Procysbi (cysteamine bitartrate)
Oral Cystinosis Therapy, Cystine Depleting Agents Oral Capsule, Delayed Release Sprinkle; Oral Granules, Delayed Release, In Packet
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 that causes build-up of a certain natural substance (cystine) in the body (nephropathic cystinosis). Cystine build-up 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)
Drug class: Oral Cystinosis Therapy, Cystine Depleting Agents
Therapeutic class: Genitourinary Therapy
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.
| Drug label | Dosing type | Instructions |
|---|---|---|
| Procysbi Dr 25 Mg Capsule | Maintenance | Take 650 mg/m2 by oral route every 12 hours 1/2 hour before a meal or2 hours after a meal. |
| Procysbi Dr 75 Mg Capsule | Maintenance | Take 650 mg/m2 by oral route every 12 hours 1/2 hour before a meal or2 hours after a meal. |
| Procysbi Dr 75 Mg Granule Pkt | Maintenance | Take 650 mg/m2 by oral route every 12 hours 1/2 hour before a meal or2 hours after a meal. |
| Procysbi Dr 300 Mg Granule Pkt | Maintenance | Take 650 mg/m2 by oral route every 12 hours 1/2 hour before a meal or2 hours after a meal. |
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.
Severe Interaction: Action is required to reduce the risk of severe adverse interaction.
Delayed-Release Cysteamine Bitartrate/Bicarbonate
SEVERITY LEVEL: 2-Severe Interaction: Action is required to reduce the risk of severe adverse interaction.
MECHANISM OF ACTION: Bicarbonate increases gastric pH. As gastric pH increases, the solubility of delayed-release cysteamine bitartrate may increase.(1) The exact mechanism behind the interaction between delayed-release cysteamine bitartrate and bicarbonate is unknown.
CLINICAL EFFECTS: Simultaneous administration of delayed-release cysteamine bitartrate and bicarbonate may result in an early release of cysteamine bitartrate and decreased effectiveness of cysteamine bitartrate.(1)
PREDISPOSING FACTORS: None determined.
PATIENT MANAGEMENT: The manufacturer of delayed-release cysteamine bitartrate states that delayed-release cysteamine bitartrate should be administered at least 1 hour before or 1 hour after administration of bicarbonate.(3)
DISCUSSION: Interactions between delayed-release cysteamine bitartrate and other acid reducing agents including H2-receptor antagonists and proton pump inhibitors were not noted in the clinical trials.(1,2) The interaction was only documented with co-administration of delayed-release cysteamine bitartrate and bicarbonate.(2,3)
There are no moderate interactions for this drug.
Contraindications
*Hypersensitivity to cysteamine or penicillamine.
There are no contraindication contraindications for this drug.
- Pregnancy
- Depression
- Fracture
- Gastrointestinal hemorrhage
- Gastrointestinal ulcer
- Idiopathic intracranial hypertension
- Increased alkaline phosphatase
- Leukopenia
- Osteopenia
- Seizure disorder
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.
More Frequent
- Dehydration
- Diarrhea
- Fever
- Gastroenteritis
- Headache disorder
- Influenza
- Lethargy
- Nausea
- Skin rash
- Vomiting
Less Frequent
- Acute cognitive impairment
- Depression
- Dizziness
- Sore throat
- Tremor
Rare or Very Rare
- Acquired genu valgum
- Anemia
- Compression fracture
- Erythema multiforme
- Fibrosing colonopathy
- Gastrointestinal hemorrhage
- Gastrointestinal ulcer
- Hyperextension of joint
- Hypertension
- Hypocalcemia
- Hypokalemia
- Hypovolemia
- Idiopathic intracranial hypertension
- Leukopenia
- Ocular pain
- Osteopenia
- Papilledema
- Scoliosis
- Seizure disorder
- Skin lesion
- Skin striae
- Stevens-johnson syndrome
- Toxic epidermal necrolysis
More Frequent
- Acute abdominal pain
- Anorexia
- Arthralgia
- Body odor
- Conjunctivitis
- Cough
- Fatigue
- Halitosis
- Infection of ear
- Pain in extremities
- Pharyngitis
- Upper respiratory infection
Less Frequent
- Constipation
Rare or Very Rare
- Drowsy
- Leg pain
- Skin atrophy
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
| Code | Type | Description |
|---|---|---|
| Nephropathic cystinosis | ||
| E72.04 | ICD10CM | Cystinosis |
ICD code information from First Databank (FDB).
Formulary status
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Last reviewed · Source: First Databank (FDB) drug data
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