KidsMD Health Topics

Short Bowel Syndrome (SBS)

  • The small intestine is an important part of the digestive tract – it’s where we absorb most of the sugars, proteins and fats from the food we eat. If a child doesn’t have enough small intestine, her body can't extract the nutrients it needs to grow and thrive. This is known as short bowel syndrome (SBS), and it’s a serious condition because if left untreated, it can lead to dehydration and malnutrition.

    Luckily, there are several ways to manage and treat SBS – including a groundbreaking surgical procedure developed by doctors here at
    Boston Children’s Hospital .

    Short bowel syndrome has only one cause: not enough intestines for your child’s body to absorb nutrients from food.  There may be many reasons for this – your child may have been born without a sufficient length of intestine, or a large section of her intestine may have been removed for surgery to correct another intestinal problem.

    • It can be more or less severe, depending on the remaining length of intestine and on how well the other organs in your child’s digestive system are working.

    • SBS is managed with IV or tube feedings, medications and sometimes surgery

    • Long-term prognosis has been improving with the discovery of a nutritional supplement that can lessen the risk of liver damage and with innovative surgical procedures.

    How Boston Children’s approaches short bowel syndrome

    The Center for Advanced Intestinal Rehabilitation (CAIR) at Children’s is one of the world’s premier programs for the treatment of short bowel syndrome, a complex and often devastating disorder caused by the loss of part of the small bowel. Each year, our program cares for about 200 children from all regions of the United States, and abroad.

    Our program is staffed by a compassionate team of experts with experience in caring for children with short bowel syndrome and their families. Our specialists include:

    • physicians trained in surgery, gastroenterology and nutrition
    • registered dieticians
    • nurse practitioners
    • nurses
    • pharmacist
    • social worker

    In 2002, our doctors performed the world's first serial transverse enteroplasty (STEP) procedure, a surgical technique developed by Tom Jaksic, MD and Heung Bae Kim, MD, which lengthens the bowels of children with short bowel syndrome. Since then, we have performed more than two dozen of these procedures with increasing success, establishing Boston Children's as one of the preeminent hospitals in the world for the treatment of short bowel syndrome.

    Call 617-355-5275 for an appointment.


    Short bowel syndrome: Reviewed by Christopher P. Duggan, MD, MPH
    © Children’s Hospital Boston, 2011

  • Background on the small intestines

    The normal small intestine varies in length between 10 and 28 feet and is divided into three main sections.

    • duodenum: A relatively short section of the small intestine, measuring about 10 to12 inches. It links the stomach to the rest of the small intestine.  

    • jejunum: the largest section, because its many folds give it an increased surface area for absorbing nutrients

    • ileum: the longest section, accounting for around 3/5 of the small bowel

    Usually, about half of the small intestine can be removed without causing significant problems, especially if the ileum is not involved.

    The extent of your child’s problems with SBS usually depends on which sections and how much of her small intestine are affected:

    • Removal of the ileum can cause problems, since it’s responsible for the absorption of fats, bile salts and vitamin B12.

    • Removal of parts of the jejunum can often be well-tolerated, since the ileum can compensate for some of the work the jejunum would do.

    Complications of short bowel syndrome

    In addition to preventing the intestine from absorbing nutrients, SBS poses another problem, too:

    1. If your child doesn’t have enough small intestine, the remaining part tries to fix the problem on its own. It puffs up like a balloon, creating more surface area to draw in nutrients, just as we might set out more buckets if we wanted to collect more rainwater.

    2. But this has an unfortunate side effect: The wider the intestine, the longer it takes for the body to move nutrients through it. (Imagine holding a tube with water flowing through it. Now imagine squeezing the tube – the water flows faster.)

    3. More time in the intestines means more time for the bacteria that would normally be swept promptly along to multiply, increasing your child’s chance of infection of her intestine, known as small bowel bacterial overgrowth (SBBO).

     

    Causes

    SBS is caused by an insufficient length of small intestine. There may be many reasons why this has happened to your child, including:

    • sugery- During surgery to correct an intestinal defect (such as intestinal atresia or stenosis), remove an intestinal obstruction or fix a volvulus due to intestinal malrotation, doctors are always extremely careful to remove no more of your child’s small intestine than absolutely necessary. But sometimes fixing the problem requires the removal of a length of intestine that compromises her ability to absorb enough nutrients through them.
    • necrotizing enterocolitis (NEC)– an illness that damages the intestinal tissues in babies and can lead to holes or areas of narrowing (strictures) in the intestines.
    • other causes – these include a traumatic injury to the small bowel that requires that it be removed, like caused by Crohn’s disease or gastroschisis.

     

    Signs and symptoms

    The signs of short bowel syndrome are essentially all those associated with the inability to absorb nutrients from food (malabsorption), including:

    • weight loss/failure to gain weight
    • dehydration
    • diarrhea
    • abdominal bloating
    • fatigue

    These may be symptoms of other conditions as well, so be sure to always have your doctor make the diagnosis.

     

    FAQ

    Q: What is the treatment for short bowel syndrome?
    A:
    Short bowel syndrome can often be treated with specially formulated tube or IV feedings and with medications, and it can sometimes be treated surgically as well.

    Q: Will my child be OK?
    A:
    Your child’s prognosis depends strongly on how much working intestine remains, and how well the other organs in her digestive system are functioning. Many children adapt well to receiving nutrition through tubes or through an IV, and go on to go to school and college and leave relatively normal lives.

    Other children may need an intestinal transplant. You can rest assured that your child’s health care team will closely monitor her condition, and keep you aware of her status and any additional treatment options that may help.

    Q: What is parenteral nutrition (PN) feeding and is it dangerous?
    A:
    With PN, a special nutritional formula is delivered directly into your child’s bloodstream through an IV (intravenous feeding). Delivering nutrients straight to your child’s bloodstream means that it can bypass her digestive system altogether.

    PN is a life-saving therapy for SBS, and many of our patients rely on PN for weeks to months to years. Our home PN program has years of experience in treating children at home with PN and other IV therapies.

    Complications are usually related to the imbalance of electrolytes and possibly infection resulting from the use of a central vein access catheter into the bloodstream.

    Call your child’s nurse if you notice that your child:

    • experiences fevers or chills
    • has stomach pain
    • has difficulty breathing
    • has tingling in the hands or feet
    • has increased urination
    • begins vomiting
    • experiences muscle weakness, twitching, or cramps
    • has swelling of the hands, feet, or legs
    • experiences increased thirst
    • your child's catheter site is warm, tender, or there is redness or swelling
    • experiences pain around the injection site

    Q: Will PN feeding make my child fat?
    A:
    Even though PN often includes lipids, it will not make your child fat. Everyone needs calories, protein and fat, in addition to other substances, to stay healthy. Listen to Christopher Duggan, MD, MPH, medical director of the Center for Advanced Intestinal Rehabilitation talk about how we can support your child through nutrition.

  • To determine whether your child has short bowel syndrome, her doctor will take a detailed medical history, perform a physical examination and run a series of tests.

    Some of these tests will determine the level of nutrients that your child’s body needs and is absorbing, including:

    blood tests - to assess the level of nutrients absorbed in the blood stream

    stool sample - to check for non-absorbed sugar and protein, and for the presence of infection or blood

    breath hydrogen analysis - to quantify the degree of malabsorption of carbohydrate or sugars as well as to determine whether excess quantities of bacteria are growing inside the bowel

    indirect calorimetry - a test to analyze the nutritional needs of your child in the form of calories, carbohydrates, protein and fat


    Your child’s doctor may also order imaging tests to show any structural problems with the intestine that could be causing the symptoms:

    abdominal x-ray - a diagnostic test which uses invisible electromagnetic energy beams to produce images of internal tissues, bones and organs onto film

    abdominal ultrasound - a diagnostic imaging technique which uses high-frequency sound waves to create images of blood vessels, tissues and organs. Ultrasounds are used to view internal organs as they function, and to assess blood flow through various vessels.

    endoscopy-  a test that uses a small, flexible tube with a light and a camera lens at the end (endoscope) to examine the inside of part of the digestive tract. Tissue samples from inside the digestive tract may also be taken for examination and testing.

    colonoscopy - a test that uses a long, flexible tube with a light and camera lens at the end (colonoscope) to examine inside the large intestine. As with the endoscopy, tissue samples from inside the digestive tract may also be taken for examination and testing.

    After we complete any necessary tests, we’ll meet with you and your family to discuss the results and the best way to proceed.

  • We understand that it can be upsetting to learn that your child has short bowl syndrome, and we look at the diagnosis as the very first step in determining the best treatment plan. Our physicians are focused on family-centered care: From your first visit, you'll work with a team of professionals who are committed to supporting all of your family's physical and psychosocial needs.

    Many children with SBS can be kept healthy with medication and nutritional support, which may be delivered at home or at the hospital. If your child is fed through an IV, there is a risk of infection from the line used to connect her vein to the feeding tube. Your child's doctors will tell you what signs to watch for. 
     

    Nutritional management

    Since your child's intestines aren't able to absorb the nutrients her body needs, she may be able to get those nutrients through either parenteral nutrition (PN) or enteral nutrition (EN).

    Parenteral nutrition (PN)

    With PN, a special nutritional formula is delivered directly into your child's bloodstream through an IV (intravenous feeding). This formula is carefully developed by your child's registered dietician and calibrated to her body's precise energy needs. Delivering nutrients straight to your child's bloodstream means that it can bypass her digestive system altogether.

    Most children requiring PN are given the solution over the course of 12 to 24 hours on a daily basis. If your child requires PN for longer-term support, we'll gradually decrease the length of each session.

    Enteral nutrition (EN)

    If your child is receiving EN, a special nutritional formula through a tube, also sometimes known as “tube feeding,” delivers nutrients she needs. There are two ways we feed children through EN:

    • gastrostomy tube (G-tube), passed through an opening into your child's stomach
    • jejunostomy tube (J-tube), passed through an opening into a portion of her small intestine.

    EN can be used to prepare the support your child's intestine while recovering from a surgical procedure.

    Listen to Christopher Duggan, MD, MPH, medical director of the Center for Advanced Intestinal Rehabilitation talk about how we can support your child through nutrition.

     

    Medicine

    Your child's doctor might prescribe medications to help manage her SBS and supplement her nutritional intake. Some of these might include:

    • imodium: used to control diarrhea by giving your small intestines more time to absorb water and nutrients

    • ursodeoxycholic acid: a naturally-occurring bile acid that is used to dissolve and prevent cholesterol gallstones and to treat primary biliary cirrhosis, a disease of the liver

    • acid blocking agents - including H2 blocker and proton-pump inhibitors

    • vitamin supplements- either multivitamins or single nutrient supplements

    • oral antibiotics – to prevent or treat small bowel bacterial overgrowth

    Surgery

    Sometimes it's possible to surgically reshape the small intestine to increase the surface area of the intestines and/or prolong the amount of time it takes for food to move through, giving the intestines more time to extract nutrients.

    Boston Children's Hospital physicians Heung Bae Kim, MD, surgical director of the Pediatric Transplant Center, and Tom Jaksic, MD, PhD, surgical director of CAIR, developed an innovative surgical procedure called serial transverse enteroplasty (STEP). The STEP procedure reshapes the small intestine in order to:

    • expose more usable surface area,similar to the way that sharpening a pencil exposes more lead
    • create a narrower space, which keeps food moving through your child's digestive tract at an appropriate pace, carrying bacteria out of her body along with the rest of the body's waste

    Learn more about the STEP procedure.

    Transplant

    Many children do quite well with intravenous feedings, but sometimes complications arise that make it no longer an option. In this case, your child might be a candidate for an intestinal transplant. There are a few different types of intestinal transplants, including:

    • intestinal transplant
    • intestinal /liver transplant
    • multivisceral transplant (the intestines are replaced, along with the liver and/or spleen, and sometimes the stomach)

    Learn more about intestinal/multivisceral transplants.

    Follow up

    Our healthcare team will monitor your child carefully – whether she's here at the hospital or coming in for regular follow-up visits - to determine how well her body is responding treatment for SBS. And please remember that we're always here to answer any questions you might have.

  • Boston Children’s Hospital is home to the world’s most extensive research enterprise at a pediatric hospital. We also have many partnerships with research, biotech and health care organizations, and we work together to find innovative ways to improve kids’ health.


    Care-changing treatments

    In 2002, Boston Children’s physicians Heung Bae Kim, MD, surgical director of the Pediatric Transplant Center, and Tom Jaksic, MD, PhD, surgical director of the Center for Advanced Intestinal Rehabilitation, performed the first serial transverse enteroplasty (STEP) procedure. This is an innovative surgery that effectively lengthens the small intestine, allowing it to better absorb nutrients and flush out bacteria.

    The STEP procedure is now being used worldwide, allowing some children to wean completely from IV nutrition and avoid the need for intestinal transplantation. In the spring of 2004, the STEP Data Registry was started to track outcomes of children who have had the STEP procedure, so that we can better understand its effectiveness compared to other bowel-lengthening procedures. Learn more about the STEP procedure.  


    Smarter nutrition

    Some children with intestinal failure can only receive nutrients intravenously (through an IV). But prolonged use of IV feedings often damages the liver, which can lead to liver failure and the need for a liver transplant.

    Boston Children’s researchers Mark Puder, MD, Jenna Garza, MD, and Kathy Gura, PharmD, discovered that the then-standard formula included a fat mixture that made fat accumulate in the liver, which may contribute to liver disease. They discovered that switching from the old fat to Omegavan, a fat mixture made from fish oil, (which has been shown to prevent fat accumulation) resulted in a formula that’s far less damaging to the liver.

    This discovery has caused a worldwide shift in treatment. Puder and colleagues are now conducting a formal clinical trial to learn how it can be used even more effectively.


    Multidisciplinary care improves survival

    In 1999, Boston Children's launched the Center for Advanced Intestinal Rehabilitation (CAIR) to provide coordinated, multidisciplinary care for children with short bowel syndrome (SBS). CAIR researchers compared 54 children with severe SBS managed by CAIR from 1999 to 2006, with 30 patients treated at Boston Children’s between 1986 and 1998. Survival was 89 percent in the CAIR group, compared with 70 percent in the controls. Notably, among patients who remained on parenteral nutrition, survival was 67 percent in the CAIR group versus 10 percent in the controls. 

  • Blaise's story

    Our experience at Children’s Hospital Boston began on Christmas Eve 2008, eight days before our daughter was born. We had a “concerning” ultrasound at 20 weeks gestation and the follow-up at 30 weeks revealed that our daughter had some kind of intestinal blockage.

    We were referred to the Advanced Fetal Care Center at Children’s. Originally, our appointment was just for a fetal echocardiogram, as intestinal blockages often come with heart defects and I had been born with a minor heart defect. We were to have a more extensive appointment two weeks later to discuss the outlook for our daughter in more depth. However, following the fetal echo, the nurse asked us if we would like her to see whether a doctor could talk with us right then. Even though his coat was on and he was heading out the door to spend Christmas with his family, Dr. Russell Jennings turned around and spent 15 minutes talking with us about possible outcomes. He was honest, friendly and upbeat. We will never be able to thank him enough for those 15 minutes.

    A week later, on New Year’s Eve, I was in considerable pain and called my obstetrician. In spite of only being 32 weeks and 5 days pregnant, I was as big as I should have been at full-term. We were sent immediately to Brigham and Women’s Hospital where I was admitted for betamethazone shots and observation. The next morning, my water broke and less than two hours later, because I was hemorrhaging and the baby’s heartbeat was becoming erratic, I was rushed into the OR for an emergency C-section.

    Blaise Ursula Conwell Balas was born at 10:47 AM on New Year’s Day 2009, seven weeks premature, weighing 3 lbs., 14 oz. She cried immediately, which was very reassuring.

    Unfortunately, she was not as perfect as she sounded. Her belly was distended and bruised looking. The Brigham staff contacted Surgery at Children’s and Dr. Christopher Weldon came over to examine Blaise. He decided to operate immediately and our family had its second emergency surgery of the day. That decision, without question, saved her life.

    During surgery, Dr. Weldon discovered a giant meconium cyst in Blaise’s small intestine. Because this cyst was so large, it had cut off the blood supply to the rest of her small intestine, most of which was dead. The extensive amount of dead tissue made it difficult for Dr. Weldon to explore further without jeopardizing Blaise’s life, so he made the decision to create an ostomy, which would divert waste away from her intestine; place a gastric feeding tube; and insert a central line so that doctors could administer necessary medications. At the end of her surgery, she had 10–12 cm of healthy intestine available to her, a length her doctor described as “not compatible with survival.”

    Blaise spent 26 days in the wonderful care of Children’s Neonatal Intensive Care Unit before being transferred to 10 East, the amazing Infant/Toddler Surgical floor. She was on full IV nutrition (TPN) because she did not have enough intestinal tissue to absorb nutrients from food. Unfortunately, the fat source typically used in TPN is known to cause liver failure in children.

    Enter Mark Puder and Omegaven. Omegaven is an alternative lipid derived from fish oil that prevents and may even help improve TPN-related liver disease. Although it is not yet approved by the FDA for pediatric use, Dr. Puder has permission to use it as part of a clinical trial. As soon as Blaise’s liver began to fail, she was switched to Omegaven; within weeks her liver function had rebounded to normal. Had it not been for Omegaven, we would probably have had to put her on a transplant list. Current survival rates for small bowel/liver transplants are 50%; survival rates on Omegaven are 98%. The choice was obvious.

    After 3 months of healing, growing and winning over all of her nurses, Blaise underwent a second surgery, this one to connect what was left of her small intestine to whatever large intestine she might have. In a truly extraordinary outcome, Blaise came out of surgery with her entire large intestine intact and more than 3 times as much small intestine as she was expected to have.

    We began the process of weaning her off the TPN and onto breast milk and formula. It took a while, but by May 25, she was receiving all of her nutrition from these “normal” sources (through her g-tube, mostly, but it was better than the IV). Finally, on May 28, 2009, nearly 5 months after she was born, we welcomed Blaise home.

    Our journey at Children’s didn’t end there. Short bowel syndrome is a permanent condition requiring long-term monitoring and treatment. We go see our friends at the small bowel clinic every six weeks or so. We make adjustments every time. Currently, Blaise is off tube feeding and gets all her food from bottles, meals and snacks. She is learning to walk, says several words and loves books and graham crackers, just like an ordinary toddler. Anyone who doesn’t know her story would never guess how extraordinary she is.

    -- Erin Conwell

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