Cysteamine

Get emergency medical help if you have any of these signs of an allergic reaction: hives; difficult breathing; swelling of your face, lips, tongue, or throat.

Call your doctor at once if you have any of these side effects (some of these may be caused by your cystinosis disorder and not by this medicine):

• a seizure (convulsions);
• unusual bruising or streaks on the skin;
• the first sign of any skin rash, no matter how mild;
• bloody or tarry stools, coughing up blood or vomit that looks like coffee grounds;
• increased pressure inside the skull--severe headaches, ringing in your ears, dizziness, drowsiness, depressed mood, nausea, vision problems, pain behind your eyes;
• severe skin reaction--fever, sore throat, swelling in your face or tongue, burning in your eyes, skin pain, followed by a red or purple skin rash that spreads (especially in the face or upper body) and causes blistering and peeling.

Common side effects may include:

• stomach pain, nausea, vomiting, loss of appetite, diarrhea;
• unusual breath odor or skin odor;
• headache, dizziness;
• rash;
• fever; or
• tired feeling, lack of energy.

This is not a complete list of side effects and others may occur. Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.

If you take too much cysteamine call your healthcare provider or local Poison Control Center, or seek emergency medical attention right away.

If cysteamine is administered by a healthcare provider in a medical setting, it is unlikely that an overdose will occur. However, if overdose is suspected, seek emergency medical attention.

Cysteamine works by reducing the amount of cystine (an amino acid) in the body.

Cysteamine is used to treat nephropathic cystinosis (NEF-roe-PATH-ik SIS-tin-OH-sis), a rare genetic condition that causes a build-up of cystine in the kidneys and other organs. Too much cystine can cause kidney failure or other medical problems.

The Cystagon brand of this medicine is for use in adults and children. Procysbi is another brand of cysteamine that is for use in adults and children who are at least 2 years old.

Cysteamine may also be used for other purposes not listed in this medication guide.

Take the missed dose as soon as you remember.

• For Cystagon: Skip the missed dose if your next dose is due in less than 2 hours.

• For Procysbi: Skip the missed dose if your next dose is due in less than 4 hours.

Do not take extra medicine to make up the missed dose.

Get emergency medical help if you have any of these signs of an allergic reaction: hives; difficult breathing; swelling of your face, lips, tongue, or throat.

Call your doctor at once if you have any of these side effects (some of these may be caused by your cystinosis disorder and not by this medicine):

• a seizure (convulsions);

• unusual bruising or streaks on the skin;

• the first sign of any skin rash, no matter how mild;

• bloody or tarry stools, coughing up blood or vomit that looks like coffee grounds;

• increased pressure inside the skull--severe headaches, ringing in your ears, dizziness, drowsiness, depressed mood, nausea, vision problems, pain behind your eyes;

• severe skin reaction--fever, sore throat, swelling in your face or tongue, burning in your eyes, skin pain, followed by a red or purple skin rash that spreads (especially in the face or upper body) and causes blistering and peeling.

Common side effects may include:

• stomach pain, nausea, vomiting, loss of appetite, diarrhea;

• unusual breath odor or skin odor;

• headache, dizziness;

• rash;

• fever; or

• tired feeling, lack of energy.

This is not a complete list of side effects and others may occur. Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.

Usual Adult Dose for Nephropathic Cystinosis:

Immediate-release capsule:
Less than 110 lbs: Data not available
110 lbs or more:
-Initial dose: 0.3 to 0.5 g/day orally divided in 4 doses. Increase dose gradually over 4 to 6 weeks if the WBC cystine level remains above 2 nmol half-cystine/mg protein.
-Maintenance dose: 2 g/day orally divided in 4 doses

Delayed-release capsule:
-Initial dose: 0.2 to 0.3 g/m2/day orally divided in 2 doses, every 12 hours. Increase dose gradually over 4 to 6 weeks if the WBC cystine level remains above 1 nmol half-cystine/mg protein.
-Maintenance dose: 1.3 g/m2/day orally divided in 2 doses, every 12 hours
-Maximum dose: 1.95 g/m2/day

Comments:
-Measurements of WBC cystine level and/or cysteamine concentration taken one half hour after dose administration are recommended for new patients after the maintenance dose is achieved.
-The goal is to keep WBC cystine levels below 1 nmol half-cystine/mg protein or 2 nmol half-cystine/mg protein (in patients with poorer tolerability), five to six hours following drug administration.

Use: Management of nephropathic cystinosis

Usual Pediatric Dose for Nephropathic Cystinosis:

Immediate-release capsule:
Less than 12 years old:
-Initial dose: 0.2 to 0.3 g/m2/day orally divided in 4 doses. Increase dose gradually over 4 to 6 weeks.
-Maintenance dose: 1.3 g/m2/day orally divided in 4 doses
-Maximum dose: 1.95 g/m2/day

12 years or older:
Less than 110 lbs: Data not available
110 lbs or more:
-Initial dose: 0.3 to 0.5 g/day orally divided in 4 doses. Increase dose gradually over 4 to 6 weeks if the WBC cystine level remains above 2 nmol half-cystine/mg protein.
-Maintenance dose: 2 g/day orally divided in 4 doses

Delayed-release capsule:
Less than 6 years: Safety has not been established.
6 years and older:
-Initial dose: 0.2 to 0.3 g/m2/day orally divided in 2 doses, every 12 hours. Increase dose gradually over 4 to 6 weeks if the WBC cystine level remains above 1 nmol half-cystine/mg protein.
-Maintenance dose: 1.3 g/m2/day orally divided in 2 doses, every 12 hours
-Maximum dose: 1.95 g/m2/day

Comments:
-Measurements of WBC cystine level and/or cysteamine concentration taken one half hour after dose administration are recommended for new patients after the maintenance dose is achieved.
-The goal is to keep WBC cystine levels below 1 nmol half-cystine/mg protein or 2 nmol half-cystine/mg protein (in patients with poorer tolerability), five to six hours following drug administration.

Use: Management of nephropathic cystinosis

In deciding to use a medicine, the risks of taking the medicine must be weighed against the good it will do. This is a decision you and your doctor will make. For cysteamine, the following should be considered:

Allergies

Tell your doctor if you have ever had any unusual or allergic reaction to cysteamine or any other medicines. Also tell your health care professional if you have any other types of allergies, such as to foods, dyes, preservatives, or animals. For non-prescription products, read the label or package ingredients carefully.

Pediatric

Appropriate studies performed to date have not demonstrated pediatric-specific problems that would limit the usefulness of cysteamine in children 2 years of age and older. However, safety and efficacy have not been established in children younger than 2 years of age.

Geriatric

No information is available on the relationship of age to the effects of cysteamine in geriatric patients.

Breast Feeding

There are no adequate studies in women for determining infant risk when using this medication during breastfeeding. Weigh the potential benefits against the potential risks before taking this medication while breastfeeding.

Interactions with Medicines

Although certain medicines should not be used together at all, in other cases two different medicines may be used together even if an interaction might occur. In these cases, your doctor may want to change the dose, or other precautions may be necessary. Tell your healthcare professional if you are taking any other prescription or nonprescription (over-the-counter [OTC]) medicine.

Interactions with Food/Tobacco/Alcohol

Certain medicines should not be used at or around the time of eating food or eating certain types of food since interactions may occur. Using alcohol or tobacco with certain medicines may also cause interactions to occur. The following interactions have been selected on the basis of their potential significance and are not necessarily all-inclusive.

Using cysteamine with any of the following is usually not recommended, but may be unavoidable in some cases. If used together, your doctor may change the dose or how often you use cysteamine, or give you special instructions about the use of food, alcohol, or tobacco.

• Ethanol

Other Medical Problems

The presence of other medical problems may affect the use of cysteamine. Make sure you tell your doctor if you have any other medical problems, especially:

• Blood problems, or history of or
• Bone problems or
• Brain disease or
• Depression, history of or
• Head injury, history of or
• Increased pressure in the head or
• Liver disease or
• Seizures, history of or
• Stomach or bowel ulcers or bleeding—Use with caution. May make these conditions worse.

Take cysteamine only 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.

cysteamine comes with a patient information insert. Read and follow the instructions carefully. Ask your doctor if you have any questions.

If you vomit your dose of cysteamine within 20 minutes of taking it, take the dose again. However, if you vomit the dose a second time, do not repeat the dose but wait and take your next dose as scheduled. Also, if vomiting occurs more than 20 minutes after you take your dose, do not repeat the dose.

For children younger than 2 years of age, the capsule may be opened and the contents of the capsule sprinkled on food or mixed in formula.

Swallow the delayed-release capsule whole. Do not crush or chew the capsule or contents of the capsule.

Take the delayed-release capsule the same way every day. This means take it at the same time and take it consistently, either with or without food. High-fat meals may decrease absorption of cysteamine.

• Without food: Take it at least 30 minutes before meals or at least 2 hours after meals.
• With food: You can eat a small amount of food (½ cup) between 1 hour before or after taking Procysbi®.

If you cannot swallow the capsule whole, you may open it and take the capsule contents with certain foods and juices:

• Taking cysteamine with applesauce or berry jelly: Open the capsule and sprinkle the granules on 4 ounces (½ cup) of applesauce or berry jelly. Mix the granules into the food. Swallow the entire mixture within 30 minutes. Do not chew the granules. Do not save the applesauce or berry jelly and granules for later use.
• Taking cysteamine with fruit juice (except grapefruit juice): Open the capsule and sprinkle the granules into 4 ounces (½ cup) of juice. Gently stir until mixed. Drink it within 30 minutes. Do not chew the granules. Do not save the fruit juice or granules for later use.
• If you are using a gastrostomy tube: Open the capsule and sprinkle the granules on 4 ounces (½ cup) of applesauce. For children receiving 1 or 2 capsules, use a minimum of 1 ounce of applesauce. Use only strained applesauce with no chunks. Mix and give the medicine through the gastrostomy tube into the stomach within 30 minutes. Flush the tube with 8 ounces (1 cup) of fruit juice. Do not save the mixture for later use.

It is important that you follow any special instructions from your doctor, such as taking dietary supplements. These supplements will replace minerals lost through the kidneys.

If you are taking medicines containing bicarbonate or carbonate, take Procysbi® at least 1 hour before or after these medicines.

Dosing

The dose of cysteamine 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 cysteamine. 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.

• To prevent buildup of cystine crystals in the kidneys:
  • For oral dosage form (capsules):
    • Adults and teenagers—The starting dose must be determined by your doctor. Your doctor may gradually increase your dose.
    • Children—The starting dose is based on body size and must be determined by your doctor. Your doctor may gradually increase your dose.
  • For oral dosage form (delayed-release capsules):
    • For patients not on cysteamine therapy:
      • Adults— The starting dose is based on body size and must be determined by your doctor. The capsules are taken every 12 hours. Your doctor may adjust your dose as needed.
      • Children 2 years of age and older— The starting dose is based on body size and must be determined by your doctor. The capsules are taken every 12 hours. Your doctor may adjust your dose as needed.
      • Children younger than 2 years of age—Use and dose must be determined by your doctor.
    • For patients switching from cysteamine immediate-release capsules:
      • Adults—The total dose is the same as the total dose that you are currently taking.
      • Children 2 years of age and older—The total dose is the same as the total dose that you are currently taking.
      • Children younger than 2 years of age—Use and dose must be determined by your doctor.

Missed Dose

If you miss a dose of cysteamine, take it as soon as possible. However, if it is almost time for your next dose, skip the missed dose and go back to your regular dosing schedule. Do not double doses.

You may take a missed dose of Procysbi® as soon as possible up to 8 hours after your scheduled dose. If you missed a dose of Procysbi® and your next dose is less than 4 hours away, wait and take your next dose at the normal time. Do not take 2 doses at one time to make up for a missed dose

Storage

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.

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

Store the delayed-release capsules in its original container. Keep it in a dry place away from light.

Commonly reported side effects of cysteamine include: lethargy, vomiting, and anorexia. Other side effects include: skin rash. See below for a comprehensive list of adverse effects.

Applies to cysteamine: oral capsule, oral capsule delayed release

Along with its needed effects, cysteamine may cause some unwanted effects. Although not all of these side effects may occur, if they do occur they may need medical attention.

Check with your doctor immediately if any of the following side effects occur while taking cysteamine:

• Abdominal or stomach pain
• diarrhea
• drowsiness
• fever
• loss of appetite
• nausea or vomiting
• skin rash

• Confusion
• dizziness
• headache
• mental depression
• sore throat
• trembling

• Convulsions
• increased thirst
• unusual tiredness or weakness

• Blistering, peeling, or loosening of the skin
• blurred or double vision
• bone lesions
• bulging soft spot on head of an infant
• change in the ability to see colors, especially blue or yellow
• chills
• continuing ringing or buzzing or other unexplained noise in the ears
• cough
• eye pain
• hearing loss
• itching
• joint or muscle pain
• loss of appetite
• loss of vision
• pain behind the eyes
• pain in the legs
• pain with eye movement
• red skin lesions, often with a purple center
• red, irritated eyes
• reddish purple lines on the arms, face, legs, trunk, or groin
• severe headache
• sore throat
• sores, ulcers, or white spots in the mouth or on the lips

Some side effects of cysteamine may occur that usually do not need medical attention. These side effects may go away during treatment as your body adjusts to the medicine. Also, your health care professional may be able to tell you about ways to prevent or reduce some of these side effects. Check with your health care professional if any of the following side effects continue or are bothersome or if you have any questions about them:

• Breath odor
• constipation
• hives or welts
• redness of the skin
• unusual drowsiness, dullness, tiredness, weakness, or feeling of sluggishness
• weight loss