Intestinal failure can be described as a state when the intestine is unable to function optimally to meet the nutritional needs or maintain the fluids and electrolyte needs of a patient. Most often, intestinal failure is due to short bowel, or SB — secondary to loss of bowel length. Intestinal failure from loss of function or disorders of motility are rare. Most patients with SB are infants, and causes of SB include necrotizing enterocolitis (inflammation of the intestine seen often on premature babies), gastroschisis and malrotation with volvulus. In older children, malrotation with volvulus is the most common cause.
The initial phase following the onset of SB includes a state of disequilibrium, complicated by fluid shifts and electrolyte imbalance. Often, these patients have stomas — a surgically created opening on the abdomen that allows stools and urine to exit the body — and managing stoma output can be challenging. This phase is followed by a prolonged state of adaptation where the bowel increases the surface area of absorption by hypertrophy of villi (small projections in the small bowel) and dilation or enlargement of the small bowel. In younger children, the continued growth of the small bowel is an advantage, and this can go on until the age of 8 to 10 years. Providing optimal nutrition is very crucial during these phases, either by mouth, nasogastric or gastrostomy along with hyperalimentation (IV nutrition) administered by a central line. Infections of the central line can become problematic with these patients, often leading to repeated line placements and sometimes loss of intravenous access. Small bowl bacterial overgrowth, or SBO, may contribute to increased stool output, and lead to malabsorption and occasionally acidosis.
Often, hyperalimentation becomes the main source to provide optimal nutrition in these patients, and if not managed carefully, it could lead to cholestasis and parenteral nutrition asssociated liver failure. PNALD could lead to cirrhosis and portal hypertension. With careful management of hyperalimentation, the risk of significant PNALD can be minimized.
Patients with intestinal failure who are on hyperalimentation should be closely monitored by a team experienced in the care of these patients. This team often consists of: physicians (a gastroenterologist), dietitians, pharmacists and nurses. The purpose of the team is to identify nutritional needs and maintain growth of these patients, so that it’s around 5 to 10 percent on the weight-for-height or BMI chart. Patients are monitored for essential fatty acid deficiency, and lipids are run at the lowest possible rate. Feeds should be started at the earliest possible time, with either breast milk, extensive hydrolysate or amino acid-based formula based on response and stool or stoma output.
Patients who are discharged to their home will need monitoring of hyperalimentation, growth and nutritional status, including trace elements, fat soluble vitamins and essential fatty acid status at regular intervals. The goal is to wean patients off hyperalimentation, and help them transition onto a diet that can be taken by mouth or given by tube-feeding. Those patients, who are predominantly dependent on and cannot be weaned off hyperalimentation, may need to be considered for bowel lengthening surgeries or a small bowel or a multivisceral transplant. A timely referral to a center that specializes in such procedures has been shown to improve outcome in IF patients and helps identify patients who may need a transplant.
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Managing Intestinal Failure in Children originally appeared on usnews.com
