OVERVIEW

Short bowel syndrome with intestinal failure (SBS-IF) is a serious and debilitating condition caused by congenital or acquired loss of bowel.1  The leading cause of IF in childhood is SBS.2

In paediatric patients, SBS-IF is characterised by a small bowel length <25% the expected length for their age group, and by the presence of intestinal failure.1–3  This results in a reduced gut function below the minimum necessary for the absorption of nutrients and fluids needed for adequate growth and health. Paediatric patients are dependent on parenteral nutrition (PN) for growth and survival.2,3–6

CAUSES OF PAEDIATRIC SBS-IF

In paediatric patients, SBS-IF mostly occurs as a result of congenital conditions requiring extensive intestinal resection, or those leading to intestinal malformation and a severely reduced small bowel length. Most common causes of SBS-IF in paediatric patients are:1–3

  • Necrotising enterocolitis
  • Gastroschisis
  • Midgut volvulus
  • Intestinal atresia

SYMPTOMS

Paediatric patients can experience malabsorption and other debilitating symptoms and outcomes such as:1–3

  • Malnutrition
  • Diarrhoea
  • Dehydration
  • Impaired growth
  • Fluid and electrolyte imbalance
  • Death

Malabsorption of vital nutrients, vitamins and minerals may affect the patient’s ability to gain weight, grow and develop normally, which may be fatal if left untreated.2

MANAGEMENT OF SBS-IF

Similarly to adults with SBS-IF, paediatric patients are treated with parenteral support (PS; parenteral nutrition and/or intravenous fluid [PN/IV]) which provides the necessary nutritional and fluid requirements needed to sustain life and support growth.1, 2

In comparison to adults, children are more likely to wean off PS; however, approximately 27% may remain PS dependent for the entirety of their lives.2

Paediatric patients can undergo intestinal adaptation, a compensatory response which increases absorptive surface area and improves absorption via numerous processes including increasing villus height and crypt depth. Whilst some paediatric patients achieve intestinal adaption naturally, others are not able to adapt on their own and may require an intestinal transplant.3,4

Click here for more information about disease management

IMPACT ON QUALITY OF LIFE

The management of SBS-IF is a challenge for both the patient and their family. The quality of life of the parents/caregivers may be significantly impacted due to the burden placed upon them.1,2

Managing the treatment of a child with SBS-IF can cause parents/caregivers to experience stress, frustration and difficulty sleeping.1 Children can also be affected as they can find it challenging to keep up with schoolwork, socialise with friends or fully thrive physically and psychologically.2,3

Transitioning from parenteral nutrition (PN) being administered in hospital to PN at home significantly improves quality of life, however, home parenteral nutrition (HPN) can be time-consuming and an intrusive procedure which interferes with daily life.2

 

MANAGING SBS-IF AFFECTS VARIOUS ASPECTS OF LIFE:1

FAMILY LIFE

WORK

SOCIAL LIFE

PS can be a time-consuming procedure as the patient may be connected intravenously for up to 10-12 hours overnight.1–3

 

POTENTIAL COMPLICATIONS

PS is a life-saving therapy, however, its long-term use in paediatric patients may be associated with serious complications such as infections, sepsis, thrombosis, kidney dysfunction and liver failure.1–5

Click here for a list of most common parenteral support complications and limitations.

Intestinal failure-associated liver disease (IFALD) is one of the most common and severe complications associated with the long-term use of PS in paediatric patients. An estimated 25-50% of patients could be affected.1–3

The development of liver and/or other life-threatening complications may lead to paediatric patients requiring intestinal transplantation, which may be associated with high risks and low long-term survival rates.2,6

The prognosis for children with SBS-IF has improved significantly in recent years; however, they still face substantial mortality with 1 in 10 children dying within 5 years.1,2,7

TREATMENT GOALS

The primary goal of medical and surgical treatment of SBS-IF is to enhance the absorptive capacity of the remnant bowel through intestinal adaptation, to ultimately achieve enteral autonomy.1

Emphasis needs to be placed on minimising the impact of the disease and its complications and treatments to improve QoL and the psychosocial and emotional needs of the patient and families in the future.2

There is a need for increased awareness and education around SBS-IF and for improved access to multidisciplinary centres with the appropriate level of SBS-IF expertise in order to ensure patients have the best opportunity to achieve their treatment goals.3–6

Date of preparation: December 2020 C-ANPROM/INT/REV/0006