As conversation centring on parenteral nutrition and nutritional complications in intestinal failure continues to pick up pace, NIHR wants to find out more. Here, we interview two members of the European Society for Paediatric Gastroenterology, Hepatology and Nutrition’s (ESPGHAN) Committee on Nutrition: Dr Jutta Koeglmeier, Paediatric Gastroenterologist at Great Ormond Street Hospital, and Dr Barbara de Koning, Paediatric Gastroenterologist at Erasmus MC, Sophia Children’s Hospital, The Netherlands.

Intestinal Failure

What is Intestinal Failure?

Patients suffer from intestinal failure when there is a critical reduction of the small intestinal gut mass or function below the minimum necessary for maintaining health or growth.

Intestinal failure can be caused by a number of factors, including substantial surgical resection or extensive intestinal atresia resulting in short bowel syndrome. Other causes of intestinal failure are motility disorders, such as chronic intestinal pseudo-obstruction, and intrinsic disorders of the epithelium (enteropathies), including rare diseases, such as microvillus inclusion disease.

What are the Signs, Symptoms and Risk Factors of Intestinal Failure?

Intestinal failure may be identified through a number of non-specific signs and symptoms. These can include diarrhoea or a high-output stoma, abdominal pain, vomiting, dehydration, weight loss and malnutrition.

The primary risk factor for intestinal failure is a resection or loss of a large part of the small bowel. Additionally, the performance of the nutrient absorption capacity of the small intestine should be taken into account when considering the likelihood of intestinal failure.

What are the Effects of Intestinal Failure on a Patient?

Patients with intestinal failure are unable to meet their nutritional requirements or hydration with oral food and drink intake. As a consequence, parenteral nutrition is given into a central venous catheter and many patients will require parenteral nutrition on a daily basis.

Intestinal failure is a complicated chronic condition which can have a profound physical and psychological impact on a patient, especially as patients often have to tolerate recurrent medical procedures such as surgery and central venous catheter insertion. A proportion will have a stoma or enteral feeding device.

Prolonged hospitalisation can be particularly distressing for children and their families, and many have to deal with distressing symptoms, such as diarrhoea. Patients with extended or irreversible intestinal failure are usually managed at home using home parenteral nutrition. Although the aim is to support as normal a life as possible, home parenteral nutrition also requires a significant amount of time and care. However, quality of life on home parenteral nutrition can be good.

What are the Treatment Options for Intestinal Failure?

The aim of intestinal failure treatment is to provide adequate macro-and micronutrients and fluids to allow for homeostasis in adults and growth in children. Modern intestinal failure management has improved long-term survival dramatically and the focus is to avoid long-term complications such as catheter-related blood stream infections, loss of central venous access sites, intestinal failure-associated liver disease, metabolic bone disease and renal problems while receiving parenteral nutrition.

Where possible, the long-term goal is to achieve intestinal autonomy and remove the need for parenteral support through a multidisciplinary support team. Good dietetic management to maximise the ability of the gut to absorb nutrients, speech and language therapy to further oral intake in patients with food aversion, and careful adjustments of the parenteral nutrition prescription according to the patients’ needs are essential.

Surgical strategies for short bowel syndrome as a causative factor for intestinal failure can be considered and include different techniques of intestinal lengthening. Evolving evidence supports the use of medication to promote gut adaptation. These drugs work by promoting mucosal growth and where possible, restoring gastric emptying and secretion.

In patients with irreversible intestinal failure who have developed serious complications or have a poor quality of life, intestinal transplantation can be considered as a cure. Life-long immunosuppression is however required and transplantation in itself is associated with mortality and graft loss.

What are the Key Considerations When Managing Intestinal Failure?

Intestinal failure patients with congenital enterocyte defects, particularly those with microvillus inclusion disease, or those with intestinal pseudo-obstruction, will have very high requirements of fluid and electrolytes. Additional intravenous fluid and parenteral nutrition are often needed.

Nutrients and fluid requirements have to be tailored to the patient’s needs. In growing children, calorie needs will change over time and should be reviewed on a regular basis. Height, weight and hydration status have to be monitored and regular monitoring of blood and urine parameters is important to avoid inadequate supply. Home parenteral nutrition is considered in patients with prolonged or irreversible intestinal failure. Children likely to need parenteral nutrition for more than three months can be discharged on home parenteral nutrition as soon as they are clinically stable. This improves quality of life, encourages normal childhood development and family life, and is associated with less complications compared to remaining admitted in hospital.

Where applicable, medication and non-transplant surgical options should be considered. Management within a multidisciplinary team is essential for a favourable outcome and avoiding parenteral nutrition should be attempted whenever possible.

What are the Nutritional Complications in Intestinal Failure?

The nutritional complications of intestinal failure can be a consequence of inadequate or excessive supply of micro-and macronutrients. Inadequate calorie supply can lead to poor growth and weight gain in children.

However, an excess of glucose should be avoided due to the risk of hyperglycaemia and the associated increased lipogenesis and fat tissue deposition in the liver. While intravenous lipid emulsions should be part of a parenteral nutrition regime used for children, an excess can again contribute to the development of liver disease. Protein is needed for growth but an excess puts a negative strain on the kidneys.

Appropriate amounts of calcium, vitamin D, phosphate and magnesium are needed for optimal growth and bone mineralisation. Metabolic bone disease is a known complication of intestinal failure and fractures have been reported in these patients.

Patients who have lost a significant amount of weight prior to the start of parenteral nutrition are at risk of developing potentially life-threatening refeeding syndrome, which is why management by an experienced nutrition care team is always advised.

Parenteral Nutrition

What is Parenteral Nutrition and When Should it be Recommended?

Parenteral nutrition is a nutritional formulation that is administered intravenously and which contains all necessary macronutrients (amino acids, carbohydrates and lipids) and micronutrients (electrolytes, trace elements and vitamins). Parenteral nutrition is formulated according to the individual needs of the patient and can either provide the total or a partial amount of their nutritional intake.

Parenteral nutrition should be administered on a patient when they are not able to function effectively or grow through enteral nutrition. This can either be caused by enteral malabsorption or a severe intolerance to enteral nutrition.

What are the Key Considerations for the Management of Parenteral Nutrition?

Each patient will require a tailored approach, considering the type and position of central venous access, as well as fluid, electrolyte, micro and macronutrient needs. It is very important that complications are avoided and they should be actively and regularly screened for. Removing the need for parenteral nutrition and introducing oral feeding should be considered as soon as possible.

Where patients have prolonged (three months-plus) or irreversible intestinal failure, and they are stable, home parenteral nutrition should be considered. Parents and patients should be trained for one-to-two weeks in the hospital to administer the parenteral nutrition and take care of the CVC. A care plan should also be introduced for intestinal rehabilitation with the aim of achieving partial or complete enteral autonomy. The social situation of each patient also requires attention and they should be offered psychological support.

It is well-known that a multidisciplinary team approach is associated with improved outcomes and an early referral to an intestinal failure rehabilitation unit should be considered.

What are the Benefits of Parenteral Nutrition?

Modern parenteral nutrition has significantly improved the survival of patients with intestinal failure and reduced the complication rates. It now allows both adults and children suffering from intestinal failure to achieve a good quality of life. It means that patients with prolonged or irreversible failure can be safely discharged home to enjoy family life, school attendance, and normal work and daytime activities.

Are there any Disadvantages of Parenteral Nutrition?

Parenteral nutrition is very time-consuming, especially for a patient on home parenteral nutrition who needs to dedicate a significant amount of time to catheter care, connection and disconnection. In addition, patients will need to visit their outpatient departments for ongoing monitoring.

Complications can occur and include CVC-related complications, sepsis, liver damage and osteoporosis. This burden can be increased by acute complications, such as catheter-related bloods stream infections, which can lead to recurrent hospitalisation, be life-threatening, and lead to long-term issues such as liver disease or loss of central venous access. Parenteral nutrition is also an expensive treatment which poses a significant financial cost to healthcare systems.

What Does the Future Hold for Parenteral Nutrition?

The management of parenteral nutrition is improving all the time as we develop our understanding of the complications associated with it, and prevent these from occurring. This is aided by the development of novel drug therapies which are expected to contribute to gut adaptation, resulting in fewer patients needing parenteral nutrition support and, in some cases, any support at all. Further improvements, such as modern lipid emulsions, heightened longevity of central venous catheters and greater multidisciplinary team working, have also had a favourable impact on the outcomes of intestinal failure.