cysteamine

What is the most important information I should know about cysteamine?

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 is cysteamine?

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.

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

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

What should I discuss with my healthcare provider before taking cysteamine?

Tell your doctor if you have ever had:

• 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.

How should I use cysteamine?

Follow all directions on your prescription label and read all medication guides or instruction sheets. Your doctor may occasionally change your dose. Use the medicine exactly as directed.

If you cannot swallow a capsule whole, carefully follow the patient instructions for opening the capsule and mixing the medicine with certain foods. Your doctor may recommend that you use cysteamine oral granules.

Do not crush or chew oral granules.

Procysbi works best if you take it without food, at least 30 minutes before or 2 hours after a meal. If you cannot take the medicine without eating, eat only a small amount (1/2 cup) of food within 1 hour before or 1 hour after you take the medicine.

Whether you take the medicine with or without food, take it the same way each time.

• Empty the entire contents of the granule packet or the open capsule into 1/2 cup of applesauce, berry jelly, water, or fruit juice (but not grapefruit juice).
• Swallow the mixture without chewing, and use it within 30 minutes after mixing. Do not save it for later use.
• Avoid eating high-fat foods within 1 hour before or 1 hour after you take Procysbi.

Procysbi also comes with instructions for giving the mixture through a gastronomy tube (14 French or larger).

Cysteamine doses are based on weight (especially in children and teenagers). Your dose needs may change if you gain or lose weight.

You will need frequent medical tests.

Cystinosis is often treated with a combination of drugs, including vitamin and mineral supplements. Use all medications as directed and read all medication guides you receive. Do not change your dose or dosing schedule without your doctor's advice.

What happens if I miss a dose?

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: If you are less than 8 hours late for the dose, use the medicine as soon as you can. Skip the missed dose if your next dose is due in less than 4 hours.

Do not use two doses at one time.

What happens if I overdose?

What should I avoid while taking cysteamine?

What are the possible side effects of cysteamine?

• 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 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.

What other drugs will affect cysteamine?

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

Other drugs may affect 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.

Where can I get more information?

Your pharmacist can provide more information about cysteamine.

Remember, keep this and all other medicines out of the reach of children, never share your medicines with others, and use this medication only for the indication prescribed.

Every effort has been made to ensure that the information provided by Cerner Multum, Inc. ('Multum') is accurate, up-to-date, and complete, but no guarantee is made to that effect. Drug information contained herein may be time sensitive. Multum information has been compiled for use by healthcare practitioners and consumers in the United States and therefore Multum does not warrant that uses outside of the United States are appropriate, unless specifically indicated otherwise. Multum's drug information does not endorse drugs, diagnose patients or recommend therapy. Multum's drug information is an informational resource designed to assist licensed healthcare practitioners in caring for their patients and/or to serve consumers viewing this service as a supplement to, and not a substitute for, the expertise, skill, knowledge and judgment of healthcare practitioners. The absence of a warning for a given drug or drug combination in no way should be construed to indicate that the drug or drug combination is safe, effective or appropriate for any given patient. Multum does not assume any responsibility for any aspect of healthcare administered with the aid of information Multum provides. The information contained herein is not intended to cover all possible uses, directions, precautions, warnings, drug interactions, allergic reactions, or adverse effects. If you have questions about the drugs you are taking, check with your doctor, nurse or pharmacist.

Copyright 1996-2023 Cerner Multum, Inc. Version: 6.01. Revision date: 3/3/2020.