Procysbi

Pregnancy Category: C

Lactation: excretion in milk unknown/not recommended

Pregnancy Categories

A:Generally acceptable. Controlled studies in pregnant women show no evidence of fetal risk.

B:May be acceptable. Either animal studies show no risk but human studies not available or animal studies showed minor risks and human studies done and showed no risk.

C:Use with caution if benefits outweigh risks. Animal studies show risk and human studies not available or neither animal nor human studies done.

D:Use in LIFE-THREATENING emergencies when no safer drug available. Positive evidence of human fetal risk.

X:Do not use in pregnancy. Risks involved outweigh potential benefits. Safer alternatives exist.

NA:Information not available.

PROCYSBI, for oral administration, is a cystine-depleting agent that lowers the cystine content of cells in patients with nephropathic cystinosis, an inherited defect of lysosomal transport.

PROCYSBI contains the bitartrate salt of cysteamine. The chemical name for cysteamine bitartrate is ethanethiol, 2-amino, (2R,3R)-2,3-dihydroxybutanedioate (1:1) (salt). Cysteamine bitartrate is a highly water soluble white powder with a molecular weight of 227.24 and the molecular formula C2H7NS • C4H6O6. It has the following chemical structure:

Each 25 mg delayed-release capsule contains 74 mg cysteamine bitartrate, equivalent to 25 mg cysteamine. Each 75 mg delayed-release capsule contains 221 mg cysteamine bitartrate, equivalent to 75 mg cysteamine. PROCYSBI contains the following inactive ingredients: microcrystalline cellulose, Eudragit® L 30 D-55, hypromellose, talc, triethyl citrate, sodium lauryl sulfate, and purified water. Capsule shell ingredients are gelatin, titanium dioxide, blue ink and white ink.

Included as part of the "PRECAUTIONS" Section

Follow all directions on your prescription label. Your doctor may occasionally change your dose to make sure you get the best results. Do not take this medicine in larger or smaller amounts or for longer than recommended.

For a child younger than 6 years old taking Cystagon, the capsule should not be swallowed whole or choking could result. To make it easier to swallow the Cystagon capsule: Open the capsule and sprinkle the medicine into a small amount of applesauce, apple juice, or orange juice.

Take Procysbi with fruit juice, but not grapefruit juice.

You may take Procysbi with or without food, but take it the same way each time. To take Procysbi with food, eat only a small amount (1/2 cup) of food within 1 hour before or 1 hour after you take the medicine. To take Procysbi without food, take the medicine at least 30 minutes before or 2 hours after a meal.

Do not crush or chew a Procysbi capsule or the medicine inside it. Swallow the capsule whole.

If you cannot swallow a capsule whole, follow the patient instructions for mixing the medicine with certain foods. Food mixing instructions may be different from one brand of cysteamine to another. Carefully follow the directions for the specific brand you are using.

• After mixing cysteamine with food or liquid, swallow it without chewing. Take the medicine within 30 minutes after mixing. Do not save the mixture for later use.
• Procysbi also comes with instructions for giving the mixture through a gastronomy tube (12 French or larger). Follow these instructions very carefully.
• You may also ask your doctor or pharmacist about the types of foods to mix with cysteamine, and how to give the mixture for best results.

While using cysteamine, you may need frequent blood tests. Your skin and your bones may also need to be checked.

Cystinosis is often treated with a combination of drugs, including vitamin and mineral supplements. Use all medications as directed by your doctor. Read the medication guide or patient instructions provided with each medicine. Do not change your doses or medication schedule without your doctor's advice.

You should not stop using cysteamine without your doctor's advice. Stopping suddenly may make your condition worse.

Store at room temperature away from moisture, heat, and light.

Seek emergency medical attention or call the Poison Help line at 1-800-222-1222.

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.

Procysbi is a prescription medication used to treat cystinosis of the kidneys in adults and children 6 years and older. 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.

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.

Seek emergency medical attention or call the Poison Help line at 1-800-222-1222.

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 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.

• Store at room temperature.
• Protect from light.
• Store in a dry place. Do not store in a bathroom.
• Keep all drugs in a safe place. Keep all drugs out of the reach of children and pets.
• Check with your pharmacist about how to throw out unused drugs.

Take the missed dose as soon as you remember.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.

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