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Procysbi

Procysbi is a delayedrelease prescription medication used for the management of nephropathic cystinosis in adults and pediatric patients 1 year of age and older. This drug helps to control the amount of a certain amino acid called cystine in the cells of your body, preventing it from building up to harmful levels. The common side effects include fever, vomiting, diarrhea, and tiredness. Your doctor will always ensure that the benefits of taking Procysbi outweigh these potential side effects.

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Frequently Asked Questions

Can I eat highfat foods while taking Procysbi?

Avoid consuming highfat meals or snacks as they can reduce the absorption of Procysbi.

Can I take Procysbi if I am pregnant or breastfeeding?

Consult your healthcare provider before using Procysbi if you are pregnant, planning to become pregnant, or breastfeeding.

What happens if I miss a dose of Procysbi?

If you miss a dose, take it as soon as you can. If it is close to your next dose, skip the missed dose. Do not take two doses at the same time or extra doses.

What should I avoid while taking Procysbi?

Limit time in the sunlight. Wear protective clothing and use sunscreen. Avoid highfat meals and snacks. Limit your caffeine and alcohol intake.

Comprehensive Drug Guide

How Does Procysbi Work?

Procysbi is a cystinedepleting agent that lowers the cystine content of cells in nephropathic cystinosis patients, bringing it closer to normal levels.

What is Procysbi Used For?

FDA indications: Procysbi is indicated for the treatment of nephropathic cystinosis in adults and pediatric patients 1 year of age and older.

Offlabel uses: Though it's not usual, in rare cases it might be used for other conditions related to cystine buildup.

Common Dosages

Capsule: 25mg, 75mg

Oral granule: 75mg, 300mg

Typical Dosing

FDA Approved Indications

The recommended initial dose is 1 gram per meter square body area per day, to be taken in two divided doses 12 hours apart. This can later be increased by your healthcare provider.
The maximum daily dose is 1.95 grams per meter square body area and should not exceed 2 grams per day.

Who Shouldn't Take Procysbi?

People who have had an allergic reaction to cysteamine or penicillamine.
Women who are pregnant, planning to become pregnant, or breastfeeding should consult their healthcare providers first.

Advice From The Pharmacist

Always take Procysbi with fruit juice (except grapefruit juice) or water.
Do not crush or chew the capsules. If you have trouble swallowing the capsules whole, talk to your healthcare provider.
Don't skip doses as it might lead to a buildup of cystine in cells.
Tell your healthcare provider about all the other medications you take, including prescription and overthecounter medicines, vitamins, and herbal supplements.
Remember, Procysbi does not cure cystinosis. Its purpose is to manage the symptoms, so it's important to take it regularly and exactly as prescribed.

Side Effects of Procysbi

Common Side Effects

Taking Procysbi might cause you to have stomach pain or discomfort.
You might experience nausea or vomiting.
Headaches or dizziness could occur.
You could experience skin rash or itching.
It might cause joint pain.

Uncommon/Severe Side Effects

Uncommon side effects:
You could feel unusually weak or tired.
You might experience a loss of appetite.
Your skin and eyes might turn a yellowish color (jaundice).
Your breathing might become shallow or labored.
Rare side effects:
You might have seizures or convulsions.
You could become unusually thirsty, showcasing symptoms of diabetes.
Your body might develop an allergic reaction to the drug which could lead to swelling of your face, lips, tongue, or throat.
Severe side effects:
If you notice darkcolored urine or lightcolored stools, seek immediate medical attention.
If you experience acute abdominal pain, especially if it's accompanied by nausea, vomiting, or fever, get to a doctor immediately.
If your skin or the whites of your eyes turn yellow, rush to get medical help.
Symptoms like rash, itching, severe dizziness, or trouble breathing could indicate a serious allergic reaction and need immediate medical help.

Risks and Warnings of Procysbi

Procysbi may increase the risk of skin, bone, and joint infections. It's important to maintain regular hygiene.
If you're on Procysbi treatment, avoid exposure to sunlight and use sunscreen while outdoors.
Patients with cystinosis are usually low on folic acid, which could lead to anemia. Your healthcare provider will monitor your blood count regularly and may suggest dietary supplements as necessary.

Interactions with Procysbi

Common Drug Interactions

Antiepileptic drugs (such as phenytoin, carbamazepine)
Medicines that can affect your liver or blood count.
Proton pump inhibitors (such as omeprazole, lansoprazole)

Alternatives to Procysbi

Cystagon (Cysteamine Bitartrate)