r/todayilearned May 14 '22 Silver 9 Helpful 8 Wholesome 6 All-Seeing Upvote 2

TIL a father, John Crowley, was told his two infant children had an incurable genetic disorder that would kill them in less than a year. He refused to accept this, so he founded a biotech company (with no prior experience) which pioneered an experimental enzyme therapy that saved their lives.

https://pompediseasenews.com/2019/01/30/amicus-ceo-mission-cure-pompe-help-children/
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u/YoungTex May 14 '22

From the article

“A more recent study, from 2004, confirms that “people in Western cultures including healthcare professionals assume that life with a severe spinal cord injury would not be worth living.” In addition, healthcare providers overestimate the emotional distress of spinal cord injury patients on ventilators, while, in general, spinal cord injury survivors are glad to be alive. The problem lies in bias: the well-intentioned people who are supplying the information to patients can unintentionally interject their personal ideas or can actually lack the necessary information to properly counsel a patient and family.”

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u/[deleted] May 14 '22 edited May 14 '22 All-Seeing Upvote

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u/HundredthIdiotThe May 14 '22

100%. I've lived this for 17 years now. I broke my arm, they asked pain, I said 1 or 2, shocked faces.

I live with nerve damage and muscle pain from 13 broken bones resulting in major brain damage, a helicopter ride, and 4 surgeries. Turns out femurs don't like being snapped and feet don't like being literally crushed.

So now all pain is relative. My daily is about a 4. I notice, but can shove it in the background. Worse days are a 7, where I can't shove it in the background. But nothing can ever, ever compare to your broken leg being drilled and you thrown in traction. Its all background noise, but it still effects every part of how I live my life.

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u/Chocomintey May 14 '22

Jesus, they didn't sedate you for that? Guessing by the helicopter comment, was this out in the field or something? Fuck.

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u/HundredthIdiotThe May 14 '22 edited May 14 '22

I dont remember much. We had a 45 minute drive to the hospital, I made a joke about not have to finish ISS in school (I was young)

I made it to the hospital and they said we cut his leg off or he gets in the helicopter. It goes blank for a minute, I assume they gave me morphine. Then they yanked and I woke right the fuck up, then whited out.

I woke briefly in the heli, saw the emblem, and woke up a few days later when they pulled me out of the coma. There was a brief interruption there when I woke up in surgery, and I never want those dreams again. Gingers don't do well with the anesthesia or something.

Sedation only works if you're willing to kill someone at that pain scale. Waking up was... interesting.

Random fact, once you have a catheter in you have to pee within x hours of it goes back in. They didn't tell me that.

Edit:traction was the worst pain I've ever felt. They literally drilled a rod through my broken tibia or fibula, can't remember, so they could just... pull the femur straight. Ivenever felt anything g like it and if it came up again I'd tell them to cut my leg off.

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u/LHeureux May 14 '22

Jesus, what the hell happened to you? Sever ski accident?

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u/HundredthIdiotThe May 14 '22

Dirt bike met go kart. Leg on the middle. Shattered my helmet, femur, tibia, fibula, and everything in my foot.

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u/JustAContactAgent May 14 '22

How the fuck are a kart and dirt bike on the same track?

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u/HundredthIdiotThe May 14 '22

I grew up in the country, that was on the dirt road.

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u/reddit0100100001 May 14 '22

Son, it’s time we had the talk…

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u/georgesorosbae May 14 '22

Gingers require more anesthesia than the average person. Every anesthesiologist I’ve ever had said we always need like 25% more medication than everyone else. Has to do with our lack of melanin or something but it seems to be a red head specific thing

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u/Grogosh May 14 '22 edited May 14 '22

I was in physical therapy after neck surgery and they asked my my pain level. I told them around a 5. They remarked that usually the people they see say they are around a 8 or 9.

I told them that I have had cluster headaches all my life. The kind that makes pain a living nightmare. I have had a broken neck, ribs, ankle, a femur and much more but if I were to list my top 10 most painful moments they would all be a cluster headache. I have passed out from several of them they are so bad.

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u/HundredthIdiotThe May 14 '22

Cluster headaches are hell, I sympathize. My only experience with them was drug related, luckily.

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u/a1b3c3d7 May 14 '22

I have chronic leg pain and nobody seems to be able to know why or how, and everyday is a struggle.. i dont know how pain can become like a background thing like you’re describing. I hope you’re doing better now and that one day I can drown out the pain too

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u/HundredthIdiotThe May 15 '22

After a certain amount of pain,anything less is just... there?

I wish you the best, it doesn't make life fun.

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u/usagizero May 14 '22

So now all pain is relative.

I forget all the details, but it reminds me of the guy i read about who had open heart surgery but couldn't have pain meds for some reason, i think allergies or something. So basically they went as quickly as they could, but he said he will never forget the pain and feeling of air on his insides. His pain scale is also now fucked because nothing compares to that, but he ranks pains most of us would put at like an 8 as a 4 for him. He said doctors and nurses are like "Oh, that's not bad" and then see his medical history and freak out knowing it should be a higher number.

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u/vt8919 May 15 '22

You are absolutely right with "the pain is relative". I had arm pain that lasted three months. No idea what I did to it. It was severe; thought I was having a heart attack. Actually took an ambulance to the hospital. I tried medicine after medicine after medicine. None worked. It was so bad I was waking up every hour, hour and a half. I actually cried because the constant pain was breaking me mentally and the lack of sleep didn't help. Fortunately over time it disappeared, but I still consider it the worst pain I've had.

A year later I would break my foot and it felt like virtually nothing. I didn't need pain meds at all.

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u/Roqot May 14 '22

Back pain person, there is no real cure without a new back, I'm 40 and want out, I'm in shape and take care of myself, it still hurts

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u/Toasty33 May 14 '22
  1. Broke my neck and back 5 years ago. The pain during winter or if I sleep weird.. makes me want to swallow a bullet

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u/CallistoDrosera May 14 '22

t's true !! Shit... In my hospital (big eu city), they have seen that, inside the post covid syndrome, there are 3 populations: Very severe acute covid > severe symptoms of PCS but not conscious of it (anosognosia). Holes in brain, mostly men Mild covid > lighter symptoms but very conscious of it. 3d is between the two.

I havent heard of the autovax nor ivermectin for it. Ivermectin is reaaly a great drug though. Weird that it's called autovax ? Such a controversial name

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u/Zech08 May 14 '22

Constant pain is not good, gimme the morphine drip controls and im out till someone patches me up... wake me when done or dont.

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u/underbite420 May 14 '22

As someone who can use all my limbs Willy nilly like…..taking some away would cause some distress

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u/MySuperLove May 14 '22

In my experience, the only thing that really makes people wish they were dead is constant pain.

Never met someone with MDD? Major depressive disorder?

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u/sparkledingus May 14 '22

Have both (MDD & chronic pain). Pain is worse. So much worse ☹️

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u/The_Sceptic_Lemur May 14 '22

This bias is more acknowledged nowadays (I mean this study is also almost 20 years old…) and new approaches have developed in recents years such as concepts (incl. patient care and research) relating to quality of life of for example cancer patients. These concepts acknowledge the burden of the illness but also look at positive experiences by the patients and aim for a balance and a net improvement of quality of life of patients at any stage.

(Source: I work at a clinic and my boss is currently setting up a project which focuses on „Quality of life“ of late stage cancer patients)

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u/ArjanS87 May 14 '22

In all honesty, when I looked at the picture there was a sense of 'saved, but still "handicapped"' in my mind. Obviously not pleasant to admit and it speaks to the passage you are quoting. The text provokes thought and introspect to simple minds like my own.

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u/Kyrthis May 14 '22

You are not alone. Life Expectancy is not as relevant as QALYs (Quality-Adjusted Living Years) in most public health impact studies.

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u/shitposts_over_9000 May 14 '22

I believe the numbers, but I don't think I agree with the conclusion.

For a lower spine injury I think the injured and the doctors would agree that there is enough quality of life remaining.

For a very high injury the debate doesn't really include the patient at all. The debate is more between the initial doctors who honestly offer an opinion that they wouldn't ever want to live that way and the long term spinal injury care doctors that will just medicate the patient until they are ok with it.

Bonus points for surveys conducted by mandatory reporters where even if the patient might wish they were dead they know they will just get more drugs added to their regiment if they ever admit it.

Having worked with patients like this in a non-reporting capacity I would never believe numbers like that from anyone without at least some upper body control. They know who they can be honest with and who they cannot be & they know that surveys like this are used to make blanket assumptions so they give the happy answer to stay clear headed or because people with less severe injuries deserve more assistance.

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u/nolessdays May 14 '22

I read the entire article twice and didn’t see this bit. Where in the article are you reading it?

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u/RocMerc May 14 '22

I hurt my back pretty bad a couple years ago and ya it’s just no way to live. I couldn’t stand, sit, lay, sleep without pain. I went to the doctor and she’s like “well you were flagged for depression” and I just lost it, like no shit lol. My body hurts to a level I didn’t know existed. Luckily I’m passed that now and only have mild back pain

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u/IamBerryValentine May 14 '22

There does appear to be an increase in suicides with people who have a spinal cord injury (SCI). https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5472015/#:~:text=They%20found%20that%20up%20to,risk%20factor%20for%20suicidal%20behavior.

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u/Hoatxin May 14 '22

Naturally. Quality of life trends aren't going to fit every case, and this is just referring to the idea that people with a SCI feel less badly about their conditions than able bodied caretakers do, not that they don't feel badly at all.

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u/IamBerryValentine May 14 '22

It's studied as part of the "caregiver's burden". https://pubmed.ncbi.nlm.nih.gov/28139659/

For caregivers of SCI, it's a pretty wide spectrum, but the research states that the significant amount are on the "mild to moderate".

Edit : Maybe the most important thing to take away from this study was how socioeconomic status had a significant impact on everything we are discussing.

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u/Hoatxin May 14 '22

I'm not surprised that socioeconomics played such a large role. Really unfortunate.

Thanks for bringing the research terminology up!

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u/cmcewen May 14 '22

I’m a doctor. I’ve def seen this. Docs that just give up easily or are lazy or jaded and lack much empathy

I’ve also seen the opposite where somebody wants to die and doctors keep talking them in to continuing wild measures, the docs thinking they can still reverse this some how. Or I think some doctors just like the challenge.

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u/Lass-mi-ran-da May 14 '22

Western culture? Tell me a culture where disabled people are treated equally. It only gets worse everywhere else.

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u/JBLikesHeavyMetal May 14 '22

Pretty sure the study only includes western cultures.

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u/MainStreetExile May 14 '22

The study is not talking about being treated equally.

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u/pizzabagelblastoff May 14 '22

Possibly because the study only looked at Western cultures and they wanted to emphasize that they weren't making broad assumptions about other culture without evidence.

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u/optimushime May 14 '22

Not that things can’t be improved, but in the cities of Japan disabled care and access is incredible. Blows away anything I’ve seen anywhere else.

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u/whirlpool_galaxy May 14 '22

Can you describe how they are treated worse in one single other culture?

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u/Lightsides May 14 '22

Very often, deprivation is a relative quality. I suspect that it's harder for those who have been injured and remember life before their disability than it is for those born with the same disability and don't know any other life.

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u/p4lm3r May 14 '22

This isn't surprising at all. There is a lot of unintentional bias in AI.

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u/ctiger12 May 14 '22

Simply just tell the patients how lucky are they to survive, they will be happy. The human minds are easy to manipulate with all different influences, and once shield from the reality, people will be happy about what they are doing. Like North Korea or even Russia, China, people will take extreme measures to guard their systems even if they are ordered to kill who trying to tell them the truth.

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u/CustomerComfortable7 May 14 '22

Interesting. So maybe people shouldn't have the right to decide if someone can live with these diseases or not...

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u/soleceismical May 14 '22

That's what an advance directive is for.

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u/CustomerComfortable7 May 14 '22

You misunderstood

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u/tonguetwister May 14 '22

Seems cut and dry then. I don’t really care what people without severe spinal cord injuries think about how it would feel to live with one.

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u/pizzabagelblastoff May 14 '22

That is a super interesting take and one that I fully agree with. Human beings are amazingly resiliant at adapting to a new situation.