Learn about Short Gut Syndrome
What is Short Gut Syndrome?
Short Gut Syndrome, or Short Bowel Syndrome, is a condition in which the bowel is shorter than normal, usually 50 percent or less, due to either surgical resection or, in rare cases, a congenital defect of the intestines. As a result, the bowel does not have the necessary surface area to absorb the water, vitamins, and other nutrients necessary to sustain life.
Symptoms and severity vary depending on which sections of intestine are missing. The primary symptom is diarrhea resulting in dehydration, malnutrition, and weight loss and/or delayed growth. Other symptoms include cramping, bloating, heartburn, weakness, fatigue, bacterial infections, and food sensitivities.
More on this topic . . .
How the intestine works
Causes of short gut syndrome
How is Short Gut Syndrome treated?
Treatment of short gut syndrome is unique as the individual case. Specific treatment is depends on what sections of intestine were removed or lost, how much remains, and how well the remaining portions adapt.
The main treatment methods are focused on providing nutritional support. Treatment may involve oral rehydration solutions, vitamin supplements, enteral nutrition, parenteral nutrition, and medications.
Mild cases are treated by managing diet, taking fluids and nutritional supplements, and taking medications to control diarrhea and bacterial overgrowth. Moderate cases may also require intravenous fluid and electrolytes.
Treatment for severe short bowel syndrome most often requires parenteral nutrition. In some cases, intestinal adaptation is possible and the patient can slowly be weaned off of parenteral nutrition. Surgical intervention may be used in order to assist in intestinal rehabilitation. In the most severe cases, adaptation is not an option and parenteral nutrition must be used indefinitely. Intestinal transplant may be an option for some patients, particularly when other treatment options have failed or when long-term TPN causes life-threatening complications.
Even in severe cases of short bowel syndrome, patients may receive enteral nutrition or continue to eat by mouth, even though nutrition is not absorbed. Feeding, whether enterally or by mouth, may stimulate the remaining intestine, the liver, and other organs to function better.
More on this topic . . .
Diet and short gut syndrome
Parenteral Nutrition (TPN)
Intestinal adaptation and rehabilitation
Surgical treatment of short gut syndrome
What is life like for a child with Short Gut Syndrome?
In many ways, a child with Short Gut Syndrome is a normal child - who needs a lot of help to stay healthy. In many cases, he or she is able to live at home with their families and go with them where they go. One of the greatest gifts you can give your child with Short Gut is to help them live as healthy of a life as possible.
Keeping a child with Short Gut healthy can require a lot of work. The most common concerns of Short Gut are diet and diarrhea. You'll need to learn how to provide proper nutrition for your child, whether by using specific foods and supplements, or by using IV or tube feedings. Also, since chronic diarrhea is a problem, you'll need to know how to make sure your child stays properly hydrated, and you'll need to adjust to the other demands of chronic diarrhea, like frequent diaper and clothing changes.
For the first few months or years after diagnosis with Short Gut Syndrome, your child will need a lot of medical attention. He or she will probably spend some time in the hospital, and even after coming home will probably have frequent doctors appointments. Where possible, the goal of treatment of Short Gut Syndrome is to help the intestine adapt so that the problems of Short Gut, and therefore the required care, can be decreased. This means that, as your child improves, the need for regular medical attention may decrease.
Of course, each case of Short Gut Syndrome is unique, as is each child. Therefore, treatments, goals, and even complications are different for each and every child. Below is a brief summary of some of the issues that a child with Short Gut Syndrome may face.
Diet & Feeding
One of the biggest challenges for a child with short gut is feeding. Short Gut Syndrome causes malabsorption, insufficient absorption of needed nutrients. Therefore, proper nutrition is the primary focus of treatment for Short Gut Syndrome.
Oral Feeding, Special Diet & Nutritional Supplements
Mild to moderate cases of Short Gut can often be managed with a special diet. Diets focus on slowing transit time of the food through the intestine, while optimizing the absorbtion of calories and other essential nutrients. Patients may be asked to avoid foods that can worsen diarrhea, such as foods that are high in sugar. Omega-3 fatty acids, such as fish oil, are often used promote liver health. In babies, special, easily-digested animo-acid based baby formula may be used. Older children may drink nutrition shakes when they cannot otherwise tolerate food. Vitamins and other nutritional supplements may be needed when the gut does not absorb needed nutrients. Some children with Short Gut develop food allergies that would further limit their diet.
Even though children with severe cases of Short Gut do not absorb many nutrients taken by mouth, oral feeding may also be used to help to trigger appetite, stimulate the digestive tract, and provide oral experiences that will help to minimize oral aversion later.
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ThriveRX's Maximize Health! online nutrition education series for consumers with intestinal failure
With enteral feeding, nutrition is delivered by a tube directly into the stomach or intestine. Some tubes are inserted into the digestive tract through the nose (NG, nasal-gastric or NJ, nasal-jejunal tubes). Others are placed surgically through the abdomen wall into the stomach (known as a gastrostomy, gastric tube, or g-tube). Generally, when enteral feeding is used in Short Gut patients, formula is dripped into the stomach continuously over several hours. The goal of continuous feeding is to stimulate the intestine, stomach, and gall bladder. Absorption is improved through the constant, slow delivery of food. Enteral feeding facilitates intestinal adaptation, allowing some patients to slowly wean off of parenteral nutrition. Enteral feeding also plays a role in protecting the liver against damage.
Your doctor and dietician will work with you to determine the best schedule for enteral feeding. If continuous feeding is chosen, a feeding pump can be connected to the feeding tube. Otherwise, bolus feeds can be given intermitently with a syringe. Caregivers will be trained in enteral feeding and arrangements will be made with an enteral feeding pharmacy before patients are sent home.
A child receiving enteral feeding can lead a fairly normal life. If a feeding pump is needed during the day, they are small and portable, allowing the child to move and play without much disturbance.
If your child has a g-tube, it is wise to keep an emergency kit on hand in case the tube is accidentally pulled out, since the gastrostomy can close in under half an hour.
Parenteral nutrition is given intravenously, completely bypassing the digestive system. Also known as PN, or TPN (Total Parenteral Nutrition), this method of feeding allows nutrition to be given when oral or enteral feeding methods cannot provide sufficient nutrition. An individualized solution containing salts, glucose, amino acids, lipids, vitamins and minerals is delivered directly into the bloodstream. Typically, TPN is infused through a central line, a more durable type of IV inserted into a major vein.
Although many people receive parenteral nutrition in the hospital, it is possible to receive TPN at home. Caregivers must be trained in proper care of the central line before taking a child home on TPN, including proper sterile technique to prevent against infection. Arrangements will be made with an infusion pharmacy for delivery of TPN and related supplies. In many cases, a home nurse may visit to help with central line care and/or to draw needed labs. Regular labwork is needed to ensure that proper nutrition is maintained with TPN, so that neither too little nor too much of any nutrient be given. A dietician specialized in TPN therapy will closely monitor lab results and make changes as necessary.
Children on Home TPN can lead relatively normal lives. TPN pumps are also small and portable, allowing children to move without much limitation.
There are risks of complications involved in long-term use of TPN, including infection of the central line and liver damage. More about these subjects is discussed under the heading "complications" below.
The greatest effects of parenteral feeding on day-to-day life are related to the central line itself, not the TPN. The line can be dislodged, and therefore must be secured and protected. This may affect what clothing you choose for your child. Because the risk of line infection is so great, sterile technique in handling the line is of the utmost importance. The dressing on the central line should not get wet or dirty. Caution must be taken to protect the line when bathing. Swimming is not recommended. Fevers, which could be an early sign of infection, are taken very seriously and most often require hospitalization, even if just for monitoring.
Frequent hospitalizations are common for children with a central line. Families will become very familiar with hospital life and hospital staff.
Oral Aversion & Feeding Problems
Many children with Short Gut Syndrome develop oral aversion. Oral aversion is unwillingness to accept food by mouth, or an unwillingness to accept an appropriate amount, variety, or type of food by mouth. In children with Short Gut, oral aversion is common because they often cannot be introduced to foods at developmentally appropriate times. Instead, their oral experience during key developmental times often involved uncomfortable medical contact with the mouth, instead. Oral aversion becomes a problem if medical treatments make it possible for the child to be fed orally, but the child is unwilling to do so.
It is possible to help prevent oral aversion from developing. Providing positive oral and facial sensory experiences, even when a child is unable to eat, can be helpful. In some cases, babies and children can be fed small amounts to maintain an interest in eating. Pacifiers, toys, washcloths, etc. can be used to provide oral stimulation as well. Help from a feeding, speech or occupational therapist can also help.
In contrast to oral aversion, some children experience excessive hunger or thirst. They want to eat or drink all the time. Ensuring that parenteral or enteral feeds meet nutritional needs can help with this problem. Maintaining a routine for feeding and finding ways to make foods last longer can also help.
Stool & Hydration
Because Short Gut Syndrome causes severe diarrhea, conversations about stool are a regular part of daily life for families of a child with Short Gut. Families must learn how to handle frequent liquid stools - including learning to deal with leaky diapers or bags, to protect skin against highly acidic stool, and to compensate for fluid and electrolyte losses caused by constant diarrhea.
Diarrhea & Diapering
The constant diarrhea caused by Short Gut Syndrome can make diapering difficult. Frequent high-volume, liquid stools (a.k.a. "blowouts") sometimes overflow diaper capacity. Leaks are a common problem, especially overnight. Families generally find a diapering system that works best for them by trial and error. Many get up at least once a night to change diapers. Mattress covers are helpful in protecting bedding.
Stool is generally more acidic for Short Gut patients, often leading to severe diaper rash. Protective barrier creams and powders can help to protect the skin against breakdown. Most families have their own "recipe" for the combination creams, powders, etc. that they've found works best for them. Diarrhea and diaper rash is worse with bacterial overgrowth, with topical yeast infections, or with antibiotics, and stronger barrier creams may be needed during those times.
For some children with Short Gut Syndrome, diapering is not a problem. Instead of stool being eliminated through the rectum, it is diverted in an ostomy, a surgically created opening in the body where the intestine is brought to the surface of the abdomen so that waste can be eliminated. Stool is collected in a pouch that covers the stoma (the end of the intestine).
The stoma is soft, pink, and round and sticks out a bit above the skin surface. The ostomy is named after the part of the intestine that has been diverted, therefore an ostomy in the ileum is an ileostomy and an ostomy in the colon is called a colostomy, etc.
Because stool passes through even less of the intestine with an ostomy, it tends to be even more liquid and generally very acidic. Therefore, care must be taken to protect the skin against breakdown. Caregivers will be taught to apply an ostomy pouch, including a skin barrier, and how to use special pastes, powders, etc. to protect against moisture and skin breakdown. This training is often done by a nurse who specializes in stoma care, also known as an enterostomal nurse. Older children can learn to change their own pouches.
Other than the need to care for and regularly empty the ostomy pouch to prevent leaks, children who have an ostomy can participate in normal activities, with the exception of rough contact sports.
Bag leaks are perhaps the most disruptive problem for children. Because stool can quickly cause skin breakdown, leaks must be attended to quickly. It is a good idea to carry an ostomy change kit and a change of clothes with you wherever you go. Babies and small children especially are more wiggly, so pouches tend not to hold as long. Bag leaks at night are common, especially for children who sleep on their stomachs. Many parents use mattress protectors and/or nighttime drainage systems at night.
If a child has both an ostomy and a central line, caution should be used to keep stool off of the line to prevent infection.
Hydration & Electrolytes
The malabsorption and excessive stool losses associated with Short Gut Syndrome can lead to dehydration and electrolyte imbalances. Therefore, maintaining proper hydration is an important part of care for children with Short Gut.
In children with mild cases, this problem might be able to be managed with oral hydration solutions such as pedialyte or gatorade. Moderate to severe cases might require intravenous fluids and electrolyte supplements.
When IV fluids are needed, caregivers may be asked to keep a record of stool output to make sure that the proper amounts of fluids and electrolytes are replaced. This may involve draining and measuring ostomy output or saving and weighing diapers.
Regular labwork may also be needed in order to make sure that electrolyte balances are being properly maintained. Caregivers will also be asked to watch for other signs of dehydration such as decreased urination.
Problems & Complications
There are several problems that may be common as a result of Short Gut Syndrome or its treatment. Not all of the complications described below apply to every case of Short Gut Syndrome.
Whenever the intestinal tract is shortened, it affects the balance of the natural bacteria living in the digestive system. As a result, bacterial overgrowth may occur. This imbalance can cause stomach upset, diarrhea, and other gastric symptoms. Overgrowth of bacteria in the gut can also translocate into the bloodstream, leading to sepsis and central line infection.
Symptoms of bacterial overgrowth include gassiness, foul-smelling gas or stool, increased watery and/or discolored stool, a swollen belly, and difficulty sleeping.
Bacterial overgrowth is most often treated with a two week course of oral antibiotics such as flagyl, kanamycin, or gentamycin that focus on the gut. Prevention often includes use of prebiotics (nutrients that promote growth of good bacteria in the gut) and probiotics (foods or supplements that contain the good bacteria themselves).
Some children require a constant, rotating course of oral antibiotics to decrease the bad bacteria in the gut. This treatment, however, can promote the growth of a specific resistant bacteria that causes a condition called d-lactic acidosis, and so doctors typically will rotate the antibiotics used in this treatment, and may prescribe periodic breaks in treatment.
Food moves more quickly through the intestinal tract in a patient with Short Gut Syndrome. However, in some cases, the stomach or remaining intestine are not strong enough to move efficiently move stool through the intestinal tract.
Dysmotility is a primary reason for creation of an ostomy. An ostomy diverts the flow of stool so it doesn't stagnate in the body, causing bacterial overgrowth among other problems.
Gastric Acid Hypersecretion
It is common for children with short gut syndrome to produce extra stomach acid. Generally this is controlled with H2 blockers like ranitidine and sometimes proton pump inhibitors like Prevacid or protonix.
In children with a central line, perhaps the most frequent complication is sepsis, or an infection of the blood. These infections can be introduced into the system through contamination of the central line, or they may result from bacterial overgrowth in the gut. In either case, sepsis can quickly turn deadly and must be treated quickly.
Because sepsis is so extremely dangerous, children with a central line should be monitored closely for signs of infection. These signs can include fever, irritability, redness or swelling at the infection site, lethargy, chills, and/or unexplained increase in stool output. Early detection and treatment of an infection is key. For this reason, giving fever reducers at home is discouraged.
Whenever infection is suspected, immediate action must be taken. In general, when a child with a central line has a fever, they will be admitted to the hospital until blood cultures can confirm that no infection is present. Frequent ER visits and hospitalizations are common for children with severe short gut syndrome.
If blood cultures do show an infection, IV antibiotics or antifungals will be used to treat it. Some organisms populate the IV catheter. If this is suspected, the central line will be removed and IV fluids and nutrients will be given through a peripheral IV until blood tests confirm that the infection has cleared.
The best prevention of infection is proper line care. It is important that all caps and connectors in the IV tubing be kept sterile. Proper, thorough, and frequent handwashing is essential, especially before disconnecting the tubing or changing the central line dressing.
TPN-Associated Liver Disease
Over time, the use of Total Parenteral Nutrition can damage the liver. When nutrients are given intravenously, the rest of the digestive system is bypassed, putting additional strain on the liver. The result is progressive liver damage.
Initially, the liver becomes inflammed. If the inflammation continues, fibrosis (or hardening) begins to develop around the vessels in the liver. Gradually, scarring will spread throughout the liver, inhibiting flow of blood and bile through the liver.
Blood tests can show the extent of liver damage by measuring the amount of bile (bilirubin) and liver enzymes (ALT, AST) in the bloodstream. Liver damage is manifested physically by a yellowing of the skin and eyes known as jaundice. As disease progressing, hardening and swelling of the liver may also be noted.
Recent studies indicate that the lipids in TPN may be the cause of most TPN-associated liver damage. Some of the methods currently being studied to address this problem include: reducing the amount and rate of lipids given with TPN and supplementing with Omega-3 fatty acids, such as ingested fish oil. Several centers are studying a promising new lipid called Omegaven that is Omega-3 based, rather than the Omega-6 based lipids traditionally used in TPN. While Omegaven is still being studied, its supporters assert that it both protects against liver damage and can reverse existing TPN-associated liver damage. It is currently being tested at several centers throughout the country, but is not yet approved for general use.
Preventative measures focus on protecting the liver against damage. Feeding is believed to slow the rate of liver damage because it stimulates the normal digestive system, returning bile to the liver through normal channels. Avoiding substances (chemicals or medications) that add to the strain on the liver is also believed to protect against inflammation and resulting liver damage.
Difficulty absorbing iron, frequent blood draws for labwork, and compromised liver and spleen function can lead to anemia in children with Short Gut. Symptoms include pale appearance and low energy.
Iron supplements, either in oral form or intravenously, are sometimes used to help with anemia. If the patient becomes severly anemic, blood transfusions may be required.
When frequent lab draws are necessary, it can help to ask the person drawing the labs to draw only the minimum amount of blood required for the tests ordered.
Growth & Development
Because getting proper nutrition is difficult with Short Gut Syndrome, children with Short Gut Syndrome may to grow slowly and to be smaller than other children their age.
Delayed growth may, in turn, lead to developmental delays when the child is not big enough or strong enough to reach developmental milestones at the expected age. Illnesses and hospitalizations can further delay development.
Because feeding is limited in children with Short Gut, it can be a particular developmental challenge. Oral aversion is a common problem. Oral aversion is discussed under "diet & feeding" above.
In general, developmental delays associated with Short Gut Syndrome are a result of the physical limitations of the condition, and not a neurological impairment. With time, most children will overcome these challenges.
Does all of this apply to my child?
The description above is a broad summary of the possible experience of a child with short gut syndrome.
As each child and each case of short gut syndrome is unique. Therefore, each child's experience will be unique. As with treatment, lifestyle depends on a number of factors, including which sections of intestine remain, and how the remaining portions function. As treatments vary, so may personal experiences. The underlying cause that caused the loss of intestines which resulted in short gut syndrome may also affect the child's overall health. Likewise, complications that arise as a result of short gut syndrome can affect your child's health and quality of life.
As children grow, as their intestines adapt or fail to adapt, as complications arise or are resolved, their health and therefore treatment and lifestyle will also change.
It is unlikely that one child would experience everything described above at the same time and, in mild cases, it is possible that very little of what is described above will apply.
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