Alfie

Alfie (b.13th June 1999 – d. 29th May 2012) is the inspiration for Laforadogs and here is his story:

The Background:

I was always keen to follow in the family tradition of having a smooth hair dachshund of my own when I grew up,  and in 1999, married and with my own family, and working from home, I was finally in a position to make the family complete with our own small addition, but my husband wasn’t quite convinced …….

Until, on holiday in South Devon, he, like the rest of us, fell in love. Out walking in the country around Salcombe, we came across a whole gaggle of Mini Wires. We were completely bowled over by their feistiness, cheerfulness, and most importantly friendliness – my childhood dogs had always been wary of strangers and it was appealing to have our overtures met by such a warm response.

We were hooked. I was all too aware of the back problems as it was this that caused the premature death of my parent’s first dog, Pippa, so I checked the internet and spoke to various breeders into other potential problems specific to MWHD and was delighted to discover that the breed had no other reported serious health concerns.

After various abortive forays, we found exactly the dog we wanted – we met father and mother-to-be, both delightful, at home in Taunton. It was made clear to us that Taggart, the dad, was too big for showing, and there was a very good chance that the pups would be the same, but we didn’t care….. all we wanted was a healthy dog from a family background with Taggart’s great temperament. As a precaution, we did check out his pedigree and were delighted and reassured to find several champion dogs including several from what, after my research, were familiar kennel names of top breeders.

Alfie on Burrington Ham

Alfie

 

Alfie was everything we’d hoped and more: intelligent, willing to try anything, including climbing to the top of rocky outcrops in Somerset (see picture) or making it to the top of Great Gable Mountain in the Lake District; ready to make up his own games ….. then persuading us to join in, such as bodyboard surfing ‘chase the ball’ in South Devon, (see Alfie video) , on the touchline in his unofficial capacity as mascot of the mini Rugby side …….  yet just as happy curled up for a cuddle on our laps.

He had his major health problems – two back ops to sort out a total of 9 calcified discs, but he bounced back stronger each time; skin allergies needing frequent treatment with anti fungal shampoos; a hernia operation, but each time he came back good as new.

The symptoms 

When Alf was 6, we were out walking with friends in woods near Tarn Hows when suddenly, with no prior warning, he keeled over and had a full blown grand mal fit, foaming at the mouth, twisting and twitching. When he came round, he didn’t recognize us and hid in terror behind a tree ….. until suddenly, like a switch being turned on he came back to us, greeting us with such relief to be back from whatever horrible place he had been.

Over the months to follow, every few weeks it happened again, and we began to recognize the triggers and the signs of an imminent fit – he’d never taken any notice of the TV before, but now, when the Rugby was on – though not football!)  –  he’d watch the screen transfixed until he fitted again. Out walking in woods with sun streaming through the leaves or playing an exciting game of hunt the ball in a cardboard box, he’d suddenly keel over as if polaxed. Our vet advised us to avoid putting him on a drug routine if possible and we learnt to avoid the triggers – he was banned from being in the room when we were watching the Rugby World Cup; we only walked in woods in dull weather and we invented new, less stimulating games.

So most of the time, we were managing to keep the grand mal seizures under control, but another distressing reaction was starting to manifest itself. Every so often, if someone passed close by him; if we threw a ball in his direction; if there was a sudden noise; we’d see him suddenly jerk back as if badly shocked. It was distressing to see. He also seemed to stumble more when walking, and he began to have difficulty catching a ball.

The vet hadn’t seen anything like it before but put it down to another manifestation of his epilepsy. I wanted to find out if there was anything we might be able to do to help lessen Alf’s symptoms, so started to search on the internet for any articles about epilepsy in dogs – nothing useful until I changed the search and added in the words ‘mini wire hair dachshund’ – and there it was…

A vet in England (Clare Rusbridge) and another in Canada had identified a genetic condition that affected 5% of MWHD. The gene was similar to one found in human teenagers who were affected by a form of epilepsy called Laforas which ultimately would prove fatal. The article said Lafora wasn’t fatal in dogs, but from its first appearance, typically when the dog was around 6-7 would “progress over many years and gradually other symptoms such as ataxia, blindness and dementia occur”. There was also a short video on the site showing a MWHD reacting to sudden movements and noise just as Alf had done, and we learnt that the correct clinical name for the reaction is myoclonus. (see below for link to her website below)

I was shocked. Why hadn’t anybody told us about this before?

We showed the article to our Vet, who confirmed he had never heard of this condition. He contacted Claire Rusbridge who confirmed that the most likely cause was Lafora. She confirmed theblood test developed by the research team in Canada (details on the veterinary neurologist website above) can identify whether a dog is ‘affected’ or not ‘, but as we were quite certain by now that Alf’s symptoms fitted Lafora, and he had already had so much medical intervention, we decided to concentrate on learning to live with it and avoid putting him on any drug regime for as long as possible.

In the early morning one day in February 2009 we heard horribly familiar noises from the room where Alfie slept and found him in the middle of another grand mal fit. We comforted him and hoped that was it, but 45 minutes later he had another ….. and didn’t seem to come out of it properly. We rang the Vet’s emergency help line and he told us to come straight down and he’d open up for us. Just as we got there, Alf had yet another fit.

In all, he had 15 fits in 12 hours. The vet dosed him up with valium, to no effect, and then anesthetic, but he was still fitting. Mid afternoon, in a desperate last ditch attempt, he gave him a dose of anesthetic that should have knocked a grown man out for 48 hours………. and finally,   Alfie calmed down and slept peacefully. An hour later, he started to come round and take an interest in his surroundings, and by the time the veterinary nurses were doing their rounds with evening meals for the other patients he was ravenously demanded to be fed and wagging his tail.

At first, when we were allowed in to see him, he didn’t appear to recognize us, but gradually, light dawned and he gave us a lovely welcome. We took him home that evening. He initially didn’t seem to have a clue where he was, but over days, he re-learnt the orientation of the house, the garden, his favourite walks.

Thanks in a huge part to the care and persistence of our Vet, Dr David Holmes of the Golden Valley Veterinary Practice for operating successfully on his back, bringing him around from the series of fits against the odds and finally operating for a hernia which manifested itself less than a month after his big epileptic episode, Alfie continued to enjoy some quality of life for several years.

As this is a genetic condition, there is no cure. The symptoms appear to be due to a build up of a starch like material in certain cells in the body, as the result of a genetic mutation. We changed his diet to a low starch diet and focused on giving him the best quality of life possible.

His daily dose of phenobarbitone was bsimilar to that which many human epileptics need to control their condition (30 mg twice per day). According to the pill bottle, he wasn’t allowed to drive or operate machinery but I’m pleased to say that didn’t affected his quality of life!). He had a few more grand mal fits since the big episode, and then started to have terror attacks – when he appeared to be in a different but completely horrific world where all he wanted to do was to desperately escape everyone and everywhere. The myoclonus, vision and hearing is gradually got worse as the starch like material built up in the brain behind the eyes . We invested in a pair of ‘Doggles’ for him, which seemed to help him walking in bright sunlight for a while but as his sight deteriorated further the dark lenses meant he couldn’t see at all so we had to abandon them.

He also gradually and gently began to show signs of losing his marbles. He no longer remembered games which he had invented when he was younger, he got confused about food times, he doesn’t recognize potential danger as he used to once (we were constantly tripping over him, whereas once he would skip out of the way). He was still our Alf and we still loved him just the same – but he wasn’t the same dog.

Alfie, age 11 Copyright Gill Key.
Alf had his first fit age 5 and went on to show all the typical symptoms, culminating in panic attacks. After two particularly severe episodes, he was put to sleep. Alf was the original poster boy for this website and is still missed every day.

 

In May 2012 after yet another terror attack, we decided the time had come to think again. We had got so used to looking after him, carrying him on to the lawn, pointing him towards his food, coping with his confusion that to us this was normal, but the terror attack caused us to take stock and finally we took the agonising decision that enough was enough. He could have carried on for days, weeks or months but what quality of life did he have left? We arranged to take him to the Bristol Veterinary Hospital where, with a tummy full of all his favourite foods and snoozing on our laps he was put to sleep. The Hospital was able to take samples from him that have been sent to Canada to help with the research and our hope is that at least some good will come out of this dreadful experience.

As you’ll have gathered from the above, it’s a horrible condition. Having used the wonders of email and forums, I’m now in touch with a few other owners of affected dogs and it seems that the actual symptoms do vary from animal to animal, which makes it more difficult to positively identify.  I just wonder how many other owners out there are struggling with dogs with some of these symptoms who haven’t been able to track down the potential cause, particularly if it isn’t widely known within the veterinary profession.

 

RIP Alf