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Hirschsprung’s disease

Posted On: February 1, 2019 By Michelle Mellet
1 Feb

Dietary management in Hirschsprung’s disease after total colectomy

Hirschsprung’s disease is a rare motor disorder of the gastrointestinal system that affects children as well as adults. The disease can cause complications that have to be managed. This article includes a brief overview of what Hirschsprung’s disease is, signs and symptoms and the treatment for the disease. The article also includes functions of the colon as well as dietary management after a colectomy as the treatment for Hirschsprung’s disease specifically in adults. 

1. Overview of the disease
1.1 What is Hirschsprung’s disease?
Hirschsprung’s Disease is characterized by the decreased number of neurons found in the distal colon. This is the result of decreased migration of neural crest cells during fetal development to the colon. These nerve cells aid as a signal for the muscles in the colon to contract to move the stool towards the anus. Without these nerves, stool doesn’t move through the colon. Hirschsprung’s disease is usually diagnosed during childhood before the age of five years, but can also be diagnosed at a later stage during adulthood. The severity of the disease depends on the length of the large intestine that lacks these nerve cells. 

1.2 Signs and symptoms of the disease
Patients present with constipation, abdominal distention (swelling of the abdomen), intestinal obstruction or abdominal pain. Constipation occurs when there is fewer bowel movements and stools can be very difficult to pass. When no stools can be passed, intestinal obstruction or blockage may occur. Other symptoms include flatulence, diarrhoea that contains blood or anaemia (lack of red blood cells). Hirschsprung’s disease is diagnosed based on the presence of clinical symptoms as well as tests. Tests include an x-ray, manometry or a biopsy. When an x-ray is performed, signs of a dilated colon can be seen or even a dilated colon with impacted stool. This means that a part of the large intestine will look narrower than normal and before this part, the large intestine will bulge. This is where the stool blocks the intestine. During Manometry, the doctor will place a balloon within the rectum and inflate the balloon. Usually, the muscles relax, but if this fails to happen, it may be an indicator of Hirschsprung’s disease. The doctor may also perform a biopsy where a piece of the intestine is inspected under a microscope. If there is a lack of nerve cells, it may be Hirschsprung’s disease. 

1.3 Treatment of the disease
Surgery is recommended in adult patients. These surgeries are extremely complicated due to the fact that the walls of the colon, sigmoid or rectum are thickened. The affected part of the colon can be removed and the two parts are then joined together, or the whole colon can be removed (total colectomy). 

2. Functions of the colon in vitamin and mineral absorption
Bacteria in the colon digest small quantities of fibre. Due to this bacterial activity, small amounts of thiamin (vitamin B1), riboflavin (vitamin B1), vitamin B12, biotin, and vitamin K are formed. Although these vitamins are formed in extremely small quantities in the colon during bacterial activity, it is not absorbed in the colon but in the small intestine. The main function of the colon is to absorb water, potassium, sodium and vitamin K. Potassium is required to maintain a normal water balance together with sodium, it plays a role in neuromuscular activity as well as maintaining a normal pH balance. Sodium regulates extracellular and plasma volume, it also plays a role in the neuromuscular function and maintains the acid-base balance. Vitamin K is required for blood clotting, bone formation and regulation of certain enzyme systems. 

3. Dietary management after removal of the colon (colectomy)
The most important part is to increase fluid intake, especially water, to prevent dehydration due to the fact that water is not reabsorbed and is lost through faeces. Other nutrients as discussed below should also be taken in larger quantities due to faecal losses. Potassium can be found in fruits, starchy vegetables, non-starchy vegetables and dairy products such as avocado, cantaloupe, mango, papaya, kiwi, cabbage, spinach, tomato, sweet potatoes, pumpkin, cooked mushrooms, and yoghurt. Sodium can be consumed by using iodized salt when cooking. Vitamin K is found mostly in dark green leafy vegetables such as spinach, broccoli, cabbage, green beans, and cucumber. Any fried foods should be avoided for example doughnuts and deep-fried meats. High sugar-containing beverages, as well as foods (cakes, biscuits, sweets, chocolates, etc.), should be avoided as this may worsen diarrhoea and thus fluid and electrolyte losses. Consumption of sugar-free drinks and sweets should also be avoided since sugar substitutes used may worsen diarrhoea and cause abdominal discomfort. When the patient has a stoma (an opening from the intestines towards the skin), special care should be taken when consuming certain foods. Some foods may cause unpleasant odours and should be avoided: onions, garlic, eggs, cabbage, fish, asparagus and broccoli. Gas-forming foods should also be avoided since it can cause the pouch covering the stoma to enlarge causing some discomfort and can also cause dislodgement of the pouch in extreme cases. These foods, for example, are onions, legumes, cabbage, broccoli, and cauliflower. Foods that may obstruct the stoma should be avoided such as apple peels, whole nuts, large seeds, pineapple, mushrooms, and corn. 

As discussed above, Hirschsprung’s disease is a rare disorder but affects children and adults. The disease can be managed by making suitable dietary changes to manage symptoms, or the patient’s diet can be altered to suit the surgery performed as a treatment for Hirschsprung's disease. 


Michelle Mellet
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