August 19, 2009

"Just Because of Jane"

(By Kathy Gooch)

August 4, 2009 – My little black Kelpie bitch, Outlaw Jane, was put to sleep today. She had just turned four years old.

August 5, 2009 – Jane’s brain was sent by courier to the University of Sydney to be used for research on CA (Cerebellar Abiotrophy)

Jane had never won a sheepdog trial. In fact she only worked sheep twice in her short life. She never had a litter of pups nor impressed any one with her working skills. Yet in the history of the breed, Jane in her difficult life and now in her death will have a greater impact on the health and welfare of the future of the Australian Kelpie than any other dog in the history of the breed.

Jane however was a movie star of sorts; starring in her own film; a DVD about CA in the Australian Kelpie. I know she was proud of that part although she never received any credit for it.

This is Jane’s story. It is time it was told.

On the 24^th of July 2005, my bitch Capree Quiver II gave birth to a litter of 5 pups sired by my outstanding cattle dog Buckaroo Chappy. Both the sire and dam had produced pups bred to other dogs with no consequences so I was not expecting problems. Jane was the only bitch in the litter and was regarded as a possible “keeper” from the start. She and her brother were the only two black pups in the litter and were both cheeky little monkeys from the start. The brightest, most active, inquisitive pups the pair were named Tarzan and Jane. But Jane was the superstar. At five weeks of age she was trying to work anything that moved; her brothers, birds, bugs and our feet. Sweeping out the kennels with a broom was almost impossible as she would cover and set it constantly. Her footwork was amazing. She was going to be the best and the brightest.

One day shortly after turning six weeks old while Jane was up to her broom chasing antics something happened that would change my life and hers for the next four years. As she stopped and set the broom her head nodded up and down. Was it my imagination or did she have a tremor? I moved the broom again and when she stopped to set it her head nodded up and down again. My blood ran cold. Something I had read years ago jumped to the fore in my mind; ataxia; a neurological condition in Kelpies. 

I frantically scrambled through my past Working Kelpie Council newsletters and found one small reference to the disease; but not much as to what caused it. I jumped on to the internet and found that “ataxia” was merely a symptom of a much more insidious condition called CA or Cerebellar Abiotrophy. The symptoms described were exactly what Jane was showing.  It seemed that it affected many breeds of dogs, Arabian horses, Angus Cattle and humans. It was progressive, degenerative and incurable. It was also an autosomal recessive; meaning both parents had to carry the gene to produce affected offspring. My heart sank. My talented pup had been handed a death sentence by her genetics. It also put an immediate stop to my planned breeding program. My thoughts immediately turned to her brothers. Were they affected? I did the same type of observations on them. None showed any symptoms at all. They were healthy happy active pups. I breathed a sigh of relief. They were okay. Unfortunately my elation was short lived.

I had lots of questions and I wanted the answers. Was it really CA? Where did it come from? Why wasn’t more information out there on the disease? If it was a recessive why did it not show up more often? What could I do about it? What was the prognosis of the disease? Was there a DNA test? My thoughts went to the sire and dam. They had each produced a litter bred to different dogs. The pups were brilliant workers and had shown no symptoms. Did this mean the dogs they were bred to were not carriers or was I just lucky? If the parents were carriers then at least one each of their sire and dam had to be carriers. My head was spinning. 

I have often been described as tenacious when dealing with something I am passionate about. Kelpies were my passion and the fact that I had bred a litter with genetic problems was eating away at me. I have always tried to breed the best to the best; carefully plotting out breedings; studying pedigrees; working traits and health issues. Obviously I had missed something and this was the consequence. I became obsessed with finding the answers to all the “whys”. Little did I know that my quest would open Pandora’s Box; evoke the ire of the Working Kelpie Council; set in motion a search for the DNA marker that carried the disease and change the course of my involvement with working dogs forever.

Meanwhile, Jane and her littermates were now 12 weeks old. Other than Jane having an exaggerated head tremor when focusing they were by all other accounts a healthy litter. When they were vaccinated I had to point out to my vet Dr. Annette Miller the head tremor as it was barely perceptible. I discussed the disease with her and while she had heard of it had never seen it. They were athletic, and cavorted maniacally like all Kelpie pups of that age. I was lulled into a false sense of security. I thought “If this is as bad as it gets I can live with it and let her have a normal life here with me.” I never even considered euthanasia. During this time whilst going through some archived Kelpie information I had I found reference to some research that had been done in West Australia by a Dr. Don Robertson in the 1980’s on CA. It became obvious to me that this disease had been around for awhile and had been frequent enough in the breed to initiate research on it. I now had even more questions. What was the outcome of the research? Was it still going on? And why had the information on this condition all but disappeared in the recent Kelpie newsletters?

I decided that a phone call to the Working Kelpie Council was in order but felt that my conversation was not very productive. Word was that if Jane carried the blood of many of the top Australian dogs then she could certainly be carrying problems, but other than that, no names were mentioned so I was left in the dark and still guessing even though I felt there was more they could have revealed. I was appalled. The Working Kelpie Council had known about this disease and apparently the dogs that were carriers since the 1980’s and nothing was being done to educate breeders, buyers and WKC members about the disease. Research started in the 1980’s by Dr. Don Robertson in west Australia had all but ceased for no apparent reason. Was it lack of funding, lack of interest or the complacency of Kelpie breeders and WKC members to find a solution to the problem? Or was it all of the above? And worse yet no research was currently being carried out to facilitate a DNA test or marker. No funding had been allocated for genetic research despite the Kelpie Council sitting on a hefty bank balance. A bank balance which had been accrued from membership and registration fees. No monies were being set aside from litter registrations for genetic research either. In fact absolutely nothing was being done with regards to CA except a brief article every couple of years in the newsletter to remind people it does exist. I spoke to other breeders and yes some were aware of it but absolutely no one admitted they had it in their bloodlines. Most of the new people to come into the breed had never heard of it. Some people when symptoms were described to them said “Yes, they had a dog like that but did not know what it was so they shot it”. Other people would tell me of other breeders that had it in their kennel but when I approached those breeders, they would not talk about it. It seemed I had uncovered the Kelpie breed’s “dirty little secret,” and no one was willing to admit to anything.

Jane and her littermates were now six months old and subtle changes were taking place in Jane and Tarz. Jane started to develop balance problems in doing some things. She was now unsure about jumping up in a vehicle and her crate. Both her and Tarz had the wide legged stance in the rear legs associated with the ataxia symptom of CA. Their muscle development became different and they looked long in the body, a condition caused by the body adapting to the balance problem. Tarz had not shown any symptoms prior to six months, but it became obvious that he was now affected. Flash and Floyd appeared okay. Mentally, they were fine. They behaved as any rowdy six months old Kelpie would and all of them wanted to work stock and so they were all started on sheep.

To put the effects of CA in terms we humans can associate with; having CA would be as if when you went to walk you were unsure where to place your feet. Once your brain became familiar with what you wanted to do it would adapt, but anything new is cause for hesitation in the placement of your feet. That is how Jane became. Once she jumped up in her crate she could do it, but not any other crate. Once she went in her kennel run she could go in fine but not another kennel run. Some days her adaptation was so good she would even make me look at her twice to remember she was affected. People that came to the farm could not pick her out as having anything wrong. Even the head tremor she had exhibited as a pup had stabilized.

Meanwhile I was on a mission to find out just how widespread this disease was in the Kelpie and more importantly to get research going again to identify the gene responsible for this insidious disease. I sent a petition out on the internet to request the Working Kelpie Council to set aside money from litter registrations for genetic research. Something I felt that should have been done years ago. I also asked people to share with me in confidence their experiences with CA and supply the pedigrees of dogs that had produced CA litters. The response from everywhere all over the world was overwhelming, except from Australia.  In Australia I had two responses from people who had affected dogs. Dogs exported from Australia had produced affected pups yet no one in Australia wanted to talk about the problem or admit they had CA in their bloodlines. Using the pedigrees of my own carrier dogs and the pedigrees sent to me of those dogs that were carriers and affected I began to see a pattern of inheritance. Using my Breeder’s Assistant pedigree program and my extensive data base of Kelpie pedigrees I was able to do some calculations that were shocking. Because of the recessive mode of inheritance a carrier must have at least one carrier parent. I took one common carrier relative that was a dog used extensively for stud. This particular dog appeared in over 115,000 Kelpie pedigrees. I realized at that moment that CA was a very extensive problem in the breed and was being compounded by breeders hiding the issue and the continuing breeding of carrier dogs.

It was at this time that I was contacted by a Show Kelpie breeder, a wonderful lady named Marie Colyer. She had bred affected dogs and desperately wanted a DNA test developed. She was the first Kelpie breeder in Australia to openly discuss the issue with me. At last a kindred spirit and one who wanted the best for the Kelpie. Sometime back Marie had sold a pet Kelpie to Terry Snow who owns Canberra airport. Terry is a great fan of Kelpies and would prove to be a key person in the search for a DNA marker.

Meanwhile my petition crusade and quest for information on pedigrees had not gone unnoticed by the Working Kelpie Council. I received a curt e-mail voicing their disapproval of my petition request for money for genetic funding through registration fees. Although the reason given was that I had done this without their approval I believe that the underlying factor was that I had brought attention to a genetic problem which for years was not given the priority it deserved. All of a sudden the WKC now became interested albeit reluctantly; in sourcing funding and a researcher to identify the genome responsible for CA. Enter Dr. Alan Wilton. A top research scientist based at the University of Sydney he is responsible for locating the gene in Border Collies for TNS and CL amongst other accomplishments. He was willing to take on the research but two key items were needed; funding and blood samples from affected dogs and carriers.  Whilst I could provide the blood from my carriers and affected dogs, global participation was needed. Samples were obtained from Sweden and the USA. My vet Dr. Annette Miller donated her time to come out and take blood on my affected dogs, carriers and anything in the kennel related to those individuals. Funding was the next obstacle.

I received a call shortly thereafter from Terry Snow. He had been contacted by Marie Colyer with a plea for help for funding for genetic research. He was prepared to tackle the problem head on. He wanted to hear from me what had transpired with regards to actions taken by the WKC and what I felt was the status of the disease. He then spoke to Alan Wilton to find out what was needed with regards to funding. Terry felt this was a worthwhile cause and agreed to donate a large sum of money for research providing the WKC also funded the project. Terry also had the brilliant concept to do a DVD at his expense about CA, with interviews and video of affected dogs. This was to be mailed out to all WKC members worldwide and all veterinarians in Australia. The Kelpie Council agreed to provide the mailing list to do so and pay for postage expenses. More on this later.

In my conversations with Kelpie owners in Australia there was a general lack of knowledge regarding CA. Education on the disease and mode of transmission was severely lacking. There was also a lot of “ostrich disease” going around amongst breeders and owners. It was time to draw attention to the problem on a national level. I contacted the “Weekly Times” a weekly rural newspaper. They sent a reporter out to do an interview and the story was featured on the front page with a degree of sensationalism. Unfortunately the reporter misquoted myself and Dr. Wilton on several things but at least it brought attention to the disease and the problems surrounding it.

While all this was going on I received another call from Terry Snow. He wanted to know if they could film the video at our farm and feature our dogs, our vet and an interview with myself on what I had experienced with the dogs. I readily agreed and so did Dr. Miller. Terry was prepared to fly an experienced film crew and himself at his expense to the local airport in a private plane if I could pick them all up and transport them to the farm. This was exciting stuff.

At this point in time there were only subtle changes happening with Jane and her littermates. I noticed what can only be described as a subtle vagueness. Also the muscle structure on the dogs seemed to be progressively developing differently. They appeared to be long in the loin area and their back legs seemed to always stop spread apart. I attributed this structural change to the fact that the body needed to adapt to the neurological changes happening in the brain. It needed to accommodate to the lack of balance that the dog was experiencing. It was at this time that I was informed by Dr. Wilton and his team the importance in keeping my affected dogs alive to monitor the physical changes and to have a source of DNA from carriers and affected dogs. I could see that this was going to be a long journey.

The day arrived for the filming. I picked up Terry and his crew at the airport. Full of enthusiasm, Terry was obviously passionate about this venture. They filmed my affected dogs with special emphasis on Jane. For those of you that have seen the DVD she is the black bitch with Dr. Annette Miller. My other dogs were filmed as well. I spoke about my experience with my affected dogs and talked about the positive aspects of having a DNA test to identify carriers and affected dogs. All the filming done on the farm that day was to be combined with interviews with Dr. Wilton and the President of the Kelpie Council, Mr. Carlon. I felt this was an extremely positive step forward in educating people about CA. My elation was short lived.

Three weeks after the filming I received a phone call from Terry Snow who was obviously very upset. They had sent a preliminary DVD to the WKC and the WKC sent back the shocking reply that they were not going to support the DVD in any way nor divulge the mailing list if it went out as it was. Their reason was that they did not want me to appear in the DVD at all. They said it was because “I was not a breeder of importance and that I was American and no one would listen to me.” I cannot tell you just how hurt I was by this action. I knew it was because the WKC board was angry at me for pursuing this venture; but I did not think they would be so petty as to exclude me from a video filmed at my place with my dogs. Terry said if I gave the word he would pull all funding from the project. I told him I needed to think about it. After many tears and a sleepless night I called Terry and told him that the breed was at stake and I needed to put my personal feelings aside and to proceed with the DVD and funding of the project. He wanted to know if I could suggest anyone to replace me in the video. I suggested Kevin Howell of Karana Kelpies, a well respected breeder to speak in my place. That is how Kevin came to appear in the video. The DVD’s were mailed and no one ever knew the true story behind the filming and how the DVD almost never came to be. That is until now. Three years have gone by and I still have not brought myself to look at the video. Now with Jane’s death I probably never will.

The gene for CA has proved to be an elusive one for the research team. With perseverance they narrowed the search to forty possible genes. Recently they have come very close to finally locating the responsible genome.

About six months ago Jane started a rapid decline. She would no longer climb on her elevated dog bed, because the movement frightened her. We had to get her a bed close to the ground so she would not sleep on the cement floor. Some days she would stand and stare when you asked her to get into her kennel and other days she would race in. She was losing condition rapidly and had to be fed three times daily to keep on weight. The other dogs constantly picked on her, sensing her weakness. She was always a barker and she no longer barked. It became apparent that her immune system was crashing as she showed signs of demodex.

A previous conversation with Dr. Wilton’s assistant Jeremy had indicated that when she was finally euthanized they would like her brain for study. This required Dr. Miller to do a necropsy immediately after death. Although she was not looking forward to this procedure she felt it was necessary to assist in the research and Jeremy felt having the brain could lead to the last bit of vital information needed to locate the genome. We made arrangements to put her to sleep on the farm. I knew taking her to the vet office would only stress her out and I wanted her last moments to be as painless as possible. She had endured enough in her short life. We said goodbye to Jane just a few days after her fourth birthday.

CA is progressive, insidious and incurable. If any one has a pup showing symptoms the kindest thing is to euthanize them early. Animals affected do not improve. It is apparent from watching my dogs that the disease may stabilize for a short while. But eventually it lives up to its name and the brain cells atrophy, causing multiple neurological problems. At necropsy Jane’s cerebellum, which in a dog her size would normally be golf ball size had shrivelled to the size of a walnut. Any breeder knowingly breeding carriers and not informing prospective buyers should not be allowed to breed dogs. I would not wish the experience of having an affected litter on anyone and I question the ethics of any breeder who knowingly passes on these genetics without full disclosure to prospective buyers. With the high numbers of potential carriers in the Kelpie gene pool a DNA test is the only way to make responsible choices in a breeding program. I hope for the future of the breed it is soon.

I still have a journey to go with Jane’s brothers. "Tarz" has regressed to a puppy state in playfulness, "Floyd" has developed a head tremor and "Flash" blanks out when you speak to him, mystified as to what you are saying and then tunes back in. They will stay with us until their change in quality of life leaves no other choice but euthanasia

August 6^th, 2009 – Jane was buried in our “dog garden” today. She will have a rose planted over her grave just as all the other dogs buried there before her have done. I think it will be a white rose to give her clarity, something she never had here on earth. And I would like to think that wherever she is, her gait is swift, she is fleet of foot and at last she is the best sheepdog in the great paddock in the sky.

(Jane in happier days)

(Jane shortly before her death showing weight loss and lack of condition)