Is there such a thing as "Atypical HUS Free" or "Remission periods"

[Bill Biermann]

Hello All,

 

The Foundation and the Scientific community are currently wrestling with the following topic:

 

DOES ATYPICAL HUS GO INTO REMISSION DURING silent episodes?

 

First, let me state that there is not overwhelming evidence to "Prove Anything "  definitively at his point.  Through the years, we have heard from adults and parents who may have said  "WE had one episode"  or  "we have years in between".   The tests to "prove" this point are simply not sensitive enough to answer this question yet. 

 

A brief or long "remission" preiod may lead one to believe that the  complement activation process can "lie dormant", and then a trigger activates the whole process, and it settles down again..    This may be true in some cases. Perhaps a certain Mututation does allow the Complement Cascade to settle down, while others do not.

 

However, i wanted to point out that there is some growing evidence that the Compliment activation process may stay active, even in silent episodes.   if that is the case, small,  unoticed "episodes"  are occurring, and their cumultuve effect may add up over time.  In fact, perhaps "major episodes "  are nothing more than a bunch of small episodes that accumulate over time. 

 

Perhaps certain mutations do settle down, and some do not.   I will state this,and it is simply from my own personal, non medical background.   It appears to me that Factor H  and Factor H Related cases seem to have ongoing activation and irecurrance, but the science behind that will either prove or disprove that statement.

 

Meanwhile, i think it is a topic that needs to be considered between your physicians and yourself when considering Treatment options.  

 

 

[debbie thelwell]

At present we feel  our son is in remission stage, he had his first episode 3 years ago and we havent had one since most of the time he doesnt have a protien leak unless he is ill, then he can. This makes me think he is in remission and awaiting the chain reaction to  trigger  off the complement proccess. We are making the most of him being well and crossing our fingers it doesnt come back again soon.

[Carrie Woodruff]

I am 38 and have been living with aHUS since I was 4.  I haven't had any significant problems since I was 8 but I will be going through some tests in the next few months to see if there are any significants silent effects going on that I am not realizing.

[Carrie Woodruff]

I am 38 and have been living with aHUS since I was 4.  I haven't had any significant problems since I was 8 but I will be going through some tests in the next few months to see if there are any significants silent effects going on that I am not realizing.

[Jessica Olivia Frysz]

Well... I can say that  my Atypical HUS seems to be controlled. I'm not in remission by all means, but when I have an episode is usually when I'm given a new medication, or a medication is changed around. But, my AHUS has been well controlled over the years, since being diagnosed in 1989.  I haven't really had broken RBCs, that's usually a sign that I would be having an episode.

[debbie thelwell]

I'm really interested in this theory as my son has been mildly Ill before and after Xmas with slight protein leak, pale, lack of energy and low fluid intake and out put, after two days of rest he seemed ok. With mcp mutation i thought we were in remission will be discussing the possibility of complimentary activity still taking place with our Dr next visit. Anyonr else's children suffer similar low key symptoms?

[Cheryl Biermann]

I am not a medical expert, but because he has the main symptoms of flaring up, lethargy, pale, and protien leaking I would definitely say this is a mild relapse.  My daughter has MCP but is asymptomatic to date, (if you recall my son, Nathan has had kidney transplant with Soliris and has mutations of both Factor H related deletion and MCP), however, if I saw my daughter behaving this way, I would be knocking on my nephrologists' door and if she had protein leaking, I would be talking to them about Soliris.  Don't wait, it might resolve, then again it might not and anyway, permanent damage may be occuring in these slight blips.  Waiting has always been a risky business for us.

[debbie thelwell]

Thanks he seems ok to date but will defiantly take on board your comments were still trying to get to grips with living with AHUS he gets really upset going into hospital so avoid if possible. Thanks as always this website is a great support and wealth of info
London was a powerful day both patient speakers moved me to tears I just hope people listen and the changes put forward for a specialist service and treatment go ahead fingers crossed

[Cheryl Biermann]

How old is he again?  With all the kids when they start very young, like Nathan, they seem to take it in stride, but there are things you can do to help him over the trauma at home.  Take pictures when you are there, talk about what happens in those areas, window shop in the gift shop. (our hospital gift shops are notoriously high priced), we got to know the gift shop manager so well, when we do come in for gum or something, she usually makes it a gift for Nathan! 

If it is a children's hospital there are usually art in the halls, take pictures or take a tour to see all the art.  Ask him about his favorite items.  My friend's child was so traumatized that she always went to the dollar store (do you have those?) before and picked out an item with her child to buy, if she cooperated with the staff, when they went home, they swung by the dollar store, and after a couple of times, Haley began saying, Mamma when are we going to the hospital again, I need a new toy!

Don't forget the wonderful Child Life Specialists who help distract and entertain while scarry things are going on.  When you know your coming in ask for them to visit you during the critical time, (wouldn't it be nice if they were there when you were leaving home?!) 

debbie thelwell said:

Thanks he seems ok to date but will defiantly take on board your comments were still trying to get to grips with living with AHUS he gets really upset going into hospital so avoid if possible. Thanks as always this website is a great support and wealth of info
London was a powerful day both patient speakers moved me to tears I just hope people listen and the changes put forward for a specialist service and treatment go ahead fingers crossed

[debbie thelwell]

Hi guys
Josh was sick at school on Thursday so Friday morning I spoke to the hospital as I was conserned there might be low level activity going on as he hasnt been 100% for a few weeks He spent the day on the ward whilst we waited for his blood results which I'm pleased to say where ok. Phew
He had gas and air when he had his bloods done this time and desided it's happy gas every time from now on so that's a relief.

Debbie x

[Cheryl Biermann]

that is a really good idea, Carrie.  Our biomarkers aren't very sensitive at
 this point though but it has been a good long time since you've had a major issue, do you have a list of the tests theyi are doing?  They think complications could be neurological, (even sleep apnea), problems with eyes, and all major organs.Carrie Woodruff said:

I am 38 and have been living with aHUS since I was 4.  I haven't had any significant problems since I was 8 but I will be going through some tests in the next few months to see if there are any significants silent effects going on that I am not realizing.

[debbie thelwell]

debbie thelwell said:

Hi guys
Josh was sick at school on Thursday so Friday morning I spoke to the hospital as I was conserned there might be low level activity going on as he hasnt been 100% for a few weeks He spent the day on the ward whilst we waited for his blood results which I'm pleased to say where ok. Phew
He had gas and air when he had his bloods done this time and desided it's happy gas every time from now on so that's a relief.

Debbie x