Nearly five years ago Global Action asked the question “ Is aHUS’s end nigh ?” In a trilogy of articles. ( click HERE for the first of the 3)
Not the end of the disease, sadly that will always be within and among us, but of the term used by doctors to describe the disease and label patients with it.
For nearly five or more years there has been speculation and no one has done more than Global Action to alert the community to this possibility.
In the month after the first patient baby girl Barbara Slammer- Frey was officially diagnosed with hemolytic uremic syndrome in July 1954 , today we have got the answer.
After three years of debate some of the aHUS clinical community, not patients, have published a report on why the name aHUS needs to change, what names could replace it and how patients old and new will be reclassified in the future. That report can be read here.
In short the recommendations are to :
- Drop the names aHUS and HUS completely
- Make the term TMA (thrombotic microangiopathy) the root of the disease name
- Have several generic categories of TMAs
- Within each generic categories have several specific disease names if the science has identified a specific TMA.
- Produce an initial list of dozens of disease names which used to be covered by just one
- Continue to revise that list of names in the future to add further specific disease names when the science permits.
So current aHUS patients will be divided up amongst the dozens of new names and then be told what they now have.
New patients would not be told they have aHUS. And as the science permits further specific names will be introduced and some patients will once more be reclassified.
This will take a lot of time to implement throughout the world as it will have an enormous number of practical issue to be worked through. An enormous number but it “doing no harm to patients” will be of paramount importance.
Eventually this will benefit all patients as the different diagnoses and then the more specific prognosis and treatments will be customised to each generic and specific TMA disease.
If needed, Global Action will do its best to help the patient community through the crossover months/ years. But at the end of the day it will be patients and their doctors to work things out between them.
In a recent survey 40% of respondents said they were aware of the possible change of name and 60% said they were not. And these were patients within the reach of global aHUS advocacy. The vast majority are not within reach so it is not unreasonable to assume that this will be news to much more than 60%. That is tens of thousands of patients and their families.
In doing so Global Action is facing it is own “identity and existential crisis” too, as will all patient organisations which use the term aHUS. It is their “brand”.
Are we TMA or just Complement advocates now?
Maybe each specific TMA group of patients will have to create their own organisations and go their own separate ways.
When the first “post aHUS” awareness day will be , remains to be seen.
That things will not be the same is the only certainty.
Viewers can give the thoughts and comment at the bottom of this article or on the the related social media posts to this blog.
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That’s odd to me. For the last 5 yrs I have called and emailed doctors who are supposed to specialize in AHUS to no avail. I cannot find a doctor yet you want to change the name? Where are all these doctors who specialize in this ultra rare disease? Why not make them available to patients in stead of making matters worse by changing the name of an ultra rare disease they know nothing about. I’m confused how people are more worried about the name than providing quality care to patients. Whoever came up with this dumb idea should create a data base for patients to interact with doctors. Show me you really care about the aHUS community. What good is changing the name when the patient dies from the disease? Everything that glitters is not gold. Sometimes good intentions turnout to be bad decisions. This is a bad decision. Have a better structure, foundation, and understanding of the disease before you change the name of something. Changing the name gets me no closer to a doctor who specializes in aHUS. It just adds more obstacles and hurdles.
Hello Reggie,
Since aHUS varies so much, physicians cannot answer patient questions about health in a simple manner, which may explain why they haven’t provided answers. Our directory is set up so physicians can transfer medical records safely, then discuss how best to move forward with treatment. With only 2 to 9 aHUS patients per million people, few doctors will ever see a single case of aHUS during their entire medical career so this directory provides free consultations to assist local physicians with expert advice. Hope this helps.
Here’s the link to the directory referred to above:
https://www.ahusallianceaction.org/ahus-clinicians-investigators/