Read Smitty's Story
Read Willi's Story

Progressive Retinal Atrophy is a disease that destroys the cells and blood vessels supplying the retina at the back of the eye. It is painless and the progressive blindness may not be noticeable for some time as most dogs adapt to blindness well. PRA occurs in every line of Tollers and appears to be late-onset, generally appearing at age 3 and onwards. The mode of inheritance in Tollers is unknown, but is suspected to be a simple auto-sommal recessive. Simple means that only one pair of genes is involved.  Auto-sommal means both sexes are affected. Recessive means that two disease genes must be present for a dog to show symptoms. If a pup receives two normal genes from its parents it will not be affected. If a pup receives one normal gene and one recessive gene, it will be a "carrier" for the disease.  If the pup receives two recessive genes from its parents, it will be affected by PRA. At present there is no way to tell a genetically normal dog from a carrier dog until a carrier dog produces an affected pup. An affected pup is automatically a "known" carrier.

Testing methods using a slit-lamp only looks at the cornea, lens and iris.  This method doesn't seem to identify affected Tollers until they are 5 years of age or older and already in a breeding program. The use of electroretinograph equipment (ERG) has provided diagnosis earlier in Tollers, generally by 3 years of age. The dog is tranquillized and the eyes are fitted with special contact lenses attached to recording equipment and coloured pulses of light are flashed and the eye's reaction observed. The equipment reads the opening and closing time of the retina and its reaction to different light frequencies. The availability of testing equipment varies.

The NSDTRC (USA) spear-headed the beginnings of a study at Cornell University in New York to determine the type of PRA that affects Tollers and the mode of inheritance which will hopefully result in the development of a blood-based DNA diagnostic test to identify genetically normal, carrier and affected Tollers at a young age. Cornell has developed a test for Irish Setters, Chesapeake Bay Retrievers, Labrador Retrievers and American and English Cockers. Toller owners on both sides of the border have continued to generously donate funds toward the  development of this test. If you would like to donate money towards Toller-specific research at Cornell you may send a cheque or money order to the Treasurer of either the NSDTRC (USA) or the NSDTR Club of Canada or directly to Cornell.
 

More information on PRA can be found at the following site:

http://www.sheepdog.com/diseases/pra/pramenu.html
 

Find out about the NSDTR Club of Canada's Hip/Eye/Heart Registry HERE

The following story was originally published in "Toller Talk" the newsletter of the NSDTR Club of Canada.

When Smitty was diagnosed with late onset PRA in June of 1995, I was devastated. There were no symptoms at all, the only reason I gook him to Guelph was because his sire had been diagnosed with PRA the year before. Dr. Kellar told me he had vision loss already, and as it grew worse, his other senses would compensate for the loss. He was 3 ½ years old.

I knew then there would be changes in our lives. Immediately, his conformation career was over. Although he had finished his Canadian championship the year before, he very much enjoyed going to a show and being in the ring. Bringing out the crates and loading the van often found him in a crate, hoping to go. Several times I have closed the crate door with him inside, put the key in the ignition,
arrived at the show site, only to find out I’ve left the leash at home. I finally got into the habit of leaving one in the glove box, just in case. He enjoys being fussed with, and often I will find him lying on the grooming table downstairs, while I do laundry. This dog was destined to be in the ring, whether an official show or a match; he loves it.

His obedience career was just in the training part of Open work, with a  promising future ahead. Field work was never a problem, as we don’t hunt nor do field training, although he has tolled geese on several occasions.

To me, the conformation ring is a place to exhibit a specimen of the breed that closely resembles the breed standard, and is free of health problems. Smitty is an excellent example of the breed, many judges told me so, but I could not in good conscience continue to show in the ring. We still go to matches just so Smitty can strut his stuff, and to answer the inevitable questions about this unusual breed.

The obedience presented a challenge. How am I to judge what my dog can and cannot see? Do I wait until he starts hitting the jumps as a friend says will happen? I talked quite a lot with Smitty’s obedience trainer, Paul Morrison of Training and Learning Centers, Waterford, Mich. Paul breeds and trains American Water Spaniels, so has experience working with challenging dogs. We came up with the following – continue working and training the Open exercises until I feel he is no longer confident in doing them.

While at the 1995 Credit Valley shows, he NQ’d three times over the high jump, and once over the broad. He would go over the high jump, retrieve the dumbbell, and then stop dead on the other side of the jump. At the broad jump, he simply walked up to the first hurdle and sat, going over with an additional command, but he did clear it in the other trials. At this point, I immediately thought, well, it’s all over – he’s retired now. Then I started thinking, well, he went over the high jump, found the dumbbell, he is just being his usual creative self, at my expense. I was actually relieved to think he was this creative. I have noticed, on the finish, he will sit a bit back from me, so that his nose is lined up with my knee, looking up so he can see me.

Of course, the true test in Smitty’s book is: can he see well enough to retrieve? I now bounce a tennis ball off the metal fence so it will give him a sound to follow. He uses his nose a lot more in the yard than in the house. Inside, he can find a ball under the deepest recesses of the couch, as well as find one under Jessica’s bed, which has who knows what under it.

Tracking is definitely in this dog’s future, as he only needs his nose, no sight required!

After the diagnosis, I came home to find a show schedule in the mail. I had entered Smitty in the UKC Premier Shows, mailed off the entry, and then promptly forgot about it. Well, I’m sorry, but $60.00 is a lot of money to throw away, and I just couldn’t blow off the entry fees, so we went with a friend who has Goldens. Much to my thrill, Smitty completed his UKC conformation championship with three Best of Breeds and a Group 4. He is also the first Toller ever to be awarded a UKC conformation championship. I did tell each judge who put him up, this was his last series of shows and why. All were very sympathetic to our cause.

I don’t know what the future holds for my dog. I have a few unanswered questions that will be answered by time alone. I only know that a lot of people have us in their prayers, and we are grateful for their support and kind thoughts.

Living with PRA doesn’t have to dominate your life, you just have to adjust your routine a bit. We enjoy our good times, and know that other owners are going through the same thing we are.

My attitude is: Okay, he has it, there is nothing I can do about it, so get on with it, and that is just what we are doing.

That was written in 1996 and following is an update from late 1998:

It is now September 1998 and Smitty is well. He has several noticeable limitations with his vision. He has no side vision, much like a horse with blinders on. To come up from behind him, and approach his head, he will startle, so we are careful to touch him gently or say something to him, to alert him as to where we are. This disease is not visible to the naked human eye. He looks like a normal dog and acts like one, too. Since the disease progression is slow and steady, he does not realise his vision is decreasing and the other senses are compensating for the loss. He has night blindness, so we leave a nightlight on for him during the night. He negotiates around the house quite well, with no problems whatsoever during the day.

His obedience career is over, he finished his US breed club CDX and got a leg on his Canadian CDX with a second in class and a let on his UKC CDX also. He lags too far behind on the heeling to qualify in that section.

He is not neutered, and I occasionally still take him to shows. At the age of seven years, he has matured wonderfully and truly looks nice.

He has a wonderful breeder, Alison Strang of Westerlea Kennels in British Columbia, Canada. She has been most supportive of us and encouraged me to keep showing him, if I desired to. He has since earned another Championship in a rare breed organisation, Federation of Rare Breeds, or FORB. With six group one placements in this organisation, his wall in the trophy room is lined with
ribbons, rosettes and silver platters. He is also the first Toller to earn a championship from this organisation as well! He still goes to the UKC Premier sows, where he has placed in the group or made the final cut every time, quite impressive for a little red dog, in a very competitive Gun Dog Group, where the different breeds total a magnificent amount of sixteen to seventeen at the
average Premier Show!

He is a very sweet dog, loving in nature, mischievous and very dedicated to his tennis ball. So much that his lower canines are ground down from fetching the ball all the time. When I show a judge his bite, I simply say “Tennis ball teeth” and get a knowing smile.

Both the US and Canadian breed clubs are working with Dr. Gus Aguirre of Cornell University in Ithaca, New York. These two clubs are supporting his research with our much appreciated dollars earned by fund raisers and other things. Dr. Aguirre and his staff are looking for the genetic marker for PRA in Tollers and are close to finding it.

We visited Cornell last year (1997) to see Dr. Aguirre in person as he wanted to see Smitty, a known Duck Toller with PRA, who was not hidden away and led as normal a life as possible. My husband and I drove him there, spent a few hours with Dr. Aguirre, where he took some photos of Smitty’s eyes and drew some blood for research purposes.

At the age of seven years, he is my best buddy and a dear dog to me. I could not ask for a better companion who I love dearly.

His formal name is CAN/UC/FORB. CH/U-CD Westerlea Seaforth Highlander CAN/4-H/SKC CD, US CDX/CGG

We just call him Smitty!

As to the PRA, we started noticing a haze in Willi's eyes about a year ago (age 10 1/2).
The vet said it was cataracts, but did not seem to be worried about it.  Recently, however, we noticed Willi was "spooked" by the dark - especially in strange places.  The haze was getting worse and we decided to take him for a consult to get laser surgery with Dr. Bussanich.  We were not expecting the diagnosis.

In hindsight, Willi has been less tolerant with other dogs and small children lately - age or vision?  He has also been sniffing far more while we are on walks - compensating for less vision?  When he sees another dog approaching, he stands still until it has approached and proved its friendliness - this is different behaviour from before.

It saddens me that we cannot do anything about PRA, but at the moment he still has decent vision during the day, and only compromised vision at night.  He can still play fetch at night along a lit driveway.  I was encouraged when Dr. Bussanich told us that because it was adult onset PRA, it may not progress very quickly.

Linda French and Willi