You don’t have aHUS any more. Well you have aHUS but not for much longer. And no you won’t be cured. You will be a patient with the “DISEASE FORMERLY KNOWN AS aHUS” You will become a Complement “whatever” TMA patient.
This has already started for some. Look at the title, and then the first sentence of this article about CM-TMA , aka aHUS.
The 10 patients included are now called CM-TMA patients. But that means aHUS. It is the Mayo Clinic that has decided to change the name. The aHUS patients themselves may, or may not , know about the change of their disease name. But among their peers the clinicians have changed the name.
But different clinicians are making different changes to the name. Confused? Many non specialist clinicians will be. They will not know what is happening.
This has happened before. About every 30 to 40 years during the past century. The same disease gets called something different. It is now time to change again. So it is not surprising. It has been coming for a while. aHUS has not been a good name for what it is for a while now . Not helped by a reclassification of different diseases seven years ago so that when someone says they have aHUS they need to explain what aHUS they have.
The term “atypical” is vague and too broad. It no longer means “not typical ” as it did when it meant “not HUS caused by E.coli”. It is not a good name for most when it comes to a rapid diagnosis that is needed. It just describes symptoms which not all patients present with. It is does not say what is causing it, just the results, which are the symptoms too in other diseases.
So it does not name the cause, and, neither does it mention the serious mechanism which makes it a medical emergency .
The serious mechanism is thrombotic microangiopathy (TMA) a rare condition that demands urgent care as would a heart attack or a stroke. Although rare there are several kinds of TMA each with different reasons for the TMA to manifest.
aHUS patients are told that it is Complement which causes their disease. Or more importantly the lack of control of Complement which cause the excessive damage to self that the excessive TMA initially tries to repair. Hence Complement mediated TMA ( CM-TMA) . But excessive is not good and organ damage follows. In aHUS the kidney organ is specifically mentioned ( uremic being a condition of a kidney problem). But CM-TMA can damage all organs.
When and how it is being decided by a group in an opaque way as far as patients are concerned . Patients included was not a pillar of patient centricity in its way of doing things.
And so WHEN (not IF) the name aHUS is replaced, welcome to the “house of fun”.
aHUS patient advocacy is but a minor part of the overall aHUS care industry surrounding the disease.
When the name aHUS is no more, aHUS advocacy will face its own existential crisis. Having heavily invested time, effort and resources to be a conduit for aHUS patients and a beacon of “aHUS awareness”. All that will fade away, bit by bit, diminished and increasingly invisible to the next generation “CM-TMA” patients. Maybe that does not matter to the bulk of the patient community.
There is another scenario too. aHUS may be divided up among different diseases, named differently and each needing their own advocacy. Patients, once they know what type of aHUS they now have , will need to chose the advocacy which is best suited to themselves. See the table at the end of the article to see to which you would belong and what your aHUS could be called.
After eight years of aHUS Awareness Day supporting, to have a ” aHUS is now a “whatever” TMA Awareness Day” does not have the same ring.
aHUS awareness is no longer needed. You don’t have aHUS any more.
Table of possible disease names and advocacy groups needed
|1. Anti FH anti bodies||Yes||afacTMA|
|4. Auto immune||No|
|5. DGKE||Probably not|
|6. ADAMTS auto||No|
|8 ADAMTS variant||No|
|14. THBD variant||Yes||ThcTMA|
|15. Cancer||Probably not|
|17. PLG variant||Yes||PLGcTMA|
|a. Pex responsive||Yes||PexcTMA|
|b. Eculizumab responsive||Yes||EcucTMA|