Preventing complications
“A knowledge of these complications is critical for those caring for these patients to be able to not only identify and treat them when they occur but also to prevent their occurrence whenever possible,” the authors wrote. Although they considered it beyond the scope of the review to outline every complication, they indicated some complications and management strategies via an included table. These complications can include cirrhosis, osteoporosis, acute kidney disease, and central venous catheter–related infection or occlusion.
Considering further surgery or intestinal transplantation
The authors noted that any further surgery should be carefully considered, with the following three contexts having possible value: “(1) to recruit unused distal bowel, (2) to augment the function of residual bowel through specific lengthening and tapering operations, or (3) to slow intestinal transit.”
Surgeons involved in managing SBS may need to confront complex intra-abdominal problems such as massive desmoid tumors, mesenteric ischemia, or complex enterocutaneous fistulae; a multidisciplinary intestinal rehabilitation team may be better able to help these patients. The authors noted that care for patients starts even before the first operation, by taking every measure to avoid massive bowel resection and the resulting SBS.
The authors noted the importance of early referral for intestinal transplantation consideration for patients with refractory dependency on parenteral nutrition or even onset of parenteral nutrition failure, which refers to complications such as intestinal failure–associated liver disease.
“At present, nearly 50% of patients being considered for ITX are also requiring simultaneous liver replacement, indicating late referral for ITX,” they wrote, citing a data from a report by the Centers for Medicare and Medicaid.
They also noted that data have shown short- and medium-term outcomes are steadily improving; however, long-term outcomes have been challenged by opportunistic infections, long-term graft attrition, and other impediments that may be preventing early referral for intestinal transplantation.
Educating patients, caregivers
Long-term PN may restrict activity for patients, but patients and caregivers should know about some modifications.
One is to cycle the PN over 10-14 hours overnight to allow freedom from the infusion pump during the day. Infusion pumps can be programmable, and some can be carried in a backpack for infusing during the day.
Authors recommend patient support groups, such as the Oley Foundation, which can help with issues surrounding body image and travel.
Because of the relative rarity of SBS, nonspecialist physicians may care for patients without a dedicated multidisciplinary team and may need education support in managing patients with complex care needs. One source the authors recommend is the Learn Intestinal Failure Tele-ECHO (Expanding Community Healthcare Outcomes) (LIFT-ECHO) project. The LIFT-ECHO project has become an online educational community with case-based learning in SBS, intestinal failure, and PN.
The authors disclose relationships with Takeda, Zealand, VectivBio, Napo, and Hanmi.