U.S. approves $2 million Novartis gene therapy for rare, deadly muscle disorder

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U.S. approves $2 million Novartis gene therapy for rare, deadly muscle disorder
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ZURICH/NEW YORK - Swiss drugmaker Novartis won U.S. approval for its gene therap...

ZURICH/NEW YORK - Swiss drugmaker Novartis won U.S. approval for its gene therapy Zolgensma for spinal muscular atrophy , the leading genetic cause of death in infants, and priced the one-time treatment at a record $2.125 million.

Novartis touched off a debate over what gene therapy is worth last year, estimating its treatment would be cost effective at up to $5 million per patient. A review in April by an independent U.S. group, the Institute for Clinical and Economic Review , concluded Novartis’ value estimate for Zolgensma was excessive.

The disease often leads to paralysis, breathing difficulty and death within months for babies born with the most serious Type I form. SMA affects about one in every 10,000 live births, with 50 percent to 70 percent having Type I disease. The FDA approval was based on a study of 15 children with Type 1 SMA, all of whom are still alive up to five years after treatment. Of 12 babies who got the now-approved dose, 11 could sit unassisted, and at least two are walking, researchers reported.Novartis acquired Zolgensma with its $8.7 billion purchase of Chicago-based AveXis last year.

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Gene therapy gets FDA approval -- and a $2 million price tagGene therapy gets FDA approval -- and a $2 million price tagThe FDA approved a treatment for a genetic disease called spinal muscular atrophy that causes infants' muscles to waste away, potentially killing them before age 2. The price tag? $2.125 million for a one-time treatment.
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