can be seen to be passed down from generation to generation in families. This means that it is possible that it is due to a genetic mutation .
What are the risk factors for developing this disease?
If someone in the family, such as a parent or sibling, has the disease, there is a certain risk that the baby will also develop it. In addition, babies with the following conditions are also at higher risk of developing Hirschsprung's Disease:
- If you have congenital heart disease .
- If you have Down syndrome .
What are the possible complications of this disease?
About 40% of babies with Hirschsprung's disease develop a condition called enterocolitis. This causes the baby's small and large intestines to become inflamed and infected. While this is mild in some babies, it can be severe enough to be life-threatening in others. If severe enterocolitis develops, the baby may have a fever and suddenly have severe diarrhea.
Important: If Hirschsprung's Disease is not treated properly, it can lead to serious, life-threatening conditions such as:
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* Large bowel obstruction: In this, the movement of food through the baby's large intestine completely stops.
* Toxic megacolon: This is a rare but life-threatening condition. The baby's large intestine becomes extremely swollen and enlarged. Gas and stool cannot pass through the swollen intestine and become blocked. This increases pressure inside the intestine, allowing bacteria to enter the bloodstream. This can lead to life-threatening conditions called `Enterocolitis` and `Sepsis`. In some severe cases, a hole (`perforation`) in the intestine can occur.
Other complications that can be seen include:
- Frequent involuntary bowel movements.
- Diarrhea.
- Fever.
- Malnutrition.
- Severe constipation.
- Abdominal bloating.
- Vomiting.
Therefore, it is very important to seek medical advice as soon as you notice symptoms.
How is Hirschsprung's Disease diagnosed?
When you take your baby to the doctor, the first thing you do is feel the baby's abdomen. They check for swelling, pain, and stool blockage. Then, they check the rectum to see if there is any stool buildup.
In addition, the doctor may perform one or more of these tests:
- X-ray: An X-ray of the abdomen can be used to check for a blockage in the intestine.
- Contrast enema: In this, a doctor inserts a thin tube called a catheter through the baby's anus. A safe liquid called contrast is sent through the tube into the intestine. Then, an X-ray is taken to see how the liquid moves through the intestine. This can help to see exactly if there is any blockage or narrowing in the intestine.
- Biopsy: This is the most important test to confirm the diagnosis. The doctor uses a special instrument to remove a small piece of tissue from the end of the baby's large intestine (rectum). Then a pathologist looks at the tissue under a microscope to see if the nerve cells we talked about are there. This test is not painful for the baby, and there is no need for anesthesia.
How is Hirschsprung's Disease treated and managed?
There is no cure for Hirschsprung's Disease. However, surgery can be used to successfully treat the condition. There are two main types of surgery: a pull-through procedure and an ostomy surgery.
Pull-through procedure
This is the most common and most common surgery for Hirschsprung's disease. In this surgery, the surgeon removes the part of the baby's large intestine that lacks nerve cells and connects the healthy part of the intestine directly to the anus. This surgery can be done either laparoscopically (through a few small incisions) or conventionally. The baby recovers quickly after this surgery.
Ostomy surgery
Some babies may need to have an ostomy surgery before or at the same time as the pull-through procedure. This can be a colostomy (a procedure for the large intestine) or an ileostomy (a procedure for the small intestine).
In this procedure, surgeons attach a portion of the large intestine or small intestine to the skin of the baby's abdomen. Instead of the baby's anus, the baby's stool comes out through an opening called a "stoma." An "ostomy bag" is attached to the baby's body to collect the stool. This is usually temporary. Later, it is removed in another surgery, completing the "pull-through procedure."
Additional treatments
In addition to surgery, these treatments may also be helpful:
- Bowel management: A method that uses medications and/or enemas to maintain healthy bowel habits in a baby.
- Sacral nerve stimulation: A surgeon implants a small device in the lower back to help control bowel and bladder control.
- Biofeedback: This is a therapeutic technique that teaches us different ways to control bodily functions that we don't realize we have, such as bowel movements.
What are the possible complications or side effects after pull-through surgery?
Babies with Hirschsprung's Disease usually recover quickly after ``pull-through surgery.'' However, some babies may experience the following problems even after recovery:
- Fecal incontinence.
- Constipation.
- Intestinal infection (Enterocolitis).
If these problems arise, your baby's surgeon and possibly a gastroenterologist will help you manage them.
How quickly will the baby recover after treatment?
If all goes well, the baby will start to feel much better within a few days of the surgery. Remember, although Hirschsprung's Disease cannot be completely cured, surgery can achieve very successful results.
How is the situation after the surgery?
After surgery, some babies may continue to have constipation, incontinence, and intestinal infections. However, with long-term medical supervision and proper management, most children can live a normal life without severe constipation and difficulty controlling their bowel movements.
Can I prevent Hirschsprung's Disease?
Unfortunately, no one can prevent Hirschsprung's Disease. If you have the disease, or if someone in your family has had it, it may be a good idea to see a genetic counselor before you start a family.
How can I help my child cope with Hirschsprung's Disease?
After surgery, there are several treatments that can help your child manage the condition:
- Nutritional guidance: You can seek advice from a nutritionist about what foods are suitable for your child and what to do to prevent constipation.
- Constant care from specialists and nurses: Supervision by a gastroenterologist and a nurse trained in this regard is important.
- Biofeedback / pelvic floor rehabilitation: These help improve bowel control.
- Support from a psychologist or social worker: The child and family can receive psychological support to help them cope with this challenge.
Always talk to your child's doctors. They will help you choose the best diet and treatment options for your child.
When should I tell the doctor about my baby's Hirschsprung's Disease?
After surgery, especially within the first year, babies can develop an intestinal infection (enterocolitis). If your baby shows any of these symptoms, notify the doctor immediately:
- Rectal bleeding.
- Diarrhea.
- Fever.
- Abdominal bloating.
- Vomiting.
Remember, just because your baby is born with Hirschsprung's Disease doesn't mean he or she can't live a happy, healthy life. The condition is treatable with surgery. However, the time leading up to surgery can be very stressful for you. It's normal to feel sad and scared when dealing with a newborn baby. Ask your family and friends for help. Let them help with household chores and cooking. Then you can spend time with your baby and get some rest.
Take-Home Message
- Hirschsprung's Disease is a condition that some babies are born with, causing difficulty defecating due to the nerve cells in the large intestine not developing properly.
- If your baby has symptoms such as no bowel movements, bloating, or vomiting within 48 hours of birth, see a doctor immediately.
- The disease can be confirmed with a biopsy.
- A pull-through procedure is the main treatment. Sometimes an ostomy surgery may be performed temporarily.
- After treatment, most babies can live normal, healthy lives. However, continued medical supervision is required.
- You are not alone on this journey. Get support from doctors and family.
Wishing your baby a speedy recovery!
👩🏽⚕️ Additional questions (FAQs)
💬 Is Hirschsprung's Disease a common problem that causes a baby's stomach to become dry?
This is not a common constipation, but a very dangerous 'congenital disease'. When a baby is developing in the womb, the nerves to the lower end of the large intestine are not properly attached. Because there are no nerves, that part of the intestine does not work, so the stool that accumulates in the intestine cannot be pushed out. Eventually, all the stool gets stuck inside.
💬 How do you know if your baby has this condition when he is born?
The main and first symptom is that the baby does not pass the first black stool (Meconium) within the first 48 hours (2 days) of birth. Also, the baby's stomach is unusually large and swollen. He vomits without drinking milk, and sometimes the vomit may be green (Bile).
💬 What are the medical treatments for this?
This is a congenital disease without nerves, so it cannot be cured by taking medicine! The only and most essential life-saving treatment for this is a minor surgery by a pediatric surgeon at the hospital to 'cut out and remove the part of the intestine that does not have nerves and does not work' and connect the remaining good intestine to the anus (Pull-through procedure).
` Hirschsprung's Disease, Hirschsprung's disease, infantile constipation, congenital megacolon, intestinal surgery, pediatrics, pull-through procedure, ostomy
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