Some additional info that might be of interest to this crowd:

He was fantastic at video games (RTS and RPG) and chess. He had impeccable hand-eye coordination. He co-founded a gaming clan. None of that is relevant anymore.

There is nothing to be done. His condition can't be solved (within his timeframe) by medicine or technology. eastdakota offered to spend whatever it took, but the least worst option was to move him across the ocean and turn him into a test subject for tests that he probably wouldn't tolerate in his current state.

He was deliberate in "minimizing toil". His university projects morphed into patents and a job at UnSpam and Project HoneyPot. Project HoneyPot's technology unlocked the core value to CloudFlare, which he cofounded. His projects and stacks were deliberately chosen for maximal reuse and to minimize wasted effort.

I thank Matthew and Michelle for keeping the story of his journey alive. I can't imagine the tech periodicals are knocking down their doors to write stories like these. Connie Loizos had another piece on him at TechCrunch in 2019[1]

[1] https://techcrunch.com/2019/08/15/cloudflare-has-a-third-cof...

In September, after we'd released our S-1 but before Cloudflare's IPO, I took two weeks off and went back to my home town of Park City, Utah. (It's actually where Lee and I had been roommates many years before.) That two week period before you go public is known as the quiet period, so I was generally avoiding social media or answering my phone or doing anything other than resting and getting ready for the pre-IPO road show.

We hadn't talked much publicly about Lee's disease, but Michelle and I had written a bit about it in the S-1 Founders Letter:

"Finally, there are two of us signing this letter today, but three people started Cloudflare. Lee Holloway is our third co-founder and the genius who architected our platform and recruited and led our early technical team. Tragically, Lee stepped down from Cloudflare in 2016, suffering the debilitating effects of Frontotemporal Dementia, a rare neurological disease. As we began the confidential process to go public, one of the early decisions was to pick the code name for our IPO. We chose 'Project Holloway' to honor Lee’s contribution. More importantly, on a daily basis, the technical decisions Lee made, and the engineering team he built, are fundamental to the business we have become."

One evening my phone rang and the called ID came up as "Lee Holloway." I checked the number and it was his. I didn't answer and the caller didn't leave a voicemail. My first reaction was that somehow his phone had been hacked or his number was being spoofed and someone was using it to somehow screw with me. Figured someone had read about his disease in the S-1 and was being a jerk.

I reported the call to Cloudflare's security team to investigate. I also texted Lee's wife to let her know to be on the lookout. She wrote back: "No, that's Lee. Sometimes I think when he's feeling nostalgic he reaches out to old friends. He never says anything, but I think it's his way to let you know he's thinking about you."

So I still shoot him text messages from time to time to let him know I'm thinking about him. And I hope, somewhere inside his mind, there's a part of him that still knows that.

Cloudflare is a great platform, and I'm glad, thanks to Ms. Upson, I got to know one of the folks responsible for it a little bit. I'll be sure to keep chasing the hard problems in my own day-to-day code, and keep a thought for Lee while I do it.

Oh man, was that a kick in the guts when I got to that bit. My Dad was diagnosed with that in the past year, (after obviously struggling for a while), and declined rapidly. He had a different variant, and indeed the one thing that DIDN'T change was his personality. In fact, that's what kind of fooled me for a while. He would still trot out his usual bad jokes and regular phrases, but after a while you realize these are almost like reflexes, and often wouldn't really make sense in context.

It was interesting to see for Lee how this seemed amplified after heart surgery. My Dad had really bad "post-operative delirium" for about a month after major heart surgery, and while he recovered somewhat, that was definitely the start of his major decline.

Sadly, after moving into a care facility in mid-March, within a couple weeks he was in hospital after contracting Covid-19. He passed away on the 4th of this month from it. The only good to come out of all this was that I'd visited him many times over the past couple years and said "goodbye" many times thinking it might be the last time, even if just mentally and not physically, I'd get to see "my Dad", as I knew him.

Apparently it is often a genetic disorder that can be hereditary, and you can get tested for the genetic markers. As a coder/manager myself who depends on my mind for work, and enjoys being mentally challenged and active, (and I also have young kids), something like this scares the crap out of me. I'm not sure I want to know if I might have it. For one thing, being in the U.S. healthcare system, if I did have the markers, would that then count as a "pre-existing condition" I'd have to disclose?

Dementia and schizophrenia are present in my family as well and caring for those in the later stages has been really hard.

I found myself torn by the same question of whether to test for genetic predisposition. After reading The Gene by Siddhartha Mukherjee, whose author is in a similar position, I have found some comfort in deciding not to test for now. As he says in an NPR interview:

> There's no one-to-one correspondence between a genome and the chances of developing schizophrenia. And until we can create that map - and whether we can create that map ever is a question - but until I - we can create that map, I will certainly not be tested because it - that idea - I mean, that's, again, the center of the book. That confines you. It becomes predictive. You become ... a previvor (ph). A previvor is someone who's survived an illness that they haven't even had yet. You live in the shadow of an illness that you haven't had yet.

https://www.npr.org/2017/05/12/528097708/the-power-of-genes-...

Otherwise it seems like whether or not you test the reality is set independent of your knowledge of it. I'd like to think that I'd want to test given that, but I suppose it's hard to know how I'd act without really being in that position.

I loved his book, but I think I disagree with him on this - feels like a rationalization of an irrational thing.

If there was a conclusive test, I would definitely take it for the sake of future offspring. Otherwise, it would likely give me more anxiety than useful information.

Insurers cannot deny health coverage, cannot charge higher costs, or subject people with pre-existing conditions to a waiting period

You may be better off getting life/long term care insurance before getting tested, but other than that there is little reason to avoid being tested.

And you must be confident that Trump will lose, since the 5-4 majority that saved Obamacare last time will not survive a few more Republican appointments.

https://en.wikipedia.org/wiki/List_of_justices_of_the_Suprem...

That said, many justices have not voted as expected. In other cases political parties have swapped positions on various issues etc.

  the 5-4 majority that saved Obamacare last time will not survive a few more Republican appointments

That is a strange coincidence. Could changes in bloodflow have an impact on brain cells?

A quick search finds a few hits, e.g. from https://perioperativemedicinejournal.biomedcentral.com/artic...

> Delirium is associated with negative hospital outcomes including a tenfold increased risk of death, a fivefold increased risk of nosocomial complications (Inouye 2006), poor 1-year functional recovery, and postoperative cognitive decline (Saczynski et al. 2012). The long-term cognitive decline seen in some patients after the development of delirium is similar to that of Alzheimer’s patients (Pandharipande et al. 2013). With reports of up to 50 % of patients over 60 years old developing postoperative delirium following cardiac surgery (Rudolph et al. 2009), this patient population poses a major burden for healthcare.

It had side effects.

For the better part of a year afterwards, I kept finding myself unconsciously substituting completely incorrect words into written correspondence, I was irritable almost constantly, my short term memory was shot, and I had hallucinations and attacks of aphasia - I’d be walking down the street, or in the shower, or in a damn meeting, and I’d not only forget where I was but I’d lose object permanence and recognition - couldn’t tell taps from cats, cars from shoes.

It got better, over time, but there was very definitely a long mental hangover from it. I do have medical quirks, like I can’t tolerate opioids (they worsen pain and make me vomit), and I’m a carrier of a whole bunch of degenerative diseases (thanks, inbred aristo dad), and when I had surgery I was weakened from years of still mysterious illness - they thought my GB was the root cause, but no - stress - leaving tech fixed me.

Anyway. I can readily see how, with someone with a poised avalanche of genetic mental disorder, it can be enough to completely push them into the abyss.

Either way my memory has not been great since. My wife is disappointed when I forget meaningful conversions from a week ago. I struggle at times to notice trivial errors, really struggle. I can look over the same email and miss several mistakes. Other days I'm clear, I don't make many mistakes and I can spot them easily.

I still managed to get an Engineering degree and hold down regular employment as a software developer. But there are periods of time where my focus and productivity are just not there. I suspect this will hold me back in my career. But I'm thankful I can function as well as I can and have survived to this point.

I don't have specific recommendations, but I think that the potential for recovery should be much higher than what people with neurodegenerative diseases suffer from, like what was described in this article.

There're a lot of risk and uncertainty trade-offs, and everyone reacts to things differently due to their genetics and what they're suffering from, but nootropic substances could potentially help here. They could possibly help as a short-term aid that improves memory and attention to detail while the nootropic is in effect, and some can actually potentially create long-term improvements.

They're very under-studied, and they definitely require a lot of careful research, gradual self-testing, and acceptance of some risk (some nootropics have almost no risk, and some have a moderate level), but it could be worth looking into.

Something as simple as a prion can completely destroy it and then there's Phineas Gage, someone whom all neurobio students learn about on day one of their course. Neuroplasticity can be quite amazing.

https://en.wikipedia.org/wiki/Phineas_Gage

I don't think it's exactly correct to say "a prion" can destroy it. One single prion I don't think would cause significant damage in most cases, if there could somehow be only a single one. The issue is that it's believed a prion will gradually cause nearby proteins to misfold and also become prions, and then it probably starts recurring exponentially like a pathogen infection, and your brain physically deteriorates and falls apart with no way to stop or reverse it. A very similar deterioration process appears to happen with other disorders like Alzheimer's and the form of dementia discussed in this article, though the root cause still isn't understood.

I mean, they had a brain tumor which could've been causing damage itself, plus radiation to the brain, invasive brain surgery, altered brain blood flow during the surgery, general anesthetic... That's serious stuff affecting the most complex machine we're aware of.

The brain can be capable of amazing feats of recovery, but it's always going to vary based on someone's pre-existing genetics, environment, age, and the specific damage they might have incurred. Recovery back to baseline absolutely may be possible for them, but there's really no way to know that for sure when dealing with something so serious and without knowing many other factors. Hope is good, but sugarcoated false hope isn't.

I don't think I'm sugarcoating, at least not in the sense of how the word is usually defined. We know neuroplasticity is real. It isn't just some placebo. Regardless of what part of the brain was affected, because of neuroplasticity, he/she can recover functionality through exercise and training. I agree with you that this won't always be the case, and at some point, brain dead is pretty much brain dead like in the case of survivors of gunshot wounds to the head for example. But given the eloquence of the poster's comment, I think the kind of situation we're looking at here is of a different class.

Here's a 2018 piece discussing research from the Mayo Clinic focused on apparent anesthetic damage in patients older than 70.

https://www.sciencedaily.com/releases/2018/07/180719112024.h...

At this point he often falls into the verbal loops described in the article, and hasn't really been able to hold a conversation for quite a while. Exacerbated by his poor hearing. He's also extremely restless, and wants to run the washing machine and dishwasher and go for groceries many times a day. When my parents still owned a dog he'd take of for a three minute walk every hour, and he drives my mother nuts by constantly wanting to help out with housework but then being unable to comprehend the simplest of instructions.

He currently spends about a week every month in an assisted care facility, and my mother seems to have finally let herself be talked into looking for a permanent spot for him, though with Covid-19 is hard to tell what will happen and when.

It was a relief when we finally got the diagnosis after he was repeatedly hospitalized for unrelated stuff in late 2018 though. It had been obvious to everyone that knew him for at least five years prior that something was wrong, but he didn't display any of the obvious signs of Alzheimer's. I guess it took extended time spent around medical professionals and a thorough checkup for them to see that something was wrong.

Also to all the young people freaking out about the inability to concentrate: Relax! From everything I've seen and heard a telltale sign of FTD is complete obliviousness on the part of the patient.

The gradual decline slowly conditions you, like the proverbial frog in the pot of water. It was strange going back and reading some of his old emails and letters recently to try and find some info I needed. It was like, "Wow! He used to be really articulate and lucid and funny". I almost forgot that guy by the end :-(

I’ve been managing a chronic condition for decades, and I’ve never had coverage denied because it was “preexisting.”

Maybe it was only if you were buying coverage outside a group it would be an issue, but I don’t know.

Regardless I many people who had preexisting conditions pre-ACA, and it never affected coverage.

For disability insurance, or life insurance, it’s probably a different story.

This mostly affected the individual and small-group markets, in which a pre-existing condition either disqualified you for coverage, made it prohibitively expensive, or explicitly excluded coverage for that condition (sometimes, the exclusion was only for a set period of time).

Typical failure modes were: individuals unable to get coverage on the open market; small businesses and non-profits that offered employees health insurance being forced to fire people because they got expensive illnesses and their insurance providers threatened to 10x their premiums; etc.

It made entrepreneurship and freelancing a much harder choice for many (typically older-than-30) folks, especially since you risked getting fired & losing insurance if you worked alone or for a small-ish firm.

Some states had remedies for these issues -- either through a high-risk pool for people who were rejected in the individual market (though often these policies had coverage limits that made them not ideal), or through guaranteed-issue insurance in the small group market (i.e., if you and a partners start a business, insurance companies in your area who serve small groups HAVE to a) insure you, b) not charge you more than 10% the average rate, and c) renew your insurance when it's up for renewal, usually annually).

California in particular had both these in place pre-ACA.

Ask me how I know. :/

Everything was controlled by a single Postgres database that made very heavy use of stored procedures, that called other procedures, that called others. It was one giant program inside the database. It took me a while to comprehend what he'd done but it was really great. The database ran everything and all those functions made sure that audit logs were kept, that the calls were allowed for the user ID being passed in, and some of these procedures made external calls to APIs including getting things like SSL certificates.

It was a magnificent monolith inside a database.

I worked on the periphery of the database (it was truly Lee's domain) and he'd tell me what output to expect or API to create and I'd code to his spec. and we'd just hook it up.

If any single artefact represents what he did at Cloudflare, it's that database. And he used to code it on a laptop we called "The Beast" because it was so crazily heavy and overloaded with memory etc. that he'd carry around a mini, test Cloudflare wherever he went.

The interesting thing is that such cases can also actually be the huge nightmare you write about, in the sense that there are tradeoffs in the original design that originally made sense but become harder to deal with later, but also in the sense that their nonstandardness can be hard for more typical engineers to work with. (This is often referred to as the 'hiring problem', though that can be misleading.) The trouble with the typicalness of more typical engineers is that they're steeped in a technical culture which they regard as the proper and obvious way to do things, but which really is mostly just the set of conventions they're used to. Those two things are easy to conflate when all your peers agree on what's proper and obvious (for example that you should never build a complex system entirely in Postgres stored procedures, or whatever the weird thing is). Those conventional assumptions are at least part of what makes such systems a 'nightmare', because if you're working on a system that feels 'wrong', then you fight it rather than blend with it, and that makes it harder to maintain. Everything that's objectively wrong with it will loom larger in your experience than it would if you didn't have the feeling that it should never have been built that way in the first place. Worse, many things that aren't objectively wrong with it, in the sense that they aren't intrinsic to the system or the domain, end up blowing up into major issues because people find they can't work with this system in the way that they're used to, and it's too hard to abandon ways that you're used to—it always feels like the system is at fault. Meanwhile, everything it got right will fade into the background as you assume that any system that had been built 'properly' (i.e. the way you'd have done it) would have gotten all those things right too, at the same cost or better.

It's hard for the later engineer who experiences the 'nightmare' to fully appreciate that if it weren't for that weird, obviously 'wrong' system, they wouldn't have their job in the first place (nightmare and all), because the business that succeeded was built on the weird system.

I don't know anything about this case other than what John posted above, but I've seen something like it in other situations, and in fact I would say that PG and YC have this in common with the case of Lee and Cloudflare as John describes it—as remote from one another as Postgres procedures and custom Lisps must be in every other respect.

What I wish we could teach younger engineers is: when you run across a system like this, pause. Then, pause. Then pause again. The creativity at the core of it can be remarkable. Often you see things that one person did which would have required a large team in other hands, and therefore would never have happened otherwise. Maybe that's one reason why these systems tend to be found at the start of companies: more conventional approaches wouldn't have worked at a stage when hiring a team wasn't an option yet; and on the other hand, the teams you do hire at the later stages tend to have more conventional engineers whose minds (or rather, whose conditioning) can't grok why you'd ever have built something so weirdly.

Usually the later engineers bond with each other around how much they dislike the 'legacy' system, and lobby to replace it, and usually get their way in the end. That's a cycle-of-life thing, but it's also sad when we prefer our conventions and assumptions over seeing, and honoring, the creativity in what came before. Sometimes masterpieces get paved over because of that. Of course, not every nightmare system is an unappreciated masterpiece. But some are, and the bias in our industry is extremely strong toward perceiving them as nightmares. I've seen people take chainsaws to masterpieces without realizing that that's what they were doing, and it makes me sad to think of it. But it makes me happy to read what John wrote to honor Lee, because that's how I'd want someone to write and feel about something I'd built, if I built something like that.

Oh, you reminded me of something fun. I had a lot of freedom under Lee when I first joined Cloudflare (that's partly how Cloudflare became a Go user) and one of my first jobs was writing a new WAF service... I made an entire working version in Arc. I almost made all our customers write rules using it.

Yes, in some similar situations, some programmers will see things that way. But have some faith and respect for individuals' abilities to reason and apply their experience. As you implied, these kinds of systems are often optimized so that one person could run them.

So it's often a trade off — "this would not be easy for a medium or a large team to work on, but it's the easiest thing for me as an individual to maintain by myself that gets the job done." And that's OK. And yes, it is natural to shift over time. But this is precisely because you now want 40 developers maintaining it instead of 1.

Overengineering is real. But architectural tradeoffs that don't work well for large teams are real too.

Just a fun fact: Parts of postgres (before the SQL days, I think) used to be in lisp. To this day we still have some weird function/macro names stemming from those (e.g. lcons).

  * Once upon a time, parts of Postgres were written in Lisp and used real
  * cons-cell lists for major data structures.  When that code was rewritten
  * in C, we initially had a faithful emulation of cons-cell lists, which
  * unsurprisingly was a performance bottleneck.  A couple of major rewrites
  * later, these data structures are actually simple expansible arrays;
  * but the "List" name and a lot of the notation survives.

However, in the example I'm thinking of, it was much more dysfunctional. The advantage of stored procedures there was that the organization's release process was so broken that it was much faster for sales and marketing to get features added by going through Carl, the DBA, instead of submitting the request to the dev team. This was because Carl was able to add the logic by sending one query rather than going through the broken months-long release process.

Carl was actually a real nice guy, although hard to get a meeting with due to his great importance and busy schedule.

I'm sure when you have a brilliant engineer like this you can make it work though.

In practice, the aversion to stored funcs/procs mostly arises from political concerns. Most devs don't understand databases very well, and in most companies, independent DBA teams and independent dev teams end up burning tons of energy fighting for turf.

One of the advantages of a startup is that the smart people are free to utilize the best tools in the toolbox for the problem at hand, rather than having to conform to the social mores around "don't use that tool because it's under the control of the other team and we'll never be allowed to touch it again".

(Tooling could be improved to make the development and debugging process more seamless.)

I mean, if you stick to a rigorous procedure of automatically exporting your stored procedures to files and committing them to git with messages... how is that different from any other development?

Serious question -- I've done tons of database work but only a little bit with stored procedures, so wondering if I'm missing a crucial detail here.

"An elegant weapon, for a more civilised age..."

That being said, there are lots of factors that influence how maintainable a specific stored procedure is. Team size, business needs, company culture, etc. Stored procedures get a bad rep from the places where they are train wrecks. YMMV.

How do you do that with a sproc? With normal application code, you can have the load balancer send an arbitrary percentage of traffic to the new instances of the service. A sproc binds the code and the data together. But you only have one database, so there's no way to do a gradual deploy of the code. That would be like doing a gradual deploy of a schema migration. It's only possible if you have a lot of databases.

Also unless you're using MS SQL server, debugging sprocs is a pain. The only workaround I've seen is to add a debug or loglevel arg to the sproc. But actually pausing execution and inspecting data? Good luck.

> How do you do that with a sproc?

Wrap the real sproc (well, the set of real sprocs, since old and new will sometimes coexist) with a wrapper sproc that probabilistically directs to the appropriate working sproc based on a table value (or anything else the sproc can read) that is updated to reflect the current stage in the conversion process, including any monitoring-based reversals.

By using the DBs error reporting features to report back errors to the application, including information about which ultimate implementation was handling the request.

It's possible to add more epicycles to try and correct for these deficiencies, but the truth is that sprocs are dangerously double-edged. Yes, your code runs next to your data, but to get parity with application code (canary deploys, debugging, logging, etc) you must reinvent lots of tooling.

> there's no way to do a gradual deploy of the code

There could be version numbers?

    some_procedure_v1()
    some_procedure_v2()

Also, for an API schema, only exposing views + stored procedures and hiding tables in private schemas makes the SQL easier to refactor. This is something we recommend at postgrest.org: http://postgrest.org/en/v7.0.0/schema_structure.html#schema-...

Sharing in case this is useful to you.

Thanks for sharing, Postgrest looks like intended for people building a web app, and who don't want to write application server code, instead, they install just a database?

Also, I imagine Postgrest is nice for internal admin apps? then one "just" needs to write Javascript and connect to Postgrest via REST?

Actually, it'd be nice if https://postgrest.org homepage explained some different use cases :-) I had to think for a little while, still not sure if I understand all cases when Postgrest can be useful.

Edit: Now I see there're explanations here: https://postgrest.org/en/v7.0.0/ " Motivation Using PostgREST is an alternative to manual CRUD programming" etc, if I just scroll down ab bit. — I didn't do that until now, I probably stopped reading at the "Sponsors" text & images previously, or I thought it was a documentation page.

This is quite cool: "Creating new views happens in SQL with known performance implications. A database administrator can now create an API from scratch with no custom programming".

It looks a bit like GraphQL also: https://postgrest.org/en/v7.0.0/api.html#resource-embedding

Nice that it's written in Haskell, was my favorite language long ago :- )

(PS. http://postgrest.org redirect to: http://postgrest.org/en/v6.0/ which is not the latest version (& not https), maybe you'd like to redirect to: https://postgrest.org/en/v7.0.0/ instead)

Besides usual webapps, I've also used it for building an IoT project(sensors logic in the db) and a GIS project(postgis/pgrouting). Some users have also used it for open data projects(OpenAPI is provided).

It's also great for internal admin apps and there are ready-made libs like https://github.com/Wellers0n/react-admin-postgrest-client.

Those are some of the use cases off the top of my head. In general, I think it's good for cases where you'd like to use the database as a "processing engine" instead of a "dumb storage".

The docs definitely need a lot of improvement. Thanks a lot for your feedback!

... Maybe that could be a way to "perview" a database migration, before running the migration for real. There could be a table 'Some_table_view_v2' that shows how that table would look, after an upcoming data migration. And then v2 app server code, would use that new view. — Then one could be more certain that the data migration, will work fine.

(At the same time, one might need to be careful to let just a small a fraction of the requests, use the new View, if the view is a bit / a-lot slower than the real table.)

Honestly though, it feels like the argument you are making here is that it's impossible in general to incrementally roll out database changes? Sure, it's definitely not as simple or easy as spinning up new cloud application instances and diverting http requests, but it's not impossible or infeasible by any means. The problem with databases is that the best solutions are always so subjective and context specific. It's very hard to write out simple, easy-to-understand, "rules," or "concepts," or whatever. Every answer, to every hard problem, is "it depends."

What are we rolling out here? Does the sproc write data or read data? Both? From the same table/schema/server/etc? Does it move data around? How much data? Do you already have read-replicas? How is data replicated to those? Do you have any parallel-write setups with eventual consistency? What parts of the higher level application/platform utilize this part of the database that we want to change? Can we spin up a new DB instance, with the new sproc, and establish data replication/change resolution with the legacy system as we are rolling out the change? Should we move everything to the new system and set up the compat piece as a _forward_ compat on the legacy side instead? etc, etc, etc.

>But actually pausing execution and inspecting data? Good luck.

There are plenty of ways to do this. Copy-paste the code of the proc into your DB IDE of choice, and execute it line by line. If the proc is using temp tables or @variables or anything else you should be easily able to instantiate those locally too and see what the values look like. Use a transaction and rollback and you can run this stuff over and over on prod if you really want to be sure of what it does. As long as you aren't locking stuff in the DB improperly.

There's also the idea of writing sprocs in a more testable/verifiable way. Maybe you have progress tracking tables with status id columns and timestamps. Maybe you split up a long sproc into phases which each write their outputs into "intermediate tables" in some sort of "processing" schema. Maybe you write things to files somewhere to be consumed by some federated monitoring system. Idk, I could think of random ideas all day here. A lot of them probably sound dumb at face value, but I really don't think any of these goals sound infeasible.

1. Verbosity and fitness for purpose: while there is a kind of beauty in pure set-based SQL querying, stored procedures by their nature tend to be a procedural paradigm, and in my opinion, they are not particularly well suited to it. They are insanely verbose by today's standards, and doing anything typically requires several times more code than the equivalent in most modern languages (your mileage may vary with different database engines!). This results in large, monolithic SPs, which are difficult to read, understand, and test. The "standard library" is generally far less extensive than mainstream languages, leading to sub-standard, verbose, slow implementations (string concatenations and manipulations are a good example in MS SQL).

2. Code navigation, dependency management: the inverse of point 1 - in general, the tooling around databases for code searching, navigation, etc, is quite woeful in comparison to non database languages. In MS SQL, dependency management is so unreliable, that the official tooling misses several classes of dependencies, just depending on the type of syntax you happened to use. In your question, you start by saying that if you have the necessary tooling already obtained, it's all good. In my experience, rarely are even "barely good enough" tools in place, let alone anything close to what exists in other environments.

3. Unit testing: since databases tend to be stateful, the very concept of unit testing is a bit of an alien one. People don't tend to write SPs with unit tests in mind, and at least in the MS SQL world, the few "unit testing" frameworks that did exist tended to be incredibly painful to use - to the point that they were generally not worth it. Brittle, expensive integration tests are not the same.

4. Execution plan caching: the way databases handle caching of SP execution plans leaves much to be desired - generally the first set of parameters that an SP gets called with end up determining the execution plan for all future calls. Even in well managed code bases, this ends up being an issue, occasionally needing a DBA to manually go in, identify a misbehaving execution plan, and evict it. Vary by param, and no-cache options do not fully solve this. While the need for such mechanisms is obvious, it is surprising that these have been a pain for 20+ years. Now, execution plan caching also happens for normal SQL too, so my point is rather that the culture around SPs tends to have an unreasonable fear of dynamic SQL, which would be far more appropriate _where the query actually is of a dynamic nature!_

I can imagine people replying "but clearly, you're doing things that you shouldn't be - that's not what SPs are for!". And I agree, in a large part that is my point too. But then, what exactly should SPs be used for?

Also it acted like a barrier, the core team who understood the business well worked on the stored procedures, keeping things fast and stable.

Another team was doing the tech to interface with the database and UX/UI was then implemented in various technologies in parallel and changed/got recycled every few years to whatever was in fashion.

The difference in culture between the two teams was huge, it seemed to work out well in their case.

In the database you don't need to do any of this. The data is there. The type information is there.

If it’s the pits, you need a better suited development environment. I use emacs and interactively code inside the DB. Python, Lua, JavaScript, PGSQL, whatever.

1) coding via repl - e.g. one emacs buffer has stored procedure code, send functions over via keyboard shortcut to SQL repl buffer connected to DB to evaluate it 'live' (jupyter notebooks are basically a copy of this mode of operation in a more consumer-friendly package)

2) w/r/t python in db, as OP mentions pgsql, pgsql has support for in-db extensibility using a variety of languages, of which python is one: https://www.postgresql.org/docs/current/plpython.html probably referring to this

As a software engineer, shit like this scares me. I've felt like I'm on a steady, slow, decline for the last ~4 years.

Is it just burnout? Do I need a new hobby?

I used to love programming... Spent 12 hours a day jamming on it. Now, I struggle to keep my mind on a line of code for more than 5 minutes.

At what point is it just burnout, or at what point is it something more? That's what's terrifying to me. I imagine that was a challenge for those close to Lee.

We just know so little about the human body. Our ability to easily query the state of the body, to assess which functional components are working, and which need help, is dismal.

I just hope that Lee, in whatever state he is in, isn't suffering.

For the last few years or so (I'm 33, also a software engineer) I've noticed a pretty big decline in my mental state. I can't focus. I immediately forget what I just looked at, read or did. I completely zone out when people talk to me. I am just not present in any situation. What is strange is that I can actually notice it, not in real-time however. It's been worrying me for the last few months so much so that I started meditating which I've never done before. My wife mentioned seeing a therapist because she thought maybe I was depressed so I scheduled an appointment but it's been rescheduled due to the virus.

I keep hearing about other programmers experiencing similar issues (yourself included) and I wonder if the nature of what we do somehow damages our brain in some capacity and we just don't know it yet. Obviously Lee's case is different but it's certainly scary. I hope he doesn't suffer as well.

On the other hand, after a long and tiring coding session just talking normal stuff gets cumbersome but that, I figure, is just tiredness and passes soon enough.

There's so much shit bombarding us every day, all engineered to provide a dopamine fix and I 100% believe it's affecting our brains.

I have to force myself to only check my phone at certain times, and ensure that I don't use it too often. Ever since I have enabled night mode on my iPhone I find myself less distracted and getting better sleep. I also put apps like instagram, snapchat inside folders on a different page so they are always out of my sight.

I'm starting to form a theory that it could be dopamine depletion. [1]

[1] https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2791340/

I'd be surprised if I had a blood sugar problem. No family history of Type-1 diabetes. I'm thin/muscular. Workout regularly (lift+run).

I suppose it's possible, but I would be surprised. Human body is a weird thing.

I tried reading the article but can't make too much sense of it.

"What makes you work hard for a big (but delayed) reward, when you could just take the easy (but smaller) reward?"

I think of my current state as, always seeking the easy reward. I utterly lack the motivation, energy, and focus, to pursue prolonged difficult tasks (like good programming takes).

When you think about it, we (humans) face this question every day. Why do you get up, and work on hard problems, when you could just sit and watch YouTube? There's got to be something motivating you to sit down, and do the work.

What they found in the study is that if they blocked D1 and D2 receptors, the rats stopped working for the big reward. They would just sit there, and take the small reward. Or, they'd do nothing at all. They became lazy, unmotivated, blobs.

If they supplemented with D-Amphetamine they were able to reverse the effect. Thereby demonstrating, that it's actually the failure to activate D1 and D2 receptors that causes rats to give up on hard work. DOPAMINE is responsible for activating these receptors, and it's these receptors that give you the "OOMF" to get up, and get shit done.

Historically dopamine is seen as the "reward" but that's not really the case. Dopamine is the fuel. It's the catalyst. It's the "mental energy" that gets you off your ass.

So my theory is that, in myself, something is either blocking D1 and D2 receptors... or I've "used up" my dopamine reserves in the brain my continually slamming them with YouTube, gaming, and general computer use. My brain now looks for this high everywhere. Reading a book stands no chance, as it doesn't release anywhere near the dopamine of clicking through YouTube or playing video games.

My baseline is fucked, essentially. So, I am going to begin the hard work of reseting this baseline... No YouTube, no Games. Will report back in a few months.

"Dopamine was originally thought of as critical in the pleasure" systems of the brain. It was thought that dopamine makes you feel enjoyment and pleasure, thereby motivating you to seek out certain behaviors, such as food, sex, and drugs. But then research began to show that dopamine is also critical in causing seeking behavior. Dopamine causes you to want, desire, seek out, and search. It increases your general level of arousal and your goal-directed behavior. Dopamine makes you curious about ideas and fuels your searching for information."

I've also noticed that if I try listening to music and my brain is being very picky with my playlist, then I know that the day will probably be filled with apathy and anhedonia.[2]

How much do you think the instant gratification society that we're living in plays a role in this behavior? The internet has made food, sex, goods, etc so easy to get, which may cause issues with dopamine management that our ancestors didn't have.

[1] https://www.psychologytoday.com/us/blog/brain-wise/201802/th...

https://www.wired.com/2011/07/sapolsky-on-dopamine-not-about...

[2]https://www.pnas.org/content/116/9/3793

Yea, my theory is that for some people (like myself), the instant-gratification society has been terrible for mental health. I have effectively retrained my brain to be stimulated by only exciting + novel things. Things that illicit huge dopamine releases due to their nature being inherently novel/new.

Take YouTube as an example. Pull down on the page, and it's like a slot machine. Page loads a whole new grid of fresh - potentially click worthy - videos!

The eyes/brain scan the page looking for a hit. BINGO you see a video that catches your interest. Click. Ahh, satisfying transition instantly to the video. Boring? Click back at the home page, and start the slot machine again. Rinse, repeat.

That cycle of chance (load the grid) --> seek (search the grid visually) --> instant reward (click the video) is what's so dangerous. Takes almost no mental, or physical effort, to carry out this cycle.

IMO, it's exploiting a very primitive system:

chance (randomness) --> stimulates effort --> get reward.

You will naturally tend toward systems that minimize effort, and maximize reward. Everything else will feel like a giant burden, or will lack a stimulating effect entirely.

You can see this same cycle in things like Facebook, Instagram, Google News, or any video game. Minimal effort required, to capture your attention (effectively blasting dopamine).

In an effort (prayer) to help fix it, I am selling my iPhone 11 Pro for a SE. I am selling my PC and iPad for a small laptop. I am also purchase 2-3 books that look interesting and plan to try reading those.

My goal is to slow down my brains access to dopamine for a time and see where that leaves me.

Just a theory!

I think you just have to retrain your brain to focus in other ways - exercise is shown to improve learning and brain function. There's definitely a relationship between physical health and mental health, and I'm guessing that physical health is an area that a lot of us software devs are neglecting.

The positive part is that experience counts for something.

I have a bad habit of remembering a past that never was. I think, man, the code used to just flow so easily, and now it requires effort. But I'm just remembering the highlights; if I try a little harder, I can start to remember other days when it wasn't so easy. I forget my missed deadlines and remember my last-minute saves. Revisiting old code now and again helps to shake the illusion loose.

Arranging life to spend a little bit less time on things that feel like work and making a little more time for things that excite you is a good medicine for this feeling and probably something everyone should do anyway.

To me it doesn't really sound like he's suffering, especially given the fact that he doesnt really have meltdowns and freakouts. It sounds more like he's just reverting to a state where his behaviors and consciousness coming from the cerebellum/brainstem, i.e. living much more instinctually and in the present, like all other beings in nature. Yes it creates a big disconnect between him and average human society, but at the same time it seems like he's also freed from a lot of the shackles that come with the human mind, free to live moment to moment.

Did he ever express any concern? Or was it entirely other people who noticed his decline, but he was oblivious?

Lots of people around him did. However, I don’t think any of us thought it was a disease. We just thought he was becoming a jerk or “couldn’t scale” as Cloudflare became a bigger organization. I was angry at him when he left. Felt like he’d abandoned this project we’d started together.

I feel incredibly sad and guilty I didn’t even question whether he could be sick until when I heard his diagnosis about 6 months after he’d left the company.

Everything I'd seen Lee do and especially some of the last meetings I had with him at work that were so difficult and I kept wondering why he seemed like such a jerk, all that suddenly made horrifying sense.

I felt so awful for my own reaction to him. And guilty.

I'm by no means qualified to make any actual statements about this case, but I don't think that the ability to reflect on degradation is perfectly coupled with a lack of it.

I've tried getting fit. I've tried improving my sleep. Vitamins, herbs, etc. The only things that work are tobacco(not nicotine, it just makes me sick) and armodafinil in small doses.

At the same time, I think I'm a better programmer than I ever was. The programming abstractions I design are better for years of work with different systems. Also, not being able to fit everything in my head as easily helps me write code that is more modular with simpler pieces.

So I feel like I've lost the ability to do the trickiest stuff, but that is a small part of coding and I'll take the skills in overall design any day.

However I think what's a huge contributing factor is gaining experience. With every structure we come up with, every function and/or abstraction we also build a mental picture of all the possible dependencies it comes with. We see the bigger picture. And we see the same (potential) paths of failure again and again and again…

I found that another favorite activity of mine didn't suffer: Designing electronics. I can still (productively) spend hours on tinkering with SPICE simulations, soldering in-situ projects, and so on.

And recently I also discovered that it's not programming itself that has become harder for me. It is programming in domains I am extremely familiar with that I'm less productive in. If it's in an area I'm not familiar with, things are much easier, because I don't see the big picture yet.

Conversations soon became impossible. Lee started chattering in repetitive, unceasing loops. He would tell Kristin: “We met at Cloudflare. We got engaged in Rome. We got married in Maui, Hawaii.” He repeated it hundreds of times a day. Then the loops got shorter, more cryptic. He spoke fewer sentences, instead muttering sequences of numbers or letters.

At the same time, given the flashes of lucidity pointed out in this article, you have to wonder if others talking about his condition so much might make him feel like a walking corpse when those hit.

I’ve really struggled to wrap my mind around his condition. I don’t think it’s frustrating for him. He seems to have lost the emotion to be frustrated.

I think we all have a sense of Alzheimer’s because we’ve all forgotten something. This isn’t that. Lee’s memory, if anything, seemed to improve and he’d bring up little details from when we first worked together I’d long since forgotten. What seemed to go away was his ability to process those memories into something more.

It’s hard to imagine losing the ability to imagine. And, as his friend and colleague, it was incredibly frustrating when we just thought he was checked out. And then devastating when we learned all this time he’d actually had a disease.

I do wonder if some of his genius came from his ability to shut down some of the other noise in his life. And if the disease, for some time before it became debilitating, was almost a superpower. I’ve never met an engineer like him.

I miss him every day.

I think that you're kind of right: I know one engineer who is like that and he it's his autism which makes him brilliant. He could operate in deep-thought mode all the time - he is extremely intelligent and extremely focused. There are other people who can hit it for patches - I could do it for 3-4 weeks at a time when I was 21. At 39 I struggle to do it for an hour a month (kids change you more than anything else).

We really know so little about how our minds and bodies work and that's something we need to change. We should be able to identify and fix conditions like this.

Makes me think that some of us here are wasting our abilities on start-ups and systems when we could be working on fixing much more complex systems.

"Be kind; everyone you meet is fighting a hard battle."

and Patrick Farley's version from his series "The Spiders"[1]:

"Show kindness to every person you meet. No matter how ill-tempered a man may seem, you have no idea what private agony he may secretly be struggling with."

[0] https://quoteinvestigator.com/2010/06/29/be-kind/

[1] http://www.electricsheepcomix.com/spiders/3.5/01_hospital.ht...

Do you feel like the article was accurate and fair to the people involved?

I got the advance copy of the article last night and it’s been an emotional 12 hours reliving a lot of the last 18 years I’ve known Lee. But I’m happy we helped create something Lee’s two sons will be able to read and see a little bit of what an incredible person their dad was.

Terribly sorry for you and your friends and colleagues’ loss. Thank you and everyone else for doing this for his children.

Thanks again for your work, both on the company and the article.

My prayer is that while this time is emotional, the process and article also provide a degree of healing as well. Again, thanks for sharing.

Yes. I can think of a few examples of this.

Lee bought a few material things, but he was mostly able to live out of his backpack for years at a time, at least before he got married. He didn't have many material desires (no fancy car, clothes, or big gaming computer). He periodically lived on other people's couches (including mine) between starting university and getting married.

I remember he drove through at least a few red lights because he was in the zone deep in thought processing some coding concepts / business plans in his mind while driving. Once I noticed this, I didn't let him drive me; instead he started to code on his laptop in my passenger seat.

When we were waiting in line to rent a UHaul-type truck, he was pacing the retail waiting area thinking through some coding for a future Unspam feature. I remember it because the guy standing behind me saw him as a stranger threat and started to get anxious that Lee was pacing in a hoodie nearby. I mention it because he never concentrated much on the small daily tasks like waiting in line and mostly overloaded those tasks to optimize his long term goals (eg. startup success).

He didn't write big architecture docs (possibly the part of his work that didn't scale much), but his mental capacity to juggle lots of small facts and tasks was pretty incredible. It worked great with a small team with very low turnover. I miss those days when I just checked in with him 2-3 times a day rather than having to spend a lot of mental effort to figure out how to serialize lots of trivial stories/subtasks into Jira.

I met back with them a year later and he was very dependent upon his cell phone to look up people on Facebook, places on Google Maps, movies on IMDB, etc. (basically all nouns) in real time during the conversation to fill in the gaps in his memories. I lived with him for maybe 5-6 years and he had to look up my Facebook profile and pictures to try to pick up on any nostalgia that might catalyze a memory.

I suspect there is an underlying current of frustration, but probably not a high level cognitive realization (at least not a sudden one) that his mind is degrading.

I’m convinced that the genetic effects that provide us with extreme visualization and problem solving skills are related to this particular failure mode, where schizophrenia is also common in the family tree.

I think about this one a lot, how easy it is for pattern recognition to misfire and synthesize conclusions and connections that are partially rational and correct, but with one bad piece of input data skewing them into fantasy. Sometimes the conclusion may be right, but its magnitude, impact, or applicability are grossly overestimated. Part of what makes such gifted people is the ability to imagine, hypothesize, and use hypotheticals. This giftedness often coincides with the ability to make many parallel what if thoughts and identify the most probable. At some point, hypotheticals morph into faith and what was once a thought experiment becomes belief. What ifs break down into belief "this is actually happening, this is reality." It becomes its own compounding feedback loop, as faulty conclusions layer on top of each other over time.

His younger brother, my uncle, was diagnosed with schizophrenia at age 20 after becoming severely erratic while away from home on an LDS mission. The diagnosis devastated their family, and has been followed by decades of difficult treatment and heartbreak.

What is it about my uncle that pushed him over the edge? Environment? Genetics? Pure chance? I wish I could know. My dad says that there's not much left of him except for a few moments here and there.

My dad had a big family, and he's said that his younger brother always showed the most promise, growing up. Smart, charming, super friendly. 30 years on he continues to greet everybody with a smile and hug, even if he doesn't know them!

It's sobering to see it in my own family, and it keeps thoughts like the one you articulated so well in the back of my mind constantly.

"The gist, then, is that someone is “himself” because countless mental artifacts stay firm from one day to the next, anchoring that person's character over time."

I have read reports that latent schizophrenia is often revealed when people travel abroad. Maybe if your environment and surroundings change drastically, your mind loses its sense of "self" that was based around anchors to the rest of the environment.

This journal article touches on this:

"Long distance travel has long been known to aggravate pre-existing psychosis.... Travel can be a destabilizing life event7 with many potential hazards to mental health, from the effects of drugs used for prophylaxis against infection, to homesickness, to disrupted circadian rhythms, to culture shock." http://www.ijtmgh.com/article_33029_738f7bb0f6124261891cf610...

Saying this as someone whose mother scored in the top 1% on the SAT and was schizophrenic.

Schizophrenia is different that it is at least partially behavioral. The brain is more like an adaptive neural network, so it's sometimes hard (or impossible) to disentangle hardware from "behavioral configuration". In the case of schizophrenia I think (not a doctor!) there is a (perhaps structural) disposition toward modes of thinking, psychosis, delusion etc. -- thought and behavioral patterns that develop if there is inadequate self-assessment in place (which is how I would explain identical twins differentially developing the condition), and experiences of stress and trauma that can disrupt benign thought.

It is a terrible condition... I have close family members with psychosis too (that's somewhere in the spectrum of bipolar to schizophrenia). For me it appears quite clear why intelligence has a protective effect (and one of the many reasons I value, and cherish, intellectual activities of all kinds so much).

Counter to opinions elsewhere (where you shouldn't live in the "shadow of the disease" through genetic screening, family history, etc.), I personally do think it's important to be mindful of it. That way at least symptoms may be recognized early and treatment can be more effective (since it is at least partially treatable and has a large behavioral aspect). Here's hoping we never develop it.

This is a beautifully written article but it hit me pretty hard, I can understand the terror having faced it for a few months, I think if I got a diagnosis of dementia I'd head to Switzerland at the point where I still could.

My heart goes out to Lee and his family.

I think degenerative mental disease like the one here is especially traumatizing since to most of the world, you're physically/visually still the same person. Also, the characteristics that formed your personality often morph into these harrowing alternations - in the case of my grandma - she was always immensely active and always on her feet. She now cannot sit for more than 30 seconds at a time and attempts to run away from home (with no particular destination in mind). She was (it feels strange to write past tense here) a great cook but now she enters the kitchen and has no idea what she's cooking or what step she is on. It is extremely difficult to keep her mind at ease at all. Her mind is so unable to focus or string thoughts together that she has resolved to loud abject shouting of gibberish (much like a toddler) since she has no idea how to convey what or how she got somewhere. But then for the briefest of moments she has complete lucid clarity and will ask me about my job and life, and offer to cook me my favorite dish.

Much like Lee's wife, I don't really know when my grandma's last "real" day was and what my next trip is going to bring sadly. It's a sad and slow torment.

I think everyone at Cloudflare was heartbroken when Matthew announced his diagnosis. It was hard to take in.

We love you Lee, you are a champ! You've left a legacy and made the world a better place. Thanks for the opportunity you and the team gave me to work at Cloudflare.

[1] https://www.health.harvard.edu/blog/autoimmune-disease-and-s...

[2] https://www.forbes.ru/karera-i-svoy-biznes/392253-kukushechk...

[3] https://www.shmabstracts.com/abstract/stress-is-the-enemy-a-...

And he was clearly overworked, by my guess.

Overwork leads to a stress. No wonder useful body cells can be attacked producing a whole range of resulting diseases.

The risk of a heart attack also significantly increases when white blood cell count gets high as their presence fuels inflammatory processes in obstructed blood vessels. Eventually this may lead to a full occlusion which is essentially what heart attack is.

[1] https://carrington.edu/blog/researchers-discover-link-mental...

EDIT: I found an email:

Dear Lee Holloway,

Excuse my curiosity, as an Engineer I am not writing this kind of emails a lot, but I have read the blog series by John Graham-Cumming about the company, and it would really help me if you can answer my question about the constant flow of engineering innovations from your company.

I am a security engineer from Europe and would like to know how is it possible, that security team will start producing such a stream of innovative things like Keyless SSL, CFSSL, SIDH implementation, Red October key management server and many more your company already published?

When the "lift and shift" from the open source or vendor stops and internal building start to happen within an organization? Is it a cultural thing where only a small startup can succeed and scale later? Is it about the budget or is it a cultural shift that is non forcible upon them? Is a prerequisite to the innovation, that almost everything is developed in house? That came to me as a contra productive strategy. I have read Skunk Works and other books, but I am still impressed how that happens regarding to the modern leading IT/infra companies.

Thank you for your time,

...

My dad was diagnosed in his 60’s with FTD and declined for years until he passed away in January. It has been really difficult, and even more so for my mom, as she had to deal with him becoming aggressive in later stages of FTD.

It’s an ugly disease, not well-known, and hits people younger than typical Alzheimer’s symptoms would. I’m so sorry to those who have to go through FTD with relatives. It’s just extremely difficult from all angles. :(

If you are dealing with this, whether with the person in the article or someone else, my contact info is in my profile.

While Lee's personality changed a lot over time, he was always the guy who got more out of hanging out in a garage building something than someone touring France/Italy/etc.

Startup != assured success.

> many of the neurologists who study it believe it is underdiagnosed

This is a tragedy. Of course it's underdiagnosed. Everyone around you would just think you're an asshole. In the article, it sounds like so many people fought right and were patient and loving. How many cases do we never hear about, get fought abusively, and push the last shattered remnants of a person into drugs, suicide, etc.

Thank you. I watched that movie with my wife 15 years ago, now, and it remains the scariest, saddest movie I've ever seen.

Are there any remotely promising paths to treating or preventing neurodegenerative diseases like these in the future? Or at least to slow down the progression? Are there any experimental drugs? Will we likely just need to wait until targeted in vivo genetic engineering is understood and mastered?

I know those are broad questions, but suffering from something like dementia, Alzheimer's, or Parkinson's, or having a loved one suffer from it, seems like one of the cruelest fates imaginable. The helplessness of the doctor saying there's basically nothing you can do other than to try to eat healthy and exercise... If I was given such a diagnosis while still lucid, I think I would want to try every experimental treatment available, if any exist. I'd accept almost any risk over that horrifying, inevitable outcome.

Exercise and healthy eating is probably still a good idea, but basically it just seems like hoping for the best without real knowledge of what may or may not be helping or hurting.

Initially found this a few years ago when I was doing a "deep dive" on Cloudflare.

Interesting nonetheless!

Search the NYTimes for "Frontotemporal Dementia", and you'll find several stories, all similar :-(

For me the saddest thing in life is the loss of potential, when someone could have done so much more, actually wanted to do more and was even trying to do more, but then something tragic happens.

My father died when I was 9.

Decades later I watched my mother in law dissolve (and she was very much aware / terrified all the time) over the course of a decade. I think it altered and traumatized my wife's entire family in a way I'm not sure they understand or have dealt with.

Sudden death seems so much more humane.

Those victims likely get written off as a worthless drain on society.

We spend lots of money on medical research but tiny amounts on researching brain illnesses. I'm not saying that we should spend less on other medical conditions - that's not such thing as an unimportant disease, but we should do much more towards ameliorating brain diseases.

The article is written like it is a rare and unfortunate story which we watch from the sidelines, but only the age of Lee makes it unique. Many of us will experience exactly the same with a beloved one ( probably a parent, maybe a significant other).

Every single part of the story except the age, all the changes in the behavior —small and large—, how hard it is to live and/or manage someone with dementia, how difficult it is to come to terms with it, etc, happens to an awful lot of people. It's just that it's not a subject people talk easily about.

Last but not least, doctors are often the worst offenders in the progress of this disease. Most doctors only care about the "now", solve the problem at hand, send the patient home and be done with it. Surgery, psychotropic drugs and other forms of treatment can speed-up dementia progress. Alas, there is no incentive to research, document and act on this.

https://www.nytimes.com/2017/11/02/magazine/why-was-their-br...

IIRC "dementia" is literally just a description of symptoms.

The real truth seems to be that we just barely have the slightest fucking clue most of the time.

I can't help but draw parallels between this story and Flowers for Algernon. It's just sad to see such a young brilliant mind be broken down by this disease.

I wasn't expecting him to be diagnosed with FTD, I will admit that reading the first part of the article painted a very different picture of what might be causing his issues.

Also, since he had a heart condition, I wonder if for some reason that might have accelerated his condition?

Cerebrospinal Fluid Leaks are something that can completely mimic a large amount of FTD symptoms, and even in the care of utmost professionals, can be near undetectable - and somehow, treatment can be as beautifully simple as a one and done injection... to a hellish guessing game and years of attempts. As Dr. Ian Carrol of Stanford's research has indicated, they seem to be highly misdiagnosed/undiagnosed as well.

I know HN has had posts about CSF Leaks before. I truly believe they're one of the worst things we face in this age that can actually be treated with 100% recovery in some cases. However, for far too many, that really means nothing as they'll be sent to a psych facility, nursing home, or become homeless, and die as a whithered husk of what was once a human before they have the slightest indication of what is actually wrong with them, let alone make their way to one of the few places that truly treat the condition.

So, the short answer is that diagnosis will be difficult, and there isn't really a test, but MRI with gadolinium contrast will show enhancement of the dura, and hopefully someone looking at it can see a leak if it is obvious. There are also cases of leaks being caused by a venous fistula, which does not image well.

For what it's worth, I had back-of-head-and-neck headaches intermittently for a while before this, most notably when riding rollercoasters (which I do miss). That is the only long term subtle 'sign' I can remember.

Hope this helps.

Edit: I should also probably add that my case is somewhat weird, caused by a bone malformation in my thoracic spine and difficult to localize with imaging. I had a T1 laminectomy to correct this. However, I'm not sure there are really 'standard' cases, except those that are iatrogenic due to LP or epidural in delivery.

I have slight regret with not double booking an appointment with Schievink when he personally called me. For some likely completely unreasonable reason, I feel like Dr. Carroll is a bit better of a choice having had a child with a leak. Had an appt with Carroll a small ways out - was in San Diego for strange reasons at the time - was going to be gone by the date of the appt and have to fly in. Schievink very much thought I had a leak from history and others diagnosis (EDS & POTS) - but thought I may be in better hands with Stanford as I already had an appointment - the main reason I went after Schievink was because I was hoping to be seen before I left SD. For some reason, I made the choice to agree with him and stick with only Carroll.

Anyways... A ton of unfortunate things fell thru with some misscheduling at Stanford, and I'm still on a list to be seen by Carroll :(

While both doctors think it's likely that I have one, which is something they've both said they typically don't say over the phone, either way - I see Dr. Carroll as an absolute hero in these strange times we seem to be living in. As selfish as it sounds, I can only hope that man pioneers research into this for the rest of his life. Being on the receiving end of at least one (possibly two with CSF) medical diagnosis in which there's less quality researchers/surgeons in the Western World than can be counted on one hand, you grow to have very strange ideals as to what's truly important in life.

I haven't interacted with Dr. Carroll, but my general impression of Dr. Schievink was very positive. Everyone in the whole process at Cedars was awesome. That said, I do still have some lingering issues recently that superficially seem spine-related, despite being assured that my saga was over. It has definitely been an improvement, though, relative to pre-surgery.

I'm sorry to hear to you're going through all of this, but there really is light at the end of the tunnel, and I hope you get there sooner than later.

If you have other questions, you can email me at my HN handle @ gmail and I will try to help as I can.

Dr. Ian Carroll is arguably the best resource in the world. Starting here is probably a good choice.

https://profiles.stanford.edu/ian-carroll

https://www.mdedge.com/neurology/migraineresourcecenter/arti...

https://youtu.be/QyvWxobqKrc

https://youtu.be/g5lsFIDzazc

No idea why I currently can't find a non walled version of the first link. It's important - and general googling would be "when to suspect a CSF Leak Ian Carroll"

Secondary to Dr. Carroll

Dr. Wouter Schievink at Cedars-Sinai also has good information... He's actually the pioneer of research IIRC.

Saved a dude from a diagnosis of dementia and spending the rest of his deluded days in a nursing home.

https://www.nytimes.com/2017/11/02/magazine/why-was-their-br...

IIRC - in the end, based on decades of patient history, he just opened this guy up as a last resort and nearly missed - but did find the leak. Near full recovery.

It seems to be that there are some very high risk groups, like those with connective tissue disorders - specifically, Ehlers Danlos. Also, from Carroll's findings, a lot of people diagnosed with POTS are often misdiagnosed and truly have some small CSF Leak. Ehlers Danlos and POTS often go hand in hand as well...

https://www.mdedge.com/neurology/migraineresourcecenter/arti...