Proper Use

Drug information provided by: Merative, Micromedex®

Take this medicine exactly as directed by your doctor. Do not take more of it, do not take it more often, and do not take it for a longer time than your doctor ordered. To do so may increase the chance of side effects.

This medicine comes with a patient information leaflet. Read and follow these instructions carefully. Ask your doctor if you have any questions.

Swallow Opfolda™ capsule whole with unsweetened drinks (eg, water, tea, or coffee with no cream, sugar, or sweeteners). Do not eat or drink for at least 2 hours before and 2 hours after taking this medicine.

Take Opfolda™ capsule 1 hour before receiving Pombiliti™ injection.

Take Zavesca® capsule at the same time each day.

Your doctor may recommend changes to your diet to help with some side effects. It is important that you follow these changes.


The dose of this medicine will be different for different patients. Follow your doctor's orders or the directions on the label. The following information includes only the average doses of this medicine. If your dose is different, do not change it unless your doctor tells you to do so.

The amount of medicine that you take depends on the strength of the medicine. Also, the number of doses you take each day, the time allowed between doses, and the length of time you take the medicine depend on the medical problem for which you are using the medicine.

  • For oral dosage form (capsules):
    • For Pompe disease:
      • Adults weighing 50 kilograms (kg) or more—260 milligrams (mg) every other week.
      • Adults weighing 40 to less than 50 kg—195 mg every other week.
      • Children—Use and dose must be determined by your doctor.
    • For type 1 Gaucher's disease:
      • Adults—100 milligrams (mg) three times a day. Your doctor may adjust your dose as needed.
      • Children—Use and dose must be determined by your doctor.

Missed Dose

If you miss a dose of this medicine, skip the missed dose and go back to your regular dosing schedule. Do not double doses.

If you miss a dose of Opfolda™ capsule, you should not receive Pombiliti™ injection. If you miss this medicine in combination with Pombiliti™ injection, restart treatment as soon as possible.


Store the medicine in a closed container at room temperature, away from heat, moisture, and direct light. Keep from freezing.

Keep out of the reach of children.

Do not keep outdated medicine or medicine no longer needed.

Ask your healthcare professional how you should dispose of any medicine you do not use.