Short Bowel Syndrome

Children get SBS when they have a large section of their bowel removed by surgery. This can happen because of problems such as:

• Necrotizing enterocolitis (nek-roh-TIE-zing ent-air-oh-co-LITE-iss), where infection causes part of the bowel to die
• Gastroschisis (gas-troh-SKEE-sis), where a child is born with their bowel outside their body
• Intestinal atresia, where a child’s bowel doesn’t form all the way before birth
• Volvulus, or twisting of the bowel
• Hirschsprung (HIRSH-sproong) disease, a problem with the bowel that keeps poop from moving forward
• Crohn’s disease, which can damage the bowel
• Bowel injuries

Intestinal rehabilitation focuses on getting your child’s bowel working again so they get the nutrition they need to thrive. Seattle Children’s is home to the Pacific Northwest’s only pediatric Intestinal Rehabilitation Program.

Our team has special training and experience in treating SBS. We use diet, medicines and sometimes surgery. Our team includes:

• Doctors who specialize in the digestive system (gastroenterologists)
• Liver doctors (hepatologists)
• Pediatric surgeons
• Nurses
• Nutritionists
• Social workers
• Pharmacists
• Occupational therapists who help with feeding

Ongoing care from intestinal rehabilitation experts is important to keep your child healthy over the long term. We keep a watch on your child’s growth, review feeding plans and check for problems that sometimes happen.

Many children with SBS need fluids and nutrients through a tube that goes into a vein. This is called IV feeding or parenteral nutrition. “Parenteral” means that feeding does not involve the digestive system.

In total parenteral nutrition (TPN), a child gets all their nutrition through a vein. TPN is a lifesaver for patients who cannot absorb enough nutrition through their small bowel. Our Intestinal Rehabilitation team is very experienced in giving children TPN in the hospital and overseeing parenteral nutrition after children go home.

When possible, doctors try to wean children off TPN to avoid problems. If TPN is used long-term, it can lead to liver disease and life-threatening infections.

An important part of feeding is giving your child some food by mouth. This helps them learn how to suck and eat, helping to absorb food. Our dietitians and feeding therapists are specially trained to teach your child these skills. We focus on feeding by mouth when possible, even in very young babies. We feed by G tube or NG tube only if needed.

Medicines help children with SBS to:

• Reduce stomach acid
• Slow the movement of food through the bowel
• Decrease diarrhea
• Absorb more nutrients
• Control bowel bacteria

Treatment may include hormones to help the bowel lining (mucosa) grow. This helps it absorb more nutrients.

We may recommend surgery for your child in certain cases to lengthen their bowel or make it narrower. Seattle Children’s has more experience performing these surgeries than most children’s hospitals.

Bowel lengthening surgery

If your child has enough small bowel, surgeons can lengthen it and restore function. The surgery is called a serial transverse enteroplasty procedure (STEP).

During the STEP procedure, the surgeon:

• Cuts notches in both sides of an enlarged segment of bowel
• Sews along the cut edges to create a zigzag shape that makes the bowel narrower and longer

As a result, food will take longer to move through the bowel and will touch the bowel lining for a longer period. This lets your child’s body digest food better and create less stool.

Rarely, surgeons cut the bowel lengthwise and sew the ends together to make a longer, narrower tube from a shorter, wider bowel. This is called the Bianchi procedure.

Bowel tapering surgery

In children with SBS, part of the small bowel may become enlarged (dilated). Surgeons operate to make this area narrower (“tapering”) so fluid and bacteria do not collect there. A dilated bowel can lead to:

• Higher chance of infections
• Less nutrients being absorbed
• The enlarged bowel not moving as well

If intestinal rehabilitation is not an option for your child or does not work, they may need a bowel transplant. Seattle Children’s has the only Intestine Transplant Program in the Pacific Northwest – and one of only a handful in the country.

• Our team has a great deal of experience diagnosing and treating SBS. Your child will get care from experts in many different specialties. These include pediatric surgery, the digestive system (gastroenterology), liver (hepatology), nutrition, nursing, pharmacy and social work. This approach is called multidisciplinary.
• We focus on how today’s treatment will affect your child as they grow and become an adult. Our experts base care plans on years of experience and the newest research on what works best for children and teens.
• Doctors across Washington and in other states refer their patients to us. We will partner with your child’s primary care provider to guide their care at home.

• Our pediatric surgery team is experienced in surgeries to treat SBS. Options to lengthen the bowel include the serial transverse enteroplasty procedure (STEP) and the Bianchi procedure.
• More cases mean our surgeons have greater skill and can best decide if surgery is even needed. All that adds up to better outcomes.
• Our treatment options include feeding assistance, IV nutrition, medicine and surgery.
• To provide the highest level of care for the sickest babies, Seattle Children’s has the state’s first Level IV Neonatal Intensive Care Unit (NICU).

Some children with SBS may need a bowel transplant. Doctors at Seattle Children’s helped pioneer this procedure. We are one of only a few hospitals where children can receive bowel transplants.

•  Having a child with a serious illness can be scary. At Seattle Children’s, your family has a full team behind you, from diagnosis through treatment and follow-up. We work together to meet all your child’s health needs.
• We take time to explain your child’s condition and help you fully understand your treatment options.
• Our doctors, nurses, child life specialists and social workers help your family through the challenges of short bowel syndrome. We connect you to community resources and support groups.
• After your child’s initial treatment, we provide follow-up care to keep your child healthy over the long term.
• We work with many children and families from around the Northwest and beyond. We can help with financial counseling, schooling, housing, transportation, interpreter services and spiritual care. Learn about our services for patients and families.

Seattle Children’s leads research in the lab and in the clinic to better understand SBS and improve treatment and quality of life for children with it. Our patients have the option to take part in research studies of promising new treatments. These are called clinical trials.

• We are leaders in starting a quality-of-life registry with centers around the world who care for kids with SBS.
• Current studies include finding the best doses of fats (lipids) for babies after surgery and tracking quality of life in patients and their families.
• Our researchers work to avoid the need for bowel transplant and improve outcomes for children who do need one.