Short bowel syndrome is commonplace to Danielle Stamm, RN, FNP-BCR. As one of the dedicated nurse practitioners in the Center for Advanced Intestinal Rehabilitation (CAIR) at Boston Children’s Hospital, she sees children with this rare but serious condition on a daily basis. But what’s familiar to Stamm and her colleagues is unusual to many other clinicians.
Children with short bowel syndrome lack sufficient length or function of their intestine to extract the necessary nutrients to grow and thrive. This condition can result from birth anomalies such as intestinal atresia, gastroschisis and long-segment Hirschsprung’s disease, or from acquired conditions such as volvulus and necrotizing enterocolitis. All children with short bowel syndrome require advanced nutritional support at some point in their lives. This support may be delivered by enteral feeding tube (g-tube), by intravenous nutrition given through a central venous catheter (parenteral nutrition) or both.
Many kids with short bowel syndrome spend a long time in the hospital and their parents tend to rely on their care team of specialists for guidance. But it’s just as important for these patients to receive regular primary care, too, explains Stamm. “We want to partner with PCPs so that our patients’ vital primary care needs are met, and so that they feel empowered to assist in their management.” Here, she shares four things every PCP should know about their patients with short bowel syndrome.
1. Specialty patients still need primary care.
Being under the care of a specialist doesn’t preclude the need for primary care. In fact, PCPs have a crucial role in the treatment of these patients, says Stamm. “We appreciate that primary-care relationship and encourage patients to see PCPs for well-child visits, vaccinations and many medical problems that are outside of our scope of practice in CAIR.” What’s more, because many families must travel great distances to receive specialty care in our program, an established connection to a trusted PCP can foster seamless care coordination — and help prevent unnecessary trips to the emergency department.
2. Short bowel kids have different GI concerns.
What may be alarming in otherwise healthy children may require different consideration in children with short bowel syndrome. For example, many of these patients experience chronic baseline diarrhea, which may be acceptable as long as it doesn’t cause dehydration, severe skin breakdown, or is not significantly deviant from the patient’s baseline. The key is to be aware of what’s “normal” for a given short bowel patient and to be able to identify changes that could signal problems.
On the other hand, the nature of the workup for occasional blood in the stool for the general pediatric population may be quite different for a short bowel patient presenting with this symptom. For children with short bowel syndrome, workup for blood in the stool is often much more comprehensive due to unique risk factors including late pneumatosis, C. difficile infection, anastomotic ulcers or strictures, chronic gastrointestinal blood loss related to diffuse intestinal inflammation, vitamin K deficiency and others.
3. Some symptoms should be taken seriously.
For most pediatric patients, fevers are typical complaints best treated with anti-pyretic medications such as acetaminophen. But for children with short bowel syndrome who have a central venous catheter in place, a fever could signal a dangerous bacterial blood infection that warrants antibiotics and admission to the hospital. “Sepsis is a significant source of preventable morbidity and mortality in children with short bowel syndrome,” says Stamm. “We are always err on the side of caution when a patient presents with a fever and a central venous catheter.”
Likewise, patients with short bowel syndrome are more sensitive to dehydration and electrolyte imbalance than their peers. “We have a low threshold to check labs in our patients as they may have labile electrolytes or develop dehydration quickly,” she says. “These developments often require titration of their advanced nutritional support or provision of extra enteral or parenteral fluids.”
4. Good bone health starts now.
Children with short bowel syndrome can experience several co-morbidities and complications, one of which is metabolic bone disease. Indeed, research conducted at Boston Children’s suggests that children on long-term parenteral nutrition (such as those with short bowel syndrome) are at increased risk for developing low bone mineral density later in life. PCPs can help mitigate this damage by encouraging patients to engage in regular weight-bearing exercise and getting optimal amounts of calcium and vitamin D.
Learn about the Center for Advanced Intestinal Rehabilitation.