Procysbi (cysteamine bitartrate)
Last reviewed · Source: First Databank (FDB) drug data
Overview
Cysteamine bitartrate is a cystine-depleting agent.
Cysteamine bitartrate is a cystine-depleting agent that lowers the cellular cystine content in patients with nephropathic cystinosis, an inherited defect of lysosomal transport. The resulting accumulation of cystine and formation of cystine crystals within the lysosomes can damage various organs, including the kidneys, bone marrow, thyroid, pancreas, intestine, liver, spleen, muscle, brain, and eyes. Cysteamine reacts with cystine within lysosomes to convert it to cysteine and cysteine-cysteamine mixed disulfides, both of which can pass through the lysosomal membrane in patients with cystinosis, resulting in cystine depletion from the cell and a reduction of cystine accumulation.
USES: This medication is used to help preserve kidney function and manage kidney damage and other problems in people with an inherited disorder (nephropathic cystinosis) that causes buildup of a certain natural substance (cystine) in the body. Cystine buildup can cause problems such as kidney problems, slow growth, weak bones, and eye problems. Cysteamine helps the body get rid of cystine.
Generic name: cysteamine bitartrate
Pronunciation: (sis-TEE-a-meen)
Reference product & biosimilars
No reference, biosimilar or interchangeable associated with this drug.
Today’s intelligence on Procysbi
Indications
The following indications are listed for Procysbi:
- Nephropathic Cystinosis
Also known as: Early-Onset Cystinosis.
Dosing
Dosage of cysteamine bitartrate is expressed in terms of cysteamine.
Initiate cysteamine therapy promptly once the diagnosis of nephropathic cystinosis is confirmed.
Cysteamine bitartrate is administered orally. The drug is commercially available as immediate-release capsules or delayed-release capsules or oral granules.
No dosing information is available for this tab.
No dosing information is available for this tab.
Drug interactions
Cysteamine bitartrate does not inhibit cytochrome P-450 (CYP) isoenzymes 1A2, 2A6, 2B6, 2C8, 2C9, 2C19, 2D6, 2E1, or 3A4.
There are no contraindication interactions for this drug.
There are no severe interactions for this drug.
There are no moderate interactions for this drug.
Contraindications
*Hypersensitivity to cysteamine or penicillamine.
There are no contraindication contraindications for this drug.
There are no severe contraindications for this drug.
There are no moderate contraindications for this drug.
Adverse reactions
The most common adverse effects with immediate-release cysteamine (>5%) were vomiting, anorexia, fever, diarrhea, lethargy, and rash.
Common adverse effects with delayed-release cysteamine in previously-treated patients >=6 years of age (>=5%): vomiting, nausea, abdominal pain, breath odor, diarrhea, skin odor, fatigue, rash, and headache.
Common adverse effects with delayed-release cysteamine in treatment-naive patients 1 year to <6 years of age (>10%): vomiting, gastroenteritis/viral gastroenteritis, diarrhea, breath odor, nausea, electrolyte imbalance, and headache.
There are no severe adverse reactions for this drug.
There are no less severe adverse reactions for this drug.
Precautions
No enhanced messaging available.
The safety and efficacy of cysteamine bitartrate in pediatric patients have been established, and the drug is approved for use in such patients. The safety and effectiveness of delayed-release cysteamine have not been established in patients younger than 1 year of age.
There are no available data on cysteamine use in pregnant women to inform any drug-associated risks for birth defects or miscarriage. Cysteamine (administered as cysteamine bitartrate) was teratogenic and fetotoxic in rats at doses less than the recommended human maintenance dose.
It is not known whether oral cysteamine is distributed into human milk or whether the drug has any effects on the breast-fed infant or on milk production. Because of the potential for serious adverse reactions to cysteamine in nursing infants, breastfeeding is not recommended.
No studies with cysteamine have been conducted in geriatric patients.
ICD codes
No ICD codes are available for this drug.
Formulary status
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Last reviewed · Source: First Databank (FDB) drug data
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