Procysbi

Generic name: Cysteamine
Drug class: Miscellaneous uncategorized agents

Usage of Procysbi

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

Procysbi 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.

Procysbi is for use in adults and children, but Procysbi should not be given to a child younger than 1 year old.

Procysbi side effects

Get emergency medical help if you have signs of an allergic reaction to Procysbi (hives, difficult breathing, swelling in your face or throat) or a severe skin reaction (fever, sore throat, burning eyes, skin pain, red or purple skin rash with blistering and peeling).

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):

depressed mood, extreme drowsiness;

a seizure;

unusual bruising or streaks on the skin;

bone pain, abnormal joint movement;

an electrolyte imbalance - increased thirst or urination, confusion, constipation, leg cramps, irregular heartbeats, tingly feeling;

increased pressure inside the skull - severe headaches, ringing in your ears, dizziness, vision problems, pain behind your eyes; or

stomach problems - pain, nausea, vomiting, loss of appetite, coughing up blood or vomit that looks like coffee grounds.

Common Procysbi side effects may include:

drowsiness, feeling tired;

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

unusual breath odor or skin odor;

rash;

fever; or

headache.

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.

Before taking Procysbi

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

To make sure Procysbi 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;

low white blood cell (WBC) counts;

depression, drowsiness, or nervous system disorder; or

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.

You should not breastfeed while using Procysbi.

Relate drugs

How to use Procysbi

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.

Warnings

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.

What other drugs will affect Procysbi

Take Procysbi at least 1 hour before or 1 hour after taking any medicine that contains bicarbonate or carbonate.

Other drugs may interact with cysteamine, including prescription and over-the-counter medicines, vitamins, and herbal products. Tell your doctor about all your current medicines and any medicine you start or stop using.

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