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Pediatric

Intestinal Rehabilitation 

Section 1: Basics of Intestinal Rehabilitation

What is the basic anatomy and physiology of the gastrointestinal (GI) tract and its importance for short bowel patients?


Step 1: The gastrointestinal tract begins at the mouth where mechanical digestion begins. Saliva in the mouth functions to moisten and lubricate food before swallowing and to partially digest food particles.

Step 2: Food moves from the mouth, down the esophagus, and into the stomach.

Step 3: In the stomach, glands secrete hydrochloric acid, which helps digest food and destroy bacteria that enter the body with the food. Little nutrient absorption occurs in the stomach. The digested particulate matter, known as chyme, moves from the stomach into the first portion of the small intestine, the duodenum.

Step 4: Small Intestine

It is in the small intestine where most of the digestion and absorption of food occurs. Special enzymes breakdown carbohydrates, fat, and proteins into smaller components that the intestinal cells can absorb and use throughout the body for nutrients. The pancreas and liver also secrete substances into the small intestine to aid in the digestion of substances.

At birth, a normal neonates small intestine is ~240 cm and colon is ~40 cm. Neonatal short bowel is considered <25 cm with ileocecal valve or <40 cm without ileocecal valve.

The small intestine has 3 parts:

1. Duodenum: Main section for absorption of iron and folate. This is also the section that bile and pancreatic enzymes enter the intestine to aid in digestion.

2. Jejunum: Major section of nutrient absorption.

3. Ileum: Major section of nutrient absorption. This is the only section of intestine that can absorb vitamin B12. If your child does not have his or her ileum, which is common in short bowel patients, they will require vitamin B12 supplementation.

Step 5: Food passes from the ileum through the ileocecal valve and enters the colon. The colon serves two main roles:

1. Acts as a storage chamber for undigested material.

2. Functions to reabsorb water.

Step 6: When initiated, contractions of the rectum and relaxation of the anal sphincter cause defecation, and stool is deposited.

Digestion and absorption rely on many components of proper contractions, enzymes, and cell function. If any of these functions are impaired, then the process may not proceed as planned, as seen in patients with intestinal failure.

For a handy graphic and overview of this process, find our 'Overview of Digestion' in our Topic of the Month Resources.




What are predictive factors for successful intestinal adaptation in a child with short bowel syndrome?


Predictors of intestinal adaptation and the ability to wean from parenteral nutrition include:

  • The length of the bowel remaining.
  • The anatomical section (i.e. location/ portion) of bowel that is remaining.
  • Presence or absence of the ileocecal valve.
  • Presence or absence of the colon.

Since different parts of the bowel absorb different types of nutrients, depending upon which section your child is lacking, will determine the nutritional deficiencies he or she will have and the extent of his or her intestinal failure.




What is a basic overview of intestinal rehabilitation?


Enteral (i.e. passing through the intestine either via the mouth or tube feeding) is critical to maintain normal intestinal structure and function. In order to transition your child from parenteral to enteral nutrition (EN), the intestinal epithelium (lining) must adapt to optimize nutrition absorption.

Your team of surgeons, gastroenterologists, and dieticians will work with you and your child to determine the optimal formula and diet to help the intestine adapt.

In general, carbohydrates may be poorly tolerated by intestinal failure patients as they are broken down by the gastrointestinal tract bacteria into small, osmotically (water) active organic acids that can cause a large amount of water to be drawn into the distal small intestine and colon, causing large fluid losses. Moderate amounts of fat are usually tolerated in the short bowel syndrome diet as well as protein.

Your child may also become dehydrated quickly due to large fluid losses from a high output ostomy, persistent secretory diarrhea, or pseudo-obstruction resulting in large gastric and duodenal losses. In cases like these, an oral rehydration solution containing glucose and sodium may be recommended. You may be required to monitor your child’s urine and stool output to assess the hydration status of your child.

If your child has short bowel syndrome or persistent diarrhea and is requiring tube feedings, a continuous feed through a nasogastric (NG) tube or gastrostomy tube may be recommended. This permits constant saturation of the carrier proteins, thus taking full advantage of the limited absorptive area of the intestine.

It is important to understand that the transition from intestinal failure to adequate intestinal function and the ability to wean from parenteral nutrition to full enteral feeding can take weeks, months, and even years.




What is intestinal failure (IF)?


Short bowel syndrome may lead to intestinal failure. Intestinal Failure (IF) develops when your child's small intestine does not provide the necessary long term nutrition needed to sustain a normal life. This results in the need for additional long term nutrition to be delivered through large veins in the body. This is called "Parenteral Nutrition" or "PN."




What is intestinal adaptation and why is this important for intestinal rehabilitation?


Intestinal adaptation is the bowel's ability to 'adapt' or adjust to the changes that have occurred within the intestine. The intestine compensates for lost function in one area of the intestine by adapting at another site. Intestinal adaptation is a slow process and can take up to two years to complete. In what ways does the bowel adapt in children with short bowel syndrome or intestinal failure? 1. The villi (little finger-like projections that line the intestine) in the remaining intestine increase in both number and size. This increases the surface area of the intestinal wall, increasing the area in which food and nutrients can be absorbed. 2. Decrease in motility. This allows for increased contact time with the intestinal wall, which allows for increased nutrient absorption.




What is intestinal rehabilitation?


Intestinal rehabilitation is the process of restoring nutritional autonomy. This means weaning from parenteral nutrition (PN) and onto oral feedings. This allows your child to live free from PN and its associated complications.




What are the components of intestinal rehabilitation?


1. Diet modification 2. Oral Rehydration Therapy 3. Vitamin and Mineral Supplements 4. Medications 5. Surgical Procedures




Who is on an intestinal rehabilitation team?


At an intestinal rehabilitation center, your child will have a team of clinicians to support them through their journey. Typically on an intestinal rehabilitation clinical team, you will find a:

  • Surgeon
  • Gastroenterologist
  • Nutritionist or Dietician
  • Social Worker
  • Child Life Specialist
  • Clinical Nurse Coordinator
  • Advanced Practice Nurse
  • Mid-Level Providers
  • Pharmacist
  • Speech-Language Pathologist
  • Occupational Therapist





Section 2: Diet and Hydration

What are the 3 general phases of diet and nutrition for infants and young children with short bowel?


The entire intestinal adaptation process, and weaning a child completely from PN and enteral feeds, can take months to years. To learn more about PN and enteral nutrition visit our page here. Phase 1:

  • First few weeks of life
  • Initial stabilization phase
  • Infant is started on parenteral nutrition (PN): nutrition through the veins to stabilize fluids and electrolytes
  • Often high output and diarrhea
  • Goal is to meet the needs for growth and development
Phase 2:
  • Start the child on enteral feeds (tube feeding), usually continuous, to stimulate intestinal adaptation
  • Begin weaning off of PN, if tolerated
  • Supplement the appropriate vitamins, minerals, and electrolytes
Phase 3:
  • Intestinal adaptation is near completion
  • Start on age-appropriate oral foods, generally food that is high-protein, low-fat
  • Try to avoid carbohydrates because of their osmotic effect (cause increases in diarrhea)
  • If the child has an oral aversion to food, consider seeing an occupational therapist or speech-language pathologist
  • Try to wean off of enteral feeds (tube feeds)




Carbohydrates


Carbohydrates are an essential energy source in the diet, but for patients with short bowel syndrome, the type and amount of carbohydrate consumed must be limited to avoid exacerbating diarrhea. Simple carbohydrates, or sugar, have an osmotic effect: this means water is drawn into the intestine and will increase your child's diarrhea. Avoid simple sugars, such as cookies, doughnuts, fruit juices. Complex carbohydrates are well-tolerated and should be roughly 50-60% of the diet (with a colon) and 40-50% of the diet (without a colon or for toddlers not yet toilet trained). Examples include bread, pasta, rice.




Fiber


Two types of fiber: Soluble Fiber:

  • Include soluble fiber in your child's diet.
  • Soluble fiber helps to slow movement through the intestine.
  • Soluble fiber passes from the small intestine undigested into the colon, where colonic bacteria ferment it.
  • One of the byproducts of fermentation is butyric acid which is a valuable source of calories, improves the integrity of lining cells, and enhances absorption of water and salt.
  • If consumed excessively, it may contribute to gas, bloating, and malabsorption.
  • Examples: Metamucil®, Benefiber®, Nutrisource®, pectin, guar gum, oatmeal, barley, legumes.
Insoluble Fiber:
  • Avoid insoluble fiber, which tends to hold water in the intestine, increasing diarrhea.
  • Insoluble fiber increases transit time and movement of stool through the intestine.
  • Examples: vegetable stems, fruit and vegetable skins, seeds.




Protein


Protein is an essential nutrient for your child's growth and development. It is recommended that protein is about 20% of dietary calories. Try to include protein with every meal. Foods high in protein include chicken breast, turkey, eggs, cheese, and yogurt.




Fat


Fat is an important energy source for the body, and it provides insulation and protection for internal organs. Fat also supplies essential fatty acids and fat-soluble vitamins (A, D, E, and K). The recommendation is for fat to be 20-30% of dietary calories with a colon and 30-40% of diet without a colon or for toddlers not yet toilet trained. Short bowel patients should prioritize polyunsaturated fats as these contain essential fatty acids (EFAs), or fatty acids that the body cannot make.




Types of Fluids


Hyper-Osmolar

  • These are fluids that contain sugar (glucose) and minimal salt (sodium).
  • These types of fluids cause water to be drawn into the intestine, causing watery diarrhea.
  • Examples: Juice, sugary soda drinks.
  • Should be avoided by short bowel patients to prevent dehydration.
Iso-Osmolar
  • These are fluids that contain electrolytes (sodium, potassium) and glucose in the same concentration as the body's extracellular fluid.
  • These types of fluids do not cause water to be drawn into the GI tract- ideal for short bowel patients.
  • Examples are oral rehydration solutions like Pedialyte® or G2®
Hypo-Osmolar
  • These are fluids that contain little to no sugar (glucose) and salt (sodium).
  • The GI tract partially absorbs these types of fluids.
  • Examples: Water, sugar-free drinks




What is Oral Rehydration Therapy (ORT)?


  • In addition to maintaining nutrient intake, your child must also maintain hydration status.
  • To maintain hydration status there must be an equal amount of input and output of fluids.
  • Fluid imbalance occurs when there is either a fluid deficit, such as losing fluid with diarrhea, or fluid surplus, such as retaining water with heart failure.
  • As a short bowel patient, your child is at high risk for fluid deficits and electrolyte abnormalities due to their large amounts of diarrhea, causing a significant loss of water and sodium.
  • Rehydration with just water and/or water+sodium will result in the continued loss of fluids.
  • The use of oral rehydration solutions (ORS) can help solve this problem.




Oral Rehydration Solutions (ORS)


Oral rehydration solutions (ORS) are a mixture of water, sodium (possibly other salts), and glucose (sugar) that utilize a unique sodium-glucose transport mechanism across the intestinal cell wall.

  • This results in the absorption of Na+, creating a gradient, and water follows.
  • There are numerous formulations, flavors, and types of ORS.
  • ORS solutions may be purchased or can be homemade.
  • Commercial examples include: Pedialyte,® DripDrop, Ceralyte,® or Liquilyte®.
  • The solutions contain the proper amount of salt, sugar, and fluid to restore the fluid, potassium, and sodium that is lost from excessive diarrhea (i.e., iso-osmolar as described above).
Check out our types of fluids sheet for more information. Sign-Up or Log-In to access under on our Topic of the Month Resources Page.




General Diet Tips for Short Bowel and Intestinal Rehabilitation Patients


  • Have your child eat small, frequent meals 6-8 times per day. Try to include protein with each meal.
  • Encourage your child to chew food well, which helps to increase digestion and absorption of nutrients.
  • Supplement appropriate vitamins and minerals, as recommended by your child's team.
  • If your child has a colon, limit intake of oxalate to avoid the formation of kidney stones.
  • Try to avoid giving your child high sugar drinks and desserts, which draws more water into their intestine and increases diarrhea.
  • Limit your child's fluids at mealtime because fluids can increase output, push food through the bowel faster, and hinder nutrients' absorption.
    • A general rule is to limit fluids to 1/2 cup (4 oz) during a meal.
    • Have your child consume the rest of their fluids between meals throughout the day, with at least a one hour buffer from food.




Why is it important to introduce oral feeds to your child with short bowel at an early age?


There are many reasons to introduce foods at an early age. Usually, it is recommended to start age-appropriate foods around 4-6 months. 1. Food moving through the digestive tract stimulates intestinal adaptation and release of trophic (growth) factors. 2. Avoid oral aversion and feeding disorders:

  • Many young children with short bowel develop feeding disorders due to bad experiences with eating/feeding, frequent NG tubes, and intubation.
  • Because of these things your child may miss out on the opportunity to develop certain feeding reflexes and not 'learn to eat.'
  • In these cases, an occupational therapist or speech-language pathologist may be consulted to help your child learn to eat and get rid of these aversions.
  • Read this article for more information.





Section 3: Medications

Possible Medications for Intestinal Rehabilitation


1. Anti-diarheal medications or medications that help decrease ostomy output. 2. Anti-secretory/ anti-acid medications 3. Bile acid binders 4. Antimicrobials 5. Growth Hormone 6. Glucagon-Like Peptide 2 (GLP-2) Analog




Anti-Diarrheal/Motility and Medications that Decrease Output


Anti-diarrheal medications slow down the movement of contents through the intestine. This increases the time contents spend in contact with the intestinal wall, thus, increasing absorption. Examples include:

  • Loperamide (Imodium®)
  • Diphenoxylate/atropine (Lomotil®)
  • Codeine
  • Tincture of opium
  • Octreotide: This is the man-made form of a hormone called somatostatin. It functions to slow down the action of the small intestine, decrease small bowel secretions, and enhances the absorption of water and salts. It helps to decrease your child's ostomy output and diarrhea if extremely high.




Anti-Acid and Anti-Secretory Medications


These medications decrease the amount of stomach acid the stomach produces. In children with a short bowel, it is common for the stomach to secrete excess acid, known as gastric hypersecretion. This can lead to increased diarrhea, heartburn, ulcers, poor digestion, and other issues. Your child's team may recommend taking one of these medications to help with these signs and symptoms. Examples include:

  • H2 blockers: famotidine (Pepcid®), ranitidine (Zantac®)
  • Proton-pump inhibitors: omeprazole (Prilosec,® Losec®)
  • Octreotide
  • Clonidine




Bile Acid Binders


These function to bind excess bile salts, which can worsen short bowel syndrome malabsorption. They can help with bile salt diarrhea, but must be taken carefully because they can also affect your child's ability to absorb fat-soluble vitamins and nutrients. An example is cholestyramine.




Antimicrobials


It is common for children with short bowel syndrome to get small intestinal bacterial overgrowth. This is treated with an antimicrobial agent or regimen. Your child's team will recommend the best antimicrobial treatment. Probiotics may also be suggested to help with SIBO.




Growth Hormone


Growth hormone, known as somatropin, is made by the pituitary gland in the brain. When released into the body, it promotes the growth and maintenance of organs and tissues. It has been made into a synthetic form. When injected into an individual's with short bowel, it has been shown to enhance intestinal adaptation and increase absorption of fluids and nutrients.




GLP-2


Glucagon-Like Peptide-2 (GLP-2) (Teduglutide, Gattex®): GLP-2 is a substance usually produced naturally by the human body. Gattex® is an injectable analog that is FDA approved for children >1 year of age with short bowel syndrome and dependent on PN. Function: Improves absorption of fluids and electrolytes by increasing the absorptive surface of the small intestine. The goal of starting the medication is to help your child have the ability to wean completely from PN.





Section 4: Surgical Procedures

What are the main goals of intestinal rehabilitation surgical procedures?


The last component of intestinal rehabilitation is reconstructing an individual's native bowel via various surgical techniques. The methods either try to optimize the function by lengthening or tapering the dilated bowel or slowing the transit, increasing the time the food and fluid come in contact with the bowel wall, hopefully increasing absorption of nutrients. Surgery may involve procedures which:

  • Narrow a dilated segment of the small intestine.
  • Slow the time it takes for food to travel through the small intestine.
  • Lengthen the small intestine.
  • Prevent blockage and preserve the length of the small intestine.




Bianchi Procedure


The dilated short bowel is divided in half (cut along its length) and sewed one end to the other, therefore, creating a longer, narrower tube with a smaller diameter.




Serial Transverse Enteroplasty Procedure (STEP)


This involves lengthening dilated small bowel by creating a row of alternating slits in the small intestine and stapling the V-shaped cuts shut, creating a zig-zag like tube. The benefit of the STEP is that it can nearly double the bowel's length, greatly enhancing the amount of time nutrients spend in contact with the absorptive intestinal surface.




What happens if my child fails intestinal rehabilitation and is unable to wean from PN?


If your child is unable to wean from PN using intestinal rehabilitation therapies, your team may recommend an intestinal transplant. Please visit our site here for a complete overview of intestinal transplantation.





Section 5: Additional Resources

Helpful Downloads from Transplant Unwrapped


Sign-Up or Log-In to Access: 1. Short Bowel Syndrome (SBS) and Intestinal Rehabilitation Handbook. 2. The Digestive System: A brief overview of the important parts of the digestive tract and how they relate to short bowel. 3. Short Bowel Syndrome At-a-Glance for Pediatric Patients. 4. The Best Fluids for Intestinal Patients.




Transplant Unwrapped Kid's Activity Book


Short Bowel Syndrome and Intestinal Rehabilitation: A Kid's Educational Activity Book.




Listing of Intestinal Rehabilitation and Transplantation Centers


Intestinal Rehabilitation and Transplantation Centers: Find a listing of intestinal rehabilitation and transplantation centers in the US.




Helpful Website: UVA Nutrition


UVA Nutrition: University of Virginia Nutrition resource webpage with numerous downloads and information on short bowel syndrome and nutrition.




Transplant Unwrapped: Learn From Others


Learn From Others: Read stories, watch interviews, and listen to audio from numerous members of the intestinal community on a variety of topics. Useful for This Section: Learn from Others- Webinars- Short Bowel and Intestinal Rehabilitation presented by Dr. Khanna.




Transplant Unwrapped: Support Programs


Support Programs: Visit the Transplant Unwrapped Support Programs page to get your questions answered, speak with others in similar situations, and feel well-supported during your medical journey.




Page References


References here.