[HN Gopher] First gene therapy for Tay-Sachs disease successfull...
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       First gene therapy for Tay-Sachs disease successfully given to two
       children
        
       Author : daegloe
       Score  : 95 points
       Date   : 2022-02-20 18:40 UTC (4 hours ago)
        
 (HTM) web link (theconversation.com)
 (TXT) w3m dump (theconversation.com)
        
       | LegitShady wrote:
       | Hopefully this is long term successful and the first step in
       | curing a lot of conditions like this.
        
         | mym1990 wrote:
         | If you're interested in learning more, the book Code Breaker is
         | a great background to some of the steps that were taken to get
         | us to where we are today! This work has been decades in the
         | making :)
        
       | drran wrote:
        
       | beams_of_light wrote:
       | >We were able to deliver these treatments to the children in our
       | ongoing clinical trials thanks only to funding from a generous
       | family whose own child is a participant.
       | 
       | Wish this were not the case.
        
         | hammock wrote:
        
           | Shared404 wrote:
           | From https://news.ycombinator.com/newsguidelines.html :
           | 
           | > Please respond to the strongest plausible interpretation of
           | what someone says, not a weaker one that's easier to
           | criticize. Assume good faith.
        
         | halukakin wrote:
         | Wish governments would spare funding to these research efforts.
        
         | mym1990 wrote:
         | It's weird to say 'only' since I assume that a lot more went
         | into this effort other than funding. There are a lot of
         | initiatives that have a lot of funding, but little success.
         | Either way, this result is certainly better than nothing, or
         | waiting years/decades for public funding.
        
         | BurningFrog wrote:
         | I expect most of the cost is regulatory.
         | 
         | Lighter regulation around rare "small market" diseases might
         | save a lot of lives. And money.
        
           | soldehierro wrote:
           | > Lighter regulation around rare "small market" diseases
           | might save a lot of lives. And money.
           | 
           | Orphan drugs for rare diseases are already subject to less
           | regulation.
        
         | bckr wrote:
         | I think it's okay to express this, but would be more valuable
         | if you added more to the conversation e.g. what you positively
         | desire.
        
           | inglor_cz wrote:
           | Rare diseases need some public funding. Not only for ethical
           | reasons, but we may also find out very nontrivial things
           | about our own biology this way.
        
             | loeg wrote:
             | They have _some_ public funding.
        
       | bckr wrote:
       | This is incredibly moving. It can feel like there's no good news
       | in the world, and that techno-optimism isn't founded.
       | 
       | But these 2 kids are a lot healthier than than they would have
       | been without this incredible invention. This is the kind of stuff
       | we can hold on to.
       | 
       | Thanks for sharing.
        
         | ch4s3 wrote:
         | They would die otherwise, painfully I might add. Gene therapy
         | is posed to be a revolution that makes antibiotics look like
         | band aids.
        
         | DaveExeter wrote:
         | These two children should have never been born. No one should
         | be born with Tay-Sachs, a genetic disease.
        
         | chaostheory wrote:
         | It's good news, but it doesn't mean that there still aren't
         | more challenges to overcome.
         | 
         | I think the article ends on a really important note:
         | 
         | "The increasing cost of manufacturing these treatments makes it
         | extremely difficult, if not impossible, to develop and test
         | gene therapy for many ultrarare diseases where the number of
         | patients worldwide is very small and profitability low.
         | 
         | We were able to deliver these treatments to the children in our
         | ongoing clinical trials thanks only to funding from a generous
         | family whose own child is a participant"
        
       | IMAYousaf wrote:
       | I don't have anything to add except that for some reason, I
       | viscerally remember the first time I heard of Tay-Sachs disease
       | in a high school classroom through a video about genetics.
       | 
       | Something made me feel so disturbed about this one disease above
       | all else because of the seeming hopelessness of the situation
       | coupled with the rampant cruelty of how it kills kids from the
       | inside and seemingly reverses developmental progress.
       | 
       | I don't know why I just remember the moment I learned about this,
       | but this is great news. Hopefully this is the first in many such
       | therapies.
        
       | CoastalCoder wrote:
       | I was a little surprised that the researcher talked only about
       | stopping disease progression, rather than reversal.
       | 
       | Especially for the 7-month old. I assumed that at that age, the
       | brain was still growing / adapting in ways that could work around
       | the earlier problems.
       | 
       | But now that I think about it, I've heard that oxygen deprivation
       | during birth can cause permanent impairment.
        
         | halukakin wrote:
         | Reading the article, I'm not seeing much info on how the
         | results were 7 months old in terms of brain development. At
         | that age, one would expect brain development to improve. I hope
         | the researchers discuss this in more detail in future news.
        
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       (page generated 2022-02-20 23:00 UTC)