Zollinger-Ellison Syndrome : Treatment and Management

This rare condition affects just one in a million people. Most common in men between the ages of 30 and 50, Zollinger-Ellison Syndrome causes the digestive system to produce excessive amounts of gastric acid. Let’s take a look at this uncommon disease.

WHAT IS ZOLLINGER-ELLISON SYNDROME?

Also called ‘ZES’ or ‘Z-E Syndrome,’ Zollinger-Ellison is a disorder of the digestive system. Normally, the body releases a hormone called gastrin whenever you eat. This hormone triggers the stomach to produce gastric acid, which helps break down food so the body can process it for fuel.

In ZES, small tumors called gastrinomas trigger the body to produce excessive amounts of gastric acid. This strong stomach acid can erode the tissues lining the stomach and small intestine, causing ulcers.

WHAT CAUSES ZOLLINGER-ELLISON SYNDROME?

ZES is caused by tumors called gastrinomas that occur in the pancreas and/or small intestine. There may be just one tumor or multiple tumors. Up to two-thirds of gastrinomas are cancerous.

A genetic disorder called multiple endocrine neoplasia type I (MEN-I) causes up to 30% of gastrinomas in people with ZES. MEN-I is inherited from your parents and often causes tumors in other areas of the body, including the pituitary gland.

You may be at higher risk of developing Zollinger-Ellison Syndrome  if you have a family history of MEN-I. If you are diagnosed with ZES, you and your direct blood relatives should be evaluated for MEN-I.

Both men and women can be diagnosed with ZES, though it is more common in men than in women.

SYMPTOMS OF ZOLLINGER-ELLISON SYNDROME

The symptoms of ZES are very similar to those of a peptic ulcer. If you have ZES, you may experience:

  • Dull, burning pain in the upper parts of the digestive tract
  • Dull, burning pain when the stomach is empty
  • Pain that is relieved by eating
  • Episodes of ulcer-like pain that come and go
  • Diarrhea
  • Acid reflux
  • Frequent belching
  • Nausea or vomiting

TREATMENT OF ZOLLINGER-ELLISON SYNDROME

Because it is so rare, ZES often isn’t diagnosed right away. Your healthcare provider may suspect ZES if you are being treated for a ‘peptic ulcer’ that doesn’t get better. Your provider may perform an endoscopy exam, in which a thin flexible tube is inserted down your throat, to look for the characteristic gastrinoma tumors of Zollinger-Ellison Syndrome. If you are diagnosed with ZES, you may need surgery to remove the gastrinomas. This is especially true if the tumors are cancerous. You may also receive treatment with antacid medications called proton pump inhibitors. These medications are widely available over the counter. There is no cure for Zollinger-Ellison Syndrome and no way to prevent it. You may need to take antacid medication for the rest of your life.

LIVING WITH ZOLLINGER-ELLSION SYNDROME

If you receive a diagnosis of Zollinger-Ellison syndrome, you can honestly say you are “one in a million.” The gastrinoma tumors of this condition are slow-growing, which means they may not be a threat to your life. Your healthcare provider may direct you to clinical trials that will help researchers learn more about this very rare disorder.