What is the life expectancy with Hirschsprung’s disease? | Hirschsprung’s disease

What is the life expectancy with Hirschsprung’s disease?

Whether or not life expectancy is limited with Hirschsprung’s disease depends on the accompanying malformations that also affect a patient. In 70% of cases, the affected children are completely healthy except for Hirschsprung’s disease. Life expectancy is not restricted and is the same as for other children.

In 30% of cases, children with Hirschsprung’s disease have other diseases. These include Down syndrome and other hereditary syndromes.Life expectancy here depends on the syndrome the child is suffering from. In rare cases, Hirschsprung’s disease occurs together with other malformations without a syndrome. Life expectancy can then be limited depending on the accompanying malformation (e.g. underdeveloped lung).

What are the symptoms of Hirschsprung’s disease in adults?

Adults with Hirschsprung’s disease must be divided into two groups. The first group consists of adults who have been diagnosed with Hirschsprung’s disease as children and have undergone surgery. Depending on the extent of the operation, these patients either have no symptoms at all or suffer from mild to severe complications.

Possible symptoms include incontinence (patients cannot control bowel movements) or a tendency to constipation. Furthermore, scarring of the bowel may develop, resulting in digestive problems or a bowel obstruction, which then requires surgery. Some patients still suffer from frequent inflammation of the bowel by bacteria even in adulthood.

The second group of patients with Hirschsprung’s disease in adulthood consists of those who were not diagnosed as children. This affects only a small percentage of people suffering from Hirschsprung’s disease. Over 90% are diagnosed as babies, and the majority of the remaining patients are diagnosed in later childhood.

Typically, patients who do not receive their diagnosis until adulthood are patients in whom only a short section of the intestine is affected. The typical symptom of these patients is constipation, which is difficult to control even with a high-fiber, healthy diet and adequate fluid intake and light laxative measures. Frequently, these patients can also stop bowel movements only by means of a klysma and suffer severe pain. Constipation – What can be done about it?