Intestinal failure

Overview

Intestinal failure occurs when a child's intestines can't digest food and absorb the fluids, electrolytes and nutrients essential to live and to grow and develop normally. The most common cause is short bowel syndrome, a problem affecting people who have had half or more of their small intestine removed to treat conditions such as trauma or necrotizing enterocolitis. Intestinal failure may also be caused by digestive disorders, such as Crohn's disease or chronic idiopathic intestinal pseudoobstruction syndrome, which causes the bowel to malfunction.

Children with intestinal failure may receive all or most of their nutrients and calories intravenously through total parenteral nutrition, or TPN. TPN is given through a catheter placed in the arm, groin, neck or chest. Patients on TPN may live for many years, but long-term use of TPN can result in serious complications, such as bone disorders, central venous catheter infections and liver disease. At the UCSF Intestinal Rehabilitation and Transplantation Program, our goal is to restore your child's intestinal function to minimize and ultimately eliminate the need for TPN. Unfortunately, not every child can be weaned from TPN. In these cases, we work to optimize the use of TPN and decrease the risk of complications.

Causes

Conditions that cause intestinal failure in children include:

• Hirshsprung's disease, also known as aganglionosis
• Diseases of the intestinal surface, such as congenital villous atrophy or microvillus inclusion disease
• Gastroschisis
• Intestinal atresia
• Midgut volvulus
• Multiple intestinal surgeries resulting in adhesions and problems with motility and/or absorption
• Necrotizing enterocolitis
• Pseudoobstruction and other motility disorders
• Short bowel syndrome

Evaluation

Children with intestinal failure must complete an evaluation process before entering the UCSF Intestinal Rehabilitation and Transplantation Program. Our team reviews each child's complete medical history, including the primary diagnosis, previous surgeries and treatments and current nutritional status, to determine whether the child will benefit from intestinal rehabilitation.

The evaluation process includes:

• Consultations with a gastroenterologist and nurse practitioner, surgeon, nutritionist and social worker
• Laboratory tests
• Radiological tests
• Additional tests when necessary, such as endoscopy with biopsy, gastrointestinal motility testing, breath hydrogen testing for bacterial overgrowth or malabsorption, and imaging tests such as an abdominal CT scan

After the evaluation process is completed, our team determines if your child should be admitted into the program. If your child is admitted, a comprehensive, individual treatment plan is designed to best meet his or her needs.

Treatment

The type and length of treatment differs for each case, depending on the child's needs and goals. Some of the treatments include:

• Counseling and education about intestinal rehabilitation
• Drug and diet modification to train the small intestine to absorb more nutrients
• Consultations to help assess and correct nutrient deficiencies and prevent damage to kidneys, bones and liver
• Placement of catheters in veins called central venous lines and management of these catheters
• Surgery, such as bowel lengthening and tapering that can help increase bowel length and nutrient absorption
• Intestinal transplant

For patients dependent on TPN, we aim to:

• Reduce or eliminate the need for TPN
• Reduce complications associated with TPN
• Decrease diarrhea or vomiting
• Help the patient gain weight
• Decrease the need for intestinal transplantation