What You Need To Know About Hirschsprung’s Disease


What You Need To Know About Hirschsprung’s Disease

Hirschsprung’s disease, also known as congenital aganglionic megacolon, is a condition that affects the large intestine, causing difficulties when passing stool. This disorder is characterized by the absence of nerves in parts of the intestine, leading to improper muscle movement in the bowel and potentially life-threatening bowel obstruction. Most individuals living with this disease are officially diagnosed around seven years of age. Therefore, parents must understand this condition so that they can provide the proper care and support. Discover what you need to know about Hirschsprung’s disease and caring for a child with this condition.

Understanding Hirschsprung’s Disease

As mentioned above, individuals with Hirschsprung’s disease lack ganglion cells in their bowels. Most people have these cells, which play a vital role in the movement of food and waste through the digestive system, a process called peristalsis. In the absence of these cells, however, the affected part of the bowel does not function correctly, making it nearly impossible to pass stool normally.

Statistically, Hirschsprung’s disease is not very common and only affects approximately one in 5,000 newborns. The condition features a male predominance, with boys being about four times more likely to be affected than girls. Additionally, it is more common in children with Down syndrome and other genetic conditions. According to the National Organization for Rare Disorders (NORD), symptoms vary from individual to individual, but most are at risk of deadly infections if their condition is left untreated.

Treatment Options

The first line of treatment for Hirschsprung’s disease often involves temporarily relieving the bowel obstruction through procedures like a colostomy or ileostomy. These procedures create an abdomen opening, known as a stoma, to allow stool to exit the body into a bag. This temporary measure effectively alleviates the symptoms and prepares the patient for definitive surgical treatment. However, it is important to note that these procedures are not curative and are primarily aimed at managing the immediate symptoms.

Surgery, usually performed in infancy or early childhood, is the primary definitive treatment for Hirschsprung’s disease. The two main types of surgery are the pull-through procedure and the Soave procedure. Both involve removing the diseased segment of the intestine and reconnecting the healthy parts. While these surgeries have a high success rate, they carry potential risks such as infection, bowel leakage, and the development of enterocolitis, a serious intestine inflammation.

Living with Hirschsprung’s Disease

Children living with Hirschsprung’s disease may need to make daily adjustments to manage their condition effectively. These may include adhering to a fiber-rich diet to facilitate regular bowel movements and reduce the chances of constipation. Regular physical activity can also aid digestion and bowel health. Beyond these physical adjustments, it is crucial to address the emotional and psychological effects of the disease. Regular counseling sessions can provide a safe space for children to express their feelings and learn coping strategies. Support groups can also offer a sense of community and understanding, helping children realize they are not alone in their journey. These resources are especially important for parents interested in helping their children with their ostomy pouch and other daily challenges.

It is important to remember that, though rare, Hirschsprung’s disease can significantly impact a child’s life. However, with early diagnosis and appropriate treatment strategies, children with this disease can lead fulfilling lives. Understanding the important information you need to know about Hirschsprung’s disease is the first step in creating a more comfortable environment for your child.


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