Complex Care Protocols Help Most Intestinal Failure Patients Avoid Transplant Surgery
Most patients with chronic intestinal failure will undergo multiple surgeries in their lifetime, but at Cincinnati Children's, the goal is to make sure an intestinal or multivisceral transplant isn't one of them. Our multidisciplinary teams of pediatric specialists employ a multifaceted strategy that includes lipid-minimizing protocols, careful nutritional management, micronutrient therapy and central line care to keep the rate of liver disease low and a child's quality of life as high as possible.
Providing appropriate lipid therapy doses is a balancing act. "High lipid doses are essential for premature, critically ill infants with intestinal failure, because lipids are an important energy source and a means of preventing essential fatty acid deficiencies," says Conrad Cole, MD, MPH, MSc, medical director, intestinal rehabilitation program. "But soy-based lipids also possess inflammatory properties and phytosterols and are deficient of certain micronutrients that can contribute to liver disease. So for patients with chronic intestinal failure—those who will be on total parenteral nutrition (TPN) for more than 30 days—we have to find the lowest possible dose that will provide optimal benefit."
The clinical team weighs multiple factors in determining lipid dose. These include growth parameters — weight, height, head measurements—as well as the patient's essential fatty acid levels. Every patient who leaves the hospital on TPN is on some form of soy-based, lipid-minimizing therapy (soy-based intravenous lipids are the only lipid source approved for pediatric patients by the Food and Drug Administration). Cincinnati Children's patients on chronic parenteral nutrition receive no more than 1g/kilo/day.
The goal is for each patient to have a direct bilirubin of less than 2mg/dL, a level that indicates the liver is healthy enough to encourage the child's growth and development. The intestinal failure team is achieving that goal in 98% of patients.
"We address other factors in the disease process to minimize the risk of liver disease as well," Dr. Cole says. "This includes providing nutritional management—looking at the patient's glucose, lipids, essential fatty acids and protein intake as we monitor their growth. We also monitor their micronutrient levels, since deficiencies can impact the development of liver disease, bone disease and overall neurological development."
Preventing central line infections also can help reduce the risk of an inflammatory cascade that could contribute to the disease process. Dr. Cole and his team utilize 70% ethanol locks during TPN windows of two hours or more. "The catheter-related bloodstream infection rate in our patients on home parenteral nutrition is consistently below two per 1,000 catheter days, whereas the national average is seven per 1,000 catheter days," he explains. "This is due to the ethanol lock protocol, as well as adjunct therapy, individualized education protocols and event analysis."
Cincinnati Children's researchers recently published a study that investigated ethanol lock efficacy and associated complications in children with intestinal failure. Published in the Journal of Parenteral and Enteral Nutrition*, the study found that ethanol lock therapy use reduces both central line–associated bloodstream infections and central line complication rates in children with intestinal failure. These results underscore the safety and efficacy of ethanol lock therapy use in this population.
Dr. Cole adds that his team's multidisciplinary approach is just as important as following evidence-based care protocols. "Gastroenterologists, surgeons, neonatologists, nurses, social workers and other professionals at Cincinnati Children's work together to identify clinical issues early and manage patients proactively," he explains. "This makes a tangible difference for our patients in terms of their health status and quality of life."
For more information or to make a referral to the intestinal rehabilitation program, please call 513-636-6155.
A Case Study: Lipid-sparing protocols, intense case management help avoid multivisceral transplant
The case of a patient with gastroschisis demonstrates how combining our protocols with intensive case management can prevent the need for multivisceral transplantation. This patient, "Jack," underwent life-saving surgical intervention at another institution that required the removal of his large intestine and left him with a 26 cm small bowel. He was transferred to Cincinnati Children's at three months of age with advanced cholestasis combined with ultra-short bowel syndrome.
The patient was listed for a liver-small bowel transplant later that year. Our team elected to increase enteral feedings as tolerated, and reduce total parenteral lipid intake from 3 gm/kg/day to 1 gm/kg/day. Employing those techniques, we achieved improved liver function and made slow but steady progress in reducing the duration of need for total parenteral nutrition.
However, after being discharged home out-of-state, the patient developed recurrent bloodstream infections, and in turn, experienced a stroke, septic shock and multi-organ failure. The patient was airlifted to Cincinnati Children's, where our team stabilized his condition.
We initiated a focused, intensive effort to educate his caregivers, local physicians and local emergency staff, providing comprehensive treatment recommendations to manage his long-term medical needs. Within six months, the patient was tolerating over half of his calories either by mouth or through continuous enteral feedings, and his bilirubin levels gradually normalized. Currently, he takes over 70% of his calories by mouth and 30% via nocturnal tube feedings.
The patient was permanently removed from the multivisceral transplant list. Our team continues to monitor him closely. Last year, we began analyzing his micronutrient levels and supplementing his diet to address deficiencies. Now nine years old, Jack receives extensive educational intervention in school but continues to meet educational and rehabilitation goals.
Please visit the Cincinnati Children's Intestinal Care Center (CCICC) for more information.
*Ethanol Lock Efficacy and Associated Complications in Children With Intestinal Failure
- Ethan A. Mezoff , MD1
- Lin Fei , PhD2
- Misty Troutt , MS, MBA1
- Kim Klotz , RN, MSN, CRNI1
- Samuel A. Kocoshis , MD1
- Conrad R. Cole, MD , MPH, MSc1
Published online before print February 23, 2015, doi: 10.1177/0148607115574745 JPEN J Parenter Enteral Nutr February 23, 2015 0148607115574745
