Just Because of Jane
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 24th 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 programme.
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 become 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 6th, 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.