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Karl Pfleger's avatar

It does seem surprising to see a phase 3 with such a small n, but Barth is very rare. The subject ages seem to be mid-to-late tens through early adults. Quite a lot of people with Barth die in infancy and more don't make it to adulthood. Looks like there are only 100-200 adults w/ Barth in the US. So that explains why the study n was so small.

Nice job reviewing some of the other trials in which the therapy failed, but I think you are being too one-sided: You could also have pointed out some of the other ongoing trials in which it's still being evaluated for other indications, at least one of which is in phase 3 so presumably had positive ph2 data. That's for dryAMD (ReNew trial). And yes, I see the positive ph2 trial record there too.

For mito myopathy it looks like the story isn't as simple as just failure: It looks like the failed ph3 MMPOWER-3 trial, rather than leading to conceding defeat instead lead to another phase 3 for mito myopathy, the NuPower trial (NCT05162768) for which I don't see results yet. (And this is after presumably positive ph2 results.)

Also, looks like the heart failure trial failure you mention was a phase 2, so you could presumably also try to comprehensively review other positive phase 2s it had. I haven't done that so not sure if there are any for conditions not yet mentioned here.

Dr. Christin Glorioso, MD PhD's avatar

Good points! I didn't thoroughly review the ongoing trials and these represent more shots on goal for SS-31. I suspect that this approval is just the beginning of the story and am hopeful that we will see it move across indications with approvals.

Dr. Linda Bluestein, MD's avatar

Great summary. Thank you!!