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What is short bowel syndrome?

Absorption of nutrients, vitamins and minerals occurs in the small bowel. Short bowel syndrome is the result of substantial loss or malfunction of absorptive intestinal surface. Absence or malfunction of 75 percent or more of the intestinal tract usually results in severe short bowel syndrome and the need for total parenteral nutrition, or TPN — that is, supplying the body with nutrition intravenously by bypassing the gastrointestinal tract.

Clinical symptoms of short bowel syndrome are similar in children and adults. Diarrhea, weight loss and malnutrition are the major features. For those reasons, patients with short bowel syndrome are unable to maintain nutritional or intestinal fluid and electrolyte balance using their intestinal tract. They become severely dehydrated, which can be life threatening. Additional problems associated with dehydration and malnutrition include weakness, fatigue and depression, as well as bacterial infections due to the breakdown of the protective layer of the intestinal tract.

Traditional treatment

Until recently, TPN was the only treatment to keep patients with short bowel syndrome alive. However, TPN is very expensive, costing between $200 and $500 per day when received at home and $500 to $1,500 per day to administer in a hospital. Annual costs can easily exceed $500,000 per year per patient. Many TPN patients are chronically ill, frequently hospitalized and subject to life-threatening complications, such as recurrent catheter infections or, especially in children, lack of vascular access. Additional problems are bone demineralization, electrolyte disturbances, gallstones, and pancreatitis. Moreover, TPN is also associated with liver damage, which can result in fatal liver failure. The reasons for liver failure in patients on TPN are still poorly understood. While the incidence of liver failure in adult patients on TPN is only five percent, it can be as high as 25 percent in children.

For patients with short bowel syndrome only, intestinal or bowel transplantation alone is a life-saving procedure. For patients with both short bowel syndrome and end-stage liver disease secondary to TPN treatment, combined intestinal/bowel and liver transplantation is a life-saving procedure that will not only eliminate the need for TPN but also cure liver failure.

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