When This Medicine Should Not Be Used:
This medicine is not right for everyone. Do not use it if you had an allergic reaction to cysteamine or penicillamine.

How to Use This Medicine:
Capsule, Delayed Release Capsule

• Take your medicine as directed. Your dose may need to be changed several times to find what works best for you.
• Read and follow the patient instructions that come with this medicine. Talk to your doctor or pharmacist if you have any questions.
• Immediate-release capsule (Cystagon®):
  
  • If your child is using this medicine, you may open the immediate-release capsule and mix it in food.
• Delayed-release capsule (Procysbi®):
  
  • Swallow the capsule whole. Do not crush or chew the capsule or the contents.
  • Take this medicine the same way every day. This means take it at the same time and take it consistently, either with or without food. Do not eat high-fat food near the time that you take this medicine.
    
    • Without food: It is best to take this medicine on an empty stomach. Take it at least 30 minutes before a meal or at least 2 hours after a meal.
    • With food: If you need to eat, you may have a small amount of food (1/2 cup) between 1 hour before and 1 hour after you take this medicine.
  • If you cannot swallow the capsule whole, you may open it and mix the contents only with certain foods and fruit juices.
    
    • Applesauce or berry jelly: Mix the granules with 4 ounces (1/2 cup) of applesauce or berry jelly. Swallow the entire mixture within 30 minutes. Do not chew the granules.
    • Fruit juice (except grapefruit juice): Add the granules to 4 ounces (1/2 cup) of fruit juice. Gently stir to mix. Drink the mixture within 30 minutes. Do not chew the granules.
    • Feeding tube: Mix the granules with 4 ounces (1/2 cup) of strained applesauce. Give the mixture within 30 minutes. Then flush the tube with at least 10 milliliters (10 mL) of fruit juice. Do not use other liquids to flush the tube.
  • Missed dose:
    
    • Immediate-release (Cystagon®): If it is more than 2 hours until your next dose, take the missed dose as soon as you can. If it is less than 2 hours, skip the missed dose and take your normal dose at the regular time. Do not take extra medicine to make up for the missed dose.
    • Delayed-release (Procysbi®): If it is more than 4 hours until your next dose, take the missed dose as soon as you can. If it is less than 4 hours, skip the missed dose and take your normal dose at the regular time. Do not take extra medicine to make up for the missed dose.
  • Store the medicine in a closed container at room temperature, away from heat, moisture, and direct light. Store the delayed-release capsules in the original container. Keep it in a dry place away from light.

Drugs and Foods to Avoid:
Ask your doctor or pharmacist before using any other medicine, including over-the-counter medicines, vitamins, and herbal products.

• Take Procysbi® at least 1 hour before or after you take medicines that contain bicarbonate or carbonate.
• Do not drink alcohol while you are using this medicine.

Warnings While Using This Medicine:

• Tell your doctor if you are pregnant or breastfeeding, or if you have liver disease, stomach or bowel problems, or a history of head injury, depression, or seizures.
• This medicine may cause the following problems:
  
  • Skin, joint, or bone problems
  • Stomach or bowel problems, including ulcers or bleeding
  • Benign intracranial hypertension (increased pressure inside your head)
• This medicine may make you dizzy or drowsy. Do not drive or doing anything else that could be dangerous until you know how this medicine affects you.
• Your doctor will do lab tests at regular visits to check on the effects of this medicine. Keep all appointments.
• Keep all medicine out of the reach of children. Never share your medicine with anyone.

Possible Side Effects While Using This Medicine:
Call your doctor right away if you notice any of these side effects:

• Allergic reaction: Itching or hives, swelling in your face or hands, swelling or tingling in your mouth or throat, chest tightness, trouble breathing
• Blistering, peeling, or red skin rash
• Bloody vomit or black, tarry stools
• Bone, joint, or leg pain
• Depression, severe sleepiness, seizures
• Fever, chills, cough, sore throat, body aches
• Skin changes, easy bruising, red or purple lines on your skin
• Severe headache, vision problems, ringing or buzzing in your ears
• Severe stomach pain, nausea, vomiting, loss of appetite

If you notice these less serious side effects, talk with your doctor:

• Headache
• Mild nausea, vomiting, diarrhea, stomach pain or upset
• Tiredness

If you notice other side effects that you think are caused by this medicine, tell your doctor.
Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088