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Informing your clinical approach

A collection of case studies are available to provide you with examples of clinical approaches to manage and prevent cystine stone formation.

Case 1: Importance of early management of cystinuria

An adult male patient experienced multiple cystine stones and underwent several urologic procedures at an early age. Years later, initiating treatment with THIOLA® (tiopronin) tablets and conservative management contributed to minimized stone burden and no hospitalizations for more than 10 years.1,a

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Case 2: Management of complex and long-term recurrent cystine stones

A 42-year-old female patient had a long and complicated stone history which led to an eventual diagnosis of cystinuria. Years later, the utilization of 24-hour urine testing to monitor cystine levels and optimized dosing of THIOLA helped reduce cystine stone formation in this patient.1,a

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aIndividual patient response. Not all patients will respond similarly to the same medical treatment.

hcp-managing_facts-icon Guides

Resources on cystinuria management

Helpful printable materials to have at your fingertips—and one to share with your patients

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AUA guidelines review

A summary of evidence-based recommendations from the American Urological Association (AUA) on the medical management of cystine stones.

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3 steps to managing cystinuria

A management plan to address the continuous buildup of cystine in the urine and help patients with cystinuria get to stone-free.

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Dosing guide for THIOLA

How to start, monitor, and adjust THIOLA dosage to optimize treatment and help patients with cystinuria get to stone-free.

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Management guide for patients

A brochure outlining the goals of treatment, therapeutic lifestyle changes, and what to expect from THIOLA therapy. Share with your patients.

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References:  1. Goldfarb DS, Grasso M. Case studies in cystinuria. Urol Nurs. 2017;37(2):90-93. doi:10.7257/1053-816X.2017.37.2.90.

INDICATIONS: THIOLA® (tiopronin) tablets are indicated for the prevention of cystine (kidney) stone formation in patients with severe homozygous cystinuria with urinary cystine  >500 mg/day, who are resistant to treatment with conservative measures of high fluid intake, alkali and diet modification, or who have adverse reactions to d-penicillamine.

Important Safety Information 

CONTRAINDICATIONS: THIOLA is contraindicated during pregnancy (except where the benefits clearly outweigh the risks), in nursing mothers, and in patients who have previously developed agranulocytosis, aplastic anemia or thrombocytopenia while on this medication.

WARNINGS AND PRECAUTIONS:

  • While no deaths have been reported with THIOLA treatment, THIOLA can potentially cause all the serious adverse reactions reported for d-penicillamine, including death. 
  • Hematologic abnormalities requiring drug discontinuation may occur: inform patients to report promptly signs or symptoms of hematologic abnormalities.
  • Proteinuria, sometimes severe enough to cause nephrotic syndrome: monitor affected patients closely.
  • Discontinue THIOLA therapy if there are findings suggestive of Goodpasture’s syndrome, myasthenic syndrome, or myasthenia gravis. If pemphigus-type reactions occur, discontinue therapy and consider steroid treatment.
  • Inform patients about potential complications; advise them to promptly report any treatment-emergent signs or symptoms.
  • To reduce the risk of serious complications, the following tests should be conducted: 
    • Peripheral blood counts, direct platelet counts, hemoglobin, serum albumin, liver function tests, 24-hour urinary protein, routine urinalysis: 3-6 month intervals
    • Urinary cystine analysis: frequently during dose optimization, and at 6 month intervals thereafter
    • Abdominal roentgenogram: annually
  • In animal studies, THIOLA has been shown to cause fetal harm. THIOLA should only be used during pregnancy if the potential benefit justifies potential risk to the fetus.
  • THIOLA should not be used in nursing mothers.
  • The safety and efficacy of THIOLA in children under 9 years of age have not been established.

ADVERSE REACTIONS:

Adverse reactions associated with THIOLA include the following:

  • Drug fever during the first month
  • Generalized rash with pruritus
  • Lupus erythematous-like drug reaction (e.g., fever, arthralgia, lymphadenopathy, positive antinuclear antibody test)
  • Hypogeusia
  • Vitamin B6 deficiency (uncommon)
  • Wrinkling and friability of skin
  • Jaundice and abnormal liver function tests (in non-cystinuric conditions)

THIOLA is associated with fewer or less severe reactions than d-penicillamine, however the following adverse reactions may occur:  

  • Gastrointestinal (nausea, emesis, diarrhea, anorexia, abdominal pain, bloating, flatus)
  • Impairment in taste or smell
  • Dermatologic (pharyngitis, oral ulcers, rash, ecchymosis, pruritus, urticaria, warts, skin wrinkling, pemphigus, elastosis perforans serpiginosa)
  • Hypersensitivity reactions (laryngeal edema, dyspnea, respiratory distress, fever, chills, arthralgia, weakness, fatigue, myalgia, adenopathy)
  • Hematologic (increased bleeding, anemia, leukopenia, thrombocytopenia, eosinophilia)
  • Renal (proteinuria, nephrotic syndrome, hematuria)
  • Pulmonary (bronchiolitis, hemoptysis, pulmonary infiltrates, dyspnea)
  • Neurologic (myasthenic syndrome)

These reactions are more likely to occur during THIOLA therapy for patients who had previously shown toxicity to d-penicillamine.

Please see full Prescribing Information.