L2HGA in staffordshire bullterrier

Ruby has been diagnosed with L2 HGA.
L-2-HGA (L-2-hydroxyglutaric aciduria) in Staffordshire Bull Terriers is a neurometabolic disorder characterised by elevated levels of L-2-hydroxyglutaric acid in urine, plasma and cerebrospinal fluid.
L-2-HGA affects the central nervous system, with clinical signs usually apparent between 6 months and one year (although they can appear later). Symptoms include epileptic seizures, "wobbly" gait, tremors, muscle stiffness as a result of exercise or excitement and altered behaviour.

Until 12 months ago I had never heard of this condition and we are now going through a distressing time. Not only have we found out my friends two dogs are carriers (unknown to her) but after some digging and research on family tree have found out that her mum & dad have the same Grandmother on all 4 sides. I have found the COI on ruby and she is 23% and both mum and dad are 21% & 23%, (breed standard 7%)

Ruby in herself isn't the same dog, there is no Staffy kisses and fuss. she is on epiphen and potassium bromide to control the seizures (which she'll be on for life), these have reduced in volume since the initial diagnosis.
she gets stuck in corners (part of the condition) and has lots of vacant moments and stands shaking and staring at nothing. her legs give way and she ends up face down on the floor. she struggles to get down the garden to toilet and regularly get stuck behind a plant pot. these last few weeks she has got progressively worse in that the wandering and banging into things and falling are getting more frequent. I didn't think she'd make her 6th birthday mid march. now i can't see past Easter.
please if your staffy is experiencing tremors, fits, odd after excitement or exercise then speak to your vet, there's a chance he/she hasn't hear of L2HGA as our were the same.

If anyone is thinking of getting a staffy pup then check if they've been tested ... if not the link ishttp://www.ahtdnatesting.co.uk/ for the swab test. £48 order on-line and results in about a week.

Thank you for sharing this information with us Jane, and we are only too sorry that you have had to experience this condition at first hand with your lovely little Ruby.

I for one had never heard of this condition until recently, and it is thanks to your courage in highlighting the condition that many of us are more aware of it, and its probable causes.

Bless you both            

Jane, thank you so much for sharing yours and Ruby's pain.

I hope that when people read this, they will see how important it is to only breed from health tested parents.  

Thank you so much for sharing it! You've done an amazing dog and my thoughts are with you and Ruby.

I've shared it on our Facebook page to raise awareness.

Such a sad and scary condition and so important to make people more aware of it. Sorry to hear that you and your little lady have to go through this.