Akcea sets off after Alnylam’s hATTR therapy in U.S., but with a handicap

Ionis spinout Akcea has its first FDA approval in the bag, getting a green light for rare disease hereditary transthyretin amyloidosis (hATTR) drug Tegsedi, but it will be burdened with a black box warning as it goes head-to-head with rival Alnylam.

Tegsedi (inotersen) has been approved for polyneuropathy caused by hereditary transthyretin amyloidosis hATTR in adults—just a few weeks after Alnylam got the okay for Onpattro (patisiran)—but will require regular monitoring for platelets and liver function.

Tegsedi has been on the market in Europe since July and has been approved in Canada, and the U.S. approval is a boost for the company after the FDA rejected a filing for its ultrarare lipid disorder therapy Waylivra (volanesorsen) last month, causing it to lay off 10% of its workforce. That rejection was also linked to reduced platelet counts.

Akcea thinks its drug is more patient friendly than Onpattro, as it can be self-administered by patients via injection once a week while Alnylam’s drug is given intravenously every three weeks. However, the FDA’s requirement for platelet monitoring at least once per week—as opposed to every two weeks in the EU and Canada—as well as liver function monitoring every two weeks could offset that advantage.

Sarah Boyce, the company’s president, said on a conference call that the amount of time in a month that patients need to devote to monitoring is “minimal” and that the Tegsedi be “the treatment of choice for many patients who want proven efficacy and the ability to receive treatment on their own terms.”

The biotech said it is ready to launch straight away and has priced the drug at $450,000 a year, which matches the list price of Onpattro, although Alnylam has said it expects the annual cost of its drug to reduce to $345,000 after discounts. Last week, however, a report (PDF) from the Institute for Clinical and Economic Review concluded that both drugs were too expensive to offer good value for money and would need to see their prices reduced dramatically.

“The pricing vastly exceeds a level commensurate with our best estimates of the treatments’ ability to improve patients’ lives, and is likely to cause financial toxicity for patients and ultimately reduce access,” said Steven Pearson, M.D., ICER’s president.

Meanwhile, both Onpattro and Tegsedi could face competition in future from Pfizer, which recently reported positive phase 3 data for its tafamidis drug in hATTR, cueing up filings and potentially first regulatory approvals in this indication in 2019.