Short bowel syndrome: causes, parenteral nutrition and treatment

The small bowel is where most of the nutrients, fluids and salts from food and drink are absorbed. A Cardiff and Vale University Health Board page explains that when the bowel is shortened there is less surface area and less time to absorb food, and over time this may leave a person dehydrated and malnourished. That state is short bowel syndrome.

NICE describes it as reduced absorption of nutrients, water and electrolytes. When the length of remaining intestine means the intestine's function drops below the level needed for absorption, this is called intestinal failure. NICE says intestinal failure is classified as type 3 when it is chronic and a person needs parenteral support over months or years.

The Cardiff page says short bowel syndrome occurs after extensive surgery that removes most, if not all, of the small intestine, and that parenteral support may be needed to keep a person hydrated and nourished.

NICE says short bowel syndrome is commonly caused by surgery needed to remove abnormal bowel. In adults the operation may be needed for conditions such as mesenteric ischaemia (poor blood supply to the bowel), Crohn's disease and radiation enteritis.

In children, NICE says it is often because of necrotising enterocolitis in premature babies, or conditions such as volvulus (twisting of the bowel) or gastroschisis. Some children are born with a short bowel.

The sources used here describe the main effects of poor absorption in general terms: dehydration and malnutrition, and, for many people, a need for nutrition and fluid support given into a vein.

NICE highlights that the condition is not only burdensome for the person but for their carers too, especially because of the need for parenteral support, which can limit family, work and social life. Patient experts told the NICE committee that many families have not had a night off from giving parenteral support in many years.

After surgery the remaining bowel can adapt. NICE says there is an adaptation phase estimated at up to 2 years in adults, which can be longer in children. During this period the amount of parenteral support needed may change. Teduglutide, in NICE's recommendation, is used once a person's condition is stable following a period of intestinal adaptation.

NICE describes current treatment as parenteral support together with best supportive care. Best supportive care includes antimotility and antisecretory medicines, fluid restriction and diet changes. Parenteral support gives nutrients and fluids intravenously, for an average of 10 to 14 hours a day for between 2 and 7 days a week according to NICE, and most people have it at home using a permanent intravenous tube.

NICE is clear that while parenteral support is life-saving, it is time-consuming, complex and its complications can be life-threatening, including blood infections, blood clots, and kidney and liver failure. It also reports that supply issues have affected parenteral support, so some people have had to adapt their care.

Diet and fluid advice depends on how much bowel remains and whether you have a stoma, so it is set by your dietitian. As an example, a Newcastle Hospitals leaflet written for people with short bowel syndrome and high losses explains that high output from the bowel or stoma, which it defines as more than 1500 ml a day, raises the risk of dehydration through water and salt loss, and that when large amounts of small bowel are removed you may not be able to stay hydrated by drinking alone.

For that group the leaflet suggests drinking about 1000 ml a day of a glucose and salt solution such as Dioralyte, restricting other fluids to around 1000 ml, and drinking between meals rather than with them. It advises a low fibre diet to reduce stoma losses, with white bread, rice, pasta and peeled potatoes suggested and fruit, nuts, seeds, raw vegetables and vegetable skins avoided, and notes that caffeinated drinks may increase stoma output. Medicines such as loperamide, codeine phosphate and a proton pump inhibitor may be prescribed to reduce output, and supplements such as magnesium, calcium and vitamin D, iron, vitamin B12 injections and multivitamins may be needed.

The leaflet stresses checking volumes with your clinical team and discussing diet with the nutrition team or dietitian. Cardiff and Vale University Health Board explains why: with a shortened bowel there is less surface area and less time to absorb food, which over time can leave you dehydrated and malnourished.

NICE guidance TA804 recommends teduglutide (Revestive) as an option for treating short bowel syndrome in people aged 1 year and above, provided the company supplies it according to the commercial arrangement. The condition should be stable following a period of intestinal adaptation after surgery.

NICE explains that clinical trial evidence shows teduglutide reduces the number of days a week people need parenteral support compared with placebo, although how much it reduces this is uncertain. Clinical experts told the committee that it could allow some people to wean off parenteral support completely or reduce the number of nights per week it is needed, and that this would greatly increase quality of life for both people and carers.

NICE's public information leaflet suggests questions to ask your team: how well it works compared with other treatments, what the risks or side effects are, how it will affect day-to-day life, and what happens if it does not work.

For many people, living with short bowel syndrome means having parenteral support at home, either giving it themselves or with a carer's help. NICE says patient experts described the responsibility of doing complicated procedures at home as potentially overwhelming and emphasised the need for treatments that offer more normality.

NICE's public information lists support organisations: PINNT and Short Bowel Survivor and Friends. Your local Healthwatch can also help. Because NICE lists blood infections and blood clots among the risks of parenteral support, it is sensible to ask your team which symptoms need same-day advice and who to contact out of hours.

NICE lists blood infections, blood clots and kidney and liver failure among the complications of parenteral support, which can be life-threatening. Contact your own nutrition or intestinal failure team for advice about symptoms such as fever, a problem with your feeding line, signs of dehydration or unexpected weight loss, and use the out-of-hours number you were given. For an emergency, call 999 or go to A&E.

The Newcastle leaflet centres on the risk of dehydration from high losses, so it is sensible to tell your team promptly if your output rises or you cannot keep up with fluids.

NICE says teduglutide is available on the NHS as a possible treatment for people aged 1 and above, and its guidance refers to NHS practice for parenteral support. NICE describes parenteral support as highly complex, so it is sensible to ask which specialist team takes responsibility for it.

Some people seek a private second opinion on surgery, parenteral nutrition or medicines. Whatever the route, it helps to be clear which team prescribes and monitors parenteral support, who to call out of hours, and how any private advice will be shared with the NHS team that provides the long-term service.