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Do You Have Zollinger-Ellison Syndrome (ZES)?

Zollinger-Ellison syndrome - stomach image

by Derek Matlock, PharmD

What is Zollinger-Ellison Syndrome?

Do you suffer from Zollinger-Ellison syndrome (ZES)? If so, you may be in rare company. According to the National Institute of Diabetes and Digestive and Kidney Diseases, Zollinger-Ellison syndrome is a rare disorder. It occurs in about one in every 1 million people. Normally, when we eat, our body releases a hormone called gastrin, which tells your stomach to make acid to help break down foods and liquids. For patients with ZES, this mechanism is disrupted by tumors or “gastrinomas.” These tumors form in the pancreas or upper small intestine and secrete abnormally large amounts of gastrin from tumors, resulting in peptic ulcers to be formed.

It Might Be Your Genes

Some people with Zollinger-Ellison syndrome may go undiagnosed as the disorder is rare and its cause is not clear. In 75% of cases, ZES is sporadic or random, whereas in 25% it is associated with MEN 1, an inherited condition characterized by pancreatic endocrine tumors, pituitary tumors, and hyperparathyroidism.  Therefore, your doctor may perform a thorough medical and family history to help diagnose ZES. Additional tests may include endoscopy or various imaging and blood tests. They may even measure the amount of acid in your stomach. For patients with sporadic ZES, the most common symptom is abdominal pain. While patients with the inherited form of ZES mostly complain of diarrhea. Other symptoms include, heartburn, nausea, vomiting, stomach bleeding, and weight loss.

Managing Zollinger-Ellison Syndrome

Currently, the goal of managing ZES is to limit complications of the disorder by suppressing acid secretions. Thus, the main medications used in ZES are proton pump inhibitors, or PPIs, like omeprazole (Prilosec®) or pantoprazole (Protonix®), prescribed at high doses. For patients who do not respond to treatment with PPIs, octreotide is used, which stops the secretion of gastrin, the hormone that tells our body to secrete acid for food breakdown. Currently, the only cure for ZES is surgical removal of the tumor or tumors, but this may not be an option in cases where the tumors have spread to other parts of the body. In that case, chemotherapy with medications like streptozotocin, 5-fluorouracil, and doxorubicin are used to shrink tumors.

Zollinger-Ellison syndrome is a rare disorder that may be suspected in patients with multiple or repeat peptic ulcers. Currently, medications like proton pump inhibitors are the main treatment option, while surgery and chemotherapy are options in certain patients. Remember, when taking proton pump inhibitors, they are best taken 30-60 minutes before a meal and may also come with their own unfavorable side effects. Be sure to talk to your doctor or pharmacist about what can be done to best optimize your treatment options for ZES.

Resources:

  1. Medscape: Zollinger-Ellison Syndrome
  2. National Institute of Diabetes and Digestive and Kidney Diseases: Zollinger-Ellison Syndrome
  3. UpToDate: Management and Prognosis of the Zollinger-Ellison Syndrome

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