Short Bowel Syndrome / Intestinal Failure
Short Bowel Syndrome or Intestinal Failure occurs when a significant portion of the small intestine is either not present or does not function normally. This can happen because the small intestine was surgically removed or the baby was born with an abnormal intestine. For more information on the symptoms, diagnosis and treatment of intestinal failure, download the GIKids Fact Sheet on Short Bowel Syndrome and Intestinal Failure.
What is intestinal failure?
Intestinal failure occurs when a significant portion of the small intestine is not present or does not function normally. This can occur if a large part of the small intestine has been surgically removed or if a baby is born with an abnormal intestine. The presence or function of the large intestine (colon) can also play an important role in how well a child with a shortened small intestine does.
Who is at risk for intestinal failure?
- Babies (usually premature) who have had surgery for NEC (necrotizing enterocolitis)
- Babies born with their intestines damaged by being outside the body (gastroschisis)
- Babies born with narrowed or obstructed intestines
- Patients who have had some intestine surgically removed due to Crohn’s disease
- Patients with intestinal pseudo-obstruction or abnormal motility of the bowel
- Patients who have had surgery for volvulus (twisting of the intestine)
- Patients who have had parts of the intestine removed for other reasons (e.g. tumors, abnormal bloog supply, strictures, etc.)
- Patients born with abnormalities of the absorptive cells lining the intestine
What are the symptoms of intestinal failure?
- Poor weight gain or weight loss
- Poor appetite
- Increased gas, foul smelling stools
- Fatigue, pallor
What are the complications of intestinal failure?
- If the child has a limited number of foods he/she will eat, try some of the following basic steps:
- Introduce foods with a flavor or texture similar to a favorite food
- Introduce strawberries if the child likes strawberry ice cream
- Try low fat frozen yogurt if the child likes ice cream
- Introduce fish sticks or fried vegetables if the child likes chicken nuggets
- Expose children to new foods gradually
- Place new foods on the table only for the first few times, then perhaps on their plate without expecting them to try the food
- Do not force your child to eat all of a new food, allow experimentation
- Allow only tastes, touch, or smell during introduction periods if needed
- Mirror desired behavior by eating new food as a family, talk about a new food positively
- Remember, changing behavior and/or accepting new things can take time, effort, and patience
How is intestinal failure treated?
The diet needs to be adjusted according to the intestine’s ability to absorb nutrients. TPN is usually required at the beginning to maintain nutrition and good hydration although it is hoped that the small intestine will adapt over time in order to allow intestinal (enteral) nutrition alone. Since the biggest problems are related to TPN and intravenous (IV) nutrition, the first goal is to try and gradually decrease the TPN while increasing enteral nutrition.
Pre-digested and hypoallergenic formulas improve intestinal absorption, and extra vitamins and minerals are often added. These formulas are usually given slowly by a feeding tube and a pump, sometimes just at night. Dripping the formula in slowly gives the intestine more time to absorb nutrients. Portable pumps are available for children who also need drip feeds during the day. Allowing the child to eat small amounts by mouth minimizes the loss of oral feeding skills.
If steady progress is not being made in decreasing the TPN, additional surgery is sometimes performed. Intestinal and/or liver transplantation can be considered for severe forms of intestinal failure or when long-term TPN has resulted in severe liver disease.
What can you expect?
The prognosis of intestinal failure depends on many factors, including the length and specific section of intestine lost, the complications experienced by the child (particularly infections and liver dysfunction) and whether other parts of the body (e.g. heart, lungs or kidneys) are working well. A good portion of children can have their TPN and enteral formula feedings discontinued and have a normal quality of life.
For more information or to locate a pediatric gastroenterologist in your area, please visit the NASPGHAN website.