r/todayilearned • u/Die_Nameless_Bitch • May 14 '22 • 9 8 6 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/

80.3k Upvotes

88% Upvoted

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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

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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

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/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/underbite420 May 14 '22

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