Progressive Retinal Atrophy - Della's Story
by Robin Camken


April 23, 2001

I received some upsetting news this past weekend when my eight year old Della was diagnosed with advanced PRA. The ophthalmologist, David Wilkie DVM, MS, DACVO, who is the head of the Ophthalmology Dept at Veterinary Clinical Sciences, Ohio State University,stated that she had, at most, 10-20% vision. I was very surprised because I had not noticed any symptoms of her reduced sight. To demonstrate, Dr Wilkie turned off the lights in the office and had me take Della to the far side of the room. She was very hesitant to move when called and slow to navigate around the table legs and garbage pail that were in her path. The room was only in semi darkness. With the lights on she could move quickly around the objects.

For a dog that is nearly blind, she shows virtually no symptoms. Della remains a very active dog that runs up and down stairs, jumps on the bed and into the van without hesitation. I have lived in four different houses in the eight years I have had Della. The most recent move was in August 2000. I have never noticed her to have any difficulty adjusting to a new environment. She has always been nervous meeting new people and will stand away back from the door when someone enters the house, until she recognizes the individual. In hindsight, this may not have been a temperament fault, but rather a function of her vision impairment.

Della was first examined by Dr Wilkie in February 1995, when she was one month shy of her second birthday. Her CERF paper has her graded as NORMAL, but with the following comment "faint linear hyper-reflective line on the dorsal to disc". Both eyes had the same line. Dr. Wilkie was not concerned about this line at the time of the first examination, other than it was an unusual finding.

Della was spayed at the time of her first CERF exam. Since she was assessed as normal, I did not see any reason to have her rechecked. I was taking my puppy to the eye clinic on Saturday, and took Della along just to see if the "line" in her eyes was still there. What a shock to find out the entire eye was now hyper-reflective. I asked if it could be anything else besides PRA that would cause this to occur. Dr Wilkie said no, it was definitely PRA.

There are several lessons to take away from my experience:

1) All dogs, whether they are in breeding programs or not, should have an ophthalmologist examine their eyes, ideally annually, but at least more than once.

2) If I had taken Della in for another examination sooner, she would have been diagnosed earlier and her parents may still have been alive and we could have confirmed their status - now it is too late.

3) Getting a clear CERF test on a Bernese Mountain Dog at 1-2 years of age does not guarantee that they do not have PRA. Ophthalmoscopy will not always detect PRA in some dogs until 4-8 years of age. I wonder how many affected BMDs there are in the population that have gone undiagnosed. I was lucky to pick up Della's case at all.

3) Della's vision problems were never noticed by a general veterinary practitioner. She had a complete physical in January 2001 for geriatric screening, which included a basic eye examination. She was assess to be in excellent condition.

4) Della showed no signs of having failing sight. Do not depend on physical symptoms to tell if you have a dog with PRA, there may not be any.

My intentions are to have Della participate in the BMDCA PRA research that Dr.Aguirre is conducting.

Robin Camken

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