What is EPS?

EPS is an uncommon but serious condition in the abdomen that can severely affect the bowel, so that it becomes partially or even completely obstructed (blocked). Most people who suffer with EPS have had peritoneal dialysis (PD) treatment for several years. The inside of the abdomen is lined with a membrane called the peritoneum. It covers the inside wall of the abdomen and most of the organs inside the abdominal cavity including the bowel. In a healthy person, the peritoneum is very thin and allows the loops of bowel to slide around easily as food passes through. In many patients who have had peritoneal dialysis, this membrane becomes thickened over time. This need not cause any problems, but if the membrane gets very thick and fibrous (sclerosed), it can restrict the bowel and stop it moving easily. Eventually this tightens so much that the bowel can no longer move freely.  When this happens, food can no longer pass through the bowel and it becomes blocked which causes pain, nausea, vomiting and weight loss.

What causes EPS?

The exact cause of EPS is not yet known and there are some current studies to investigate why and how the condition develops. We do know that there are some risk factors that make EPS more likely to develop.

  • Length of time on peritoneal dialysis (PD). It is very uncommon for someone to develop EPS if they have been on PD for less than 2 years. Most people have been on PD for more than 5 years.
  • Severe peritonitis caused by bacterial or fungal infections.
  • The use of a lot of strong glucose dialysis bags whilst on PD may contribute.
  • Many patients seem to develop EPS shortly after stopping peritoneal dialysis. This could be because they have swapped to haemodialysis or because they have had a kidney transplant.

However there are many patients who do not develop EPS even though they have been on peritoneal dialysis for many years, have used strong glucose dialysis bags and have had episodes of peritonitis. It is not known why some people experience severe problems with EPS whilst others do not.

What are the symptoms of EPS?

When the bowel becomes encased in the thickened layer of fibrous tissue, food that has been eaten cannot easily pass through the gut and can get stuck, causing a blockage in the small bowel.

The build up of food causes vomiting and this can make it very difficult to eat enough, so patients often experience dramatic weight loss and consequently can become very weak. Patients may experience watery diarrhoea as some liquid may be able to get past the blockage, but more solid food cannot. This maybe accompanied by bouts of abdominal pain.

A special kind of x-ray called a CT scan (computed tomography) of the abdomen helps the doctors decide if the symptoms vomiting, weight loss and pain could be caused by EPS. Before this scan, patients are asked to drink a liquid called Gastrograffin that highlights the bowel on the scan and they are also given an injection in the scanning department. They will be asked to lie in the scanner which moves them back and forth to take the scans. The scan images can help the doctors see if there are any blockages in the bowel and if the bowel has become trapped. Sometimes it shows that the bowel wall has become thickened. It is also very common to find fluid collections in the abdomen that would not normally be there; these collections may contain several litres of fluid and can make the patient feel very bloated.

To summarise, the symptoms and signs of EPS can include:

  • Nausea and vomiting
  • Weight loss
  • Weakness
  • Pain in the abdomen
  • Distended abdomen
  • CT scan showing a blockage in the bowel or collections of fluid in the abdomen

How serious is EPS - is the surgery risky?

The operation involves carefully removing the fibrotic membrane encasing the bowel. The aim of this is to allow the bowel to move more freely, allowing food to pass through again. The surgery can take a long time, typically between 4 – 8 hours, but much longer on occasions. EPS is a serious condition which needs treatment – without treatment up to 4 in 10 (40%) patients might die from it. The surgical treatment for EPS is not without risk and we estimate that 1 in 10 patients may die shortly after surgery as a consequence of the treatment and its complications; although this depends on how fit the patient was before surgery.

What could happen if EPS is not treated?

Once someone has developed EPS and has the diseased membrane covering the bowel, it will not go away without surgical treatment. Symptoms are more severe for some people than others so that many patients will need hospital treatment and surgery fairly immediately, whilst others can remain at home and try to manage their symptoms by modifying their diet until they can come into hospital. In many instances, if EPS is untreated then the condition can result in death. Even with an operation some people will die, but 7/10 people who have had surgery are well one year later.

If lot of fluid accumulates in the abdomen because of the EPS, then this could need draining. Sometimes it can be difficult to breathe deeply and this fluid can even accumulate around the lungs. Even if it is possible to drain the fluid, it may come back unless the EPS is surgically treated.

If EPS is not treated surgically there is a danger of bowel perforation. Should this happen then the patient will need emergency surgery and will need to have a stoma made. Recovery after this situation can take a lot longer and patients are much more likely to have severe complications and infection.

 

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