Episodic Ataxia

Hi all,

My 18 year old soon-to-be-sister-in-law (s2bssil) has Episodic Ataxia. She has had symptoms since she was born, and whilst she has had several different (incorrect) diagnoses in her lifetime (including cerebral palsy) in her teen years the specialists identified her symptoms as Episodic Ataxia.

I have joined this forum as I am hoping to make contact with someone who has suffered with Ataxia their whole life (or from a very young age) as opposed to developing symptoms as an adult.

My s2bssil is a real sweetie and quite quiet. She never complains and doesn't always tell family/friends when she is having an attack. She is around close family the majority of the time, and they can always tell when she is having an attack and take the appropriate steps following this - however, I'm hoping to start spending more time with her and unless we're walking around, I don't always pick up on the fact she is having an attack right away.

When she is having an attack, her speech becomes slower and more heavily slurred (though it is always slower and more slurred than the average joe). Also when she has an attack, her limbs become very heavy and she is not able to stay on her feet... which is why if we're sitting together having lunch or watching a movie, I don't always notice when she is having an attack, and she doesn't always tell me!

When she has an attack we sit her down somewhere sturdy, get her something sugary (like a chocolate bar or banana) and a drink of blackcurrant and after a few minutes she starts to come around - so it is not a distressing experience (for those around her any way, I don't know what is it like for someone that suffers with ataxia) its just something that I know she finds frustrating.

I know that she has a spoonful of cornflower in her drinks (like blackcurrant) which is meant to help reduce the frequency/likeliness of attacks, but I don't know how often she does this (daily, weekly, etc) or how helpful she finds it.

I also know that when she gets excited an attack is more likely (for example, when she comes to visit us she always has one, and at Christmas too).

Basically, I'm wanting to understand what its like for someone who suffers with Episodic Ataxia. As I said earlier, she is a really good kid who is very smiley and loves being with friends and family and getting involved, however she doesn't say much, and she definitely doesn't say much about her condition. Anyone that can shed some light on:

* what an attack of this nature is like (is it distressing? or more of a nuisance, like a migraine?)
* is there anything you find helpful that reduces these attacks?
* anything you would like to share that you think would be useful for me to know.

I love her to pieces and just want to do my bit to be more aware and understanding of what she goes through on a regular basis.

Thanks in advance! Hana

Hi Hana - you’ve come to the correct place. I commend you for finding out more. Everyone’s different, and here are some links to previous discussions on EA:

http://www.livingwithataxia.org/main/search/search?q=Episodic+ataxia

Hi, how fantastic that you want to support your sister in law in this way :slight_smile: I have had EA 2 since I was a child. The first attack I can remember was when I was 8 but I had symptoms before then. Diagnosed at the age of 37 after many misdiagnoses. I find the attacks distressing and often cry afterwards but mine are longer and don’t go without sleep. I have never heard of corn flour reducing attacks!! Sounds strange to me. I am on fantastic medication that has reduced the frequency of attacks with great success but it only works for some people with EA2 and she may have EA 1.
I need quiet when I have an attack and can’t stand people fussing I just want to be left alone as it is so embarrassing for me.
Any questions, feel free to ask. Also on Facebook there is a fantastic episodic ataxia support group - any question there is answered by some wonderful people all of whom either has EA or has a relative with it :slight_smile:
You Re welcome to email me.
Louise

Hi I dont really know much about Episodic Ataxia as my dad had a different strain (which remains unknown) I just had to comment on how lucky your sister in law is to have someone so kind and caring as you for joining this forum and taking the time to find out as much as you can to help her. She sounds as though she has a fantastic family and your post bought tears to my eyes as your love for her really shines through. I hope you can find the answers to your questions and that she remains her happy self, much love and best wishes to you and your lovely sister in law.
Gail xx

Hi,
I was diagnosed with E.A in 2010. Now in 2013 a second opinion reveals Progressive Muscular Atrophy!!!
When I had E.A. I could not feel an attack but during the day there were times I could walk better and enjoyed good balance. No consistent health and altered daily activities. The present is limited activity and I use a walker indoors and wheelchair outdoors. Exhaustion and tiredness are the primary symptoms after an activity and sufficient rest relieves them. Your love will be of great strength to your niece. Good luck.

Hi Hana,

How wonderful that you want to support your soon to be sister-in-law in this way....

I am Dawn Wooldridge age 47, I have had EA2 since birth, although it took docs until I was 35 to correctly diagnose me.... up until then I had many mis diagnosis..... I have a daughter who is now 24 who has also had EA2 since birth......I also have a 6 month old granddaughter who has already been diagnosed with it too......

For my daughter & I , sleep is the only help for us.... at least 8 hours, before I even think about coming out of an attack.... the symptoms you describe sound typical of this condition..... although I must say I have never heard about the cornflour and it's effects and would be interested to know more....

Any change in emotion can cause an attack, worry, excitement, fatigue, so it's important to try & keep calm at all times... but other triggers for me too are coffee, alcohol, bright lights, sudden loud noises.... etc.... to many to list....

I take acetazolomide which many with EA2 find beneficial, although with some it has no effect....

Well Hana, I have told you about me and a little of my famiily..... but the best advice I can give you is sit and talk to your soon to be sister-in-law..... find out what her triggers are and ask her how she would like you to help her....

I get VERY embarrassed when in an attack, as do many..... and I don't like attention drawn to me....

But the one thing I have learnt is that all Ataxia including EA affect everyone in an individual way...... we all have different triggers etc... and different ways of coping..... which is why I suggest you talk to your soon to be sister-in-law....

I wish you all well and as Louise has said there is a facebook page titled "Episodic Ataxia Support Group" which is solely for those with EA, their friends and families....

Feel free to contact me if you wish to chat further or I can be of any other assistance.... or feel free to email me direct too on ■■■■■■■■■■■■■■■

Best wishes to your family

Dawn x

Hi All!

Re the cornflour. Maybe this is to thicken the drink, making it easier to swallow,

and to try and reduce the chance of choking....... ? I know this is recommended

by therapists. xB

I thought the same Beryl. My Dad used to have thickener in his drinks to stop him choking, looked disgusting as if wallpaper paste had been put in his drinks. He hated it so went without a drink if poss (we would on odd occasions sneak him a drink without!) then the lack of fluids were making him worse and water infections would not clear. He never moaned bless him