Short bowel syndrome is an extremely rare disease of the intestines. Only about 10,000 to 20,000 people in the U.S. have it, including adults and children.
Here’s what you should know.
What Is Short Bowel Syndrome?
When you have short bowel syndrome, a part of your small intestine is either missing or damaged and doesn’t work. The small intestine is the part of your digestive tract that absorbs most of the nutrients you get from food. When you have short bowel syndrome, your body can’t fully absorb all the nutrients you need.
Short Bowel Syndrome Causes
Most of the time, short bowel syndrome is a side effect of surgery to remove a piece of your intestines because of another health problem. It’s possible, however, for a baby to be born with it. It’s not inherited. If a newborn has short bowel syndrome, it’s usually because of necrotizing enterocolitis. This is a serious condition that causes a baby’s intestinal tissue to become inflamed, which causes the tissue to die.
Short bowel syndrome in infants
Short bowel syndrome can be a birth defect. In this case, the small intestine didn’t grow properly when the baby was in the womb. As a result, the small intestine may be too short, not fully formed, or missing a section.
Small bowel resection
More often, short bowel syndrome happens after a piece of the small intestine is removed to treat another health problem in infants, children or adults. This surgery doesn’t guarantee you’ll get short bowel syndrome. It depends on many other factors, including:
- Which portion of your small intestine is removed
- How much of your intestine is removed
- The length and condition of your remaining intestine
- Your age and overall health
- Whether rehab after your surgery is able to help the remaining intestine adapt
Who Gets Short Bowel Syndrome?
People with short bowel syndrome either needed some of their small intestine removed to treat a serious health problem or they were born with an abnormally short bowel.
Who gets small bowel surgery?
The most common health problems that lead to small bowel surgery are:
Necrotizing enterocolitis. This is a serious, life-threatening condition that can happen to babies born early or underweight. It’s the most common reason a baby would need surgery to remove part of the small intestine. Severe inflammation of the intestine allows bacteria to get in and will lead to a loss of blood flow to the intestine. This causes that tissue to die.
Gastroschisis. This condition happens when a baby develops inside the womb. It’s when the intestines grow outside the body.
Artesia of the small intestine. A birth defect in which several parts of the small intestine are too narrow to function.
Volvulus. In this condition, the intestine is twisted around itself. It can be a birth defect. Or it can happen to adults because of another health problem.
Crohn’s disease. An autoimmune condition in which your immune system attacks your digestive tract and severely damages a part of your intestine.
Intussusception. This is most common in babies. A piece of the intestine slides into itself, like a telescope, and blocks blood flow and the passage of food. It can happen to adults, too.
Who is born with a short bowel?
Doctors and researchers don’t know a lot about what would cause a baby to be born with a short bowel. Some cases, they believe, are because the baby inherited a gene that caused the intestinal problem. There are two genes that may cause this: CLMP and FLNA.
Short Bowel Syndrome Symptoms
The first and most common symptom of short bowel syndrome is diarrhea that doesn’t go away. In the early stages, you may have other gastrointestinal symptoms. As time goes on, you can become dehydrated and malnourished, which can lead to other symptoms.
Symptoms of short bowel syndrome vary a lot from person to person depending on the length of the remaining intestine, how well it works, and how well it picks up the slack for the missing part.
Early signs of short bowel syndrome
Besides diarrhea, the early signs of short bowel syndrome might include:
- Greasy, pale, especially bad-smelling poop
- Abdominal cramping
- Heartburn
- Fatigue and weakness
- Weight loss
Serious symptoms of short bowel syndrome
Malnutrition and dehydration from short bowel syndrome are serious. This means your body isn’t getting enough nutrients or water.
Untreated dehydration is a medical emergency that can lead to organ damage and shock. These are urgent signs of dehydration that can come from short bowel syndrome:
- Peeing less than usual (or babies, that means no wet diapers for 3 hours or more)
- Dark-colored urine
- Stiff, non-elastic skin (when you pinch and release it, it doesn’t snap back in place)
- Extreme thirst and dry mouth
- Feeling lightheaded, dizzy, or fainting
- Eyes or cheeks that look sunken
- For babies, no tears when crying
Malnutrition can cause:
- Swelling or bloating in your belly
- Muscle loss
- Dry, flaky skin
- Swollen feet and legs
- Hollow temples
- Poor growth in babies
A lack of vitamins, iron, and zinc each have their own set of symptoms beyond the general symptoms of malnutrition.
Short Bowel Syndrome Complications
Short bowel syndrome can cause the following serious problems.
Diarrhea
Severe and ongoing diarrhea can cause malnutrition, extreme dehydration, and death.
Esophagitis
In short bowel syndrome, your stomach can make too much acid, called gastric hypersecretion. Unchecked, this can lead to heartburn, ulcers and, in rare cases, inflammation of your esophagus, also called esophagitis. The inflammation can make it difficult and painful to swallow and difficult to eat, too. The condition may cause chest pain as well.
Fatty liver disease
As many as 50% of people with short bowel syndrome can develop fatty liver disease, also called hepatic steatosis. This happens when too much fat builds up around the liver and messes with its proper function. Untreated, it can lead to cirrhosis, liver cancer, and liver failure. The risk is highest for people who have to be on long-term IV nutrition, also known as parenteral nutrition.
Gallstones
Short bowel syndrome raises risk for gallstones, especially if you’re on long-term IV nutrition. People who are on IV nutrition very often get a buildup of calcium salts in their gallbladder that can lead to the formation of gallstones.
Kidney stones
These often form when your body doesn’t absorb fat properly, which is the case in short bowel syndrome. Fat is left behind in your colon, where it combines with calcium and forms an acid that travels to the kidneys and forms stones.
Nutrient deficiencies
Your body can’t absorb nutrients properly in short bowel syndrome. A lack of certain nutrients leads to other problems:
- Vitamin A: Damage to your eyes.
- Vitamin B: A host of problems throughout the body, including in your immune, circulatory, and nervous systems.
- Vitamin D: Soft bones (rickets) in children. Thin, weak bones (osteoporosis) and fractures, muscle spasms, and nerve problems in adults.
- Vitamin E: Abnormal fluid collection and swelling in your legs and feet.
- Vitamin K: Bleeding for longer than normal, internal bleeding, easy bruising that happens too often, and other skin color changes.
- Iron: Pale skin, misshapen fingernails, weakness, and extreme tiredness.
- Zinc: Hair loss, slow wound healing, and eczema-like skin inflammation.
Metabolic bone disease
This is another complication that’s more likely to happen in people who are on long-term IV nutrition. It’s caused by nutritional deficiencies. Metabolic bone disease refers to a group of diseases that weaken your bones. The most common ones are osteoporosis and osteopenia (which is less severe).
D-lactic acidosis
Also called D-lactic encephalopathy, it happens when carbohydrates left behind in your colon join forces with bacteria in your colon to form d-lactic acid. When this acid builds up in your bloodstream, it can lead to confusion, poor muscle control, and slurred speech.
Short Bowel Syndrome Diagnosis
Doctors might need to run multiple tests to confirm a diagnosis of short bowel syndrome. Here’s what you might expect.
Blood tests
Because short bowel syndrome leads to malnutrition, your doctor may order blood tests (such as a blood chemistry test) to measure levels of vitamins, minerals, and other nutrients in your blood. Abnormal levels could point to short bowel syndrome.
Stool tests
In short bowel syndrome, your gut is unable to absorb fat the way it should. Your doctor might order a fecal fat test to see how much fat is in your stool (your poop). An abnormally high level could help confirm suspicion of short bowel syndrome.
Typically, this test is done with a home test kit. You place plastic wrap over the toilet bowl to capture the poop. You may be asked to capture all your poop for 24 hours to 3 days. You’ll place it in the provided container and send it to the lab.
For babies in diapers, you’ll line the diaper with plastic wrap to collect the poop for a specific amount of time, place it in the container, and send it to the lab.
Imaging
Your doctor might need pictures of your small intestine. Here’s how those pictures might be taken:
X-ray. You’ll lie on a table under a machine that uses a small amount of radiation to take pictures of the inside of the body.
Upper GI series. You’ll swallow a chalky liquid that helps make abnormal things in your upper GI tract, including your small intestine, more visible on an X-ray.
CT scan. You’ll lie on a table on a machine, and your midsection will be inside a large ring that will capture images of your gut through X-rays and computer technology.
MRI. You’ll lie very still inside a tube within a machine that uses radio waves and a large magnet to take pictures of the inside of your body.
CT or MRI enterography. You’ll swallow dye or get it through an IV so that the CT scan or MRI can better show any problems in your intestines.
Liver biopsy
Short bowel syndrome can lead to liver damage. A liver biopsy can further confirm the diagnosis and show the extent of the condition by uncovering liver damage.
In most cases, doctors can do a percutaneous liver biopsy. That means they insert a needle through the belly and into the liver to remove a small piece of liver tissue. The tissue is then looked at in a lab.
Bone density test
Malnutrition from short bowel syndrome can lead to bone-thinning and bone-weakening diseases. A bone density test can show whether the short bowel syndrome has already advanced to that degree.
A bone density test is also known as a DEXA scan for “dual-energy X-ray absorptiometry scan.” It’s a low-radiation X-ray.
What to do when you are diagnosed with short bowel syndrome
If you or your child gets a diagnosis of short bowel syndrome, the next step will be to work with your health care providers to come up with a treatment plan.
Short Bowel Syndrome Treatment
For many people with short bowel syndrome, the other healthy intestines adjust and reach full function. Until that time, the goal of treatment is to make sure you get the nutrition you need and avoid complications until your GI tract is fully working. Your individual treatment plan will depend on the severity of your condition, what part of your intestines is affected, and whether your colon is involved.
For mild cases of short bowel syndrome, it might be enough to slowly increase how much you eat and drink while your intestines adjust. During this time, you may work with a dietitian to carefully plan what you eat and drink. You might take supplements to ensure you’re getting all the nutrients you need. You may also need anti-diarrhea medicine.
For moderate to severe cases, you might need total parenteral nutrition. That means you’ll get all your nutrition from fluids given to you through an IV. Severe cases might also require a bowel transplant.
Whenever possible, the goal will be to get you off of parenteral nutrition since long term use of it raises risk for serious complications. As you wean off of IV nutrition, you may move to enteral nutrition. That’s when you get food delivered directly to your intestine through a tube. You’ll gradually increase your eating and drinking, which stimulates your intestines and helps them adapt and begin to absorb nutrients as they should. You’ll work with a dietitian to make sure you eat and drink the right things.
Some people improve but don’t fully recover and need IV nutrition for life.
Surgery
Some people need surgery to deal with certain aspects of short bowel syndrome.
You might need surgery to:
- Put a tube, called a catheter, into a vein so you can get IV nutrition and fluids
- Put a feeding tube in your stomach or small intestine to give you enteral nutrition
- Fix or remove other damage or problems in your intestines
- Make your intestine longer or change its structure so it can work better
- Get an intestinal transplant in serious cases when your intestines don’t return to proper function, you need long-term IV nutrition, and you develop serious complications
Other treatments
Medicine will play a role in your care, too. You might receive one or more of the following:
Teduglutide (Gattex). This medicine might improve the structure and function of your intestine so that it can absorb nutrients and reduce your need for IV nutrition.
Glutamine. It's a human-made form of a substance made in the body that helps cell growth and function to improve the function of your intestine and lower the need for IV food.
Somatropin (Genotropin, Humatrope, Norditropin, Nutropin, Serostim, Zorbtive). A human-made form of human growth hormone, a substance given off by your pituitary gland, that can help your intestines absorb better.
You may also get prescription diarrhea medicine and antacids.
Home remedies for short bowel syndrome
Over-the-counter medicines for diarrhea, pain, and heartburn may be a part of your care. You might need dietary supplements for nutrition, too. But whatever you take for your symptoms, it’s important that you clear it with your health care provider first.
Short bowel syndrome treatment timeline
Treatment for short bowel syndrome can take years. Here’s a look at what you might expect.
Acute stage. During the first 3 to 4 weeks of short bowel syndrome, you may get:
- Parenteral nutrition, where you receive the nutrients you need through an IV
- Enteral nutrition, where you receive liquid food through a tube implanted in your digestive tract
- Small amounts of food and drink that gradually increase as you're able to take them in
Adaptation stage. It may take your intestine several years or more to get used to its short length and get back up to full power. During this phase, you might get:
- Less IV nutrition as your intestinal function improves
- More enteral nutrition as your intestine gets better able to absorb it
- More real food and drink
Maintenance stage. At this point, your intestines have healed and improved as much as they are going to. You may need to take up permanent lifestyle changes to make sure you can get all the nutrients you need to stay healthy.
When Treatment Doesn’t Work
If, once your gut has stopped adapting, it still can’t absorb enough nutrients to keep you healthy, you have what’s called chronic intestinal failure. In this case, you might need long-term IV nutrition or surgery.
Clinical trials for short bowel syndrome
If standard treatments don’t work, you might consider a clinical trial. In a clinical trial, you can get the latest experimental medication or procedures that aren’t yet available at all health care facilities. People often look into clinical trials when they have a condition that hasn’t responded to other available treatments.
Taking Care of Yourself
Once you’re eating and drinking regular foods and beverages, you’ll need to follow a special diet to make sure you get proper nutrition and prevent complications.
Short bowel syndrome diet
You may work with a dietitian to get your diet just right. Here are some rules of thumb for foods to add to your diet and others to avoid.
Make sure you get plenty of:
- Protein, such as eggs, tofu, fish, meat, dairy products, and smooth peanut butter and nut butters
- Refined carbohydrates, such as white bread, white rice, skinless white potatoes, and white pasta
Steer clear of:
- High-fat foods, such as deep fried foods
- Sugar and sweets
You might need to avoid:
- High-oxalate foods, which can cause kidney stones, such as caffeinated drinks, nuts, beans, soy, leafy greens, and many others
- High-fiber, complex carbohydrates, such as whole-grain bread, immediately after surgery
Managing short bowel syndrome
You’ll need change not only what you eat but how you eat so you put less stress on your intestines:
- Eat whenever possible, and aim for four to six small meals a day
- Chew slowly and chew well
- Only drink a half cup of liquid with each meal
Depending how much your intestine eventually adapts, you may be able to go back to three larger meals per day, chewing a little faster, and drinking more liquid with meals.
If you have frequent diarrhea, your doctor might recommend an oral rehydration solution. This isn’t the same as a sports drink. Ask your doctor what to buy.
If you need to gain weight, the doctor or dietitian might suggest protein shakes that you can buy at the drugstore. You might have heard of brands like Ensure and Muscle Milk.
You might also need a daily multivitamin or specific vitamin supplements. Ask your doctor what is best.
Information for Caregivers
Short bowel syndrome may need lifelong or long term care and upkeep. Whether you’re the parent of a child with the condition or the caregiver to an adult with it, you can help them:
- Get to routine medical appointments for follow-up care and monitoring
- Stick to diet recommendations to get complete nutrition
- Keep up with supplements, oral hydration, and nutrition shakes as needed
Short Bowel Syndrome Prognosis
Short bowel syndrome can improve significantly over time or become a lifelong condition.
Can short bowel syndrome be cured?
With treatment and time, some people with short bowel syndrome get better. Their remaining intestine adapts and they’re once again able to absorb all the nutrients they need from regular food. Other people may never get back to a fully functioning digestive tract.
What to expect with short bowel syndrome
You should expect for the recovery process to take up to a few years. How much function you regain in your intestines depends on many factors, such as:
- How much healthy intestine you have
- Which part of your intestine was affected
- Whether your colon is still intact
- Your age
- Your overall health
Getting Support
Living with short bowel syndrome can be very challenging and take an emotional toll, especially if your gut never returns to full function. Learning from and sharing with others who have the same condition can help. You can find support groups and educational resources at the Short Bowel Syndrome Foundation.
Questions to Ask Your Doctor
- How well do you expect my intestines to adapt?
- Do you expect me to get back to normal?
- How long until my intestines have adapted?
- Would a clinical trial be right for me?
- Will I need surgery?
- Is there anything I can do to improve my chances of a full recovery?
Takeaways
- You can get short bowel syndrome from an operation for an intestinal problem or due to a birth defect.
- Recovery is possible, but it’s a long road that may take a few years.
- You should expect to need medication, IV nutrition, an implanted feeding tube, and a special diet.
