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Rare disease

Miglustat

Brand: Zavesca®

PBAC's latest decision on Miglustat: Not recommended (2011). Considered for Treatment of progressive neurological manifestations in adults and paediatric patients with Niemann-Pick disease Type C (NP-C) disease.

PBAC outcome
Not recommended
2011
ICER (AUD/QALY)
Not modelled
no economic evaluation
Submissions
3
first 2008
Submissions
3
2008 → 2011

Eligible population

adults and paediatric patients with Niemann-Pick disease Type C (NP-C) disease

Therapy area
Rare disease
Evidence base
RCT | Other
Key trials
OGT918-007a, OGT918-007p, OGT918-007a(ext), OGT918-007p(ext)
Comparator
placebo plus standard medical management
ICER note
No updated modelled economic evaluation was presented. At July 2010 PBAC meeting, PBAC accepted that miglustat is unlikely to be cost-effective, but no specific ICER value stated in this re-submission.
ICER (historical)
$10.0M/QALY in an earlier submission (2010) — the latest submission carried no numeric base case. No single PSD states this combined range; see source PSDs.

Why PBAC said no

Reasons cited in the latest PSD: Clinical evidence did not demonstrate miglustat superior to placebo in effectiveness; videofluoroscopy swallowing studies lacked blinded outcome assessment and control groups with no consistent improvement shown; case series data based on small patient numbers with large variability and unvalidated scales; survival analysis comparison between treated and untreated cohorts had unmatched baseline characteristics and unclear comparability, confounding cannot be excluded; chain of argument from dysphagia treatment effect to lifespan extension via aspiration pneumonia reduction not sufficiently established; applicability of dysphagia-pneumonia associations to NP-C patients uncertain; failed to meet LSDP eligibility criteria 4 and 5

Submission history

Similar precedents

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