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VenxxivaCareFirst (Caremark)

Prevention of cystine stone formation in adult and pediatric patients ≥20 kg with severe homozygous cystinuria not responsive to high fluid intake, alkali, and diet modification alone

Initial criteria

  • Diagnosis of cystinuria established by one or more of the following: biallelic mutations/variants in the SLC3A1 or SLC7A9 gene confirmed by genetic testing; stone analysis revealing 100 percent cystine calculi; presence of pathognomonic hexagonal cystine crystals visualized on urine microscopy
  • Requested medication is being used as an adjunct to high fluid intake, alkali, and diet modification
  • Member has elevated urinary cystine levels at baseline

Reauthorization criteria

  • Member is experiencing benefit from therapy as evidenced by a decrease in urinary cystine levels compared to pretreatment baseline or reduction in stone formation/growth

Approval duration

12 months