So what is atypical HUS anyway?

This is going to be a very boring post so feel free to ignore if you have no interest in things medical.

Some of you are probably wondering what is this thing that has nuked my kidneys.  It is one of those things that you learn about in medical school and hope you never really need to know.  Hemolytic Uremic Syndrome (HUS), and it’s sister condition Thrombotic Thrombocytopenic Purpura (TTP) belong to a group of conditions called TMAs – thrombotic microangiopathies.  Basically a series of events cause blood clots in the small vessels of the body.  If the condition attacks your kidneys it is HUS, if it attacks your brain it is TTP.  It is literally a one in a million condition – lucky me.  For some reason most of the research in this area has been done in Europe and there are experts in Italy, France and England that we have conferred with.

HUS is thought to be caused by a genetic defect in the complement system – a group of proteins the are part of the body’s immune system.  There are two kinds – typical HUS is more common in kids and usually develops after eating bad meat that has a certain type of e. coli bacteria.  Kids usually recover although a few may progress to renal failure.

Atypical HUS is mostly in adults and a few really unlucky kids.  In a large percentage of people a defect is found in one of the genes that make proteins that stop the complement system. After several trials and tribulations i was able to get my DNA to italy for testing to see if i have one of the defects.  So far all the genes tested have been normal.  That MAY mean i have a better prognosis that usual.

That is the kicker – people with aHUS do not do well with kidney transplant.  The aHUS recurs in the transplanted kidney.  It is like this lowgrade fire, and the kidneys are the fuel.  After my own kidneys burned out the fire went out, or at least burned down to embers.  Adding a new kidney in the mix is like adding fuel to the fire and reigniting those embers.

That is not to say there is no hope – there are case reports in the literature that suggest a way forward and we are going for it.  This is after much research and talking to experts all over the world.  The first part is plasma exchange.  Here they will  take off my plasma, removing the bad humors that may cause the HUS and replace it with donor plasma.  The second part is the use of a drug called eculizamab.  It is a monoclonal antibody (think designer drug) that stops the compliment system and has the dubious distinction of being the most expensive drug on the market.

So my pre-transplant schedule looks like this: on Friday i get a dose of ivig, which is to stop my body from attacking Sarah’s kidney.  On Monday, i get plasma exchange, ivig and eculizumab.  On wednesday, another plasma exchange and ivig.  Then eculizumab on the big day.

After transplant i will have more plasmapheresis and eculizumab while we watch my labs to see if there is any evidence the HUS is recurring.  Unfortunately the HUS can recur at any time – even years after the transplant.

So there you have it – this is how i ended up where i am and in a million years i never would have guessed i would be here!


4 thoughts on “So what is atypical HUS anyway?

  1. yeah…not so excited about that post! You’re going to scare the bejeebers out of my family. And I think you are just showing off with big words.

  2. Sarah: I’m excited for your journey. Sorry you’re going to have pain but what you are doing is just amazing. Improving others lives is really all that matters. I can’t wait to hear that you & Lisa are both doing great and that Lisa’s new kidney is working! You’ll have a life long bond with her and you get to enjoy it since your a living donor. It is wonderful where life takes us….you were meant to do this! Love u. I’ll call you tomorrow…still hoping to come out to CO or NM.

    • Dawn – I found a job for you! Well, a job helping me… look forward to chatting, and with my new plan, you’ll get to both Colorado and New Mexico. Lucky you! 🙂

  3. Hi Lisa, Am a friend of your Aunt Adrienne, have been following your ups and downs, and want to wish you all the best. My son in law donated his kidney to his Aunt 8 years ago and BOTH are doing phenomenally well. Saunda

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