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Children's Intestinal Rehabilitation Program
Intestinal failure is the inability of the small bowel to absorb adequate amounts of nutrients, fluids, and electrolytes. The causes of intestinal failure in children are usually grouped into three major categories: short bowel syndrome (SBS), neuromuscular diseases involving the gastrointestinal tract (such as total aganglionosis, long-segment hirschprung disease, or chronic intestinal pseudo-obstruction), and congenital diseases of the intestinal epithelium (microvillus inclusion disease). The most common cause of SBS, especially in premature infants, is necrotizing enterocolitis. Other etiologies of SBS in children include gastroschisis, intestinal atresia, and mid-gut volvulus.
Regardless of the etiology, children with intestinal failure require very complex medical care. This care includes providing appropriate nutrition for normal growth, maintenance of optimal fluid and electrolyte balance, management of potential complications, and provision of the specialized care for the subset of patients who will require small bowel and/or liver transplantation. The goal of intestinal rehabilitation is to optimize bowel function through the use of multiple therapies and to eventually wean patients with intestinal failure from parenteral nutrition.
The new multidisciplinary Intestinal Rehabilitation (IR) Program at Children’s National provides individualized, comprehensive medical, dietary, and surgical treatment for often critically-ill children. The medical treatment focuses on aggressive dietary management with very precise control of metabolic balance and prompt and effective treatment of complications. Aggressive use of specialized enteral feeding programs by the experienced medical team helps to maintain nutrition and hydration, which are important factors in longterm survival. Another important component of the program is on-going parent education and support.
Children’s Intestinal Rehabilitation Program is a part of the joint program of Liver and Small Bowel Transplantation developed between Children’s National and Georgetown University. Although the highly specialized Program is new at Children’s, team members have extensive experience in managing these challenging patients.
Clarivet Torres, MD, director of the Intestinal Rehabilitation Program at Children’s National is a nationally recognized leader in this field, based on work at the University of Nebraska. She is a long-time colleague of Stuart Kaufman, MD, who is medical director of Georgetown’s Pediatric Liver and Intestinal Transplantation Program. Other members of Children’s team include Anthony Sandler, MD, division chief, General and Thoracic Surgery; Parvathi Mohan, MD, a nationally recognized expert in liver and bowel disease; and Pat Zavosky, RN, who has more than 15 years of experience managing critically ill children with bowel disorders. The team also includes a dedicated staff of nurses, nutritionists, and social workers.
Until recently, the outcome for many children with intestinal failure was very poor. For example, previous studies indicated that children with less than 40 cm of small bowel length and no ileocecal valve (ICV) or children with less than 15 cm of small bowel with an intact ileocecal valve were unlikely to be able to be weaned from parenteral nutrition. In addition, children with direct bilirubins more than 2.5 mg/dL were more likely to die.
Although it is challenging to successfully treat children with intestinal failure, newer data provide hope for the attainment of more successful outcomes. Dr. Torres recently published a summary of her experience from the past six years. During this period, 74 parenteral nutrition dependent intestinal failure patients were evaluated. The majority of the patients had only jejunum with a median intestinal length of 38 cm (range 8 cm to 150 cm). The median daily caloric requirement by parenteral nutrition at the time of evaluation was 100 percent (range 55 to 100 percent). Fifty-one of the 73 patients had cholestatic liver disease: 12 with cirrhosis, 27 with bridging fibrosis, and 12 with portal fibrosis. Forty-seven patients had 64 different intestinal surgical repairs, including 15 bianchi procedures and 17 step enteroplasties. Forty-one of the 51 with direct bilirubin greater than 2.5 mg/dL normalized the serum bilirubin with treatment.
Ten patients required transplantation; two are listed for transplant and seven patients died. Of the remaining 55 IRP patients, 39 have been weaned from TPN, including 25 with severe liver disease marked by varying degrees of fibrosis or cirrhosis. Seventeen additional patients are in the process of weaning from parenteral nutrition, and have decreased their median parenteral nutrition requirements from 100 percent to 40 percent. Several laboratory measurements also improved in these 55 patients. The mean direct bilirubin dropped from 6.3 mg/dL to 0.4 mg/dL (p = <0.0001), the mean albumin increased from 3.0 to 3.7 (p = <0.0001), and the mean platelet count increased from 200,000 to 300,000 (p = 0.0007). One of the most clinically important outcomes was the improvement in growth. The surviving non-transplant patients have had statistically significant improvements in their mean weight Z scores and height z-scores. The survival rate of the patients in the Intestinal Rehabilitation Program study was 90 percent.
These data are dramatically better than those published in previous medical literature and provide real hope for an improved future for children with intestinal failure, including those with chronic liver disease. More aggressive intestinal rehabilitation can be very effective in improving the outcomes for patients with intestinal failure, including those who have risk factors previously identified for poor prognosis in terms of parenteral nutrition weaning or survival. Even the patients who subsequently required transplantation improved their general state of health while in the program and were better able to tolerate surgery and immunosuppression. None of the Intestinal Rehabilitation Program patients on the transplant waiting list in Dr. Torres’ study have died or were transplanted in critical condition.
The initiation of the Intestinal Rehabilitation Program at Children’s National provides children with intestinal failure the chance to receive comprehensive medical and surgical care, giving them the chance for improved long-term survival, including weaning from parenteral nutrition and avoidance of the need for transplantation and long-term immunosuppression. Early referral of patients with intestinal failure prior to the development of advanced liver disease is recommended.
For inquiries about patients with intestinal failure or early referral of patients, contact the Division of Gastroenterology, Hepatology, and Nutrition at 202-476-3032 and ask for Pat Zavosky.
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