Hey what’s new?
I have found out something new , did you know that aHUS is not the official name for what we have
Wait what? aHUS is not official
It has just been what most people have called it for the past 25 years or so
Well does that not make it official.
No.
Well what would make it official?
Being classified as such with an official descriptor in the World Health Organisation list of diseases.
OK? But didn’t Alexion use the name atypical Hemolytic Uremic Syndrome when it got approval for eculizumab? Is that not official enough?
Yes it did. Having used “Atypical Hemolytic-Uremic Syndrome” in its trials but also the aHUS abbreviation But it was acceptable to the FDA etc as the name that most clinicians would use at the time. So good enough with both parties agreement to use it. It had a specific context. But it did not make it an official name, That would be what the WHO called it at the time.
So nothing official exists?
Yes Hemolytic Uremic Syndrome is the official name.
HUS is the official name?
Yes and it was only three years ago that the official descriptor of HUS was changed to to Hereditary HUS and Other HUS and aHUS was mentioned as being included in those official names..
Just include have never seen those names used and so they don’t matter.
No they do they are the official ones and disease codes are used in medical records and billing Doctors can use aHUS like the rest of their peers do.
How can they do that, they can just call it aHUS?
Yep they can, it is like the name became customary by a kind of “Deed Poll”
Wait what that can happen?
Yes and if they wanted doctors could change it again if enough of them accept a new name.
What they could change it to this complement mediated thrombotic microangiopathy or cTMA name that is being bandied about at the moment?
Yes and many are and with different versions of it . For many now aHUS is almost like the artist formerly known as Prince if you know what I mean It is not official it is a preference. Why is it TMA when it is a two word term. I digress.
But they would not say that to patients .
Nothing to stop them. New patients could be told that they have cTMA. They still would need to use the existing HUS official codes.
But they use HUS in the official name it’s confusing too.
Even some doctors may not see the difference between HUS and aHUS. But even when aHUS is mentioned it means it is included in the hereditary HUS which (includes “Genetic aHUS”) and the other HUS includes “nongenetic aHUS” ;
And no one has ever mentioned this to patients. Where would those who have a genetic mutation be coded but who did not inherit the mutation from their parents? Or those who are tested who are idiopathic.
Not to my knowledge when the codes were changed they did not have to not even to patient advocate organisations . But you ask a good question because in the first case it is not something that one of their parents had but it is genetic change.. According to the official name it would be hereditary because it was genetic. In the second instance it would be “other HUS” , which includes non genetic aHUS
And if someone does not have a genetic test done what then ? Not all do?
Toss a coin?
Time for a lie down this disease naming business is like trying to square the circle
Or what I like to call “impossible geometry”. Can i join you?
Article No. 820
