Home Drug Guide Cysteamine Bitartrate

Drug Guide

Generic Name

Cysteamine Bitartrate

Brand Names Cystagon, Procysbi

Classification

Therapeutic: Antirheumatic, Mucolytic

Pharmacological: Cysteamine analog, free thiol agent

FDA Approved Indications

  • Cystinosis (a rare inherited lysosomal storage disorder)

Mechanism of Action

Cysteamine reduces cystine levels inside lysosomes by forming cysteine-cysteamine disulfides that can exit the lysosome, thereby decreasing cystine accumulation.

Dosage and Administration

Adult: Start at 1.3 to 1.95 grams/m2/day divided into 2-4 doses; titrate based on response and tolerability.

Pediatric: Dosing based on body surface area; adjustment necessary for renal impairment.

Geriatric: Use with caution; closely monitor renal function.

Renal Impairment: Adjust dose based on renal function, with close monitoring.

Hepatic Impairment: Data limited; use cautiously and monitor for adverse effects.

Pharmacokinetics

Absorption: Rapid gastrointestinal absorption.

Distribution: Widely distributed in body tissues.

Metabolism: Metabolized in the liver and tissues to form metabolites.

Excretion: Primarily excreted via urine.

Half Life: Approximately 3 hours.

Contraindications

  • Hypersensitivity to cysteamine or any component of the formulation.

Precautions

  • Monitoring for nephrolithiasis, bone marrow suppression, and nephrotoxicity. Use with caution in patients with liver disease. Regular blood tests required. Consider pregnancy and lactation; risk-benefit ratio needed.

Adverse Reactions - Common

  • Gastrointestinal symptoms (nausea, vomiting, diarrhea) (Common)
  • Odor of body and urine due to cysteamine (Common)

Adverse Reactions - Serious

  • Nephrotoxicity (Rare, but requires monitoring)
  • Bone marrow suppression (neutropenia, thrombocytopenia) (Rare)
  • Allergic reactions (rash, anaphylaxis) (Rare)

Drug-Drug Interactions

  • Amifostine may increase risk of neutropenia.
  • Other nephrotoxic drugs may increase risk of renal impairment.

Drug-Food Interactions

N/A

Drug-Herb Interactions

N/A

Nursing Implications

Assessment: Monitor renal function, blood cell counts, and for signs of nephrolithiasis.

Diagnoses:

  • Risk for infection related to neutropenia.
  • Risk for renal impairment.

Implementation: Administer doses as prescribed, monitor laboratory values, counsel on hydration.

Evaluation: Assess for reduction in cystine levels, monitor for adverse effects, ensure compliance.

Patient/Family Teaching

  • Take medication exactly as prescribed.
  • Report any signs of kidney problems, blood disorders, or allergic reactions.
  • Maintain adequate hydration.
  • Understand the importance of regular blood tests.

Special Considerations

Black Box Warnings:

  • Potential for nephrotoxicity and myelosuppression requiring careful monitoring.

Genetic Factors: Consider genetic counseling for families with cystinosis.

Lab Test Interference: May interfere with certain laboratory tests; inform lab personnel.

Overdose Management

Signs/Symptoms: Nausea, vomiting, diarrhea, hypotension, hypersensitivity reactions.

Treatment: Supportive care, monitor vital signs, renal function, blood counts, and provide symptomatic treatment.

Storage and Handling

Storage: Store at room temperature, away from moisture and light.

Stability: Stable as per manufacturer guidelines for the duration specified.

🛡️ 5 Critical Medication Safety Tips for Nurses

1

Triple-Check High-Risk Medications

Always have another nurse verify insulin, heparin, warfarin, and chemotherapy drugs. These "high-alert" medications cause the most serious errors. Check concentration, dose calculation, and pump settings twice.

2

Know Look-Alike, Sound-Alike Drugs

Common mix-ups: hydromorphone/morphine, Celebrex/Celexa, Zyprexa/Zyrtec. Always use BOTH generic and brand names, read labels twice, and use barcode scanning when available. One wrong letter can be fatal.

3

Assess Before AND After Giving Meds

Check vitals before cardiac meds, pain levels before analgesics, and blood glucose before insulin. Always reassess within 30 minutes to evaluate effectiveness and watch for adverse reactions.

4

Watch for Drug Interactions

Common dangerous combinations: warfarin + aspirin (bleeding), ACE inhibitors + potassium (hyperkalemia), digoxin + diuretics (toxicity). Always check drug interactions before administering new medications.

5

Educate Your Patients

Teach patients medication names, purposes, major side effects, and what to report. Informed patients catch errors and improve compliance. Always encourage questions - an educated patient is a safer patient.

⚡ Remember: When in doubt, don't give it out! It's always safer to double-check than regret later.

⚠️ Medical Disclaimer

This drug guide is for educational purposes only and is NOT intended for clinical use. Always consult current prescribing information, healthcare providers, and institutional protocols before administering any medication. Do not use this information for patient care decisions.