The CUIRE Program at Johns Hopkins All Children’s Hospital provides a comprehensive range of services for rehabilitating patients diagnosed with short bowel syndrome, severe gastrointestinal dysfunction and intestinal failure.
Intestinal failure in children is commonly caused by short bowel syndrome (SBS), which means the small intestine cannot absorb enough of certain nutrients and fluids. Such a disorder may occur from surgical removal of large sections of bowel either because of diseased bowel, intestinal trauma or loss of blood supply to the gut. The degree to which patients suffer from consequences of SBS and develop intestinal failure depends largely on the anatomy of the child.
Many patients with extensively compromised intestines that require large sections to be removed require a feeding method that bypasses the gastrointestinal tract. This method, called total parenteral nutrition (TPN), involves providing nutrients through fluids in the vein. TPN saves thousands of lives each year, but with long-term use, some patients experience complications, including central line associated blood stream infection (CLABSI) or blood clotting known as thrombosis.
Intestinal rehabilitation is the science that stimulates the amazing ability of the intestine to adapt to different and unexpected medical and surgical conditions. It encompasses pharmacological, dietary and surgical options that can maximize intestinal adaptation and function.
The CUIRE Program at Johns Hopkins All Children's Hospital will coordinate care for patients with complex intestinal disorders in the hospital, after discharge and if necessary upon readmission.
Short bowel syndrome and similar disorders in which the muscles of the digestive system do not work normally can lead to instestinal failure. Those that requre intestinal rehabilitation include:
- Necrotizing enterocolitis (surgical NEC)
- Intestinal atresia
- Intestinal pseudo-obstruction
- Microvillus inclusion disease
- Autoimmune enteritis
- Intestinal polyposis
- Hirschsprung Disease
Managing Instestinal Failure, a Team Approach
The pediatric intestinal rehabilitation program is devoted to preventing liver failure using medical, surgical, and nutritional rehabilitation strategies with a goal of avoiding transplantation through intestinal rehabilitation. The benefits lead to shorter stays in the intensive care unit and fewer complications.
This team evaluates patients meeting criteria for rehabilitation as they focus on ensuring continuity of care by integrating treatment plans among the team members, the patient and the family. Patient and provider education along with improved clinical outcomes is the cornerstone of the program.
The clinical team consists of:
- Pediatric Gastroenterologist
- Pediatric Surgeon
- Pediatric Hospitalist
- Registered Dieticians
- Registered Advanced Nurse practitioner (coordinator)
- Clinical Pharmacist
- Speech therapist
- Clinical Social Worker
After the initial evaluation is complete, our team of experts will discuss each patient’s case, determine a management plan, and review the patient’s medical care with the referring team on a regular basis. This approach ensures the creation of an integrated comprehensive patient/family-focused plan of care with emphasis on continuum of care and improving clinical outcomes.
Strategies used by the team include:
- A focus on nutrition to help patients maintain normal growth
- Meticulous parenteral management to minimize TPN-associated liver disease.
- Prompt monitoring to deter and/or treat blood stream infections.
- Management of procedures like serial transverse enteroplasty procedure (STEP), a technique used to increase bowel length and help increase intestinal absorption.
- Prompt surgical intervention as needed, which can increase the quality and function of intestines to improve the patient’s nutrition, growth and development.
- Coordinate and collaborate inpatient care with primary and specialty care physicians, including a neonatologist, gastroenterologist, pediatric surgeon, hospitalist, nutritionist and other ancillary services.
- Development of a plan of action for following patients from birth to discharge, in addition to monitoring any subsequent hospital readmissions with complications.
- Prepare patients for home care with parenteral nutrition, ensuring patients and families are aware of all public benefits and arrange adequate follow-up care.
- Coordinate nutritional and gastrointestinal care and transition programming.
- Coordinating care in the outpatient setting with speech and occupational therapists to minimize complications with eating disorders.
- Track long term neurodevelopmental and feeding outcomes in an outpatient clinic
The CUIRE Program takes advantage of the collaborative efforts and databases at Johns Hopkins All Children's Hospital to use best practices for parenteral nutrition associated with liver disease (PNALD). The team maintains a data base of patients to track outcomes as well as monitor and improve growth and long term neurological outcomes of patients with chronic illnesses. The CUIRE program at JHACH participates in the Pediatric Intestinal Failure Consortium (PIFCON), a study of the pediatric intestinal failure population, including the medical, surgical, nutritional and therapeutic management of these patients.
Our team of experts, including residents and neonatology fellows, help patients and families by providing information about medical and surgical options, prognosis, lengthy hospital stays and transition care at home. The team uses an aggressive medical/surgical approach so that patients with intestinal failure and advanced liver disease can avoid transplants. Studies have shown that patients in intestinal rehabilitation programs have improved clinical outcomes.