Not too long ago, a breeder acquaintance whelped a new litter of Westie pups. All seemed normal until Day 3 when one puppy's yet-opened eyes suddenly bulged. A quick trip to the vet eased the problem: the eyes were gently forced open, releasing pus, and an anitbiotic and ophthalmic drops were prescribed -- normal therapy for neonatal conjunctivitis. This condition is not rare and occurs when bacteria gains entrance to the closed space behind the lids, either via the bloodstream or through a small scratch near the eye.

As the puppy grew, the initial blue cloudiness one sees in just-opened eyes did not disappear. At eight weeks, the pup was seen by a veterinary ophthalmologist who determined that it was not the early infection causing the situation, but rather that persistent pupillary membranes were clouding the eye. This pup was partially blind.

The breeder was very disturbed to learn that this is a disorder seen in West Highland White Terriers and is one of three breed-related eye disorders recognized by the ACVO (American College of Veterinary Opthalmologists). In the newly-updated volume published by the ACVO, Ocular Disorders Presumed to be Inherited in Purebred Dogs, three disorders are listed for Westies, as follows:

The "Breeding Advice" option given is determined by the significance of the condition to vision and/or very strong evidence or heritability. NO means substantial evidence exists to support the heritability of this problem AND/OR the problem is suspected to be inherited but does not represent potential compromise of vision. Based on accumulated evidence, BREEDER'S OPTION may be upgraded to NO or, if sufficient evidence becomes apparent, may be deleted completely.

What are these three disorders, and how are they treated?

Persistent Pupillary Membranes (PPM) is a birth defect caused by persistent blood vessel and iris tissue remnants in the anterior chamber (space between the cornea and iris) which fail to regress normally during the first three months of life. In the Westie, these strands often bridge from the iris to the lens and may result in cataracts with resultant vision impairment.

The genetic potential is unclear and both sexes can be affected. The vast majority of pups will retain adequate vision. The usual treatment involves using pupillary dilators, such as atropine, while the eye is growing to allow for adequate stimulation of the retina during its development.

Cataracts are partial or complete opacities in the lens of the eye and are found to be inherited in many breeds. Other (non-genetic) causes are diabetes, trauma, inflammation, puppy-milk replacers (nutritional deficiencies), or PPM.

The type of cataract described in the Westie initially involves the posterior Y sutures (the Y-shaped junction of the lens fibers) and may progress, resulting in vision impairment. The age of onset is less than six months of age and a recessive mode of inheritance is suggested by the pedigrees which have been studied.

In 1981, a study of cataracts in the breed was done in Sweden; of ninety-seven inter-related Westies (ages six weeks to thirteen years). seven of the twenty who were less than six months old were affected while forty-two of the seventy-seven adults were likewise diagnosed with cataracts. This study resulted in the recommendation that neither affected dogs nor their sires or dams should be used in a breeding program. To limit the spread of this condition, litter mates should not be bred as each litter mate carries a 0.67 likelihood of being a carrier.

The only treatment for cataracts is surgical removal. There is the option to monitor the eyes for complications which might occur, such as inflammation, glaucoma, or lens luxation. Dogs are very good at adjusting to gradual vision loss as long as their environment is not changed.

Cataract surgery is elective and requires a significant commitment of time on the owner's part as eyedrops must be administered both before and for about six weeks after surgery in addition to multiple check-ups during the healing period. Cost is considerable as this surgery is performed only by specialists. One vet lists costs of approximately $400 pre-op and surgical costs of an additional $1400 for one eye, $1700 for both. However, the success rate is generally over 90%.

Keratoconjunctivitis siccs, or Dry Eye Syndrome, or simply KCS, is a condition resulting from a lack of tear production. Tears normally function to keep the surface of the eye moist and healthy. Conversely, a lack of tears causes the eye to become irritated and scarred. The clinical signs of KCS include heavy mucus production, redness, rubbing at the eyes, cloudy eyes, corneal ulcers, and even general lethargy. After an extended period of dryness, the surface of the eye (cornea) begins to accumulate dark scar tissue and blood vessels, to the point of blindness . . . similar to having a complete layer of mud on your glasses.

The cause of dry eye is usually unknown but congenital disorders in puppies, infections such as canine distemper virus, and sulfa antibiotics are all know causes. KCS is seen very commonly in Cocker Spaniels, Shih Tsu, Lhasa Apsos, Bulldogs, Schnauzers and Westies.

In a study of KCS in England, it was found that 35% of two hundred affected dogs were West Highland White Terriers and that 70% were female. In this study, the range of development was foud to seven years and there was a familial tendency. Many of the dogs also had concurrent dermatitis and otitis externa (outer ear infection).

In America, ten million humans suffer from Dry Eye, which is more common in women than in men. The leading cause appears to be autoimmune disease, such as rheumatoid arthritis and lupus, and also other disorders such as thyroid, diabetes, asthma, incurable disorder, of which 90% are women.

It is interesting to note that although no direct connection has yet been proved, some feel our Westies have a weaker immune system than other breeds of dogs, and it is an intriguing theory that perhaps this may predispose them to skin, ear and eye problems, all conditions which have been proven in certain cases to be immune-mediated inflammation of the tear-secreting glands.

Treatment for KCS has three objectives: (1) to stimulate tear production; (2) to lubricate the cornea; and (3) to decrease the scar tissue. For the pst decade, specialists have used the drug Cyclosporine to accomplish all three objectives. Cyclosporine is an immunosuppressant which is used primarily in human patients after organ transplants. For topical ophthalmic use, a 2% solution in oil was advocated. However, this solution has not yet been approved by the FDA, although veterinary ophthalmologists have been using it for years with an approximate 80% success rate. In most cases, the drug must be used indefinitely as KCS is a life-long problem.

In severe cases, parotid duct transportation is indicated. One of the salivary ducts from the mouth is transplanted up into the corner of the eye. Saliva then takes the place of tears and keeps the eye lubricated. This operation is usually successful but often has cosmetic side effects as the constant flow of "tears" can badly stain the hair around the eyes.

One major drawback of preventing KCS is that it is often not apparent until a dog has finished his or her breeding career; thus a popular stud who may be affected later in life could pass this trait on to offspring unbeknownst to his owner, and the dog may even have died of old age before his progeny exhibit any signs of KCS. Many specialists lean towards the suggetion that KCS does have a genetic basis, but the mode of transmission is unknown.

One veterinary ophthalmologist we consulted has not seen any major change in the number of cases of these three Westie eye disorders he has seen in the past ten years. However, it must be remembered that not all Westie owners bring their dogs to specialists, hence many cases may be unreported.

Likewise, very few Westies are reported to CERF (Canine Eye Registration Foundation). CERF was founded by a group of concerned owners/breeders with a goal of eliminating heritable eye diseases in purebred dogs through registration, research, and education. CERF cooperates with the ACVO to maintain a registry of purebred dogs that the ACVO Diplomates (members) examine and have found to be unaffected by major heritable eye disease. CERF also maintains a data base containing information generated by all examinations done, which helps breeders identify trends in eye disease and breed susceptibility.

From 1991 to 1995, 153 Westies were reported to CERF versus a total of 50,462 Westies registered with the AKC from 1990 to 1994. And more recently, in 1996, only six Westies were cleared by CERF and assigned a CERF number.

From 1991 to 1996, CERF reports that 68 Westies (29 males and 39 females) were examined and reported to have cataracts. Twenty-four Westies (16 males and 8 females) were found to have PPM. It must be remembered that these figures in no way are indicative of the number of Westies affected as most are not seen by specialists.

Why is it important to register a Westie with CERF? Not only does CERF registration support the AKC's role in promoting responsible breeding and ownership, but it shows your support for the mutual goal of eliminating these diseases and provides puppy buyers and other breeders with proof of your commitment to maintaining the highest quality bloodlines. For further information, CERF now has a Website at http://www.vet.purdue.edu:80/~yshen/. They may also be contacted by phone at 317-494-8179.

For several years, AKC has published CERF numbers for cleared dogs both in the quarterly Show Awards and printed on the dog's registration, and those of the dog's progeny. However, effective December 1, 1996, AKC has begun to publish ONLY CERF (and OFA) numbers for dogs positively and permanently identified at the time of testing, by either microchipping and/or tattooing. CERF requires that each dog be re-certified each year while AKC will allow that certification to stand for two years before removing the CERF number from the dog's name. CERF is presently working on a system whereby breed clubs will be given the option to exercise breeder option on certain diseases, setting guidelines for controlling the breeding of affected dogs.
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