Procysbi (Cysteamine)

Description

Procysbi (cysteamine bitartrate) is used to treat nephropathic cystinosis in adults and children 1 year of age and older. People with cystinosis have a high level of cystine (an amino acid-related substance) inside cells, especially in lysosomes (cell parts). Procysbi helps reduce these levels by converting cystine into forms that can exit lysosomes.

Directions

After you buy Procysbi (Cysteamine) with a valid prescription from your doctor, your dose depends on several factors, including body size, weight, and cystine levels in your white blood cells. This prescription medication is typically taken twice daily, every 12 hours.

Patients who take cysteamine for the first time usually start with a lower dose, which is gradually increased. Because Procysbi is a delayed-release medicine, patients switching from immediate-release cysteamine typically start with the same total daily dose they were taking before, split into two doses taken 12 hours apart.

Procysbi is available in two formulations: delayed-release capsules and oral granules.

Capsules:

Swallow capsules whole unless you have difficulty swallowing. In this case, the label allows opening them and using the contents as directed. For example, you may sprinkle the medication over apple sauce, berry jelly, or fruit juice (except grapefruit juice).

Granules:

Do not crush or chew. Use the granules as directed in the instructions for use and sprinkle them over apple sauce, berry jelly, or fruit juice (except grapefruit juice).

If you miss a dose of Procysbi, take it as soon as you remember, unless your next dose is less than 4 hours away. If so, skip the missed dose and take your next dose at your regular time. Do not take 2 doses at once.

Ingredients

The active ingredient in Procysbi is cysteamine.

Cautions

The following cautions apply to both Procysbi delayed-release capsules and granules:

• This medication may cause skin, bone, and joint changes. Your provider may monitor you for related issues during treatment.
• Notify your doctor immediately if you develop a significant rash, as Procysbi can cause severe skin reactions.
• Watch for symptoms of intestinal ulcers or bleeding. Your provider may need to adjust your dose if you experience severe side effects.
• If you experience severe or worsening stomach pain, vomiting, bloody or persistent diarrhea, or trouble controlling bowel movements, immediately contact your doctor. You may be experiencing a serious bowel condition called fibrosing colonopathy.
• This medication has been associated with symptoms of the central nervous system, such as seizures, unusual sleepiness, lethargy, depression, or confusion. Do not drive or operate machinery if Procysbi makes you feel impaired.
• Your doctor may monitor you for low white blood cell counts and/or elevated alkaline phosphatase before and during treatment. You may need a treatment adjustment if the results stay abnormal.
• Contact your doctor right away if you have a headache, dizziness, ringing in the ears, nausea, double or blurry vision, vision loss, or eye pain. These symptoms may stem from increased brain pressure. You may also need an eye exam.
• Tell your provider about any other medications you take. Some drug interactions can increase stomach pH, while others can affect how Procysbi releases medication.
• Avoid alcoholic beverages while taking Procysbi.
• Animal data show harm when Procysbi is taken during pregnancy. While there is not enough human data available, you should talk to your clinician if you are pregnant or planning a pregnancy.
• Do not breastfeed while taking Procysbi.

Contraindications

Do not take Procysbi if you have had a serious allergic reaction to penicillamine or cysteamine.

Side Effects

Relatively common but often manageable side effects of Procysbi include vomiting, nausea, diarrhea, stomach pain, and bad breath.

More serious side effects and risks include stomach and intestinal ulcers and bleeding, fibrosing colonopathy, increased brain pressure, serious symptoms affecting the central nervous system, and skin, bone, or joint problems (Ehlers-Danlos–like syndrome).

You may experience additional Procysbi side effects. Contact your doctor if you notice any persistent or concerning side effects from Procysbi, or if you have any additional questions about Procysbi’s cost, coverage, and more.

Buy Procysbi today at a low cost!

References

• PROCYSBI (cysteamine bitartrate) Prescribing Information. Deerfield, IL: Horizon Therapeutics USA, Inc.; 2022.
• PROCYSBI (cysteamine bitartrate) Prescribing Information. Lake Forest, IL: Horizon Therapeutics USA, Inc.; 2020.

Product Code : 15506