Readers who know anything about me at all know my wife passed away a little over four years ago from Huntington's Disease (HD), a fatal genetic disorder that causes the progressive breakdown of nerve cells in the brain. It deteriorates a person's physical and mental capabilities in the prime of their life and it has no cure. HD is the quintessential family disease because every child of a parent with the disease has a 50/50 chance of inheriting the gene and HD. Today there are around 30,000 symptomatic Americans and about 200,000 potential cases. Therein lies one of the primary issues with HD - our wonderful for-profit medical system and so-called Big Pharma simply have little incentive to work on solving HD. It is as if President Trump's southern border wall has been erected to hamper HD study. HD families have no Michael J Fox or Stephen Hawking to help focus attention on HD. Woody Guthrie is probably the most famous person to have ever had HD - luckily his son Arlo did not inherit the gene. Woody died in 1956.
Some of you will say that HD afflicted people simply should not have children. That may indeed be correct but the genetic test that is 100% accurate was not developed until 1993. My children were born in 1977 and 1982. Lynn was tested and the results of the pre-genetic test were negative, Doctors basically did an MRI and saw no trace of HD (brain shrinkage). Oops - 1n 1999 some friends of ours noticed some odd behavior and strange physical traits in Lynn. She was genetically tested and I will never forget the phone call from the Neurologist's office. I answered the phone at work and there was silence on the other end. Finally the caller said I am very sorry but Lynn has HD.
She lived until 2014, but, we had two children. You can see where this is going. Our daughter was diagnosed with HD last week after the genetic test was administered. For what it is worth, the older test came back negative 4 months ago.. Each of her three children has a 50/50 chance of inheriting the defective gene. Our son refuses to have the test because he fears it will destroy his attitude about life and he is trying to assure his family has a stable future whether he is there to share it with them or not. I understand his reticence as Lynn gave up on life almost immediately after her diagnosis. It was part of the progressive degeneration of her brain.
So what does this have to do with the weekly 2-on-1 blog? Enter CRISPR. If you wish to examine the technical jargon please click CRISPR. Long story short, CRISPR has the potential to eliminate some 6000 genetic disorders - including HD. 60 Minutes has done a story on CRISPR.
Genetic engineering is here and it is real. With a bit of luck my six potential HD inheritors will benefit from CRISPR if we can manage to navigate the slippery slope that genetic engineering has become. But, to ignore the benefits available through the judicious use of genetic engineering is simply immoral in my opinion. Opponents claim genetically modifying embryos to prevent disease is tantamount to playing god. I say if god screwed up, correct the mistake. Opponents say genetic engineering is unnatural and does not take into consideration the desires of the future generation. I say so what? Is not setting a broken arm unnatural? Is not plastic surgery unnatural? Is not transplanting organs unnatural? What about artificial limbs? And who would not want to have HD removed from their life if given a chance? Perhaps some of the faithful might be willing to risk their life on the power of prayer. or god performing a miracle. I am not one of the faithful.
To see what Ramana has to say on the matter please go to Ramana.
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i didn't know Woodie Guthrie had HD.
ReplyDeletethe only thing i can honestly say about it all is that i don't know what i think. it's like a maze in my mind. you know... the kind where there is an end point and you are to find your way there. i think one thing until someone points out an arguably sensible reasoning then i think oh. yes. i see that. but then i see the other way too. and i'm back to square one!
i guess i'm mostly with your son. quality of my life now is important to me. and now is all we ever have. even if they continue to figure this all out and how to alleviate things like HD and ALS and cancer it will then be their own "NOW."
people like Michael Douglas and a few non famous people i know have survived the cancer that took down my Bob. there is no point in my feeling bitter about it (which i almost did once but caught myself) or even envious i suppose. it's just that sometimes we're simply born into the wrong point in time regarding that affliction. my great grandmother died at 36 of pulmonary disease which wouldn't threaten her life now presumably. not the most intuitive or best answer i'm sure. but there it is. and i'm back to square one! SH
It is black and white to me Tammy. Chop the gene, eliminate the disease, up to 6,000 of them. Of course that means little profit for Big Pharma and that is what they really care about.
DeleteA very good piece SM, and one I have to say I agree with wholeheartedly. As my own wife battles nsc lung cancer, she is benefitting (for now) from an immunotherapy drug that goes after the cancer cells and stops them from growing. The bet is that further research will improve upon its effectiveness and find a way to eliminate them altogether. It is trying to modify her system to fight something it doesn’t know how to fight on its own. Thanks SM for our thoughts on GM here as I truly believe our bodies are amazing machines that just need outside help occasionally to do its job better!
ReplyDeleteI understand the concerns of the naysayers but we need to clear this hurdle and proceed. Best wishes for your wife and her battle and to you for being there for her.
DeleteWords simply fail me. My approach has been entirely impersonal and abstract whereas you have brought in personal and realistic information to be pro GM. I have however not completely expressed my no to GM but, have just said that I am indifferent. It is easy to be so when one is not affected and I appreciate what you have brought to light about the subject matter.
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