Somewhere Under My Left Ribs: A Nurse’s Story (2018)

The article hints that the operation described took place 20 years ago. Already, in that time, we've made significant progress treating cystic fibrosis: we've developed ivacaftor, the first 'designer drug' that can really fix the disease, means that were they born today it's more likely that they wouldn't need the transplant in the first place. In some forms of cystic fibrosis, there is a specific genetic deficit that prevents the chloride channels in the lungs from functioning properly, meaning ultimately that patients get thick and sticky mucus in their lungs that becomes very easily colonised by bacteria (as well as many other problems elsewhere too). If they have the "right" form of cystic fibrosis, Ivacaftor specifically binds to their chloride channels, and improves the transport of chloride through the ion channel by binding to the channels directly to induce a non-conventional mode of gating which in turn increases the probability that the channel is open. It's a lifechanging / lifesaving drug, and it came on the market in 2013.

The design and implementation of ivacaftor relied upon knowledge from physics and mathematics to clinical medicine. I am a physical scientist working in healthcare (in medical imaging) and I feel overwhelmingly lucky that I am able to work on problems like this. But I am acutely aware that it is the end of a very long road: I use aspects of what was theoretical physics, worked out in the 1960s and 70s (especially by a French/Russian physicist called Anton Abragam) together with giant chunks of mathematics and statistical understanding -- all to work on complex problems in biology and medicine. Just because you do not work on medical problems directly does not mean that your work is not of use to us! I, my team and my colleagues, benefit hugely from technology ranging from the Linux kernel to "how I hacked this" guides on the internet.

Unfortunately, Ivacaftor is an insanely expensive drug. In the USA, it apparently costs "over US$300,000 per year" [1]; my copy of the BNF (79 -- March to September 2020) lists the price that the NHS pays as around £14k per month (~£168k pa). This is a lot of money -- and it will save a life. Unfortunately, it is my opinion that many of the largest problems facing the healthcare systems around the world at the moment are political. The nurse who wrote this article was employed in the UK, where taxpayers make the extremely altruistic generosity of the NHS a (strained, breaking) reality. We get economies of scale. But the system is utterly close to collapse -- there are around 100,000 job vacancies within it, there aren't enough healthcare professionals, and many in the profession are leaving for elsewhere due to extreme overwork and the effects of the pandemic. Some countries have it far better (e.g. Denmark) and others have it much, much worse. One of the things I am personally proud of is that the NHS and universities system in the UK explicitly do not have profit as a motive and the horrible decisions of "is it worth spending this amount of money to have a chance at saving a life" are taken out of the hands of patients and almost all clinicians: there are guidelines, they are evidenced-based and aim to be objective, and they are, broadly speaking, followed. It is for that reason that the mother of the child in the story "only" has to deal with the horrendous emotional trauma associated with his illness, and not necessarily the punishing financial cost associated with being a transplant recipient, dependent upon cyclosporin for the rest of his days, and with the threat of other problems later on (e.g. 'too small for size' syndrome, requiring another transplant). Other parents, and other children with cystic fibrosis elsewhere in the world, are not so lucky.

---- [1] https://en.wikipedia.org/wiki/Ivacaftor

Thought I fully agree with your sentiment, I found working as a coder/scientist in a renowned hospital a braindeadening experience. On all sides walled in by commerce and vested interests, there is very, very, very little room for innovation. If there was, you'd be selected to be painting within the lines set out for you. Papers were glorified changelogs, and rather unsubstantive ones at that. I got very close to a burnout, on account of the vacuousness of the work. Personal marketing was the job, not actually coming up with better ways of doing things.

I have a very rare immune-mediated neurological disease affecting my peripheral nervous system, that started at age 5, in 1994. The disease was discovered on NIH research grant funds in the 2000s. But, the grant funding is never remotely enough, respective to need, for society.

In 2012, my medical problems became so severe that I scavenged the medical literature. I was tremendously lucky to come across such literature, ultimately describing the disease I have. At the time, there were only 38 articles written about it. Anyways, I was tested for it, and I had the antibody.

Soon after, I literally had a near-death experience. Anyways, I was 22, and I was basically a kid. My parents and my twin brother pretty much saw me on my deathbed.

I am in pharmaceutical remission now, after trying about 10 different treatments, plus combinations of them. It was never statistically likely that I would end up like this, let alone be alive.

I am culturally American, but I hold dual citizenship also with Croatia, so I am a European Union citizen. I am not as sentimental about being American as other people. Honestly, the only reason why I would never renounce my American citizenship is because the US ultimately saved my life, along with others around the world.

I think nurses do get a lot of respect in the hospitals I've worked in. You certainly wouldn't be long tolerated if you were dismissive to them, at least.

I spent a lot of my childhood in hospitals. After a few years of fighting, my situation almost stabilized. Until a high ranking doctor decided to do an experiment on me, which eventually cost me all of my eye-sight. From my side, nurses get 10x more respect then doctors.

Oftentimes nurses have to choose between sacrificing themselves or another human in massively profit optimized enterprise to keep someone alive or to let that person suffer to make management realize certain shortcomings of that system.

Keep in mind that unless nurses keep their work/life boundaries there is actually no way for numbers oriented management to realize issues within certain doctor run departments.

It isn't that different from other corporations per se, but in the case of the nurses they have to choose between letting a patient suffer consequences for the sake of transparency or not. It is a moral dilemma.

Nurses pretty much run my local hospital.

This hospital might the be the only place, I have seen, where doctors egos are put in check. Maybe too much?

I remember feeling sorry for this one doctor, whom seemed like a decent guy.

It's higher variance than you're implying. Some nurses are angels, some are... not. This shouldn't be surprising, given that nurses are human just like the people who work in any other profession.

> Doctors get status and respect, but often it's the nurse

I don't buy this, because the "nurse who knows more than the residents" has been a stock character for a while. Nurses haven't been unsung for most of this century.

If anything, the perception has totally flipped, and another stock character (at least in the US) are doctors as "yes-men" and "butchers", preoccupied with getting back to playing golf, who only know how to prescribe a pill because the drug rep advertised it to them.

Nurses are lionized, and almost no one is saying "just a nurse" anymore:

https://news.gallup.com/poll/274673/nurses-continue-rate-hig...

I disagree. If you are willing to kill a person why would you care whether the person willingly agreed to a donation? You could also just fabricate a letter of consent.

I live in a country with Opt-out and the only thing I think that should be changed is that you should be informed about it actively (e.g. a letter on your 18th birthday or so), even though that might reduce the numbers of donators.

> but it is quite likely that certain black and gray market would be created.

I see your point. But, on the other hand, having so few organs available is what makes black market actually work. In Germany we already had a scandal where people bought their way up the waiting list.

Both situations are horrible, but I think that the opt-out solution is probably the better choice for avoiding/reducing black markets.

I don't think someone ready to murder people to take their organs will have problem with finding ways around "consent".

In fact, if more organs are available, it should limit the need for a black market. Imagine you are rich and in desperate need for an organ. You are not fundamentally evil, but you are really desperate, losing morality and ethics as the disease progressed. If someone can find you an organ before you get to murder then it will save the life of an innocent and your sanity, and maybe also your own life too, for the quicker response.

We allow wealth and resources from dead people to do the equivalent, when that could do far more good if redistributed.

One $million cash-rich person dying might lose a pair of useful kidneys but children are dying from dehydration through diarrheal disease that's relatively easy to treat and that persons cash could save more than 1 or 2 people, probably 100s (or more) ... so why is the moral equivalence not made. Surely taking someone's stuff by default is less imposing of a state than taking someone's flesh by default?

That's out of date, the UK is now "opt out" [1]. Oddly, this is still called "organ donation".

[1] https://www.organdonation.nhs.uk/helping-you-to-decide/about...