Submitted by Debbie Johnson

It has been almost 20 years since I first experienced Cerebellar Ataxia.

I had spent several years acquiring a number of quality working bitches before I decided to purchase a stud dog that would compliment my acquired bitch’s pedigrees. Before long my first resulting litter whelped, I still remember the feeling of pride and success, unaware of what was yet to come. When the litter was two weeks old their eyes opened and the world for two of the puppies began to spin.

Their heads bobbed and banged on the side of the whelping box and they struggled to nurse. Just as terriers are known for their strong will and determination, they began to compensate and it actually appeared as if they were getting better. They stood with a wide stance and when eating they developed a pecking motion, quickly picking up their food and returning to an up right position. When walking they would weave and stepped high like a goose or duck, but could manage to get from point A to point B.

I contacted the breeder of the bitch and stud dog, but neither had ever experienced such a novelty litter. “Must be in the bitch line,” said the stud dog breeder. And – you guessed it – “It must be in the stud dog line,” claimed the breeder of the bitch. Both offered the same advice, to put the puppies down and to not repeat the breeding. I was also advised it would be best if I did not speak a word of this to anyone.

My vet diagnosed the affected puppies with ataxia, but he wasn’t aware of it being in puppies. Ataxia is something normally only found in cats, yet somehow the disease had reached my litter. Weeble and Wobble were 3 months old when our second litter whelped: four puppies, two being ataxic. My vet then recognized this as a serious problem. He referred me to the University of Georgia, where I met with Dr. Paige Carmichael and Dr. Joan Coates. The ataxic puppies were donated and so began our journey. We spent the next five years collecting data and the up hill battle of recognition that the disease existed within the breed.

Within two years of my first litter I found I had four carrier bitches and a carrier stud dog that I had purchased. Each was from a different kennel, but with related terriers in the second and third generation. I then acquired a second stud dog with only a couple of related dogs in the third generation. He too was discovered to be a carrier. “Why me?!” I began to ask myself, and according to everyone else it was only me.

Eventually the mode of inheritance for Cerebellar Ataxia was determined to be recessive, meaning both parents must carry a copy of the gene to produce it. Through seminars, education and determination, breeders slowly became aware of the disease. Over a ten year period 28 affected puppies were donated to research. There were many other cases reported but they didn’t make it the University of Georgia; vets had not understood procedure and the tissue had not been preserved properly.

In 2005 I requested the ataxia research at UGA be transferred to the University of Missouri, where Dr. Coates could continue with researcher Dr. Gary Johnson. Then suddenly our numbers began to decline, three years ago two puppies were donated and none in the last two, leaving researchers to conclude we had bred it out. Unfortunately, that is not the case. It has raised its menacing head once again. The fact is you can’t breed out carriers; we just slow it down or manage them. But when we have a popular line, whether it is a carrier dog or bitch, carriers will continue to be produced at an accelerated rate. When a carrier is bred to a non-carrier half the litter will be carriers. If a popular carrier sire is bred to five normal/non-carrier bitches and 20 pups are conceived, 10 will be carriers. These numbers are based on a punnett square.

If you are a breeder and you are aware that your stud dog or bitch is producing carriers, you are obligated to disclose this to your puppy buyers, both ethically and legally. If you continue to deny this or refuse to own it, it’s only a matter of time before it will bite you in the back side.

In order for researchers to acknowledge Cerebellar Ataxia still exists in our breed we must report all cases. Puppies must be six weeks old before donated. Please contact me or Cheryl Costello, and we will assist you in getting your puppies donated at no cost to you. With determination we will one day see a genetic marker for ataxia.