Short bowel syndrome

We understand that you may have a lot of questions when your child is diagnosed with short bowel syndrome (SBS):

• What is it?
• Why is it a problem?
• What are the treatments?
• How will it affect my child long-term?

We’ve tried to provide some answers to those questions here, and when you meet with our experts, we can talk with you more about your child’s condition and treatment options.

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, Crohn’s disease orgastroschisis.

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 areusually 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.

Questions to ask your doctor

After your child is diagnosed with short bowel syndrome, you may feel overwhelmed with information. It can be easy to lose track of the questions that occur to you.

Lots of parents find it helpful to write down questions as they arise – that way, when you talk to your child’s doctors, you can be sure that all of your concerns are addressed. Here are some to get you started:

• What’s the best treatment for my child right now?
• What is the plan for how my child’s treatment will progress?
• How much experience does this institution have with short bowel syndrome?
• What other questions should I be asking about my child’s situation?