Tag Archive for: Torres

Human Digestive System Anatomy

Intestinal rehabilitation program demonstrates high survival rates

Human Digestive System AnatomyIn a new study, researchers at Children’s National Hospital analyzed outcomes in children with short-bowel syndrome (SBS), parenteral nutrition dependence (PND) and intestinal failure-associated liver disease (IFALD) who were treated in the Children’s National Intestinal Rehabilitation Program (IRP) from 2007 to 2018.

The study, led by Clarivet Torres, M.D., director of the IRP at Children’s National, reviewed charts of 135 patients with SBS-PND at the time of their enrollment in the IRP. Of these, 89 patients had IFALD, defined as sustained (>2 months) conjugated bilirubin (CB) of ≥2 mg/dl at enrollment and/or abnormal liver biopsy, showing stage two through four fibrosis.

Historically, IFALD was a major cause of mortality among patients with intestinal failure and accounted for the high percentage of pediatric small-bowel transplants. Over the last two decades, outcomes of patients with SBS, PND and IFALD have been improving with creation of intestinal rehabilitation programs.

Recent data from the International Intestinal Failure Registry Pilot Phase showed improved outcomes, with 8% mortality. However, IFALD was the cause of death in 7% and almost 50% of patients were still PN dependent at 12 months of follow-up.

The study at Children’s National reveals normalization of the conjugate hyperbilirubinemia in 99% of the patients with IFALD, over a median time of 9.5 weeks after enrollment, with nontransplant treatment. It also demonstrates a transplant-free survival in 97% of these patients. Although the median percentage of expected bowel length in the study population was only 23% and over half had no ileocecal valve, 81% of patients were successfully weaned from PN over a median time of 5 months of being enrolled in the IRP.

Dr. Torres and her team demonstrate high rates of transplant-free survival and enteral autonomy among SBS children with IFALD who do not have underlying primary motility/genetic disorders.

Despite a high acuity of symptoms and pathology (for example, ultrashort bowel, initially high PND, initial CB >4 mg/dl, significant fibrosis/cirrhosis), they reduced their PN needs relatively quickly and improved IFALD. As one of the larger IRPs in the United States, Children’s National demonstrates high survival rates of patients without the need for liver or intestinal transplantation.

Other Children’s National authors include Vahe Badalyan, M.D., and Parvathi Mohan, M.D.

Torres C, Badalyan V, Mohan P. Twelve-year outcomes of intestinal failure associated liver disease in children with short bowel syndrome: 97% transplant -free survival and 81% enteral autonomy.  Journal of Parenteral and Enteral Nutrition. 2021;1–10. https://doi.org/10.1002/jpen.2112

Unbelievable survivability rates for short bowel patients

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When other doctors ask Clarivet Torres, M.D., how she is getting the best survivability rates for patients with Short Bowel Syndrome (SBS), she says her success is because of teamwork.

The Intestinal Rehabilitation Program (IRP) at Children’s National, started in 2007 when Dr. Torres joined the health system and became the program’s director, has shown 98 percent survivability for patients with SBS over a period of nine years. That’s compared with a recent study from the Pediatric Intestinal failure consortium (Predictors of Enteral Autonomy in Children’s with Intestinal Failure: a Multicenter Cohort Study), which showed that 43 percent of the patients died or underwent transplantation over a median follow-up of 33.5 months.

Intestinal failure often prevents these patients from digesting enough nutrients and fluids to maintain proper growth, and they often require parenteral nutrition (PN). Dr. Torres’ team has helped to wean 91.3 percent of patients from PN, compared with the above study, which showed that enteral autonomy was achieved in 43 percent.

Based on the outcomes for the first 120 children with SBS treated in Children’s National’s IRP from 2007 to 2016, Dr. Torres says that with meticulous and aggressive medical/surgical management, even patients with advanced liver disease can show improvement in liver functions and nutritional parameters with the ability to discontinue parenteral nutrition and avoid the need for transplantation.

“These are very, very good results for any program and ours has been growing substantially in the last 10 years,” Dr. Torres says. “We are like a family, we are very good at teaching so everyone knows how to care for these patients.”

Cross-departmental collaboration

Her main focus as director has been spreading the word about SBS across the departments. For example, the ER knows to start IV fluids on these patients right away or to keep watch for sepsis symptoms. From nurses, pediatric residents, and surgeons to radiologists and the ER, Dr. Torres has encouraged the sharing of knowledge and teaching how to respond to SBS patients.

Dr. Torres also attributes the success of the Children’s National’s program to having a multidisciplinary intestinal rehabilitation team who are trained to follow up with these highly complex patients with SBS.  “In general, these patients have a very high morbidity-mortality rate, and it’s important to be close to follow up.”

Members of  the IRP includes, a dedicated surgeon, Anthony Sandler, M.D., and four supporting GI doctors (Parvathi Mohan, M.D., Vahe Badalyan, M.D., Sona Sehgal, M.D., and Muhammad Khan, M.D.).

Other important members are one physician assistant, two nurse practitioners, two coordinators, one dietitian, one social worker, one case manager, and devoted nurses who work in the specialized Intestinal Rehabilitation Unit.

Having a dedicated director and surgeon also is a new perspective. Focusing on this group of patients allows Drs. Torres and Sandler to become experts in the medical and surgical management of the patients with short bowel and intestinal failure.

A closer look inside the program

The goal of the IRP is to optimize bowel function through the use of multiple therapies and to eventually wean patients with intestinal failure from parenteral nutrition. The medical treatment focuses on comprehensive dietary management with very precise control of metabolic balance and prompt and effective treatment of complications.

Pro-adaptive surgery, such as stoma closure, ostomy in continuity, stricturoplasty, enteroplasty, and autologous gut reconstruction, with the longitudinal intestinal lengthening and tailoring (LILT) and serial transverse enteroplasty (STEP) procedures, may produce dramatic clinical improvement in patients with SBS.

The use of specialized enteral feeding programs by the experience medical team helps to maintain nutrition and hydration, which are important factors in long-term survival. Other important components of the program are ongoing parent education and support, and promoting an optimal quality of life. Intestinal transplantation with MedStar Georgetown University Hospital is an option for patients who fail treatment.

“The Intestinal Rehabilitation 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,” Dr. Torres says.