What is intestinal failure?
Intestinal failure occurs when the small intestine does not work properly. There are a variety of reasons why this may happen. It is therefore important to investigate your child’s gastrointestinal tract thoroughly so that the correct treatment can be offered.
Great Ormond Street Hospital has a large nationally-recognised gastroenterology unit. The unit specialises in the assessment and treatment of diseases of the gastrointestinal tract and has all the facilities required for the investigations, tests and treatment your child may need.
The process of assessment involves an entire team of specialists (multidisciplinary team), some or all of whom you may meet during your child’s hospital admission:
How does the gastrointestinal tract work?
The gastrointestinal (GI) tract is a complex organ that extends as a hollow tube from the mouth to the anus. Its main function is to break down food so it can be absorbed into the bloodstream. The process of moving food through the GI tract involves a complex interaction between hormones (chemical messengers) and nerves, so that food is squeezed rhythmically through the system (peristalsis).
Once food has been processed in the stomach, it empties into the small intestine where the majority of digestion and absorption occurs. Here it is mixed with bile and pancreatic juice containing enzymes (proteins that cause or speed up a chemical reaction).
The broken down nutrients are then small enough to pass through the wall of the small intestine, which contains small finger-like structures called villi, and then absorbed by the blood. The blood is carried away from the small intestine through the hepatic portal vein to the liver, where it is filtered, toxins are removed and the nutrients are processed. The residue then passes into the large intestine, where water is absorbed to form solid faeces (poo).
The small intestine is the most important part of the gastrointestinal tract as this is where nutrients are absorbed from food and converted into a form that can be used by the body. It is about two metres (seven feet) long in newborn babies and grows to about six metres (20 feet) long by adulthood.
It is made up of three parts:
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Duodenum – the first part connected to the stomach.
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Jejunum – this makes up about one third of the entire length of the small intestine.
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Ileum – this is the lower and longest part of the small intestine and leads to the large intestine.
The wall of the GI tract is made up of four layers, which are essential for absorption and contain immune cells to protect against disease. If a child’s body is unable to absorb enough nutrients and fluid, he or she will usually show one or more of the following symptoms:
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weight loss
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poor growth
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dehydration
What is intestinal failure?
Intestinal failure occurs when there is reduced absorption by the intestine. Supplements of macronutrients (such as proteins, carbohydrates and fats) or electrolytes (such as minerals, salts and water) are needed to maintain growth and development. If no treatment is given, or the body does not compensate for reduced levels of macronutrients or electrolytes, undernutrition and dehydration can occur.
What causes intestinal failure?
There are two main reasons for intestinal failure:
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The small intestine is too short to digest food properly. This is caused by a section of the intestine needing to be removed to treat conditions such as gastroschisis, volvulus, trauma or necrotising enterocolitis.
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The small intestine is present but does not work correctly due to:
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Disordered motility – such as pseudo-obstruction, neuropathic (nerve) or myopathic (muscle) gut disorders.
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Autoimmune causes – where the immune system mistakenly attacks itself as ‘foreign’ causing inflammation.
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Congenital (present at birth) or genetic (inherited) conditions such as microvillous atrophy, tufting enteropathy or congenital diarrhoea.
Information sheets about some of the above conditions are available. Please ask the team for details.
As each of these conditions needs to be assessed and treated in a different way, a thorough assessment is needed to confirm or rule out each diagnosis.
How will the team plan treatment?
When your child is first referred to GOSH, he or she will be offered an inpatient stay or outpatient appointment depending on the information given in the referral and the needs of your child.
During this visit, a doctor will discuss your child’s history in great detail and carry out a full examination. He or she will review any previous tests from your local hospital if results are available and make a decision about whether any other tests are needed.
Diagnosing intestinal failure is a complex process with many steps so it can take some time to reach a decision. We will do our best to keep you informed of our progress towards a diagnosis.
What tests and scans will my child need?
Your child my need a variety of investigations to diagnose the underlying gut problem and find its cause. Some of the investigations that might be suggested include:
Manometry
This is a test to measure how well the muscles and nerves in the stomach and small intestine work. A catheter (flexible plastic tube) is put through your child’s nose, into their stomach and then to the small intestine.
Fluid is flushed through it, which causes the nerves and muscles in the digestive system to start to move as if your child had had something to drink. The signals made by the muscles as they do this are picked up by sensors contained in the catheter, and these are then recorded by a machine.
Gastroscopy and colonoscopy
These are tests which allow the doctor to look directly at your child’s GI tract. To do this test, an endoscope (a thin, flexible tube with a bright light at the end) is passed either through your child’s mouth down into their stomach for a gastroscopy or through your child’s anus into the large intestine for a colonoscopy.
Video capsule endoscopy
This test is used to view the middle section of your child’s small intestine. Your child will be asked to swallow a capsule containing a tiny light and camera, which transmits pictures of the digestive system to a data recorder worn on a belt around the waist. Read more about video capsule endoscopy in the Procedures and treatments section.
Biopsy
This is a procedure to take a tiny sample of tissue which can later be analysed in the laboratory. This is usually carried out during a gastroscopy or colonoscopy using an endoscope. If a full thickness biopsy is required, your child will be referred to the surgeons.
Gastric emptying study
This is a scan which shows how quickly the stomach passes a liquid into the small intestine. The liquid contains a dye that shows up well on X-rays.
Transit study
This is a test which shows how quickly food passes through the digestive system. Your child will be given some pellets to swallow at home and then a few days later have an x-ray to see how far they have travelled through the GI tract.
Electrogastrogram (EGG)
This measures the activity of the nerves in the lining of the stomach, before, during and after food using electrodes (which look like round, blue stickers) stuck to the skin of your child’s tummy.
Ultrasound scan
This uses sound waves to take pictures of your child’s body. The sound waves are of a much higher frequency than normal so you cannot hear them. A jelly is used to help conduct them inside the body. It is the same as the ultrasound scans used during pregnancy. Read more about ultrasound scans in the Procedures and treatments section.
Impedance study
This scan is used to measure whether food and stomach acid is travelling back up your child’s oesophagus. A thin flexible tube is passed through your child’s nose into their stomach and taped in position. While the tube is in place, you will need to note down what your child does, eats or drinks, so that it can be matched up with the results from the tube.
pH study
This is a test that measures gastro-oesophageal reflux (GOR).
Contrast follow through
This scan uses a contrast, a liquid that shows up well on x-rays, to show your child’s GI tract. Your child will need to swallow some of this contrast as a drink and then have an x-ray around 20 minutes or so later.
Magnetic resonance imaging (MRI) scan
This uses a magnetic field rather than X-rays to take pictures of your child’s body. The MRI scanner is a hollow machine with a tube running horizontally through its middle. Your child will lie on a bed that slides into the tube. Read more about MRI scans in the Procedures and treatments section.
Angiogram
This scan is used to give a picture of your child’s blood vessels and organs using a dye that shows up well on X-rays.
In addition to these investigations, other general assessments may be suggested to look at particular areas more closely. These could include the immune system, metabolic system, genetics, liver and kidneys but will not be needed for every child.
Discussing the results of these tests and scans
If your child has these scans during an inpatient stay, the results will be discussed at the twice weekly ward round, following which the proposed treatment plan will be explained to you and your child. This may be a plan for discharge, for further tests and scans or for further treatment.
If your child has been discharged from hospital or has not had an inpatient stay, we will arrange an outpatient appointment for you to discuss the results and proposed treatment plan.
What are the options for treatment?
It is difficult to predict which treatment will be most suitable for your child as this is dependent on the cause of intestinal failure and the results of the tests and scans.
However, the options generally include the following, although some may not be suitable for every child. Before you make a decision about any of these treatments, we will discuss the advantages and disadvantages with you and explain why each one has been suggested.
Diet
A full feeding history forms part of the assessment process. This is important to see which feeds and feeding methods have been tried in the past, how your child tolerated these and what impact they had on his or her growth and weight gain.
As a result of the full feeding history, the dietitian may suggest altering your child’s feed in some way, either in which feed is given or how it is given. These could include:
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A different milk feed.
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An altered diet, excluding certain foods, such as cow’s milk or limiting certain macronutrients, for example fat or sugar.
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An altered method of feeding, such as oral feeding or tube feeding (nasogastric, gastrostomy or jejunostomy).
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For some children, despite trying various diets and feeding methods, they are unable to thrive. Parenteral nutrition, a way of giving a solution of all the nutrients needed for growth directly into the bloodstream, might be suggested.
You will be involved in the decision making process and we will give you full instructions before you go home. Booklets for care of nasogastric tubes and gastrostomy/jejunostomy tubes and about parenteral nutrition are available from the team.
Medication
Your child may need to take medicines to help with intestinal failure and absorption difficulties. There are various different types of medicine, some of which require regular monitoring with blood tests.
All medicines have some side effects and the aim of this monitoring is to balance the benefits brought by the medicine with any unwanted side effects.
We will make sure you understand fully about the medicine and monitoring required before your child starts taking it. Any medicine prescribed and dispensed through the pharmacy at GOSH will come with an information leaflet.
Other treatments
Some severe intestinal failure conditions may need input from other teams at GOSH and sometimes other hospitals.
Small bowel transplant
If this is suggested for your child, he or she will be treated under the joint care of GOSH with Kings College Hospital (KCH) in South London. Together the teams will carry out a full assessment of your child’s suitability for transplant and make a treatment plan for the future after discussion with you and your child.
The assessment procedure will involve a short inpatient stay at KCH. For more detailed information about the assessment and transplant procedure, please see the booklet produced by KCH, available from your clinical nurse specialist.
Bone marrow transplant (BMT)
This will require your child to be referred to the bone marrow transplant team at GOSH, who will carry out detailed assessments to check your child’s suitability for a BMT, before either looking for a donor within the family or on the international register. The BMT clinical nurse specialists will explain further about this process and give you more detailed written information.
Other surgical options
Surgical procedures such as a STEP (serial transverse enteroplasty) to lengthen a short gut may be an option. If this procedure is suitable for your child, he or she will be referred to the surgeons, who will discuss it fully with you.
What next?
Following discharge after an inpatient stay, we will send a short summary of what happened to you, your family doctor (GP), local consultant and any other members of your child’s healthcare team. Depending on the diagnosis and treatment plan, further outpatient appointments or inpatient stays will be arranged for your child.
