Cystagon

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.

Follow all directions on your medicine label and package. Tell each of your healthcare providers about all your medical conditions, allergies, and all medicines you use.

You should not use this medicine if you are allergic to cysteamine or penicillamine.

To make sure cysteamine is safe for you, tell your doctor if you have:

• any type of skin rash;
• bone problems (including fractures);
• epilepsy or other seizure disorder;
• liver disease;
• a history of low white blood cell (WBC) counts;
• a history of depression, drowsiness, or nervous system disorder; or
• a history of stomach ulcer, or bleeding in your stomach or intestines.

It is not known whether cysteamine will harm an unborn baby. Tell your doctor if you are pregnant or plan to become pregnant.

It is not known whether cysteamine passes into breast milk or if it could harm a nursing baby. You should not breast-feed while using this medicine.

Procysbi should not be given to a child younger than 2 years old without a doctor's advice.

>10%

Vomiting (35%)

Anorexia (31%)

Diarrhea (16%)

Fever (11%)

Lethargy (11%)

1-10%

Rash (7%)

Frequency Not Defined

Seizures

Ataxia

Encephalophathy

Somnolence

Depression

Nightmare

Impaired cognition

Hypertension

Urticaria

Bad breath

Cystagon is a prescription medication used to treat cystinosis of the kidneys. Cystinosis is a rare condition that causes cystine, a protein building block, to build up in the cells of the body.

This medication may be prescribed for other uses. Ask your doctor or pharmacist for more information.

Take Cystagon exactly as prescribed. Do not increase or decrease the amount of medicine without your doctor's approval.

• Cystagon is available as a capsule and is usually taken four times a day. 
• If you or your child misses a dose of medicine, it should be taken as soon as possible. If it is within two hours of the next dose, skip the missed dose and go back to the regular dosing schedule. Do not double dose.
• Do not give intact Cystagon capsules to children under six years of age. They may not be able to swallow them and may choke. For children under six years of age, the capsule may be opened and the contents sprinkled on food. Talk to your doctor for complete directions.
• Your doctor will arrange for regular blood tests to be done to measure the amount of cystine inside white blood cells. This will help to determine the correct dose of Cystagon. Regular blood and urine tests will also be done to measure the levels of the body's important electrolytes. This will help your doctor to correctly adjust the doses of these supplements.
• Your doctor may also want to do certain tests to find out if unwanted effects are occurring. The tests are very important because serious side effects, including ulcers or bleeding in the stomach and intestines (digestive tract), can occur.

This section provides information on the proper use of a number of products that contain cysteamine. It may not be specific to Cystagon. Please read with care.

Take this medicine 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.

This medicine 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 this medicine.

• 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 this medicine 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 this medicine 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 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.

• 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 this medicine, 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.

Along with its needed effects, a medicine 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:

• 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 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

Other side effects not listed may also occur in some patients. If you notice any other effects, check with your healthcare professional.

Call your doctor for medical advice about side effects. You may report side effects to the FDA at 1-800-FDA-1088.

Use Cystagon as ordered by your doctor. Read all information given to you. Follow all instructions closely.

• To gain the most benefit, do not miss doses.
• Keep taking this medicine as you have been told by your doctor or other health care provider, even if you feel well.
• Swallow whole. Do not chew or crush.
• You may sprinkle contents of capsule on soft food or liquid. Do not chew.

What do I do if I miss a dose?

• Take a missed dose as soon as you think about it.
• If it is less than 2 hours until your next dose, skip the missed dose and go back to your normal time.
• Do not take 2 doses at the same time or extra doses.

• If your symptoms or health problems do not get better or if they become worse, call your doctor.
• Do not share your drugs with others and do not take anyone else's drugs.
• Keep a list of all your drugs (prescription, natural products, vitamins, OTC) with you. Give this list to your doctor.
• Talk with the doctor before starting any new drug, including prescription or OTC, natural products, or vitamins.
• Some drugs may have another patient information leaflet. Check with your pharmacist. If you have any questions about this medicine, please talk with your doctor, nurse, pharmacist, or other health care provider.
• If you think there has been an overdose, call your poison control center or get medical care right away. Be ready to tell or show what was taken, how much, and when it happened.

This information should not be used to decide whether or not to take Cystagon or any other medicine. Only the healthcare provider has the knowledge and training to decide which medicines are right for a specific patient. This information does not endorse any medicine as safe, effective, or approved for treating any patient or health condition. This is only a brief summary of general information about this medicine. It does NOT include all information about the possible uses, directions, warnings, precautions, interactions, adverse effects, or risks that may apply to Cystagon (cysteamine capsules). This information is not specific medical advice and does not replace information you receive from the healthcare provider. You must talk with the healthcare provider for complete information about the risks and benefits of using this medicine.

Review Date: October 4, 2017

Cystagon® (cysteamine bitartrate) Capsules are hard gelatin capsules which provide 50 mg or 150 mg of cysteamine free base as cysteamine bitartrate:

Cystagon® Capsules, 50 mg are white, opaque capsules printed with CYSTA 50 on the body and MYLAN on the cap. They are available as follows:

NDC 0378-9040-05
bottles of 500 capsules

Cystagon® Capsules, 150 mg are white, opaque capsules printed with Cystagon 150 on the body and MYLAN on the cap. They are available as follows:

NDC 0378-9045-05
bottles of 500 capsules

Store at 20° to 25°C (68° to 77°F). [See USP for Controlled Room Temperature.]

Protect from light and moisture.

Dispense in a tight, light-resistant container as defined in the USP using a child-resistant closure.

PHARMACIST: Detach Patient Information Leaflet at each perforation and give leaflet to patient.

Mylan Pharmaceuticals Inc.
Morgantown, WV 26505

REVISED JULY 2007
CYST:R13p

The following information is available for patients and/or guardians in the patient leaflet.

Applies to cysteamine: oral capsule, oral delayed release capsule

General

The most common adverse events were vomiting, abdominal pain, nausea, anorexia, fever, diarrhea, lethargy, headache, and rash.[Ref]

Gastrointestinal

Very common (10% or more): Vomiting (35%), diarrhea (16%)
Common (1% to 10%): Nausea, bad breath, abdominal pain, dyspepsia
Uncommon (0.1% to 1%): Gastrointestinal ulceration and bleeding, constipation, gastroenteritis, duodenitis[Ref]

Metabolic

Very common (10% or more): Anorexia (31%)
Uncommon (0.1% to 1%): Dehydration[Ref]

Nervous system

Very common (10% or more): Lethargy (11%)
Common (1% to 10%): Dizziness, headache, encephalopathy
Uncommon (0.1% to 1%): Somnolence, seizures, convulsions, ataxia, confusion, tremor, hyperkinesia, jitteriness
Postmarketing reports: Benign intracranial hypertension with papilledema[Ref]

Other

Very common (10% or more): Fever (22%)
Common (1% to 10%): Fatigue, asthenia
Uncommon (0.1% to 1%): Decreasing hearing[Ref]

Dermatologic

Common (1% to 10%): Rash, skin odor
Uncommon (0.1% to 1%): Hair color changes, skin fragility (molluscoid pseudomotor on elbows)
Frequency not reported: Urticaria
Postmarketing reports: Skin lesions, skin striae, skin fragility[Ref]

Cases of Ehlers-Danlos like syndrome and vascular disorders on elbows have been reported in children chronically treated with high doses of different cysteamine preparations. In cases where histopathological examination of the skin was performed, the results suggested angioendotheliomatosis.[Ref]

Hepatic

Common (1% to 10%): Abnormal liver function
Frequency not reported: Elevated alkaline phosphatase[Ref]

Cardiovascular

Uncommon (0.1% to 1%): Hypertension[Ref]

Genitourinary

Uncommon (0.1% to 1%): Abnormal urine odor[Ref]

Hematologic

Uncommon (0.1% to 1%): Leukopenia, anemia[Ref]

Immunologic

Uncommon (0.1% to 1%): Anaphylactic reaction[Ref]

Musculoskeletal

Uncommon (0.1% to 1%): Joint hyperextension, leg pain, genu valgum, osteopenia, compression fracture, scoliosis
Postmarketing reports: Molluscoid pseudotumors[Ref]

Psychiatric

Uncommon (0.1% to 1%): Nervousness, abnormal thinking, depression, emotional lability, hallucinations, nightmares[Ref]

Renal

Uncommon (0.1% to 1%): Nephrotic syndrome, interstitial nephritis, renal failure[Ref]

Cases of nephrotic syndrome have been reported within 6 months of starting therapy with progressive recovery after treatment discontinuation. In some cases, histology showed a membranous glomerulonephritis of the renal allograft and hypersensitivity interstitial nephritis.[Ref]

Some side effects of Cystagon may not be reported. Always consult your doctor or healthcare specialist for medical advice. You may also report side effects to the FDA.