How is Short Gut Syndrome treated?

Treatment of Short Gut Syndrome focuses on maximizing proper nutrition and hydration, minimizing complications and symptoms, and encouraging the intestine to adapt, so that it absorbs as much nutrition as possible. To meet these goals, doctors use a mix of nutrition support, diet management, medications, and surgical intervention.

The exact approach to treatment is as unique as each patient and depends on 1) which sections of the intestine were removed or lost, 2) what sections remain, and 3) how the remaining portion functions and adapts.

Mild cases are treated by managing diet, maintaining fluids and electrolytes, taking nutritional supplements, and using medications to control diarrhea and bacterial overgrowth. Moderate cases may also require tube feeding and/or IV fluid and electrolytes.

For severe short bowel syndrome, treatment often involves parenteral nutrition, a form of IV nutrition also known as TPN. In some cases, intestinal adaptation is possible and the patient can slowly be weaned off of TPN. Medication or surgery may be used as a part of intestinal rehabilitation. In the most severe cases, sometimes known as "ultra short gut," complete adaptation may not be possible and TPN must be used indefinitely. Intestinal transplant may be an option for some patients, particularly when other treatment options have failed or when long-term TPN causes life-threatening complications.

Even in severe cases of short bowel syndrome, patients may receive tube feeding, or enteral nutrition, and/or continue to eat by mouth, even though nutrition is not absorbed. Feeding, whether enterally or by mouth, may stimulate the remaining intestine, the liver, and other organs to function better. Feeding can also decrease oral aversion and increase social inclusion.

On this page, you will find information about:

• Intestinal rehabilitation
• Nutrition support
• Medication and homeopathic therapies
• Surgical treatments
• Intestinal and multivisceral transplant
• Transplant evaluation, a path to IRP

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Intestinal Rehabilitation

Intestinal rehabilitation is a multi-disciplinary approach to treating intestinal failure that combines nutrition support, diet, medication, and surgical treatment to help intestinal failure patients' intestines to adapt and absorb more. The goals of intestinal rehabilitation are maximizing intestinal absorption, managing symptoms, reducing dependence on TPN, and avoiding intestinal transplant by avoiding the complications that make it necessary, (namely, liver damage, lost central venous access, and recurrent sepsis.)

An intestinal rehabilitation program or IRP, as the name suggests, is a program that specializes in intestinal rehabilitation. An IRP team usually includes at least a gastroenterologist, a dietitian, and a surgeon. As these programs are rare in the United States, patients often travel to receive treatment from these experts.

Read more about the team members that make up an IRP here.

Find a list of Intestinal Rehabilitation and Transplantation Centers on Transplant Unwrapped

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Nutrition support

• Total parenteral nutrition (TPN) is nutrition that bypasses the digestive system and is given intravenously (through an IV) instead. Each specialized sterile formula is a custom recipe designed for an individual patient. TPN includes fluids, electrolytes, sugars (dextrose), vitamins, minerals, and fats (in the form of IV lipids).
  
  
  • Lipids are the fats included in TPN. There are three kinds of lipids currently available in the U.S. Intralipid is made of soy and high in omega-6 fatty acids. Because omega-6s can irritate the liver, intralipid can cause liver inflammation and even damage over time. Omegaven is made of fish oil and is high in omega-3 fatty acids. Omegaven has anti-inflammatory properties and has been shown to reduce liver inflammation. SMOF is a combination of lipids of both types that is believed to be more similar to the balance of fats consumed in a normal diet. Research to compare the effect of SMOF on the liver compared vs. other types of lipids is ongoing.

• Enteral Nutrition is liquid nutrition and generally refers to tube feeding through a tube placed in the digestive system.
  • G-tubes or gastrostomy tubes are placed directly into the stomach via a hole in the abdominal wall. This can be a long tube known as a PEG tube or a short tube that longer extensions can be attached to called a "button."
  • NG or OG tubesare long tubes placed through either the nose (nasogastric or NG) or mouth (orogastric or OG) down the throat and into the stomach. These are often taped to the cheek to help hold them in place. They do not require surgical placement. A nurse or trained caregiver can place one bedside in the hospital or at home.
  • J tubes and GJ tubes are tubes is inserted into the jejunum, the top part of the intestine, in order to bypass the stomach. Jejunostomy tubes (J tubes) are usually used when the stomach is not functioning properly. A GJ tube includes two tubes, one inserted into the stomach (g for gastric) and the other into the intestine (j for jejunum). Jejunostomy tubes are placed by an interventional radiologist in the hospital to be sure they are properly placed.
  • Medical formulas are used for tube feeding. There are many commercially available liquid formulas on the market. Some are sold as liquids while others are sold as powders that need to be mixed with water. There are formulas made for babies, children, and adults. In some cases, normal over-the-counter formulas, such as baby formula or nutrition shakes may be used. As a general rule, though, short gut patients do not usually tolerate OTC formulas high in sugar and dairy, and they need a more specialized formula that can better offset poor digestion. One type of formula is called a peptide formula. In peptide formulas, proteins are already partially broken down (hydrolyzed) so they are easier to digest. However, even peptide formulas may not be well-absorbed in SBS. In this case, patients turn to elemental or amino acid formulas, such as Elecare and Neocate. These formulas are completely dairy free and the proteins are completely broken down so only their building blocks, amino acids, remain, making them easiest to digest and absorb.
  • Blenderized diets, or whole-food diets, are made of fluids and foods blenderized into a liquid form. In general, these blends are lower in dairy and sugar and therefore may slow digestion more than traditional medical formulas. There are a few commercial "whole food" formulas on the market. However, many families blend their own blenderized diets, sometimes working with a dietitian to develop balanced recipes based on the patient's needs and food tolerance.
• Diet guidelines. A key to managing short bowel syndrome is a carefully managed diet focused on slowing digestion and maximizing absorption of key nutrients. Most intestinal rehabilitation programs include a dietitian who can advise patients in choosing a diet that is best for their anatomy and tolerance. In general, an SBS diet focuses on simple carbohydrates, lean meats, and healthy fats and avoids sugars, including dairy and fruit. Certain vegetables are allowed as tolerated, but high-fiber vegetables, whole grains, and red meats are limited.
• Oral hydration. Fluid and electrolyte losses are common, so patients need to take in additional fluids to make up the difference. There are some commercial oral rehydration solutions (ORS) that are ideal for this. There are also recipes for ORS that patients can make at home. Drinks with electrolytes are preferred over water because they are more easily absorbed, while water can actually increase dumping in some patients and cause dehydration.
• Nutrition Supplements.
  
  
  • Vitamin and mineral supplements. Because certain vitamins and minerals are only absorbed in certain sections of the intestine, supplements are sometimes needed for balanced nutrition, at times in high doses. When possible, these can be taken by mouth or given by feeding tube. When anatomy prevents absorption, though, sublinguals or injections can be used. Doctors will monitor bloodwork to look for and treat deficiencies.
  • Probiotics. Probiotics are live bacteria or yeast that are good for you, like the ones found in yogurt. Probiotic supplements help manage bacterial overgrowth by helping to restore the balance of good and bad bacteria in the gut. There is debate about the safety of using probiotics when someone has a central line. In some cases, probiotic bacteria have been found growing in central lines. Believing this risk is low, many doctors recommend them with success. Others avoid them.
  • Fish oil. Because intralipid, a fat emulsion commonly used in TPN, does not contain omega-3 fatty acids, there have been studies done into providing fish oil enterally to patients. This has a two-fold benefit. It helps supply needed fats and also helps to reduce liver inflammation. Effectiveness, however, depends on the patient's ability to absorb it.
• Feeding therapy may help patients overcome oral aversions. A speech and/or occupational therapist, sometimes along with a behavior specialist, use oral exercises and gradual exposure to foods to improve eating skills. Feeding therapy is usually outpatient, but there are some intensive inpatient feeding therapy programs.

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Medications & homeopathic therapies

• Promoting bowel adaptation. GLP-2 analogues. Gattex is the first and best-known GLP-2 analogue, though there are others currently being studied. These are specific orphan (rare disease) drugs made specifically for TPN-dependent patients with short bowel syndrome. The intestine is lined with villi, which are like small fingers that catch and absorb food as it passes through the intestine, somewhat like velcro. GLP-2s lengthen the villi. Studies have shown that a daily injection increases intestinal absorption, decreasing the need for TPN.
• Managing bacterial overgrowth. Prophylactic antibiotics, antifungals, & oregano oil. Bacterial overgrowth is a complex problem as there are millions of bacteria in the intestine, both good and bad. To manage bad bacteria, doctors may prescribe antibiotics that target common bacteria. They usually use antibiotics that pass through the digestive tract without being absorbed. However, with so many different bacteria, patients may need to start and stop treatment or rotate antibiotics and/or antifungals. Some patients and families also use a homeopathic option. Oregano oil has antimicrobial properties that can manage or prevent overgrowth. Importantly, oregano oil is a "hot oil" that must be diluted in a carrier oil to prevent harm to the digestive system.
• Reducing diarrhea. Antidiarrheals, bile acid sequestrants & pectin. Medications to treat diarrhea work in many different ways. Lopermadine is an over-the-counter anti-motility medication that slows down food as it moves through the gut. Cholestyramine binds bile acid, essentially making digestive secretions and stool less acidic. Turning to food, pectin can be added to enteral feeds. Pectin has been shown to make the intestine healthier and make food move more slowly through it, decreasing diarrhea.
• Decreasing acid hypersecretion. H2 blockers and proton pump inhibitors. Abnormal digestion and motility can lead to acid hypersecretion. This can be treated using common heartburn medications that reduce stomach acid.
• Motility stimulants and laxatives. Although diarrhea is common with short gut, some patients experience the opposite, dysmotility, or poor gut movement. Motility stimulants can prevent stagnation of bile contents that can lead to bacterial overgrowth, translocation, dilation, and other problems. Because motility issues can sometimes cause constipation and blockage, in some cases, laxatives may also be prescribed.
• Reducing central line infections and clotting. When a central line is not in use, it is filled with a sterile liquid solution and "locked" to preserve it until future use. Commonly, saline and heparin are used to lock IVs. However, to reduce central line-associated bloodstream infections, an antimicrobial lock may be used instead. Antimicrobial lock therapies include antibiotics, ethanol, and sodium bicarbonate.

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Surgical treatments

Surgical treatment of Short Bowel Syndrome works to improve gut function. In SBS, sections of the intestine may not work as they should. This happens because of scar tissue or adhesions that can cause tie up or bind the gut, restricting its movement. It can also happen because the intestine is damaged in some way, essentially malfunctioning so it doesn't move as it should.

Peristalsis is a word for the wavelike, squeezing movement of the intestine wall. This squeezing motion moves food through the gut much in the same way that toothpaste is moved by squeezing a tube of toothpaste.

When there is an obstruction, whether physical or functional, the food and fluid in the gut will back up, causing it to become stretched out, or dilated. Once dilated, it is too wide for peristalsis to effectively move gut contents. The squeeze is too weak for the larger tube.

Surgery can help remove physical obstructions like scar tissue. But many of the surgical treatments commonly used for SBS decrease, therefore improving motility, absorption, and nutrition.

• Intestinal tapering (Tapering enteroplasty)is usually indicated when there is significant dilation of a portion of the bowel. Tapering reduces the width (caliber) of the dilated bowel excising, or cutting off one side of the intestine, closing the side with a row of staples. Once narrowed, the bowel's movements work to move food through the bowel again.
  Another version of tapering is known as plication. This procedure is similar, except that instead of cutting off a portion of bowel, it is folded in and stapled or sutured. Although this procedure also helps the bowel to work again, the suture lines eventually break down, allowing dilation to return.
• Intestinal lengtheningprocedures are another treatment option for dilated bowel. However, instead of removing the excess tissue, the intestine's shape is changed surgically, leaving all of the intestinal tissue, but narrowing the intestine so that peristalsis works as it should.
  
  
  • STEP procedure (Serial Transverse Enteroplasty): In this procedure, the dilated bowel is lengthened by making a series of cuts with a surgical stapler from alternating (transverse) sides of the intestine in a kind of zig-zag pattern so that the dilated bowel can be unfolded and stretched out like a slinky. The STEP procedure can nearly double the bowel length while restoring motility.
    There is debate within the SBS community regarding the safety and efficacy of the STEP procedure. For years, it has been treated as a gold-standard surgical intervention for short bowel syndrome. Supporters point to the many patients who wean fully off of TPN following the procedure. Opponents argue that the transverse cuts interfere with normal gut movement and disrupt the blood supply. They argue that dilation returns and that repeating the procedure has less success. Worryingly, some patients develop anastomotic ulcers at the staple lines that bleed chronically and sometimes severely. Evidence shows poor outcomes, such as continued TPN dependence and other complications like bleeding and returned dilation, happen most often in patients with a history of gastroschisis. There are few studies into this issue and those that exist were small in scale. Most evidence is anecdotal -- hence the controversy. One point on which all sides agree, however, is that the procedure is most successful when done by a surgeon who has abundant experience with it, such as a surgeon in an intestinal rehabilitation program.
  • Bianchi procedure (longitudinal intestinal lengthening and tailoring, or LILT): The first bowel-lengthening surgery, this procedure divides a dilated section of the bowel into two parallel tubes by cutting the intestine down the middle and then sewing or stapling down the center. Cuts are then made to separate the tubes and the ends are sewn together. The result is that the intestine is narrowed, improving motility, and the intestine is nearly doubled in length. The advantage of the Bianchi, or LILT, procedure is that it works with the normal anatomy of the intestine, following the normal blood supply and muscle movement. It also involves fewer cuts and reconnections. It has a high rate of success in weaning patients from TPN and overall survival. However, it is very technically challenging and is not often performed. As a result, there is not much literature on it, and even less comparing it to the STEP procedure.
  • SILT procedure (spiral intestinal lengthening and tailoring): This is a relatively new technique in which the bowel is spirally cut, stretched, and then closed again, making a tube that is longer and more narrow. Its advantages are that, unlike the STEP procedure, SILT causes less damage to the blood supply and nerves, and it works with the regular muscle movement of the intestine. It can also be performed when there is less bowel dilation. However, it does open the bowel, risking contamination and infection. As a new and infrequently performed procedure, there is little information regarding its success and it has not been widely adopted.
• Ostomies: an ostomy is a surgical opening in the abdomen through which bodily fluids drain. For intestines, an ostomy brings the side or end of the intestine to the surface of the skin so that stool can empty into a collection bag. This ostomy is usually named after its location in the intestine, (ileostomy, jejunostomy, colostomy, duodenostomy, etc.) Sometimes, during the surgery that led to SBS, surgeons will create an ostomy to drain stool. Ostomies can also be used as a way to address obstruction or missing anatomy, and to treat dilation.
  When an ostomy is no longer needed, it may be taken down and the small and large intestines are reconnected. This surgery is known as reanastomosis.
  
  
  • Ostomy-in-continuity: some centers have begun to use another type of ostomy to treat dilation. The common name "ostomy-in-continuity (OIC)", refers to either a Bishop-Koop ostomy or Santulli ostomy. Both procedures connect two ends of the intestine in a sort of y shape with the end of the y coming out of the abdomen as an ostomy. This ostomy creates an escape valve, of sorts, through which pressure can be released, allowing the intestine to decompress. Thus, the body gets the benefits of a fully connected colon - better absorption and feeding tolerance, along with a healthier liver - but relief from pressure, dilation, and motility problems. This is a fairly new but promising technique.

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Intestinal and multivisceral transplant

Occasionally, intestinal adaptation may not be possible, or at least may not be fast enough to outpace other life-threatening complications. In these cases, transplantation may be the best treatment option.

With intestinal transplant, the damaged or shortened small bowel is removed and replaced with a healthy bowel from a donor. A patient may receive an intestine alone or may receive multiple organs from the same donor, which is known as a multivisceral transplant. Because of the size of the intestine and its complicated associated vascular, nervous, and lymphatic systems, this is a complex and difficult procedure. Additionally, during the procedure, the stomach's shape is often changed surgically to help it to empty into the donor intestine. Sometimes the spleen and gallbladder will also be removed to make space for the donor organ, which will usually swell and take up space. Removing these organs can also head off other common complications. Doctors will create an ileostomy as part of the procedure, which makes it easier and more comfortable to perform biopsies. The ostomy will be reversed later, once there is less chance of rejection.

Isolated intestinal transplant vs. multivisceral transplant

• Patients may be listed for either an intestine-only transplant or a multivisceral transplant, meaning a transplant involving multiple organs. The most common multivisceral intestinal transplant is a liver-intestine transplant. Because intestinal failure and TPN dependence often lead to intestinal failure-associated liver disease, some patients will require a liver along with the intestine. Though not part of the common name, liver-intestine transplants include transplantation of the pancreas because of shared anatomy with that organ. Sometimes other organs may also be included in the transplant if they are also diseased or damaged. For example, some multivisceral transplants will also include a stomach or a colon.
  There are pros and cons of both isolated vs. multivisceral transplants. The main advantage of an isolated transplant is that, if there is a problem with the donated organ such as rejection, the donated organ can be easily removed and the patient can simply be put back on TPN while awaiting another donor. Also, wait times are shorter for isolated intestinal transplants, as there is lower demand for intestines than livers. Isolated intestine transplants, however, have higher rates of rejection than multiorgan transplants. Liver-intestine transplants have a lower rate of rejection. Unfortunately, if rejection occurs, the liver cannot be removed without another donor. Liver-intestine transplants have longer wait times, sometimes years, depending on the transplant center and blood type.

Indications for intestinal transplant

Short bowel syndrome, even fully TPN-dependent SBS, is not an indication alone for transplant. Patients can live long, mostly typical lives while receiving parenteral nutrition. Below is a list of common indications for transplant.

• Loss of central venous access: because there are a limited number of veins through which a central line can be placed to give TPN, the current leading cause for intestinal transplant is the loss of central venous access. If a central line can't be placed, it is no longer possible to provide IV nutrition. Further, without central access, a transplant procedure cannot be performed. Therefore, if a patient has lost most of their central veins to scarring, clotting, infection, etc., they may be listed for transplant.
• Liver disease: intestinal failure and long-term TPN use can cause liver disease. In cases where liver damage is severe, a transplant becomes necessary. At present, only liver health is scored to determine status on the wait list by severity. Better management of lipids in TPN and a growing understanding of intestinal failure-associated liver disease have improved liver health in transplant patients.
• Frequent sepsis: bloodstream infections are a risk for all TPN patients. However, some patients are much more prone to infection than others. Advances in lock therapies have helped to reduce this risk in many patients. Nonetheless, frequent, severe sepsis can be a sign that transplant is necessary for long-term survival.

Risks of transplant

Besides the risks of the surgery itself, there are many ongoing risks associated with intestinal transplants. Because of these risks, transplantation is not considered a cure for intestinal failure. Instead, transplant patients trade one set of problems and risks for another. Below is a list of some of the most severe complications of intestinal transplants.

• Immune suppression: to protect the donated organ, patients will take immune-suppressing drugs for the rest of their lives. This puts them at increased risk of illness and infection. This problem is compounded because immunosuppressants can make vaccines less effective for some transplant patients. In a post-pandemic world, this means that patients need to exercise exceptional caution regarding illness.
• Rejection: in some cases, the immune system may attack the transplanted organ. This is treated by increasing immune suppression but may lead to death and loss of the donated organ (graft).
• Graft vs. host disease (GVHD): in other cases, the immune system of the transplanted graft may attack the body of the transplant recipient, or host. This is more common in intestinal transplants than in other organ transplants because of the large lymphoid content of the graft. This is a serious condition and may be fatal.
• Post-transplant lymphoproliferative disorder (PTLD): Transplant immune suppression creates a risk of a form of lymphoma. Though not strictly cancerous, PTLD can evolve into cancer if not treated early. PTLD is usually triggered by the Epstein-Barr Virus (EBV), commonly known as mono.

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A note about transplant evaluation

It can be worrying and even scary when a doctor suggests a referral for transplant evaluation. Evaluation does not mean transplant. When a doctor suggests transplant evaluation, they usually recognize they don't have expertise in SBS and want to transfer the patient to a higher level of care.Any quality intestinal transplant program also includes an intestinal rehabilitation program. In fact, good intestinal transplant surgeons in general are the most committed of all doctors to avoiding the need for transplant. Transplant evaluation does not require, or even guarantee, that a patient will be listed for a transplant. However, an intestinal transplant evaluation may be helpful in a few other ways.

• It may be easier to get insurance preauthorization for transplant evaluation than to get authorization for an intestinal rehabilitation program. Transplant evaluation usually represents a transfer to a higher level of care. Most insurance plans have policies written for transplants while few have policies regarding intestinal rehabilitation programs. After evaluation, the center can work with your insurance to get approval to participate in their intestinal rehabilitation program. Though still a long process, this path to authorization is often easier than transfer for purposes of an intestinal rehabilitation program alone.
• Evaluation provides valuable information about the patient's current status including liver health, remaining central access, intestinal length, intestinal function, and nutritional status. Better yet, the testing is performed and interpreted by experts in the field of intestinal rehabilitation.
• Evaluation starts the process of transplant listing, should that option be needed later. The process of insurance approval, medical evaluation, and listing for transplant can take weeks if not months. Having these steps already completed may save critical time if a transplant becomes an urgent need in the future. Some patients even choose to be put on the transplant wait list with a low status so they accrue wait time while working with an IRP to make sure they never actually need the transplant.

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