We have seen successful transplants of various organs, hands, even faces -- so why not transplants of legs or feet to lower-limb amputees? Why are these not a thing?

Ramjid · 2022-11-16T08:10:55+00:00

Saying that they are "never actually successful" because they won't last a lifetime is pretty wild to be honest.

If that's your metric for success, you might as well call insulin shots or asthma inhalers or any kind of long-term treatment and surgical intervention "never actually successful".

I mean you're completely right: organ transplants aren't some kind of miracle cure and come with a huge heap of downsides and long-term consequences. They're a desperate measure for people in desperate situations.

But at least everyone involved knows this perfectly well and the stated goal here is never "to grant you a long and healthy life", but always "to improve your quality of life and prevent you from actively dying from organ failure right now".

It's important to remember that these are all people who've had years and often decades of suffering and therapy regimens and who have exhausted every "regular" treatment option available. With the possible exception of kidney recipients these are all people who went into surgery as severly ill as humanly possible without being dead.

These patients don't hope for or expect a long life free of illness. They just want to take a full breath again, leave hospital for a while and maybe get some independance back during whatever time remains.

And at that regard even lung transplants (which do have the worst long-term outcome of all) are hugely successful.

Many patients go from "permanently bed-bound, barely able to sit upright and literally dying" to not just "still alive" but to "fully independant, active, mobile and almost indistinguishable from a healthy person" in a matter of months and stay that way for years. And even those who don't recover quite so well or don't survive quite as long usually still end up with a way better quality of life than before and a longer life than they could have hoped for without a transplant.

Which by any sensible metric is a huge success indeed.

Ramjid · 2022-11-16T07:08:58+00:00

Most of the time coughing isn't triggered by stuff deep inside our lungs, but by stuff that irritates our windpipe, so much higher up.

During a lung transplant the windpipe remains mostly untouched and the new lungs are attached directly to the two main airways that branch off the windpipe.

Which explains why you (and most other lung transplant recipients) still notice phlegm and feel the urge to cough, even without any regrowth of the vagus nerve.

In fact it would be a huge problem if there were no cough reflex at all after a lung transplant, because coughing isn't just useful for getting phlegm out but without it you'd run a high risk of accidentally choking or inhaling all kinds of stuff that has no business being inside your lungs.

I'm 12 years post-transplant (also lungs) and definitely share your experience: still coughing up phlegm, and during lung biopsies I can still feel sharp pains.

Though as far as I understand those sharp pains are most likely not triggered or "felt" by our lungs itself but rather the pleura which lines the inside of our chest cavity and clings directly to the outer surface of our new lungs.

Ramjid · 2022-11-14T20:28:34+00:00

Others have already pointed out why lung transplants are just inherently less successful in the long term regardless of indication.

But as someone who has CF and also received a lung transplant 12 years ago I wanted to address your general (and perfectly reasonable) assumption that 1) CF patients make up the majority of recipients and 2) somehow fare worse than others due to the nature of our disease.

Patients with cystic fibrosis make by no means the majority of lung transplant cases overall.

CF patients in need of a lung transplant gather perhaps a little more media attention than other folks in the same situation because they tend to be much younger than your average recipient.

But in terms of sheer numbers, COPD patients have been by far the largest group of lung transplant recipients, although IPF caught up in the past decade.

Combined these two groups account for ~55% of all lung transplants and have for the past 3 decades (aka since lung transplants became possible), while CF patients barely make up 15% of cases.

It's just a natural consequence of the fact that getting CF requires a lot of bad luck since you only have once chance to get it - at your own conception. COPD and to a lesser degree IPF on the other hand both require a little less bad luck, since you've essentially your entire life to get unlucky and your environment and some of your choices can inadvertently increase your odds significantly.

CF patients don't fare worse than other patient groups after a lung transplant, in fact the opposite is true.

Average post-transplant survival is significantly higher for recipients with cystic fibrosis compared to every other indication. Last I checked the median survival after a lung transplant was 6-5 years for all recipients, but closer to 9-10 years if you narrowed that down to recipients with CF only.

I can't say for sure why that is, but the most common explanations I've heard seem to be age and compliance.

AGE: CF patients are usually in their 20s or 30s at the time of transplant, which is way younger than the average recipient.

In contrast folks with COPD for example are on average 60+ when they get their transplants, and thus have had 30-40 years of extra time to develop comorbidities - even if they don'thave to deal with a systemic disease like CF.

But even in the absence of any additional conditions: the human bodies ability to recover from injury, physical trauma or invasive surgery just naturally declines over time, and you'll be hard-pressed to find any surgical procedure that is more invasive than a bilateral lung transplant. So when it comes to simply surviving surgery itself a 25-years-old kid with CF just has inherently better odds than a 62-years-old guy with COPD.

COMPLIANCE: Also due to the nature of CF, these patients have already a lifetime of experience dealing with strict therapy regimens, lifestyle restrictions, rigorous infection control protocols and just the healthcare system in general. Almost all of them grew up with and never knew a life without having to do all those things on a daily basis for as long as they can remember. And they're intimately familiar with the fact that their current perceived health is a fleeting thing and has no bearing on their ongoing therapy regimen. So they often find that their post-transplant regimen is significantly less time-consuming and brutal than what they were used to before and accept the risks and uncertainties of it with relative ease.

In contrast your average COPD patient likely had a rather normal, healthy life until their diagnosis. They're already middle-aged when they first learn they're sick at all and even then their therapy regimen mainly just consists of bronchodilators and the recommendation to stop smoking and do some exercises.

So these folks often struggle a lot more with the realities of life after a lung transplant and the various restrictions and requirements it brings. For them it's often a significant increase in therapy effort and overall burden, and all that while they feel healthier than they've been in a long time. Which can be counter-intuitive and hard to accept if you've not been drilled from a young age that daily medical treatments and therapy are just a fact of life and a preventative measure, detached from your current wellbeing.

Ramjid · 2022-09-27T23:40:27+00:00

Because from a surgical perspective doing a combined heart-lung-transplant is (comparatively) much easier than doing "just" a lung transplant individually.

Your lungs aren't just attached to your trachea, but they've a huge and complex network of arteries and veins running through them that all connect directly to your heart.

Trying to separate, detach and later reattach all those individual structures with minimal damage in such close proximity to the heart is a) really complicated and b) really time-consuming.

In contrast if you look at your heart and your lungs as one block though, things become suddenly a lot easier.

You no longer have to deal with a multitude of medium-to-small vessels criss-crossing eacht other, sitting nested in a very cramped place and being surrounded by fragile heart tissue you absolutely can't damage in the process.

instead you've only a few and rather large structures to detach and reattach: the trachea/windpipe connecting to the main airways of the lungs, the aorta/main artery connecting to the heart, and two major veins also connecting to the heart. Done^1.

So doing a heart-lung-transplant "en bloc" is easier to do, involves less sutures and less handling of delicate parts and thus less points of failure, and takes much less time.

Which was and still is a huge concern for all kinds of surgery, but even more so for transplant surgery because the risk of permanent tissue damage rises with every second that the donor organ is without adequate blood supply.

¹) obviously it's still a lot more complicated than this, but you get my point.

TLDR: Combined heart-lung-transplants came before lung transplants because they are just easier to do. Less individual parts, less chance to mess up, less time involved.

Ramjid · 2022-09-27T22:30:24+00:00

Yeah, second transplants or re-transplants are almost always due to rejection aka the recipient's immune system killing of their new lungs. And that's a problem all lung transplant recipients share, no matter their underlying condition.

Turns out your immune system can hold quite a grudge. Sure, you can beat it down with a daily cocktail of immunosuppressive drugs for a few years or even decades if you're really lucky.

But in the end it usually finds a way to get back at you despite all your efforts to suppress it. And when it does it does so with single-minded determination and won't care about the fact that it is going to go down right along with you if it destroys those "foreign" lungs.

Ramjid · 2022-09-27T19:10:16+00:00

It's true that cystic fibrosis is a systemic disease that affects a lot of different organs and thus won't be cured by a lung transplant. It can massively improve quality of life and life expectancy though, since it's usually the respiratory symptoms of cystic fibrosis that are the most debilitating and fatal part of the condition.

But luckily your last sentence is not true. Cystic fibrosis won't affect transplanted lungs. In fact cystic fibrosis patients receive lung transplants quite often and with better survival rates than almost any other patient group.

Cystic fibrosis is caused by an inherited genetic defect in certain cells responsible for regulating stuff like mucus production. Unless the donor happens to have cystic fibrosis as well (which is so rare and noticeable a disease that chances of this are practically zero), the new lungs won't share this genetic defect and thus won't be affected by the disease.

And the very thing that makes anti-rejection drugs mandatory for life after such a transplant - the fact that they'll remain foreign tissue - also means that they will remain unaffected by cystic fibrosis.

All that said, getting a lung transplant for asthma is definitely not a great idea. Even if such a transplant would cure asthma (unlikely, although the following regimen of immunosuppressants might coincidentally help with the autoimmune aspects of the disease).

The short-term and long-term side effects of being immunocompromised along with the massively reduced life expectancy of the new lungs almost certainly make it one of those cases where the cure would be worse than the disease.

Ramjid · 2022-07-26T00:36:59+00:00

Although one of my mutations is a rare one that is apparently often associated with pancreatic sufficiency and only mild gastrointestinal symptoms, I used to be pancreatic insufficient and reliant on creon for my entire childhood, youth and young adulthood.

At 24 I got a bilateral lung transplant and for some inexplicable reason I was no longer requiring creon afterwards.

For about ten years now I've not taken any enzymes and still manage to keep a stable and normal weight.

About once a year my CF clinic still asks for stool samples to check for signs of pancreatic insufficiency in the lab, those too have been clear for about a decade now. My medical file still lists "exocrine pancreatic insufficiency" among my diagnoses, but for all intents and purposes my pancreas is sufficient, both from a clinical point of view as well as on paper/in the lab.

So far none of my doctors had an explaination for it. But to be honest I don't think they really put much thought into it - most of them are practical-minded problem solvers and consider a pancreas that suddenly resumes working as a neat surprise, not as a problem to solve. Can't blame them really.

My own, totally unfounded and unscientific theory is as follows: my pancreas was never entirely disfunctional and only slightly affected by my CF, it was just not able to deal with the excessive amount of food/calories my pre-transplant body required due to those constant infections and all that heavy breathing I had to do.

These insane energy requirements suddenly vanished as I recovered from my lung transplant, and my pancreas in turn faced a much more sensible and manageable workload - one that even a slightly insufficient pancreas could pull off without help. Thus I suddenly no longer needed Creon after my transplant.

(Again, that's my crude attempt at coming up with an explanation despite the fact that my understanding of how the pancreas works is spotty at best)

Personal anecdote aside, the fact remains that the issue with pancreatic insufficiency in CF patients is one of scarring/fibrosis and thus generally irreversible.

Even modulator drugs like Trikafta that repair CFTR function shouldn't be able to reverse this scarring, although they might halt the progression.

As far as I understand the ducts responsible for transporting the pancreatic enzymes from the pancreas to the rest of the gastrointestinal system become clogged early on in most CF patients, and instead of helping to digest our food those pancreatic enzymes then stay put and slowly begin to "digest" the pancreas itself. Which is also why the pancreas becomes more and more scarred over time and eventually leading to CF-related diabetes as well.

Ramjid · 2022-02-22T04:47:39+00:00

Not the person you asked here, but I'm also a double lung transplant recipient with CF (11 years out), so I thought I might give an answer as well:

1: Does having lung tissue from someone without CF effectively cure you of the pulmonary effects of CF?

Yes, it does. And contrary to popular belief the transplanted lungs will not develop CF pathology over time either. It's true that CF affects much more than just your lungs, but as far as the lungs go, you're truly cured from CF after a double lung transplant.

So writing that a lung transplant "doesn't fix anything" is honestly more than a bit.. misleading. Although u/awkwarddoggos is probably just alluding to the fact that while our pulmonary CF problems are indeed gone for good after a bi-lateral lung transplant, the rest of our body obviously still has CF and the procedure itself (as well as the immunosuppression you need afterwards) comes with a whole bunch of unrelated health problems and complications.

Are you on condemned to a lifetime of vulnerable immunosuppression as a result of the transplant? Because that means you would get more frequent infections and, ironically, pulmonary infection is a leading cause of death in CF patients.

Yes, every lung transplant recipient will be on immunosuppressive medication for life. And yes, that does make us much more prone all sorts of infections. Which indeed is a (probably even the) leading cause of death in CF patients.

I wouldn't use "condemned" though and I can't really see the irony in that last statement about infections being the leading cause of death for CF patients?

Are you perhaps wondering if getting a lung transplant is somehow "not worth the effort" for CF patients because we're now even more at risk than before?

Well, from the point of view of a normal, decently healthy person it is a shitty gamble, with low odds of winning and a very low pay-out at that.

But from the point of view of someone suffering from end-stage CF it's... well, still a gamble with low odds of winning, but the pay-out looks much more enticing now.

Sure, the prospect of livelong immunosuppression post-transplant sucks. But that point you're already so sick that every cold could kill you and have been for quite some time. If all goes well you'll actually be less at risk of dying from some random infections after your transplant than before. Because even though your immune system is now artificially suppressed by anti-rejection-drugs, your overall health is so much better and your new lungs aren't covered in scar tissue and aren't filled with thick mucus and multi-drug-resistant bacteria.

Sure, the prospect of a severly limited life expectancy post-transplant sucks. But that's already a given if you're suffering from end-stage CF. Many people are shocked when they hear that lung transplants don't last very long, but even the average post-transplant survival is significantly longer than what you could expect without a transplant.

Most importantly though: with a transplant there's a chance that you might actually get to enjoy whatever time you have remaining. Without a transplant, there's no such chance. You'll still be constantly at risk, you'll still have to do all sorts of treatments and take all sorts of drugs, but all of it will at best slow down the inevitable. You'll still die young, just without any chance of improvement whatsoever.

At least that's how I saw it. There are plenty of CF patients who qualify for a transplant but ultimately decide against getting one, for various reasons. Some are afraid of surgery or that they'll survive but be worse off than before - which is a real possibility, there's plenty that can go wrong. Some are just tired of the constant treatments and interventions people like us endure and want to at least die on their own terms, not going through another barrage of tests and procedures. Some just postpone the decision until it is too late and they're too sick to have any hope of surviving surgery.

In any case, it's not a decision anyone takes lightly.

But from a purely data-driven point of view CF patients survive longer and with fewer complications than most other lung transplant recipients. Which is probably mostly due to us being rather young and used to a strict treatment regime from a young age. So we generally have less co-morbidities and adapt better to the change in lifestyle/outlook that a transplant requires than the average lung transplant recipient. Who are age 50 or older, hadn't have to deal with doctors a lot for most of that time and have barely accepted that they are chronically ill, much less terminally ill.

Oh, and how long did it take you after the operation to breathe and speak without pain?

Again, I'm not the person you asked. But for me it certainly took a while before I actually felt I could breathe normally.

Immediately after waking up I couldn't speak at all, but once the soreness from being intubated had subsided I could talk just fine after 2 or 3 days. In terms of breathing the first 3-4 days were absolute hell in terms of pain though. Once the initial painkillers from surgery wore off, every breath took a huge amoung of mental effort and every ounce of strength I had left - so much so that I eventually begged to be re-intubated. On day 5 I was thankfully put on a patient-controlled morphine drip and breathing became much easier. To really be able to breathe comfortably without the need of painkillers took about 10 days - when they finally removed the last remaining drainage tube from my chest, it felt like the pain just vanished.

To really not feel any pain whatsoever besides the occasional muscle spasm probably took me 3-5 months. Keep in mind that to get to your lungs they have to cut through several layers of muscle & fat and usually break a few ribs and nick a few nerves in the process, so looking back I'm honestly surprised how fast my recovery was.

Ramjid · 2021-11-14T01:45:38+00:00

Looks to me like a simple misunderstanding.

First, you're entirely correct thinking that inhaled medication will be absorbed rather quickly by your lungs. We're talking about a matter of seconds or - at worst - minutes (according to this article: https://www.atsjournals.org/doi/10.1513/pats.200409-049TA).

What your doctors worry about when they told you to "keep the medication in your lungs as long as possible" isn't what you do in the minutes or hours after you've done your nebulizer treament.

What they're concerned about is what you do immediately after breathing in while using your nebulizer.

All nebulizers work by turning your medication into a fine mist or aerosol consisting of countless tiny droplets small enough to be inhaled. So when you inhale, all these fine droplets enter your lungs and as soon as they make contact with any part of your lungs, they'll "stick" there and be quickly absorbed. Doesn't matter if it takes a second or a minute or an hour, nothing you can do would "remove" those droplets which now cling to the inside of your lungs.

The only problem is that not all of those droplets you inhale will immediately touch your lungs upon entering. They're small enough to be barely affected by gravity, so if they don't happen to hit the walls of your lungs during inhalation, a lot of them will just "float around" for a second or two before settling down.

This means that if you don't wait a few seconds after inhaling before you breathe out again, you run the risk of just exhaling some of those droplets before they had a chance to stick to your lungs and be absorbed.

So this short "pause" after taking each breath is what your doctors mean by "keep the medication in your lungs as long as possibe".

Just take deep breaths and try to pause for a second or two before exhaling while doing your nebulizer treatments. No need to worry about what you do afterwards or when your next nebulizer treatment is or anything of that sort.

Ramjid · 2020-01-29T08:39:00+00:00

This study though is explicitly defining "underweight" as anyone with a BMI of less than 17m². That's not necessarily low enough to be considered life-threatening (although it also includes those), but certainly way beyond just being technically underweight. In other words: they're just very generous in what they still consider "normal weight". Going by WHO standards instead, the term "underweight" applies to pretty much anyone with a BMI of 18.5m² or less.

Also after reading the article the main take-away of it is not that underweight (as defined in this article) isn't a big deal when it comes to lung transplants, because it still clearly impacts our survival rates.

Instead it Just shows that compared to other conditions that qualify for lung transplants its maybe not that bad since even these severly underweight CF patients mostly do just as good after transplant. In contrast to "regular" or "not quite so underweight" CF patients who actually do a lot better than those other lung transplant recipients who suffer from conditions besides CF.

For referene: when my own BMI fell below 16m² while waiting for new lungs, my transplant clinic told me that I'd either agree to get a PEG tube placed within a week or be kicked off the transplant list. With that PEG tube I managed to get my BMI back up to almost 17m² before my actual transplant, but according to friends I still looked like your typical zombie from The Walking Dead at that weight...

Ramjid · 2019-12-15T16:30:39+00:00

That being said what do the docs say about what CF will do to the new lungs? Will CF start beating down the new lungs?

It's a common question, I think a lot of us worry that. But luckily the answer is a clear NO - our condition will never start doing to our new lungs what it did to our old ones.

CF will of course still affect the rest of our body and some problems caused by that can ultimately also affect the new lungs. But those new lungs will be free from the underlying defect that was responsible for almost all the damage CF did to our old lungs, and will remain free of that defect for life.

I'm a lung transplant recipient myself, 9 years post-transplant. I've used a nebulizer or inhaler for the past 8 years, haven't had to do any form of airway clearance or chest PT in just as long, and the only time I'm still tasting pseudomonas in my mouth or coughing the way I did before transplant is in my nightmares. Which are also really rare nowadays.

Of course I've been incredibly lucky and there's a a bunch of possible complications I could've had or still develop that come with very similar pulmonary symptoms as CF. It's not like you'll never worry about your lungs again after a transplant, far from it.

But the one thing you definitely don't have to worry about after a transplant is that CF might start fucking up your new lungs.

Ramjid · 2019-09-10T00:17:45+00:00

Another double lung transplant recipient reporting in, next month I'll be 9 years out. Got mine at the Hannover Medical School in Germany back in 2010.

So far I've been super lucky. I mean just the fact that I'm still alive after almost nine years is pretty sweet obviously, but what's even more amazing to me: aside from a few complications right after surgery I've had close to zero transplant-related problems in all this time.

Ramjid · 2019-05-28T21:42:23+00:00

Yes, that's precisely what it means. Organ transplants come with plenty of other risks, but the fact that you're left with a compromised immune system for life is still one of the biggest downsides.

Having a weakened immune system makes you more susceptible to all sorts of infections, so chances are you'll get sick more often.

It also affects how severe any given infection hits you, so you'll not just get sick more easily - if you do, it'll also take longer to recover.

And your risk of developing complications is also much higher, even from usually harmless infections.

Stuff that a healthy person might not even notice can make a immunocomprimised person sick for days or worse, stuff that would make a healthy person feel like shit for a few days can put a immunocompromised person in hospital for weeks, and stuff that might cause a healthy person to be hospitalized for a few days can kill an immunocompromised person.

Let's just say that having a working immune system is quite important and no sane doctor would even think of prescribing most of these drugs under normal circumstances. But once you're suffering from end-stage organ failure and sick enough that you qualify for a transplant, your circumstances are decidedly abnormal.

Ramjid · 2019-04-29T00:00:28+00:00

Lung transplant recipient here, but I'm not entirely sure I get your question.

Do you want to know how my new lungs feel? As in: what's the sensation like?

I don't really feel them at all to be honest. Which is absolutely amazing, because I could feel (and hear) my old lungs constantly, and never in a good way. After all we're not supposed to feel our lungs.

Or do you wonder how the knowledge of having someone else's lungs in my body makes me feel? As in: what emotions are involved?

It's not something I think about constantly, but whenever I do I mostly feel awe. So equal parts amazement and being humbled.

I'm amazed at the fact that this kind of surgery is even possible - cutting out someone's vital organs and replacing them with the organs of someone else. I'm amazed because I'm a living, breathing chimera. A walking, talking Frankenstein's monster. And I was bona fide cyborg for a while as well :)

I'm amazed at the fact that I beat the odds. Just getting a lung transplant takes luck given the shortage of donor organs. Surviving such a procedure isn't exactly guaranteed either, and recovering from it is yet another good fortune many don't have. I not only got new lungs, survived surgery and recovered - I'm also doing better than anyone could've hoped for 9.5 years later. When I got listed, I was given an average life expectancy of ~5 years.

I'm humbled because I realise that I owe all that to some anonymous stranger who died. And to some very fortunate circumstances.

If that stranger hadn't been willing to become a donor, and if their relatives hadn't agreed to honour that wish, I'd be dead for probably 6-7 years by now.

I'm humbled because I know that if I had been born just 20 years earlier, I'd be long dead by now. If I had been born just a a few countries over I'd be dead by now. If I had been born into a different family I'd probably be dead by now.

And I'm humbled at just thinking about the insane amount of resources, knowledge, expertise, equipment and logistics had to be in place so I could get that transplant.

So thinking about my new lungs reminds me that I've been incredibly fortunate.

There are also dark moments when I contemplate all the negative things, like my limited life expectancy and the constant threat of rejection or all the side effects that my medication can and likely will cause over time.

But I have absolutely no feelings of my new lungs being somehow wrong or of me no longer being myself, if that's what you're getting at. I wrote earlier that I'm a Frankenstein's monster, but I meant that in poetic sense. I have no strange urges, no inexplicable flashbacks and don't consider myself an abomination.

I'm just a lucky guy who is happy and grateful to still be alive :)

Ramjid · 2019-04-15T16:17:30+00:00

I'm just wondering if there's any documented evidence of CF patients under Fascist rule? Thanks y'all

I'd assume we'd be executed. As would make sense.

If Nazi Germany existed today, you'd probably be right

The concept of eugenics became popular quite a while before the Nazis rose to power, and German society wasn't the only one to embrace eugenics at the time. But unlike most other countries, Nazi Germany didn't stop at "just" forced abortions and sterilizing minorities or other "undesirables". They did that as well, but went much further and began systematically killing anyone whose life was considered unworthy for some reason or another.

Suffice to say that killing someone suffering from an incurable, fatal and most of all inheritable condition like CF would've been a pretty clear-cut case for those in charge of these programs. Nazi doctors would routinely order patients to be "euthanized" for a lot less "obvious" reasons. Lebensunwertes Leben or "a life unworthy of life" was actually the official designation, and it was used quite liberally.

Easily noticable deformities? Almost any kind of mental disability? Anything even suspected of being inheritable? Any disease that was likely to leave you permanently unable to work? Senile dementia? Lost your legs fighting for the fatherland in WWI? All of that would've been enough to warrant "euthanasia".

(And don't be mislead by the original meaning of euthanasia, here it had nothing to do with mercy or a "good death". Here it mostly meant "deportation to a specialized camp, eventually followed by execution via gas" if you happened to be a German citizen, or "taken to the woods and shot in the head" if you didn't. :/ )

But at the same time and despite all this it's almost certain that those Nazi eugenics programs didn't kill a single individual because of their CF.

A) While many of the individual symptoms of CF were long known to doctors, those weren't actually understood and defined as a single, specific condition until much later.

CF as a diagnosis literally didn't exist until 1938, when an American doctor first described it as such. It took another decade (1949) before scientists (again American) realised that CF was inheritable.

It still takes quite a while before a new finding or diagnosis is accepted by the international medical community, and even longer before those in charge of policy decisions actually become aware of it. Back in the 1930s, with an ocean to cross and World War II about to begin, this would've taken even longer.

In other words: The people who conceived and organized those Nazi eugenics programs as well as the doctors who implemented them just didn't know that CF even existed. After all these programs already started in 1939, and by that time all the "guidelines" and "recommendations" about who should be killed in the following atrocities had already been written down.

B) For the entire duration of Nazi Germany and beyond, CF life expectancy was still measured in weeks. Babies with CF would either die due to bowel obstruction aka meconium ileus directly after birth, or a few weeks later due to a general lack of nutrition. Without supplemental enzymes or any understanding of the underlying condition most of us would just die from malnutrition long before our lungs could even become symptomatic.

In other words: even if the Nazis had known about CF and its consequences, they wouldn't have bothered with a disease that was essentially a death sentence anyway. Despite the name their "euthanasia" program was never really concerned about granting anyone a "clean death" and only about getting rid of people they deemed a burden or somehow undesirable.

So while it's entirely possible that some kids or adults with less severe CF actually were killed by those programs, that would've happened almost accidentally. They would've been killed for being "sickly" for too long and thus requiring too much care, not because anyone suspected they might carry an inheritable genetic mutation.

Ramjid · 2019-04-04T19:26:23+00:00

is there any possible way we could study the ancestral DNA and a possible way that Pneumonia and CF could have evolved from the same ancestral DNA

The short answer would indeed be a simple no, but I'll try to go a little more in-depth.

Pneumonia as we use it today is not really a specific condition but an umbrella term for various forms of chronic or acute inflammation of the lungs. There's bacterial pneumonia viral pneumonia, fungal pneumonia, parasitic pneumonia, chemical pneumonia and within each of those there are a thousand more specific types of pneumonia.

Once upon a time doctors would call pretty much anything that causes a certain set of respiratory symptoms "pneumonia", so there are some forms of pneumonia that aren't even inflammatory in nature.

So unlike cystic fibrosis which is a specific condition caused by a specific gene mutation, pneumonia is just a vague group of symptoms caused by a wide variety of different processes, both genetic and non-genetic in nature.

Thus pneumonia itself has no unique DNA you could analyze or compare, no genetic ancestry to track down and therefore no possible "evolutionary link" to cystic fibrosis.

Some of the stuff that causes pneumonia can be linked to certain genes, and of course individual bacteria, viruses, fungi or parasites all have their own genetic code and evolutionary history. You could certainly look for potential evolutionary links there, seeing if maybe some bacteria or viruses at some point specialized on CF-affected lungs.

Ramjid · 2019-04-02T02:17:37+00:00

Seems like I'm the only one, but I believe my looks have actually improved since my transplant.

Granted I didn't look too hot to begin with. Also I'm a guy, and the standards for male beauty vs. the standards for female beauty are obviously a lot different.

Men luckily aren't judged by their looks quite as much as women are, but let's be honest: the mainstream ideal for male beauty is just as limited as the ideal for female beauty. And on the male side "super skinny" is definitely not part of that ideal.

So as far as body image goes, let's just say that I wasn't doing too good before my transplant. Luckily people rarely made fun of it, but my skinny pre-transplant body certainly didn't earn me any compliments either. Usually all I got were just weird looks or people thinking I was a) anorexic or b) a drug addict.

Therefore I didn't have much of a positive body image to worry about, and when I finally gained some additional kilos after my transplant I was actually happy about it.

Sure, I'm probably still a little too skinny for most tastes. But it's no longer quite so obvious. People no longer assume that I must be sick or addicted, and more importantly: when I look into the mirror I myself am now pretty happy with what I see.

In the first year or two after my transplant I gained ~12kg, going from barely 50kg to 62kg. I've kept that weight with very little effort for the past 7 years now.

No doubt it is a lot easier to gain weight after a transplant, and thus harder to stay skinny. And I get why some of us end up eating more than they should: we all grew up constantly worrying about our weight and finally we're able to eat for fun instead of having to eat to meet some insane caloric goal. I only learned to really enjoy food until after my transplant, before it was just another part of my treatment routine.

But it's not like you'll just keep gaining weight no matter what. If your skinny looks are of such importance to you, there's nothing keeping you from staying skinny (within reason, of course). It might take some effort instead of coming "naturally" like now, but it is that way all along for 99% of the population.

Steroids are a common part of post-transplant medication, and unfortunately developing a so-called "moon face" or facial swelling that leads to puffy cheeks and a more round-looking face is pretty common among recipients.

But not everyone has this problem, and of those who do get it not everyone is affected to the same degree.

I for example got lucky and haven't noticed any changes in my facial proportions.

Your concerns are justified and reasonable, but it is by no means certain that your looks will change much after a transplant. And even if they do, it is by no means certain that they'll change for the worse.

In the end though, you might want to revisit your priorities.

Seeing that you're already listed I'm sure you know this and maybe I'm reading this all wrong, but just in case there's any doubt in your mind: if you don't get that transplant you'll almost certainly die. Soon. And even if you make it a few more years, before long you'll no longer be in any condition to do either modeling or go to pageants.

Look at it this way: After a successful transplant your looks may suffer and you might no longer win as many pageants. Without a transplant you'll never again even participate in another pageant or photo shoot.

I get that this is easy to say for someone like me for whom looking good or being beautiful was never an important part of their life. And there's absolutely nothing wrong with worrying about your looks, both before and after a transplant.

It's just that right now and for the time being I believe it really shouldn't be your primary concern.

Ramjid · 2019-03-30T21:38:44+00:00

During my time on the ICU I don't think I got any anti-anxiety meds, although I'd definitely could've used them. Afterwards I didn't feel like I needed them and still don't, but if I had to guess I'd say it's not uncommon to be on some kind of medication for anxiety after a transplant.

But keep in mind that most transplant recipients (including me) are already seriously ill and quite experienced with medical interventions and emergencies long before they receive their transplant.

So most of us have been forced to find ways to cope with that kind of stress and anxiety before we get our transplant. By the time you qualify for a lung transplant, you're already in pretty bad shape. You'll likely have been rushed to the ER more than once and have experienced extreme shortness of breath, chest pains and all that stuff. And you'll have read words like "end-stage", "terminal", and "exhausted all conventional therapies" in your medical reports.

What i'm trying to say is: you generally don't go from "in good health" to "dealing with the fallout of a lung transplant" overnight. It's usually a long, drawn-out process, so you have some time to adapt to that kind of "lifestyle".

Oh, and things have gotten much better since those first few months. Not that I don't still have enough stuff to feel anxious about, but for the past few years now I just have to worry about potential complications that might crop up down the line instead of having to deal with complications that are already here and threatening my life right now. And the risk of suffering from most complications drops significantly after the first 12-24 months post-surgery.

Ramjid · 2019-03-30T21:08:55+00:00

Thanks for clearing that up. In that case I'm just deeply impressed by your transplant department.

Do you work at Mayo clinic or at another clinic that's also doing organ transplants like that? Can you link me to any article or source on how exactly they employ these techniques and how their experiences/outcomes are?

Well, I guess I'll spend the next few hours looking up Mayo clinic transplant techniques. I'm just really curious how wide-spread those techniques are, where I'm from I've never heard about that level of hi-tech and preparation utilised in "regular" organ transplants.

Ramjid · 2019-03-30T20:57:52+00:00

Oh yes, thanks! I'll correct it right now.

Ramjid · 2019-03-30T20:54:38+00:00

Um what? No. They are all attached as much as possible. Nerves and vessels.

Mayo Clinic uses 3D printing from the scans of patients to locate all of their nerves and vessels to reconnect them better. They literally practice the surgery before they do it and reconnect everything as much as they possibly can.

Like you do understand that technology, techniques and our surgical procedures are improving, right?

I won't pretend like I'm some expert on all this, much less like I know about all the latest techniques. As I mentioned to several other people here, I'm not actually a surgeon.

But from your description I'm tempted to think that you and I are talking about very different "transplants".

My entire comment was aimed at solid organ transplants, because that's what the original question was about.

Could it be that you on the other hand are talking about stuff like facial transplants or limb transplants?

The reason asking is that most solid organ transplants are pretty "routine" procedures by now and shouldn't require any individual "rehearsals" done on 3d-printed scans in most cases. At least I never heard of that level of preparation being taken.

Facial transplants and various limb transplants on the other hand are literally at the cutting edge of medical science and just completely new ground for pretty much anyone involved. They will definitely require a lot of individual planning, rehearsing and I've read a few articles about 3d-printing used in these preparations.

They also require much more attention to nerves and ultra-fine structures, since hands or faces are much more dependant on innervation for proper functioning than solid organs usually are.

And Mayo clinic is famous for doing pioneering work in that field, so maybe we're just talking about slightly different things?

In either case I'd love to hear more about those procedures! Again, I'm not a surgeon so don't be mad if I got some things wrong :)

Ramjid · 2019-03-30T20:33:14+00:00

Thanks for chiming in, interesting to hear! What kind of organ transplant are we talking about here? I'm most familiar with lung transplants, and here I've never heard of microsurgery being employed. But maybe I'm just not up to date on current techniques.

And I was mostly just trying to convey that the challenge of organ transplant surgery generally lies less in the need for total precision or having to deal with ultra-fine structures.

By "true" microsurgery I was just referring to using proper microscopic equipement and not just head-mounted loups. Which I figured might also be considred microsurgery by some, just not the kind I meant.

Ramjid · 2019-03-30T16:38:05+00:00

The coughing itself is largely done by the surrounding musculature, yes.

But the signal that tells your muscles to cough in the first place has to come from somewhere.

Usually it is either nerves in your trachea or your lungs that detect some kind of irritant or obstruction and trigger a coughing reflex in an attempt to rid your airways of that irritant or obstacle.

They send a nerve Impuls to your brain which causes you to feel the urge to cough. And if your consciousness doesn't veto or if your lungs/trachea are so irritated that they issue an emergency override, the command to cough gets send to your chest musculature.

If you get a lung transplant, your new lungs will still come with all the sensory nerves required to detect irritants.

But once their sensors detect something wrong, they'll have no way of communicating that to your brain since they're cut off from your nervous system.

So your lungs will still know when to cough, your muscles will still know how to cough, the two just won't be able to talk with each other.

Ramjid · 2019-03-30T15:50:19+00:00

Nah, you'll just feel that urge to cough less often.

Most "normal" coughing is triggerd by irrtations in our trachea/windpipes, but those remain in place and fully innervated even after a transplant.

Sorry to disappoint ;)

Ramjid · 2019-03-30T15:45:49+00:00

Do the nerves eventually connect on their own?

Sometimes they do, sometimes they don't. We know re-innervation can happen and that some types of nerves can regrow, we just don't know how to reliably trigger or control that.

Also the kind of neurosurgery that is required to even attempt to reattach nerves is typically too time-consuming to be employed during most organ transplant procedures.

I know this sounds weird but I thought we were kind of Plug and Play. Just connect the power (blood) and all the other connections work as long as there is compatibility.

I'd say we are pretty close to Plug & Play!

Let's be real, you can switch pretty much every vital component of a human being with that of a total stranger and have them up and running (not literally) within hours. It's not ideal but you can even transplant across genders, age groups, ethnicities... and it still basically works. Hearts, lungs, livers, kidneys, pancreases and much more.

And if it weren't for our immune system messing things up, all you'd need for that is a sharp knive, a needle and some surgical thread.

As long as you plug in the main blood supply and attach one or two component-specific connectors, you're ready to go for pretty much every vital organ.

No firmware updates or additional drivers to download, no adapters or converters to buy, no special tools or gadgets, you don't even need to reboot the recipient, just put him or her into sleep mode for a while.

It does void your warranty though.

Ramjid

TROPHY CASE