Oh, the small intestine. It just doesn’t get any press.
Unlike the more glamorous colon (“Don’t forget your colonoscopy!”) or the stomach (“Doc, the pain just won’t go away”), the small intestine just quietly goes about its job, somewhat like the second assistant director on a film: unsung, but you can’t get the job done without her.
Until something goes wrong.
In short bowel syndrome (SBS), something has gone radically wrong: The small intestine can no longer do its job. According to the Short Bowel Syndrome Foundation, the condition occurs when “parts of the intestine are removed surgically,” making it more difficult for the remaining intestine to process food. In the journal Nutrition in Clinical Practice, Loris Pironi stated that the syndrome defines “the clinical feature associated with a remaining small bowel in continuity of less than 200 centimeters from the ligament of Treitz.”
This disorder can manifest a host of uncomfortable and debilitating symptoms, including bloating and flatulence, vomiting, food allergies/sensitivities, cramps and heartburn, sleep deprivation and fatigue, irregular bowel habits, and difficulty maintaining weight, among others.
A severe potential effect of SBS is chronic intestinal failure (CIF), leading to symptoms such as diarrhea, electrolyte disturbances, malnutrition and dehydration. Pironi wrote that CIF is “defined as the ‘reduction of gut function below the minimum necessary for the absorption of macronutrients and/or water and electrolytes, such that intravenous supplementation (IVS) is required to maintain health and/or growth’ in a metabolically stable patient.” This is known as parenteral nutrition, in which the patient receives part or all their nutrition through an IV, bypassing the digestive system.
When the lack of absorption isn’t as severe, the patient may be classified with intestinal insufficiency (II), or intestinal deficiency (ID).
While SBS is relatively rare, affecting an estimated 10,000 to 20,000 people in the United States, the past 40 years have seen the number of cases double. Endo, et al., wrote in the journal Regenerative Therapy, “Treatment for SBS is mainly supportive, consisting of supplementation, prevention and treatment of complications, and promotion of intestinal adaptation. While development of parenteral nutrition and drugs promoting intestinal adaptation has improved clinical outcomes, the prognosis of patients with SBS remains poor. Intestinal transplantation is the only curative therapy, but its outcome is unsatisfactory.”
The Short Bowel Syndrome Foundation has resources to help patients cope with the nutritional demands of their condition.
Deep in the Weeds
In a CME Outfitters webinar, Miguel Regueiro, M.D., AGAF, FACG, FACP, chair of the Digestive Disease and Surgery Institute at the Cleveland Clinic, discussed with Doctors Kishore R. Iyer and Donald F. Kirby the challenges of SBS and how to create a “medical neighborhood” to help patients cope with—and thrive in spite of—the disorder.
There are three types of SBS according to anatomical criteria. Type 1 is end-jejunostomy, which is the most severe, according to Donald F. Kirby, M.D., FACP, FACN, FACG, AGAF, FASPEN, CNSC, CPNS, the medical director of the intestinal transplant program and professor of medicine at the Cleveland Clinic. “It has the worst prognosis because it has the shortest amount of surface area,” he said. Indicators include rapid transit, acid hypersecretion, poor adaptation, large fluid losses, and malabsorption.
The most common is Type 2 SBS, jejuno- colonic, which has rapid transit, poor adaptation, B12 and bile salt malabsorption, and variable calorie and fluid absorption. “To really stay off parenteral nutrition in this type, you need more than 65 centimeters of jejunum to at least half or more of colon,” Kirby said.
Jejunoileo-colonic, Type 3, has adequate absorption until about 75% resected. It also has good adaptation and slower transit. But although it offers the best prognosis, it’s uncommon—only about 9% of patients are Type 3. They do well, Kirby said, because “they’ve got that ileocecal valve … These patients are the easier ones to rehabilitate and get off parenteral nutrition.” However, adaptation can take two to three years, so patience is critical.
The disease is tough enough to deal with on the day to day, but according to Kishore R. Iyer, MBBS, FRCS (Eng), FACS, professor of surgery and pediatrics at Mount Sinai Medical Center in New York City, it gets really difficult when patients encounter social situations. “Think about one thing any one of us does socially that does not involve food and drink. So for patients living with this disease, it is really quite devastating at many levels,” he said. Worries about the stoma bag leaking, embarrassing digestive noises or urgency to go to the toilet can substantially impair quality of life for patients.
And while total parenteral nutrition (TPN) is lifesaving, it can also impose burdens, especially if patients are infusing their nutrition at night: If the machine’s alarm is constantly going off, and patients have to wake up constantly, this will interfere with their sleep cycle. If you’ve ever had a colicky child or a medical condition that constantly rouses you, you know what it can do to your daily productivity.
Kirby concurred. “Being on home parenteral nutrition is harder than being on dialysis,” he said. “You go to a dialysis center, you give them an arm, they hook you up, you sit there, watch TV for three, four hours, and then you go home, eat, drink, be merry.
“For TPN, you’ve got to take the bag out of the refrigerator, you need to let it get to room temperature. You then may have to add a couple of additives, and then you put it up on the pole, and then you prime the pump, and then you set it up and let it go, and then the pump beeps in the middle of the night, and then you’re infusing things at 200 to 300 milliliters per hour, so what does that mean? Well, everybody’s going to get up and start peeing in the middle of the night, so nobody gets a good night’s rest. If you can give them a night off, you have suddenly made a best friend. It’s really important to realize the burden on these patients.”
Partial parenteral nutrition can be paired with regular eating, but patients on TPN should avoid eating. Some report hunger cravings, while others do not. So much depends on the patient, the amount of digestive tract, and the team.
Creating an Interdisciplinary Team
Patients benefit when all their healthcare professionals are on the same page.
The key to facilitating communication, whether in clinical practice or research, according to Pironi, is careful and “homogenous” categorization of the disease. This categorization may include anatomy (e.g., length of bowel), evolutionary factors (maintenance phases), pathophysiological factors (whether there is a “colon in continuity”), clinical factors (level of malabsorption), and severity of chronic intestinal failure (CIF).
Kirby said the first place to start in achieving an early and accurate diagnosis is by taking a comprehensive and accurate medical history. This may require some digging. When surgeons measure the amount of remaining small bowel and make a note of it, that’s very helpful for members of the medical team as they follow up and create a treatment plan. Blood work and fecal fat testing reveal a lot of information, but endoscopy and colonoscopy are important, too, Kirby said, especially if you’re dealing with patients who might have mucosal disease, as this can affect nutrient absorption.
Iyer said that a good intestinal rehabilitation team should have “medical expertise in the form of a gastroenterologist, [and] ideally, a hepatologist as well. There should be a surgeon with an interest in intestinal failure. We also have an intestinal transplant program. Our intestinal transplant program rests squarely within the intestinal rehab program. We view intestine transplant as just one extreme piece of the intestinal rehabilitation team. But that’s only the physicians.”
Kirby added that one of the team leaders could be the gastroenterologist or pediatric gastroenterologist, but this depends on the program you’re working with. “Many home parenteral nutrition programs in the U.S. … have an endocrinologist, or maybe it’s embedded in surgery.” He works closely with transplant, reconstructive and general surgeons, depending on whether the patient needs a feeding tube, assessment or reconstructive surgery. “We may have to work with intensivists when our patients are admitted with sepsis from another catheter infection … and we have excellent interventional radiologists that helped us get the line in, keep the line in, and maintenance of that line, which is absolutely imperative.” And of course, nurses who help with line care and ostomy care.
One factor teams often overlook is involving the health insurance company early in the process. Other team members critical to rehabilitation include those with expertise in nutrition, dietitians and nutritionists, Iyer said. Incorporate someone with expertise in medical management. You’ll likely also need social workers, pharmacists, infectious disease specialists and radiologists. The team may be large, but that’s necessary to provide the patient—and the patient’s family—with the best possible care.
So is education. Kirby includes medical educators on his team. He provides an educational series for parenteral patients, and he goes so far as to have them sign a patient agreement, acknowledging they’ve seen it.
Pharmacists do their part on the team, prepping the TPN and flagging potential drug interactions and drug/nutrient interactions, which can manifest quickly and may have lethal consequences. Tomczak, Stawny and Jelinska wrote, “In addition to drug incompatibility, other intravenous medication administration errors may affect a patient’s condition, including the risk of death and the increased cost of medical care resulting from a prolonged stay at the hospital.” The standardization of PN admixtures has helped reduce medical errors, but it’s still important to have a pharmacist on board.
And parenteral feeding can induce complications, including overfeeding, sepsis, GI atrophy and fluctuating blood sugar levels. On the long term, parenteral feeding can cause a form of liver disease, gallbladder problems, and demineralization of bones.
Nutrition assistance and counseling is critical to patients. Kirby called the registered dietitians at his facility “probably the most important member[s] of my team.” While it’s easy to set down a bunch of rules about what a patient can and cannot eat, the dietitian personalizes it for each patient, depending on multiple needs. “We try to get into their lives and figure out the best diet we can for them,” he said.
According to Kirby, the best place to start when it comes to diet is by determining how much colon a patient has left. “If they have a colon, you’re going to have to put them on a lower fat diet than if they go straight to an ileostomy or a jejunostomy.” Key to success is keeping patients on small meals: five or six smaller meals or light snacks in a day. “We don’t want to overload their surface area,” Kirby said. He advises that patients eat slowly, avoiding simple carbohydrates and simple sugars, as well as limiting lactose.
Avoiding sorbitol is important to limiting diarrhea, and even too much water can be problematic. Kirby recommended oral rehydration solutions—often used for patients where intravenous fluids are unavailable or impractical—and again, slow consumption.
To help deal with practical considerations like home envi- ronment, needs and finances, Kirby ensures social workers are a part of the team. He also includes a psychologist; pa- tients and their families can become depressed or anxious about the patient’s situation. “This affects every member of the family,” Kirby said. “Sometimes [even] the pets.”
Iyer said that a good intestinal rehabilitation team should have “medical expertise in the form of a gastroenterologist, [and] ideally, a hepatologist as well.
In pediatrics, Iyer said, team members should include occupational and behavioral therapists, as well as people with expertise in speech and swallowing mechanisms. The composition of the team will depend on the type of diagnosis and needs of the patient. Pediatric cases may include experts in behavioral health, dietitian, nutrition, medicine, surgery and radiology. Kirby said that probably more important for pediatrics is having a speech pathologist. The tiniest patients may never have been taught to eat; some don’t even have a suck reflex.
The importance of child and family support for pediatric patients cannot be overstated, so Kirby includes a child life specialist.
Pediatrics can be tricky. “For the patient … we want to achieve freedom from parenteral nutrition to the extent that’s possible,” Iyer said. “That should be goal one.” For some patients, this is impossible. So goal two is “freedom from complications.”
The third goal is improving quality of life. Iyer’s team works to understand, empathize and see how they can help. “The therapy itself is burdensome; the disease is burdensome,” Iyer said. “It’s not for me to comment on a patient’s quality of life. I need to listen and hear from the patient and his or her family—what is their quality of life, and is there something I can help with?” It might be as simple as recommending the patient carry their parenteral nutrition bag in a backpack during the day so they can get a better night’s sleep.
“This really takes a village,” Kirby said. “To be successful, you need to have a team of people [who] are interested, dedicated and want to work together.”
“We’re in academic medicine in a distressing disease field that is now going through almost tumultuous development for the better,” Iyer said. “So yes, research is a responsibility.
“You can improve time to freedom from PN, you can reduce complications, you can improve survival, you can improve quality of life. So an intestinal rehabilitation program should be involved in the care of these patients.”
For article references, visit www.EndoProMag.com.
