Please wait while the formulary information is being retrieved.
DRUG IMAGES
PROCYSBI DR 25 MG CAPSULE
PROCYSBI DR 75 MG CAPSULE
The following indications for PROCYSBI (cysteamine bitartrate) have been approved by the FDA:
Indications:
Nephropathic cystinosis
Professional Synonyms:
Early-onset cystinosis
Indications:
Nephropathic cystinosis
Professional Synonyms:
Early-onset cystinosis
The following dosing information is available for PROCYSBI (cysteamine bitartrate):
Dosage of cysteamine bitartrate is expressed in terms of cysteamine.
Initiate cysteamine therapy promptly once the diagnosis of nephropathic cystinosis is confirmed.
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 | DOSING INSTRUCTIONS |
|---|---|---|
| PROCYSBI DR 25 MG CAPSULE | Maintenance | Adults take 650 mg/m2 by oral route every 12 hours 1/2 hour before a meal or 2 hours after a meal |
| PROCYSBI DR 75 MG CAPSULE | Maintenance | Adults take 650 mg/m2 by oral route every 12 hours 1/2 hour before a meal or 2 hours after a meal |
| PROCYSBI DR 75 MG GRANULE PKT | Maintenance | Adults take 650 mg/m2 by oral route every 12 hours 1/2 hour before a meal or 2 hours after a meal |
| PROCYSBI DR 300 MG GRANULE PKT | Maintenance | Adults take 650 mg/m2 by oral route every 12 hours 1/2 hour before a meal or 2 hours after a meal |
No generic dosing information available.
The following drug interaction information is available for PROCYSBI (cysteamine bitartrate):
There are 0 contraindications.
There are 1 severe interactions.
These drug interactions can produce serious consequences in most patients. Actions required for severe interactions include, but are not limited to, discontinuing one or both agents, adjusting dosage, altering administration scheduling, and providing additional patient monitoring. Review the full interaction monograph for more information.
| Drug Interaction | Drug Names |
|---|---|
| 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) |
EFFER-K, GAVILYTE-C, GAVILYTE-G, GAVILYTE-N, GOLYTELY, KLOR-CON-EF, KONVOMEP, OMEPRAZOLE-SODIUM BICARBONATE, PEG 3350-ELECTROLYTE, PEG-3350 AND ELECTROLYTES, Q-CARE RX, SODIUM BICARBONATE, VAXCHORA BUFFER COMPONENT |
There are 0 moderate interactions.
The following contraindication information is available for PROCYSBI (cysteamine bitartrate):
Drug contraindication overview.
*Hypersensitivity to cysteamine or penicillamine.
*Hypersensitivity to cysteamine or penicillamine.
There are 0 contraindications.
There are 1 severe contraindications.
Adequate patient monitoring is recommended for safer drug use.
| Severe List |
|---|
| Pregnancy |
There are 9 moderate contraindications.
Clinically significant contraindication, where the condition can be managed or treated before the drug may be given safely.
| Moderate List |
|---|
| Depression |
| Fracture |
| Gastrointestinal hemorrhage |
| Gastrointestinal ulcer |
| Idiopathic intracranial hypertension |
| Increased alkaline phosphatase |
| Leukopenia |
| Osteopenia |
| Seizure disorder |
The following adverse reaction information is available for PROCYSBI (cysteamine bitartrate):
Adverse reaction overview.
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.
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 38 severe adverse reactions.
| More Frequent | Less Frequent |
|---|---|
|
Dehydration Diarrhea Fever Gastroenteritis Headache disorder Influenza Lethargy Nausea Skin rash Vomiting |
Acute cognitive impairment Depression Dizziness Sore throat Tremor |
| Rare/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 |
There are 16 less severe adverse reactions.
| More Frequent | Less Frequent |
|---|---|
|
Acute abdominal pain Anorexia Arthralgia Body odor Conjunctivitis Cough Fatigue Halitosis Infection of ear Pain in extremities Pharyngitis Upper respiratory infection |
Constipation |
| Rare/Very Rare |
|---|
|
Drowsy Leg pain Skin atrophy |
The following precautions are available for PROCYSBI (cysteamine bitartrate):
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.
Contraindicated
Severe Precaution
Management or Monitoring Precaution
Contraindicated
| None |
Severe Precaution
| None |
Management or Monitoring Precaution
| None |
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.
The following prioritized warning is available for PROCYSBI (cysteamine bitartrate):
No warning message for this drug.
No warning message for this drug.
The following icd codes are available for PROCYSBI (cysteamine bitartrate)'s list of indications:
| Nephropathic cystinosis | |
| E72.04 | Cystinosis |
Formulary Reference Tool