[metasean]

I first heard about prions and Creutzfeldt-Jakob Disease after getting a letter saying I may have been exposed.

I had surgery after someone with CJD, but before that person died, was autopsied, and diagnosed.

That was over a decade ago. So far no signs of it. I did make peace with the fact that I have a much higher probability of dying from it than the average American. The only substantivu changes in my life are:

(a) My family and I have had very frank discussion about death earlier than we would have otherwise.

(b) I no longer donate blood.

(c) I removed myself as an organ donor. Although my family knows I still want my body to be available for science.

[developer2]

>> My family and I have had very frank discussion about death earlier than we would have otherwise.

It would benefit any family to have that discussion, even if both parents are in their 20s and there has been no "scare scenario". Many people die every single day due to unpredictable events, with no warning whatsoever. Unfortunately, too many people have never really pondered their mortality, leading them to be incapable of even considering what it would mean if man/woman/child died tomorrow. Having a sit-down meeting is likely to be brushed off as a "meh, it'll never actually happen to us".

Any one of us could be dead in the next 5 minutes. That's more reality than paranoia. The trick is to get to the point where you understand just how fleeting life is (you or a loved one might be gone any day now), but without letting it negatively control your life.

>> I did make peace with the fact that I have a much higher probability of dying from it than the average American

You should stop thinking that way. A decade past the initial scare, you're likely no more at risk than anyone else. You weren't given the disease, you were exposed to a small possibility of it. It's still the responsible thing to not donate blood or be an organ donor, but as far as your day-to-day life goes you should consider yourself squarely in the "average" category.

[metasean]

re: Discussions of death

I completely agree that it should be talked about more and plans made. Unfortunately several family members don't see things the same way and this provided the impetus for discussion.

> You should stop thinking that way. A decade past the initial scare, you're likely no more at risk than anyone else.

I'm not an expert, but the last time I researched it, I wouldn't even develop symptoms for at least a decade. So I'm actually at the point where I could actually find out that I have it.

> You weren't given the disease, you were exposed to a small possibility of it.

I absolutely agree! That said, my probability is still drastically higher than the _average American_. I was dating a science teacher at the time and she did some research into it. Basically the only other Americans with similar odds are military personnel stationed in Europe during the Mad Cow scare. There are other populations, e.g. Europeans during the Mad Cow scare, that are at similar or higher probabilities. But among Americans my odds are definitely higher than average.

> but as far as your day-to-day life goes you should consider yourself squarely in the "average" category.

I'm anything but average ;-) but I definitely don't let this impact my day-to-day life. It's much more of an intellectual curiosity and medical awareness for my family and doctors in case I should become symptomatic.

[ekr]

Surely, after a decade it should be fairly easy to detect Prp_sc levels, or at least extract bone marrow sample for examination. That should eliminate beyond any reasonable level of doubt, any reason for worrying about the matter.

[cat199]

Certain categories of people with exposure are to this day disallowed from being blood donors as a result due to the risk and difficulty in testing.

Red Cross rules: " You are not eligible to donate if:

From January 1, 1980, through December 31, 1996, you spent (visited or lived) a cumulative time of 3 months or more, in the United Kingdom (UK), or From January 1, 1980, to present, you had a blood transfusion in any country(ies) in the (UK) or France. The UK includes any of the countries listed below. ... "

http://www.redcrossblood.org/donating-blood/eligibility-requ...

[metasean]

According the always correct source of ultimate wisdom</s> - wikipedia, there isn't currently a way to test for it while alive, but there are some promising areas of research.

>At present, there is virtually no way to detect PrPSc reliably except by examining the brain using neuropathological and immunohistochemical methods after death. Accumulation of the abnormally folded PrPSc form of the PrP protein is a characteristic of the disease, but it is present at very low levels in easily accessible body fluids like blood or urine. Researchers have tried to develop methods to measure PrPSc, but there are still no fully accepted methods for use in materials such as blood.

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

[maxerickson]

The other side of it is that you can't really prepare yourself for the sudden loss of a close relationship.

Having a life insurance policy sized to the needs of children is probably more beneficial than having a frank conversation with them.

[developer2]

>> you can't really prepare yourself for the sudden loss of a close relationship

I wholeheartedly agree. I didn't mean to sound like you can prepare yourself so far as to avoid any mourning when someone close to you kicks the bucket. I do believe that once you've been through 1-3 such rounds of death, an average person will finally "understand" and take future deaths with a grain of salt.

As an aside, I personally find it frustrating when a family decides to hold a closed-casket funeral. Actually seeing death up front and personal is the only way to truly become acquainted. The number of times I've heard "think of the children"... which is really just adults being in denial about their own eventual death, and perpetuating that fear into their children. Death is a natural eventuality for all; the younger someone can figure that out, the better off they'll be.

[peternicky]

Wow, what a frightening letter to receive! Thank you for sharing and best of health to you and your family.

[metasean]

Thank you!

I definitely went through a range of emotions from confusion to anger that more wasn't done to minimize the likelihood of incidents like this.

As I learned more, I realized how improbable this situation was in the first place. My current understanding is that while our* probability is higher than the average American, we're still highly unlikely to develop CJD. More importantly to me is that there isn't anything I can do about it, so I try not to let it consume a lot of my mental energy.

*I think there was a two week window, so anyone operated on at that hospital during that window is in the same situation.

[oh_sigh]

Shouldn't autoclaves destroy all proteins, prion or otherwise? What exactly is the exposure vector?

edit: Unsourced statement from wikipedia[1]: "However, prions, such as those associated with Creutzfeldt–Jakob disease, may not be destroyed by autoclaving at the typical 134 °C for three minutes or 121 °C for 15 minutes.[citation needed]"

[1] https://en.wikipedia.org/wiki/Autoclave

[antisthenes]

In an autoclave, prions can be deactivated by using a temperature of 270 F at 21 psi for 90 minutes. [1]

[1] http://www.bseinfo.org/priondefinition.aspx

[metasean]

> Normal sterilization procedures such as boiling or irradiating materials fail to render prions non-infective.

https://en.wikipedia.org/wiki/Transmissible_spongiform_encep... (The quoted sentence is from the paragraph immediately above the TOC block.)

Remember it was the hospital that sent me, and anybody else who had surgery during the same period, a letter saying I may have exposed. While they followed required sterilization procedures, those standards were apparently not enough to statistically ensure prions were destroyed. I'm not sure if they knew what was required in this regard, because one of the things mentioned in the letter was that all related surgical equipment had subsequently been destroyed.