The hidden disorder in staffies

Have you heard of the neurometabolic in stafforshire bull terriers, commonly called L2-Hga (L-2-Hydroxyglutaric aciduria)?

I didn’t either, until I read Jazz’s story.  This disorder sees elevated levels of hydroxyglutaric acid in urine, plasma, and cerbrospinal fluid.  L2-Hga has affects on the central nervous system, and symptoms ususally occur 6-12 months old.  The symptoms normally include uncoordinated movement and epilepsy like behaviours.

This disease is found on one gene that is autosomal recessive, so easy to breed out if breeders DNA test and breed only carriers to clear dogs, and aim to produce clear dogs long term. Unfortunately, not all breeders are committed to this cause.

Wildbunch Knight in Amor ("Joker") is not L2-Hga affected - but boy is he cute!

Wildbunch Knight in Amor (“Joker”) is not L2-Hga affected – but boy is he cute!

This is Jazz’s story, written by a staffy owner in South Australia.

My husband, John, had always wanted a female, brindle staffy.  In December 2008, John drove a distance and returned to surprise our boys with our staffy puppy who was born in October, 2008.  We named her Jazz.  Staffies hadn’t excited me greatly – I had never known a staffy and I already had my beautiful cocker spaniel, Merlin who was three at the time.

Before John bought Jazz, I did some research about Staffies and understood that they should be L2HGA and HC clear by parentage.  I explored this a bit further and advised John.  John enquired about the condition with our local vet (also a staffy lover) and with the registered breeder who sold us our puppy.  Neither the vet nor the breeder was aware of L2HGA.  Our boys, Flynn and Archie, fell in love with Jazz immediately.  She became their best friend.  We had lots of fun times with two boys and two dogs in our large backyard.  Merlin and Jazz became great friends and Jazz soon learnt that Merlin was a great play mate.

One morning, at the end of January, Jazz freaked out and we couldn’t understand why.  She was racing around, barking and panicking and seemed quite disturbed about something.  It was like she was trying to get away from herself.  We didn’t know what the problem was.  We took Jazz to the vet; he described her as lethargic with possible abdominal pain.  She was admitted for observation and remained lethargic.  We brought her home in the afternoon and she seemed like her normal self.

Jazz seemed to have a problem with one of her legs.  Sometimes she didn’t put her weight on it.  She also seemed to run a bit strange – she’d run forwards and in a wonky kind of way.

In the middle of March, Jazz had another episode similar to what had occurred at the end of January.  We took her to the vet again and he recorded that she had “sudden onset of barking and apprehension which continued for about 20 minutes, all systems normal on examination, no apparent cause”.

I took both dogs on a walk on 11th April, 2009.  Jazz was five and a half months old.  It was a brisk walk and, despite some short walks around the block this was Jazz’ first real brisk walk.  Jazz seemed very excited when she was on the walk.  So much so that someone commented “the dogs love their walk don’t they?”.  This was Jazz’ first and last ‘normal dog walk’.  We had been walking for 15 minutes and Jazz, while still on her lead, looked around and then ran off the footpath into a shaded area under trees.  Jazz raced around in circles on her lead, she wet herself, was panting and barking and had diahorrea.  It took me 15 minutes to move both dogs around the corner into a quieter area where there was a tap.  Jazz continued to race around on her lead in a circle in a mad panic, diahorrea was shooting out, she lay down and then stretched out, she was yelping and wouldn’t move.  As a mother of two young children, this was a rare occasion that I had gone out without my mobile phone.  Jazz wouldn’t move on, I thought she was at risk of a heart attack or something and that she may die.  I tied her to a gate and raced to a nearby shop with Merlin.  I tied Merlin to a heavy chair and raced into the TAB (John liked to have a bet or two).  I raced to the counter, quickly explained I was John’s wife (John who likes to have a bet), advised that John’s dog was in trouble and asked if I could use the phone.  The TAB owner was more than happy to help.  John turned up in the car shortly after and picked Jazz up and drove her home.  John spent some time calming Jazz down and she eventually seemed fine.

On 14th April relatives called past with a small fluffy dog.  Proudly, we brought Jazz out the front to show them how much she had grown.  Jazz lost balance a couple of times and fell over.  It was at this time that my mind wandered back to L2HGA as I had remembered the reference to ‘wobbly gait’.  I checked the internet, read the description of L2GHA and I remember that night suggesting to John that Jazz may have L2HGA.

The husband of a friend is a vet and I mentioned Jazz, our experience and my thoughts, to him.  He wasn’t aware of L2HGA and he advised that it could be any of a number of things.  I agreed with him, his response was appropriate, I wasn’t qualified to make a diagnosis and he had not met or assessed Jazz.

There were further incidents in May, June and July, (an open fire, a loud noise outside and another open fire) all resulting in arching of the back, wobbly gait, constant barking and panic usually later followed by further wobbly gait.

Our family and friends were concerned about Jazz’s behaviour – generally they commented that something is not right.  Flynn and Archie understood that Jazz had special needs and that her immediate family needed to provide her with extra help at times to make her feel OK.

I had a lengthy conversation with a vet whose name was on the internet on the L2HGA page and a member of the Staffordshire Bull Terrier Club. He explained that there is no treatment or cure for L2HGA, of all the dogs tested he had only had one ‘affected’ result.

Jazz was tested for L2HGA in September 2009 with the results confirming that she was “affected”.  We expected this would be the test result and it provided some explanation of the occasional behaviour that we found difficult to observe but had come to expect.

This diagnosis gave us an opportunity to accept that Jazz had a genetic disorder.  We understood that positive or negative excitement often caused Jazz a problem.  This had never stopped her racing around the back yard with so much energy and having so much fun with Merlin.  They raced around and played until Jazz was too exhausted to play any more.  Merlin had always tired earlier but seemed to enjoy the energy of his heavy set young friend.

John advised the breeder of Jazz’ condition.  The breeder was apologetic and offered to provide us with another puppy.  We felt the breeder needed to accept responsibility for selling a L2HGA puppy.  John drove the distance again and collected what we briefly owned and knew as ‘Little Jazz’.  We sold Little Jazz to a lovely home.  We had not intended to replace Jazz.  Jazz was still our special dog.  We were lucky to spend the time we had with Jazz and we were also glad that, despite seeking an adventurous, go anywhere pet, we had a very gentle, loyal pet and we were prepared to assist her with her special needs.  We were pleased that she hadn’t been bought by someone seeking a show dog because, despite her willingness to please, Jazz would not have been able to deliver.

Jazz was happy, always loving, a great play mate for Merlin, Flynn and Archie and she was quite normal at least 95% of the time.

As a family, we had learnt how to calm her down after one of her episodes and how to help her recover.  That was our objective and, from our observation, we think that is what we achieved.  Flynn and Archie learnt how to help Jazz recover.   She would sometimes have a little barking episode around my bedtime or just before I was due to wake up in the morning.  I would heat some milk for her and reassure her and she was generally OK.

We were somewhat proud of our ability to ‘manage’ Jazz’ condition.  One day we took Jazz and Merlin to the local dog park for a play.  They were the only two dogs there the whole time and they had a great time.  We were happy that we had taken Jazz out and it seemed to have been a success.  A few hours later, Jazz stood still in the back yard and then did back flips like an uncontrolled wind up dog.  We have a great, large backyard that is enjoyed by the whole family.  Jazz no longer left her backyard.

We had many more random episodes and we dealt with them as they arose.  Jazz recovered and life went on as normal.

We booked a dog friendly holiday in January 2011.  At the last minute we decided to leave the dogs at home so that they were in a familiar environment.  This was our only holiday for the year and we decided that there was too great a risk in taking Jazz with us.  We knew she was most comfortable in her own familiar backyard.

Flynn, Archie and I had given John a hammock for Christmas.  When John was lying there, Jazz decided that this was a good place to be and she decided that a rest alongside John in the hammock was a good way to end the day.  When I saw Jazz do this I thought about taking a photo.  As an obsessive hobby photographer, it was a bit unusual that, on this occasion, I decided not to race in and grab the camera. I’d do it next time.  There was no next time.  Jazz had an episode in the morning on 14 January, she couldn’t stand up.  She couldn’t stand up that evening and she couldn’t stand up the next morning.  John took Jazz to the vet and Jazz went to Heaven on 15 January 2011 – aged 2 years and 2.5 months.

We hope that by telling Jazz’ story, this will help to eliminate L2HGA.  We also hope that the owners of every staffy puppy will not have the worry associated with living with a dog with such a significant genetic disorder.  The opportunity to share a lifetime with a staffy should not be cut dramatically short by the effects of L2HGA.

Read more about L2-Hga from the Staffordshire Bull Terrier Club of Western Australia or the Swansea SBT Ring Craft Club.

24 thoughts on “The hidden disorder in staffies

  1. I had not heard of this, but wow, what a heartbreaking story. Good for this family for sharing their story in the hopes that it will raise awareness and stop this from happening. So sorry for their loss.

    • This is such a beautifully written yet heartbreaking story. Brought tears to my eyes. I’m so sorry for your loss. Jazz sounded like a beautiful puppy dog.
      I’m also an owner of two staffies. They are the most loving and happy dogs. I would be lost without my two furry children.
      Thank you for sharing your story and raising the awareness to other staffy owners.

  2. This is happening sometimes to my daughters English staffy
    He is 8 moths old it only happens when he was scared or over excited we told vet and she said it could be little seizures

  3. How is this a story about loss!?
    You heartless people gave away your dog because you couldn’t be bothered dealing with his condition. If one of your kids develops a disorder will you abandon them? Shame on you, all my sympathy goes to Jazz.

  4. So heartbreaking, my Pup Ruby was never diagnosed with that disorder but I’m positive she had it, she had episodes almost exactly the same as jazz, she stopped eating, lost vision, was uncoordinated, and couldn’t not stand up for the last 3 weeks she was with us, Ruby passed away August 5 2016, I know your pain.

  5. I had never heard of this disorder. My partner had his staffy euthanised today after 11 years and vet told him that his dog had a neurological disorders. All symptoms she had were the same.

  6. We have just lost our beautiful staffy male benny to epilepsy at 10:07pm on Christmas Eve 2016, it has been the most traumatic experience of our lives, benny was 8.5 years old and is sorely missed

    Staffies in my opinion are the most affectionate dogs bar none, our benny would come to us for a kiss and a cuddle all the time and just enjoyed being with people, anyone that ever met benny loved him, and he met a lot of people.

    I would never hesitate to suggest to anyone looking at adopting a dog to get a staffy, we did and it has been the best experience we have ever had.

    I know exactly what the people that wrote this story went through, we didn’t know anything about L2HGA when we got benny but we do now.

    To the best little mate ever BENNY

      • Hi
        So sad to read the story of little jazz x bless her.
        I have a 10 year old staffy who has never been ill . But recently has his ‘quiet days’ where it may start with a yelp as he jumps onto the bed. I have put this down ti pissible arthritis and the vet agreed. And when this happens he kindof sulks. Doesnt play or eat for the day and takes to his bed. But once every now and then he shakes all over only very slightly. And it happened again this morning his body shakes then stops for about 10 15 secs then shakes again this went on for about two mins. Any thoughts or advice would be very welcomed.
        Have had him since he was old enough to leave his mum. So i know all his medical history.
        The vet has said i pribably inly have a couple of years or so left with him as staffies dont live much more than 12 or 13.

  7. Just an up date to say my boy didnt have this hidden illness he had cancer of the spleen and lost his fight yesterday. Put to sleep at 10am. 27th feb.
    Feels soo empty and horrible without him.
    Deb

      • Leema
        Thank you for your kind words. He was cremated today and i can bring him back home next week.
        Cant bring myself to pack his things up yet. And keep expecting to see him on the sofa with his head up and tail wagging when i walk in the door xx

  8. Evening everyone. I am reading this because I wanted to see if there were charities relating to this horrific disorder.

    I had Missy, a gorgeous white staffy who was my best friend. I had her from 11 months old from someone her actually named her mystery due to the weird way she’d sit or walk. But he told me that she was caged pretty much for 5 months of her life by a previous owner.

    I would walk her and the slightest pull on the lead and she would be flat to the ground. After many times of this happening I took her to the vets. They put it down to her not having muscles in her back legs.

    Anyway years passed and she had muscly legs, a bit on the chubby side as she refused to walk far. I put that down to her very chilled out behavior.

    5 years pass and she is absolutely fine, well in my eyes she was perfectly normal. Things she did and the way she acted just made me appreciate her so much more, she was calm, cuddly, playful and had a fantastic happy attitude. As years went by she would always suffer during her heat cycle, she’d bleed for 3-4 weeks and always get cystitis meaning she’d always want a wee even though nothing would come out, she’d always be on antibiotics for that every season.
    Again nothing too out of the ordinary really. Then one day I went downstairs and normally she’d be up with me ready to get her breakfast, she’d try to get up, raising 1 paw and not move, I instantly thought she hurt her leg, I picked her up and took her downstairs, straight down on the floor she fell. She then led in her bed, ignoring her food (definitely not like her!) I went to work for the next 10 hours, came home and found her still in her bed, not moved and not touched her food.
    Alarm bells raised I took her to the vet. They then diagnosed her with an infected womb, so toxic it would have killed her, this turned out to be the scariest 6 hours of my life while they operated on her. When they tried to get her to wake up it took another 2-3 hours for her to come round.
    After a stressful no sleep night, the next ay I got her back…. she was fine, back to what I believed was normal.

    A year later, she goes back to the vet for another leg problem. However, the vet was very intrigued by how she walked. Under the vets table was a bar, that acted as a support for the table. Missy would have had to lift herself over this bar to carry on going forward. However she wouldn’t, she instead would just stop once the bar touched her legs. I explained to the vet that she had always done this, with walls, fences and anything a little bit taller than her she would stop.
    This made the vet test her version, turns out missy could see perfectly fine, however only things straight in front of her, so she would have been oblivious to the bar once it was underneath her vision.
    This led on to the vet watching her walk, she then had an idea of what it could be…… she had a blood test….. and yes it was the vile genetic disorder the vet guessed it was.
    The vet told me it is very very rare, completely incurable and will get progressively worse.

    Refusing to research this disorder or hear anything about it. Not knowing how long my girl had left, I spoilt her, treated her even more like a kid and spent so much more time with her.

    As the months passed, every now and then she would get a little less responsive, she slept most of everyday. She’d come to you if you called her. Looking at her though she would just randomly twitch. I knew deep down why she was doing this. She would go for walks but just randomly stop, then refuse to move any further, she had always have moments where she’d space out and stare in to nothing throughout her whole life, but walking her was so much worse than that.

    One night I asked my lodger to bring her to my work as I closed. When she got out of the car, she fell over and smacked her jaw on the ground. She got herself up….. but she started frothing at the mouth and looked as if she was chewing her tongue. Freaking out I called the vet. She explained that missy was having a seizure, a bit part of her condition, after a few minutes it passed. The vet explained that these could become more frequent or could be months later.
    She was better that’s all that mattered.

    Again months passed and only minor seizures occurred after falls.
    This made me know I did not have much time left :(

    On December 3rd 2016 missy fell over going into the garden, again she had a seizure which lasted less than a minute. After taking her upstairs to clean her down it triggered another seizure. A big one. Missy went blind and for the first time ever barked in discomfort. After a few hours of on off seizures I took her to the vet. She gave her an injection to stop the seizures. It didn’t work.

    On December the 3rd at 9am missy was put to sleep…. the seizure would not subside and it was the only thing that could put her out of that misery. 5 months have passed and there isn’t a day where she isn’t on my mind.

    In January she would have turned 7. In my eyes 6 is still too young.

    I know I have rambled on a bit and I apologize but I think it is so good to talk about this disorder because it comes in so many different varieties. I was told that that angelic behavior of missy was not right and that should have had a bit of a boisterous side to her, which she didn’t, she was well behaved, loyal, affectionate and very loving.
    I was told that she would not have lasted 2 weeks after she was diagnosed, but she held on for a good year and a half. And that seizures were the most common way for a dog to go from this vile disorder. There is no timeline, no cure and no way of slowing it down. Again the main reason I even looked into this was to see if there are charities out there, or whether this horrible thing is being looked into.

    • Hi Carl.

      Sorry to hear about your experience with Missy.

      There are no charities that I am aware of regarding L2-Hga, but it is an easy disease to stamp out if breeders would just test their dogs before breeding!

  9. Carl.
    So sorry for your loss. At least she found a living forever home with you. My boy was put to sleep on feb 27th this year due to cancer. I have his ashes at home and say good morning to him every morning. My autistic son was not the same after my old boy died at 10 and a half. So we brought another staffie male pup. Who is a bundle of energy. Felt a bit guilty getting another dog so soon after and still feeling really upset whenever anyone asks about my old boy. They really are fantastic dogs with so much love to give. He was my best friend. And my new bundle of joy is a mini monster but love him the way he is. Its great going through the training and out for walks again.

    Time is a good healer.

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