Nov 16, 2024
The Bay Horse Tavern is lit once again
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The Bay Horse Tavern is lit once again
wheresonichedgehogwnt replied to your post “I start radiation in the next 30 days (well he said ‘within the month’...”
This is going to sound weird, but thank goodness your doc has a panel of their peers to take your case to, because that sounds super complex and sometimes it takes twenty opinionated experts citing papers and smacking each other down for three hours to come to a good decision in complex cases Which doesn't make it any less frustrating for you, but I'm glad a bunch of experts are going to have to come to a hard-fought consensus for you, if that makes sense?
Yah, it’s the nature of the disease though. Like no one with SDHD or SDHB would be surprised to have this happen:
So far I have been discussed amongst: a panel of endocrinologists (within the state and Australia-wide), a team of vascular surgeons, a team of ENT surgeons, a team of a neuro/vascular/ENT surgeons (each the heads of their respective departments and taking point on my case) and registrars, and now a panel of radiation oncologists (and presumably their registrars).
Like, for this condition there is:
- No grading system for the severity of the disease. (Not recognised globally or per country).
- No understanding of how the genetic defect affects the body outside of the tumours. ‘Surely Succinate Dehydrogenase defects re: the ATP cycle is bad but there’s no funding for that so just...tell us your symptoms and we’ll note them down and be unable to confirm if that’s the defect or not cheers.’
- Not being able to determine if the primary tumours are benign or malignant. Latest science says they are now all malignant because of the way they behave and the complexity of treatment, even in the absence of metastasis.
- No consensus on whether it should be called ‘cancer’ or not (now leaning towards ‘cancer’ - but not all my specialists agree. Surgeons call it cancer. GP calls it cancer. Endo says ‘well we don’t know yet because the paperwork isn’t official’ and RO says ‘um.’ Differs by country and field of speciality. Oncologists say ‘it’s basically cancer, but also not quite, but basically, but not - but we treat it like it is, so...baSICALLY...’)
- No understanding of why some people’s conditions metastasise and others don’t. As in: no determining risk factors, and with no grading system, no way to go ‘stage 4 has X chance whereas stage 1 is X.’
- No understanding of why some get tumours younger and some don’t, they think it’s down to gene penetrance, but they’re not sure.
- No global agreement on treatment protocols (even on surgery vs. radiotherapy vs. lutate (chemo) vs. abstaining).
- No agreement on the basic testing protocols like should it be plasma metanephrines, 24 hour urinary metanephrines, or dopamine / tyrosine tests? (Recent leanings is plasma metanephrines, but many doctors prefer urine despite accrued case studies indicating it’s no better and more inconvenient to patient).
- No agreement on how to respond to hypertensive crisis as caused by shoving a scalpel near tumours that dump fucktons of cortisols and adrenaline into the body if you so much as look at them wrong. Some surgeons like beta blockers. Some surgeons like intra-surgery management. Some surgeons like nothing at all, believing it’s too rare for the risks of beta blockers.
- No agreement on what kinds of surgeons should be taking point on the surgery. Often leading to gross surgical mismanagement across the world, as say, a vascular surgeon or gastrointestinal surgeon takes something that should be at least partly-managed by an endocrinological surgeon who understands the treatment protocols for hypertensive crisis as caused by paras and pheos. It can take months-to-years for the body to adjust to a para/pheo being removed, for example, and that often needs hormonal treatment. Especially in the case of pheos where someone has SDHB. Let’s not even get started on SDHD / C etc. where they know even less.
- No agreement on if tumour removal should be followed by radiotherapy, and no agreement on how to determine if someone is in remission. (Pro-tip, if they have the genetic defect, they never will be - but ten years with clear scans means you don’t have to have scans for the rest of your life. Sometimes. Not in Australia - no wait, not in Perth).
Oh and finally:
- No agreement on how often surveillance scanning should be done or when it should be started if you have the genetic defect. Some say from age 5, to have whole body MRI every two years. Some say from age 8. Some say from age 11. Some say every six months, one year, three years.
Once you have active tumours, there’s:
- No agreement on how often surveillance scanning should be done or when it should be terminated. Some say it should never be terminated (the prevailing opinion). Some say 3 months, some say 6 months, some say 12, some say 2 years, some want a varying scale
OH AND ACTUALLY FINALLY:
- No agreement on what kinds of imaging should be done. In Western Australia, it’s PET scan every five years with Octreotate, and for me a whole body MRI with gadolinium every 6 months (more regularly right now) for the rest of my life.
Elsewhere in the world (largely funding / machine dependent), it’s CT with contrast only, or only MRI (PET and MRI is considered superior as a combination, but a lot of places won’t fork out for this). Or MRI with no contrast. Or PET with non-DOTA chelators.
...
And so it goes on.
*
Even in the US with their shitty, shitty healthcare, people with Paras/Pheos who have the genetic defect are usually sent to major centres of research to get involved with trials and multi-disciplinary teams.
It’s not uncommon to actually be allowed to conference call with your teams, because there’s so little consensus at every step of the way that you might as well be fucking involved. The specialists tell you to self-advocate, and they say: ‘you will have to explain this to everyone who doesn’t know about this disease.’
Sucks if you don’t want to do that because you’re being crushed by the disease in the first place.
Doctors common responses: ‘Para what now?’ ‘SDHD - what does that stand for?’ ‘Oh, so it’s...benign? No wait.’
*
At the 2017 Symposium on Para/Pheos in Australia, the world’s best specialists all flew to Sydney and basically argued diplomatically with each other (along with the patients, who were invited to the Symposium alongside specialists at a discount, because there’s so few of us that we’re often all intimately familiar with our specialists and we’re almost all in fucking case studies lol like ‘oh that’s Darren from Pacak’s study right? Oh yeah, I remember talking to him about X doctor regarding this study and didn’t he shit talk that trial...’) about the situations as they present themselves.
Sometimes they come to good decisions. Sometimes they don’t. Sometimes they throw their hands in the air and say ‘twenty people in the world have this type of tumour and you have it in an entirely new way so we just don’t fucking know.’
The joy of rare disease.
Oh hey, it’s rare disease day tomorrow! *thumbs up*
Rare diseases suck. I have been a case study already. I have been a ‘how not to do things’ case study (re the first surgery) and a ‘how to do things right’ case study (re the first surgery, LOL).
It sucks to have panels discussing your case. Truly. Like on the one hand yay people get to learn things!
On the other hand, boo they know almost nothing about my disease or how to treat me because there’s hardly any peer reviewed research that might increase my survival and quality of life odds that isn’t based entirely in a minimal number of patient studies and the statistics are lacking and highly variable.
hey?!! just listened to songs of a lost world in its entirety for the first time. it’s so fucking good i can’t really process this & it’d be too overbearing to text my friends with all my feelings about it ahhhh
MY CARRD IS COMPLETE
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After Decades, the Air Force Retires 8-Inch Floppies From Missile Control Systems
After over 40 years, the Strategic Automated Command and Control system (SACCS) no longer runs on venerable 8-inch floppy drives from circa 1972. As of this past June, the entire system has moved to a “highly-secure solid-state digital storage solution.” That’s according to Lt. Col. Jason Rossi, commander of the Air Force’s 595th Strategic Communications Squadron. The federal government, for obvious reasons, declined to say much about the exact methods it uses to network the strategic operations center for the ground-based nuclear arsenal.
The status quo, however, could not have endured indefinitely. Back in 2016, a GAO report drew attention to the fact that the SACCS was still running on an obsolete IBM Series/1 computer, which was first developed in the 1970s. The United States Air Force has defended the system in the past, with references to how its obsolescence can actually be a strength in certain ways. This is true. A system that isn’t connected to the internet and doesn’t rely on anything resembling modern hardware could be harder to penetrate in certain regards. At the same time, however, the age and difficulty of securing replacement hardware mean that the Air Force was previously forced to repair components rather than replacing them. Below, we see a group of USAF personnel attempting to breathe life back into a damaged unit.
Defense News has a detailed discussion of how the USAF uses civilians for repair work while airmen handle the component diagnosis and system repairs. If you’re thinking “Wait, I thought we just junked all those floppy drives,” it’s not clear that the rest of the System/1 systems went out with their floppy, eight-inch…. disks.
Look, it was either the above or a joke about a “SACCS of disks.” Also, I’m actually an eight-year-old with a college degree and an unusually large vocabulary. Photo by Wikipedia
According to Defense News, Lt. Colonel Rossi only acknowledged that the Air Force was seeking an overall replacement to SACCS, not that it had already replaced the entire system. How’d they manage to equip the machines with a solid-state storage solution if they haven’t actually retired the computers themselves? I’m genuinely not sure. However, one thing I am sure of is that this is eminently possible. The Series/1 computers supported a substantial number of I/O ports, including standards like RS-232, also known as a serial port. I can’t find any literature on anybody hooking an actual SSD (or, let’s be honest — a USB thumb drive) up to an IBM Series/1, but people have found ways to interface solid-state storage devices with the original IBM PC.
It only took the entire day but now I have a SSD hard drive inside my IBM PC 5150! Big thanks to @ne1for23 for making a 360k boot disk! pic.twitter.com/UNewcswkqq
— FozzTexx (@FozzTexx) July 23, 2017
To be clear, I’m not actually claiming that somebody jerry-rigged a generic USB thumb drive up to a 42-year-old IBM Series/1. I’m saying that the Air Force is clearly still concerned with the maintenance of these old computers (based on the Defense News article) and that it may well be perfectly possible to connect solid-state storage to a computer that’s this ancient. Hopefully, the replacement procurement process is a bit more formalized than a hobbyist project.
Now Read:
The United States nuclear system still runs on eight-inch floppy disks
Semi-Autonomous, Nuclear Decommissioning Robot Sees With Microsoft Kinect
Japan Removes First Nuclear Fuel Rod From Fukushima Power Plant
from ExtremeTechExtremeTech https://www.extremetech.com/computing/300543-after-decades-the-air-force-retires-8-inch-floppies-from-missile-control-systems from Blogger http://componentplanet.blogspot.com/2019/10/after-decades-air-force-retires-8-inch.html
After Decades, Rangasthalam Creates An Unique Record!
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After Decades, a Death Sentence Depends (a Little) Less on Where You Live
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