What You Need to Know About Hirschsprung’s Disease
Hirschsprung’s disease is a condition where the colon enlarges beyond a normal size. The bowels are only affected by about 30 centimeters. This disease was discovered in the late 1800s by Harald Hirschsprung. There were two children, newly born, that died due to swollen midsections. After examining the bodies the doctor discovered that they had enlarged colons that led to their death. After that the disease became known as “Hirschsprung’s Disease.”
It is a congenital disease that occurs in young children, generally infants when they are born. The symptoms are usually painful constipation, and some infants may not be able to relieve excrement at all. It is very important that it is treated as soon as possible, because the stool will begin to collect and cause severe problems. Infections, colon bursting, and death are all possibilities if it goes untreated.
Explanation of Symptoms
The constipation that babies experience when they have HD is painful and uncomfortable. While the child cannot express their discomfort, they may cry often and make signs of struggling. The nerve cells in the large intestine that help push out waste are damaged or nonexistent in babies that have HD. The waste then continues to build up when it is not being released.
Infection will start to develop if the waste is left there for too long. That is why immediate action to fix to the problem is required for the safety and health of the baby.
Abdominal swelling and vomiting bile are two major signs that the baby has HD. In some cases the symptoms don’t even appear until later in life, such as the teenage years or adult years. A newborn should have a bowel movement shortly after being born. This is a sign that everything is working fine and the baby is healthy. If the child does not have a bowel movement, which may mean that they have HD.
Causes of HD
This condition is genital, therefore it develops while the child is developing in the womb. Essentially, something stops the nerve cells from developing. There isn’t any significant research behind HD that tells us why it develops. Children with other disorders may be born with HD.
If you plan to have children in the future, there is a small chance that the baby will also be born with HD. It completely depends on hereditary factors.
Mothers cannot do anything to prevent this from happening, so if you are a parent feeling guilty or upset about your baby having this condition, know it is not your fault.
Testing and Diagnosis of HD
The doctor will prescribe a number of tests to determine if HD is the problem or if another condition is at fault. A barium enema x-ray is one of the easiest methods. The Barium Enema basically “highlights” any problems in the bowels, and the x-ray identifies them. A manometry is another procedure that tests the bowels and intestines to determine if anything is wrong with the body. The procedure involves small balloon inflation inside the rectum. A healthy rectum would relax immediately, but if it doesn’t relax then HD may be a problem.
Biopsies are an accurate method for testing for HD. A small piece of intestine is extracted and examined. The doctors can then look at the cell framework and determine what the problem may be. Some doctors prefer to do all of the required testing, but others may only need one test to determine a diagnosis. If you want all tests to be performed for safety reasons, then simply ask. If you have doubts about the surgeons credibility, see a different doctor or a specialist.
Treating an Infant with Hirshsprung’s Disease
Obviously treating Hirshsprung’s Disease is extremely important and will result in death if treatment is not followed. There are a few different methods for treating this disease. Some or all may be used to accurately treat the baby in the least painful manner.
Colostomy is the first part of treatment. The surgeon will surgically cut the colon where there is an abnormal growth or problem. The waste will then excrete into a special bag. This bag must be cleaned and taken care of to prevent infection. It may smell strongly but it gets the job done.
As the child grows, doctors will need to keep close tabs on weight, and age. They will know when part two is required. This part basically repairs the colon by connecting the parts to each other so that they can work normally. Pull-through surgeries are constantly changing and becoming more efficient. There are a few different pull-through methods, all of which work just as well. Some doctors prefer a specific method because that is their expertise.
Some infants may not need a colostomy. It purely depends on the severity of the condition and how well the doctor is familiar with this disease.
Prognosis After Treatment of Hirschsprung’s Disease
After a successful treatment the majority of patients are able to live a normal and fulfilling life. There are no restrictions on their lifestyle. A small percentage, around 15 percent, must rely on a laxative for the rest of their life. After surgery the child may start staining underwear or having accidents. This goes away in time. If the child has to use a laxative, they are able to choose their own laxative types (tea, pills, herbal, or chemical) and adjust their dosage accordingly. Patients that become dependant on the use of laxatives may need to take extra precaution in staying hydrated. This will prevent colon problems as well as other bodily conditions caused by dehydration.
The only other thing to monitor after surgery is food and liquid intake. Since the intestine is made shorter, it is extremely important for the child to eat and drink more. Consuming more liquids and food will ensure that the child is getting adequate nutrients to sustain good health. Healthy foods are a must. The child should eat good whole grain fiber, as well as fruits and vegetables.
Combating Infection after Surgery
If fever, swelling, vomiting, diarrhea, bleeding, and lethargy are present it is extremely important to rush the child to the emergency room. These symptoms are common when infection of the intestine is present. Infection can either be caused by the surgeon that performed the procedure, or inadequate healing. Antibiotics and IV treatments are used to help fight the infection. Doctors will need to perform regular rinsing of the intestines. Extended hospital stay may be required depending on the severity of the infection.
Important Facts about HD
• It is a congenital disease of the large intestine. It is not caused by something the mother did or didn’t do.
• Symptoms involve no first bowel, swollen midsection, and slow development.
• Sometimes symptoms do not appear until the child is older.
• Infection is a potential problem before surgery or after.
• If treated immediately there will likely be no further complications.
• Prognosis is positive.