🪦 Deceased Gwen Hartley & The Hartley Hooligans - Attention Seeking Horrorshow Mom of Two Dead Gremlins & Finally Free Human Son

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The problem with ASD is that the major damage is present since birth, not shortly after like with PKU.
All cases have shown severe microcephaly and cerebral atrophy in womb, as well as other issues etc
So far, they suspect ASD is worse than the other types of synthesis defects, the others have varied success with replacement therapy, but ASD seems to be pure potatodom. It'd be cool if they could, but I think it's highly unlikely. They could maybe halt the progressiveness, but what's the point if not a single part of the brain presents normal from birth?


Over at the AKA Gwen's Derange Hooligan channel on YT, there seems to be a Gwen capo that was posting on people's profiles, prior to the news about Claire's death being made public, apparently.

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Pinned by Shlemiel, Schlemazel, Hasenpfeffer Incorporated
Shlemiel, Schlemazel, Hasenpfeffer Incorporated1 week ago
Originally posted 3 Days ago Traveling Heart 10 hours ago "Awww so sad your other channels are getting shut down. Lol. Good. This one is next. But we aren't done with you all quite yet. Something big is coming. I managed in the last few months to uncover most of your identities. :) Going to wait for Christmas to show everyone so you all have a big...fat surprise. :) Blessings" Traveling Heart 10 hours ago "Fun what you can do when you have the knowledge to uncover your ISP addresses. Lmao" Shlemiel, Schlemazel, Hasenpfeffer Incorporated 1 minute ago "If you and/or your warped and perverted "friends" successfully follow through on exposing ANY of the PRIVATE CITIZENS that are expressing their PERSONAL, PRIVATE, FIRST AMENDMENT RIGHT to their point of views expressed on my channels, for the sole purpose to ruin their respective life, which is exactly what you people are focusing on: My channels and I are NOT RESPONSIBLE for ANY potentially PHYSICALLY LETHAL/FATAL HARM/actions that may happen to any of you, by SOMEONE ELSE OTHER THAN ME, INDEPENDENT OF ME, UNRELATED TO ME, UNKNOWN TO ME. I do NOT condone violence of ANY sort. People who lose everything, feel they have nothing else to lose by coming after YOU. You found them, they WILL very easily FIND YOU, too. LETHAL adjective 1. of, relating to, or CAUSING DEATH; DEADLY; FATAL: 2. made or carried out to CAUSE DEATH: 3. CAUSING GREAT HARM or DESTRUCTION: FATAL adjective 1. causing or capable of CAUSING DEATH; mortal; DEADLY: a fatal accident 2. causing DESTRUCTION, misfortune, RUIN, or failure: 3. decisively important; fateful: Take a screen shot of this entire comment. Make your associates aware of the gravity, of what you and they are embarking on. You and you alone, are responsible for your own actions."
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Mars Jupiter
1 week ago
What the fuck?


VICTORIA V
2 days ago
SEE!!! This is the same group of lunatics that are harassing me!! Traveling Heart left me a message on some new random YouTube channel, about Claire passing away before it was even announced to the public!! It was under the Victoria A, Volpe account, NOT ME!! The one that they made up!! These people are FANATICS!!


VICTORIA V
2 days ago
It just goes to show that these people are really CRAZY!!! To think, that some anonymous people's comments on the internet are going to cause the death of a already medically, fragile, child??‽! One that wasn't even supposed to live past birth?‽ AND I'm sure they're going to sue the doctor that said she was incapable of life, too!! And we're all going to be prosecuted and thrown in jail!! Traveling heart good luck with that!! I know I'm sitting here shivering and it isn't from you!! Ha!!! And instead of celebrating the 17 years Claire had on this Earth? They're busy on the internet! Obsessed with social media!! JUST like Gwen Hartley! And it certainly wasn't too hard to find out who I am, because I've never used an alias to begin with!! I don't have multiple accounts! I really don't care! My opinions are mine!! I will share them to the world! I would say the same things in person, that I write on the internet. It just makes me angry that somebody would made up a fake profile of me to say horrible things that I would NEVER say! And I NEVER have said!! Just so "THEY" can screenshot this fake profile of me with my real picture and put it on their damn website, to get more attention for themselves! JUST like Gwen Hartley in real life! It was never about her kids, it was about the attention it would get her!! Rest in peace, dear little Claire! And I mean that from the depths of my heart! ♥️
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Tanya Shearer
2 days ago
We didn't kill Claire you fucking idiot. She died on her own.


Tanya Shearer
2 days ago
Victoria, this traveling heart person is nuts!!! Claire had no idea we exist so how can we have killed her?


NYC girl
2 days ago
These empty threats are ridiculous & almost everyone knows who I am, who cares. Ip addresses mean nothing & if these idiots want to talk about prosecution just keep threatening harm on internet users who make fun of others, it doesn't matter who. Law enforcement will not be bothered with You Tube insults. You must be either really young or really dumb to think otherwise. Many here have the means to sue the shit out of you, you know tie you up in court. Grow up & take up an important cause like the homeless, abused animals, kids that will not have a Christmas present due to no money. If you think protecting the likes of Brittney & Brandon Buell is meaningful take a course in humanity & while there enroll at school & try to find a course in different types of humor. Your anti bully site has not gained any respect from real anti bullying organizations because although making fun of Jaxon & the Hartleys is tasteless & can appear mean neither Jaxon or the Hartley girls will ever know anyone spoke a word about them. Now defaming someone by making fake profiles using their name & likeness to make derogatory comments is prosecutable. If you don't believe me ask a detective who specializes in internet crime. All those comments have been saved & will be used when appropriate. I am sick of being accused of making fun of Jaxon & the Hartleys, I don't. If I did it would be my business. Grow up
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NYC girl
2 days ago
@VICTORIA V We've had our say with these idiots & now its time to ignore & let them keep threatening us, I do not believe a word of it. Keep all the fake profile posts as they are prosecutable because they were posted to cause harm to a specific person/people being you & I. Are they actually blaming Claires death on anyone? I have been talking to Gwen by PM for years & I can tell you & anyone else they are not behind this cyberbullying, fake profiles, empty threats at all. They've been dealing with a sick child for quite awhile & if these idiots were so very close to them they'd know internet comments are not on their minds. As parents I'm sure they are devastated & drowning in sorrow. I have been there. But these idiots exacting revenge on people who make comments they don't like. I'd love to see them in court & I'd drag it on for years. Just exactly what they'd be suing us for is a mystery. Time to ignore Tori.
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NYC girl
16 hours ago
SSHI I can tell you with 100% certainty Gwen & Scott are not behind this internet nonsense with Traveling Heart. Also trying to find Ip addresses without the help of law enforcement is impossible. There are easy ways to block anyone but I know its not necessary. Gwen is grieving deeply & doesn't bother with detractors. She doesn't read the comments & doesn't answer them. There was a time I thought some of these dumb comments by Good Times A GoGo was Gwen but it isn't. I personally have spoken to Gwen, believe me or not any trolls out there I know they are not backing you up. By the way trying to get Ip addresses mean nothing at all. SSHI have a Merry Christmas
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Sandy Y.
14 hours ago
@NYC girl So who in hell is "Traveling Heart"??? I see above where he/she made threats but see no actual comment here or on any other videos. Did he/she make threat comments then delete them? I swear, these loonies with multiple accounts & screennames keep coming out of the woodwork!

http://archive.is/Bp2IW

It'd be interesting to know who it is.
Sidenote : Claire looked fucking terrible in this video on her birthday. I know it was posted at the time, but in retrospect she looks even worse.

https://youtube.com/watch?v=Yocz22C4018

Wow. That's really weird.

I thought Claire looked pretty bad in the bowling video. It's not just seizing. She looks like she's about to faint. Constantly. And she was very yellow. She should not have even been taken outside. It's not like she could have enjoyed bowling. Bowling alleys are filthy germ palaces. Not a place to take sickly, severely disabled children.
 
There are valid reasons to include certain kids with severe disabilities even if they can't fully participate or engage in activities. But shit like dragging pallid, brain-dead Claire to a bowling alley to nudge a ball with her bony knees is just pointless and mean.
 
Thank god that thing is out of its damn misery and pure non-existence. I can't believe the mother went to such lengths to pretend it was a living being that could think, feel, emote, process, produce, eat, drink, love, hate, etc. It might as well have been a fucking brain stem.

It blows my mind we waste time, resources, and emotion on these “people”, that are so cripplingly disabled that they will never have productive, fruitful lives. Not even productive lives, they don't have lives at all, they literally can't do the extreme majority of the functions our species is built to do. She exists just for the sake of existence, and is a burden on society and her own family.

Put aside religious and/or right-wing pro-life hardliners and fee fee loving leftist hardliners, and give me one good argument for putting these people through life... In the natural world, before humanity was dominated by the culture of massive societies, these people would have been left to die in infancy, and it would have been understood that that is the natural thing to do. They were the weak link, they jeopardized the survival of the rest of the family... Not putting someone that fucked up through the suffering of life is akin to a fetus being miscarried or a baby being stillborn. It just fucking happens, why do we sit here and play God like this?

Not to mention these cunts spends taxpayers money - OUR money in getting these literal vegetables healthcare. What is this: Bubble Buddy? Seriously, what has this world come to? And the media love it. CNN actually did a report on these cunts and painted them as heroes.

Isn’t it hilarious and ironic that our society go all out when promoting abortion - labeling it has strong and independent, and how “It’s their right” (having to end the life of a living healthy human in your womb only because it's inconvenient to you is not your right). But when there’s a literal useless vessel that’s a drain on society while being in pain 24/7, there’s suddenly a need to feel sympathy? It’s now heroic. The morality is non-existent. Because now it makes a good story. It's the pinnacle in terms of virtue signalling. That kid is the victim, and so are we: the society who have to face the consequences because a roastie couldn’t get over the fact that her child was shot to shit.

The parents are probably going to divorce out of spite and built-up denial of their faulty genetics after the second abomination dies.

1 down. 1 to go.
 
Ostatnio edytowane:
I think that once it’s better understood it could potentially be treatable to the point where the kid only has mild disability or is normal. There is a similar condition called phenyl ketouria or PKU where instead of the defect being in the asparagine enzyme, it’s in a phenylalanine enzyme. The condition is very treatable if diagnosed at birth with a heel prick test, and if you eat a low phenylalanine diet (no meat and only Phe free protein supplements) you actually live a normal life. For this disease, you could eat an asparagine supplimented diet if you can’t make it yourself, much like the PKU treatment.

No, absolutely not. If this were treatable with asparagine supplements, it would be common practice. PKU means your body builds up an excess of an amino acid. In the girls' case, there's an amino acid that isn't being produced at all, and (this is most critical) hasn't been produced while they were gestating. Their metabolic pathways are all jacked up, and attempts at asparagine supplementation on kids with this disease have been made...but they are not very successful. You can't put that genie back in the bottle with some quick amino acid pills.
 
Idk even know if “normal” was what Gwen wanted, because she has definite snowflake leanings but the girls not being typical pretty little girls was something that really bothered Gwen no matter how hard she tried to pretend otherwise. She made a number of comments that showed such thoughts at various times, including that “at least Claire was cute” when she was a baby.

I think a large part of it can be chalked up to Gwen being very big on the superficial and appearances so having the girls really fucked with her worldview. I mean the constant photo-shopping, filtering and group photos are so over the top.

I did find it hilarious that I had no clue Gwen is quite the short chunky monkey because she expertly hides it in photos. A random candid shot showing her full body shocked me.



This. It was Gwen showing her sassy, wild side hence the “fucking” part.



...and the fact both potatoes were actual dwarfs made it extra witty for Gwen.
I never realized Gwen was short and fat either! Dang I’d love to see a candid shot... a previous poster talked about Gwen wagging her fat little finger at poor South American women in favelas for not wanting to birth a potato and the mental image just makes me cackle
 
I have sympathy for Gwen until I remember she refuses vaccines, muscle relaxers, anything that could make her childrens’ hellish lives any easier. Bragging in the face of doctors trying to help and knowingly allowing another tortured body into the world that she had other people care for.

I didn't realize she didn't even allow them muscle relaxers. Now their contorted miserable little bodies make more sense.

It seems like she insisted on keeping them alive for as long as possible at the expense of giving them a decent quality of life. I genuinely hope the girls lack any kind of higher function, because as cruel as it is to think that all they knew was pain, it feels crueler to think that they were capable of any kind of fully formed thoughts and awareness that they were just props for their mother's amusement.
 
No, absolutely not. If this were treatable with asparagine supplements, it would be common practice.

Nothing related to a disease with less than 20 known cases is going to be common practice. Also by the time you're born with this you are severely microcephalic or dead anyway. You'd just get a slightly healthier potato even if you could treat it.

The only real prevention for this is just to abort the fetus.
 
No, absolutely not. If this were treatable with asparagine supplements, it would be common practice. PKU means your body builds up an excess of an amino acid. In the girls' case, there's an amino acid that isn't being produced at all, and (this is most critical) hasn't been produced while they were gestating. Their metabolic pathways are all jacked up, and attempts at asparagine supplementation on kids with this disease have been made...but they are not very successful. You can't put that genie back in the bottle with some quick amino acid pills.
PKU does cause an amino acid deficiency though, of tyrosine, because tyrosine is produced in the body by phenylalanine hydroxylation. In PKU this stops so the phe can’t become tyr. I’m not sure if there is any other pathways to make tyrosine, but I’m wondering why missing asparagnine syneghsis is potatoe for life but you can get by without tyrosine if you avoid accumulating its precursor. This is especially curious since tyrosine is needed to produce thyroid hormones.

If I had to take a guess at why, it could be that aspargnine doesn’t cross the blood placenta barrier as well, whereas tyrosine does, so you can be asymptomatic before birth because you can get tyrosine from your mother. But for legit, I am actually curious about this, and other unborn errors of metabolism and how they are treated.
 
I’m not sure if there is any other pathways to make tyrosine, but I’m wondering why missing asparagnine syneghsis is potatoe for life but you can get by without tyrosine if you avoid accumulating its precursor.

Because if you're born without a brain you can't just grow one.
 
Yeah, that's what I conjectured, that tyrosine can cross the blood placenta barrier but not asparagnine. That makes sense.
They are pretty medically fascinating, that's for sure.

It seems like such a tiny metabolic flaw but the results of it are breathtakingly catastrophic.

The variation of it that was posted a few times in the thread, just recently a page or two back, with the little girl who definitely has issues but is no where near potato level. But from what I’ve read she is lucky or at least one of the few not totally potato cases found so far.
 
It seems like such a tiny metabolic flaw but the results of it are breathtakingly catastrophic.

The variation of it that was posted a few times in the thread, just recently a page or two back, with the little girl who definitely has issues but is no where near potato level. But from what I’ve read she is lucky or at least one of the few not totally potato cases found so far.
That could mean that there are many genetic variations that can cause the loss of function or reduced function leading to the disease, and some types may be less serious/ more potentially treatable than others.

In general, mutations that lead to a total loss of function are recessive, because most enzymes are haplosufficient (one copy that works is good enough to keep you normal under most circumstances). Its when you have two copies that don't work then you're in trouble.

This is true even for more innocuous loss of function mutations. Blue eyes are recessive because they result from not making melanin in your eyes, and if you have one gene that works then you can still make some melanin.
 
Ostatnio edytowane:
Yeah, some pages back I also discussed the 3 kids who've ever been born with a known synthetase deficiency for glutamine, which is even more catastrophic to lose out on than asparagine. Two of the three were absolute monsters who never made it out of the hospital, the third had some unknown mechanism that was causing him to produce small amounts of glutamine in spite of the genetic problem.

All three were products of "consanguinous relationships" (incest).

I wonder if the Hartleys are second cousins or something.
 
In light of Claire’s passing, I decided to do something.
It’s the holiday season, and regardless of her mother’s behaviour, I want to take a moment to imagine the girl Claire could have been, had she not been born with such a debilitating condition, and to mourne the loss of that girl.
I’d like to imagine this is what she would look like in heaven, if there is one. :optimistic::feels:
I have photoshopped a photograph of her, so we can look at it and bid her farewell with emotions in mind other than horror and abject disgust.
Rest In Peace Claire.
2EFBDEFD-1FB6-4F6C-99AC-3118EB218FF5.jpeg
 
Yeah, some pages back I also discussed the 3 kids who've ever been born with a known synthetase deficiency for glutamine, which is even more catastrophic to lose out on than asparagine. Two of the three were absolute monsters who never made it out of the hospital, the third had some unknown mechanism that was causing him to produce small amounts of glutamine in spite of the genetic problem.

All three were products of "consanguinous relationships" (incest).

I wonder if the Hartleys are second cousins or something.
This makes sense because the condition is a rare recessive mutation, and the reason why inbreeding is bad is because if you have one copy of a bad gene, your relatives are more likely to have that same bad gene, so your kid gets the disease with two copies of the bad gene.
 
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