I Don’t Know What My Disease’s Name is Anymore

Patients were ghosted in the rename process and I’m mad.

Nobody likes being ghosted. Dear Reader, I know this post might be in-the-weeds of my aHUS community, but I’m mad and I want to share my thoughts here even if no one else wants to listen.

I spend a lot of time saying our patient voices matter - and nothing makes me more disappointed than watching in real time when they are discounted.

I wrote in September about the efforts around the renaming of my disease. Here:

When Your Disease Has a "Bad" Name, Should Patients & Caregivers Be Part of the Discourse?

After the article, the leaders of the renaming project at the National Kidney Foundation agreed to meet with patients. Len Woodward at aHUS Alliance worked on the scheduling and then… crickets.

And more crickets. 

Today, Len posted an article which I recommend titled All is quiet on the aHUS naming front.

The article outlines the challenges as well as the long list, that feels more like a Cheesecake Factory menu of acronyms. 

So instead of aHUS, I don’t really know what my disease name is.

It might very well be Pregnancy-induced Complement-mediated No Known Gene Thrombotic Microangiopathy!

OR PCNKG-TMA - REALLY ROLLS OFF THE TONGUE.

A reminder, Thrombotic Microangiopathy or TMA means tiny blood clots, the hallmark of aHUS and the common cause of kidney failure in patients.

Despite this being a ridiculous acronym, I see other inconsistencies.

For example, HELLP is a Pregnancy-Induced Thrombotic Microangiopathy (P-TMA) - which already throws doctors off course. They thought I had HELLP at first. 

But now, they suggest the names should also have “fluidity” to address the changes in the disease WHICH FEELS LIKE THE OPPOSITE OF PICKING A NAME. 

(Sorry for the all-caps, but I feel anger).

So I might have been P-TMA at first, and then after genetic testing I’d be PNKG-TMA, but now that I’m not pregnant - I have CNKG-TMA ?

This isn’t a disease name. These are descriptors of a fluid situation.

I have to live with this name. Every day. For the rest of my life.  And my valid concerns are being ignored.

If you want to pick a “fluid” name, I suggest you’re picking no name at all. 

A new disease name opportunity is rare. It could be a change to help save lives, create a faster path to diagnosis - but to not consider or listen to patients and advocates is a missed chance to make something that works for everyone.

I can’t google PCNKG-TMA. No one is fundraising around PCNKG-TMA. And there is no community for PCNKG-TMA.

Why not choose a name that has a pathway to support and community too? 

I am saddened this new naming pushes people with a very rare disorder into tinier and tinier silos. 

Any new patient with a diagnosis with the new nomenclature will be even more alone - and that’s a scary place to be when you’re already in a scary, rare place.

Additionally, the experts involved in the new naming also don’t all agree with this naming methodology. So in my mind, this doesn’t sound like a process that is working.

Someone hire me to project manage. 

To ignore patients and silencing our voices, when we are the most impacted is profoundly disappointing. 

So far the whole process has been totally murky, but now it also feels disrespectful.

I want a name that saves lives and brings us together with clarity.

For now the biggest barrier, besides implementation challenges, is money. NKF wants more money to continue the dialogue and seems to see the name process as complete as far as they are concerned, unless funds are added. 

Happy Kidney Month, indeed. 

Thanks for reading my concerns. At least here, I have a voice.

For now, the situation remains FLUID. 

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If you’re new here and wondering, “what happened to this lady?” read:

I started writing this when I was on dialysis. It’s intended to be both memoir and a practical tool to help folks who might be going through something similar or those caregivers and family supporting someone with a challenging diagnosis. NOTE: This is not intended to replace actual medical guidance. Please consult your doctors on your individual challenges and situations. Please talk to your clinicians before adjusting any of your care protocols. Also names have been changed for most of my medical staff.

Thank you new paid subscriber Sarah Ellis. Your support means so much! Thinking of you and your family! Love to Paul.

Also lbboren!! So grateful for your support!

Thank you to CC Couchois, Roy Lenn, and Dr. Richard Burwick for your founding level donation.