I would like to share Posey's story and its unusual coincidences with you and ask that any of you who may have similar stories will offer any information that will help us to learn more about a possible "new" Westie problem.
My beloved alpha bitch Posey (Purston Primrose), now fifteen years old, began coughing earlier this year - an occasional dry unproductive cough that followed no particular pattern. Fearing congestive heart failure, I had Posey radiographed which confirmed that she had an enlarged heart. She was given Lasix, a diuretic, but the cough continued.
Early in September, I decided I wanted a second opinion and so took Posey to Dr. Sydney Moise, a cardiologist at Cornell's Veterinary Hospital in upstate New York. Sophisticated testing - echocardiogram, doppler (ultrasound), more radiographs and a CBC panel - was done. Dr. Moise determined Posey's heart was normal and explained that Westie hearts tend to be larger for the size of the dog, as is true for other breeds with their shape of body.
However, Posey indeed did have a problem. Dr. Moise determined that she has probable West Highland White Terrier Lung Disease, for which there is no cure.
I was dumbfounded and queried Dr. Moise about this disease as I had never heard of it. She explained that this is a pulmonary fibrosis seen in middle-aged to older Westies. Several cases had been seen at Cornell and it appears limited to Westies (although the only mention I was able to find in the literature stated "terriers and some toy dogs could develop pulmonary fibrosis.") Clinical indications are troubled breathing, and in some dogs, coughing. There are radiographic changes and the blood gasses are abnormal, showing hypoxia, or lack of oxygen to the tissues. The cause is unknown. Therapy, such as bronchial dilators, can only "comfort" the dog. There are no medications that can cure the disease and dogs affected can live for months or years, depending on the severity.
Three weeks later, Posey and I returned to Cornell for more testing, including a tracheal wash to determine if Posey had any bacterial infection that might cause bronchitis; she did not. Dr. Jeffrey Toll concluded that Posey's cough could also be caused by a chronic or an allergic bronchitis, in addition to her probable lung disease, and that she would benefit most from very low doses of cortisone.
One week before Montgomery, I shared my discovery with Dorinda Dew, whose nine-year-old bitch Anna, had just been diagnosed with possible COPD (chronic obstructive pulmonary disease) and was also having a tracheal wash and other tests. Anna's symptoms had begun with rapid breathing, and tests showed she had a high white blood count that was being brought under control with antibiotics so that steroids could be given.
A few days before Montgomery, I contacted Anne Sanders who had just received information that one owner had three Westies die of this disease, and that at the same clinic, six of seven Westies had been diagnosed with the same problem over the past year. We planned to discuss the subject at Montgomery.
On Sunday afternoon at Montgomery, while waiting ringside for Best in Show judging to commence, Gale McDonald came up and handed me a veterinarian's report on "Progressive Pulmonary Disease in Westies." Dumbfounded, I asked how she came by this information. A couple living in North Carolina had contacted Gale through the WHWTCA information phone number for help when their much-loved Westie died - of Westie lung disease. Incredibly these owners so wished to share their vet's report and to meet Gale that they drove more than 700 miles round-trip to hand-deliver the findings. Their vet felt that the condition, occurring usually between nine and eleven years of age, is considered idiopathic (unknown cause) and hereditary. Their dog was treated for 1 1/2 years, using conventional medicine and acupuncture, but could not be cured.
I mulled over the similarities, wondering if hereditary could play a part. Once home, I contacted Gale to see if she had been given a pedigree with the vet's report. As she read it off to me, I nearly dropped the phone! A well-known champion appeared three generations back on the sire's side on both Posey's and the deceased Westie's pedigree, and another very well-known champion appeared on the dam's side of both pedigrees! Could there be some truth that this might be inherited, or was it merely an interesting coincidence???
Where do we go from here? Do we have a new Westie disease, possible one with a hereditary basis? Will we look at each of our Westies who may be coughing or exhibiting breathing abnormalities and panic? Is this a known disease, or has it been branded Westie Lung Disease because one veterinarian, somewhere in the United States, just happened to examine two Westies within one month who had breathing irregularities and blithely put a name on it?
Anne Sanders suggested I refer to Malcolm Willis' book, Practical Genetics for Dog Breeders (New York, 1992). In Chapter 12 (also presented by Dr. Willis at the AKC Parent Club Genetics Conference in October 1995), he warns of jumping to genetic conclusions. He presents a flowchart as a route to take when trying to determine whether or not a defect or disease is inherited. (Please refer to the chart at the end of this article.)
Willis points out that if a genetic cause is suspected, then more data must be collected an that this will usually involve an initial questionnaire through the breed club. He feels no defect or disease is the province of one group, but affects all clubs, breeders, and owners within that breed and all have a moral duty to collaborate. Regarding pedigree study, he cautions that frequently breeders with access to "affected" pedigrees eagerly label "Dog X" as the cause for no other reason than Dog X appears on each side of the pedigree. Without mathematical testing for proof of mode of inheritance, such statements are merely harmful and not helpful in any way.
It would seem that we are just beginning at the first step of Willis' flow chart. Judging from the case histories collected in the last three weeks, it would appear that some Westies have a problem. These Westies seem to share the same conditions or clinical findings. What about the clinical and biochemical parameters, or boundaries? Do all the affected Westies have the same lab results? Do they all respond to the same treatments? Are we talking the same disease in these six Westies?
We do not yet have definitive proof that Westie Lung Disease even exists, and therefore must determine what the problem is. If already reported in the literature (scientific journal) and the cause is known, then the answer is very simple: We know that Westie Lung Disease exists. However, if not previously reported, we have a tedious and very time-consuming task ahead of us, beginning with an attempt to determine the cause. Many possible causative agents are possible. Is it a manufacturing defect (something that goes wrong at a particular stage of pregnancy)? Is it related to diet? Is it disease-related? Or is it genetic? Or might it even be caused by environment?
We have a long way to go before we can even begin to brand Westie Lung Disease with a genetic stamp. We need as much information as we can collect on similar cases.
For additional information, please refer to the following sites and their health articles on Pulminary Fibrosis:
www.westieclubamerica.com;
www.westiefoundation.org.
DEFECT REPORTED
Define the problem, describe the
condition accurately, try to find clinical
and biochemical parameters.
|
SEARCH PUBLISHED LITERATURE
(include in your search similar defects in other
species not just the dog)
|
----------------------------------------
| |
ALREADY RECORDED IN NOT PREVIOUSLY REPORTED
LITERATURE AND |
CAUSE KNOWN INVESTIGATE THE CAUSE
| |
| -----------------------------------------
| | | | |
| GENETIC MANUFACTURING DIETARY DISEASE
| LIKELY Advise accordingly
| |
| SEEK MORE DATA
| |
| -----------------------------------
| | |
| APPROACH BREED CLUBS REPEAT
| OR BREED COUNCIL MATING
| (send out questionnaire, collect
| other cases, sexes, litter sizes
| pedigrees)
| |
| ------------------------------
| | |
| FAMILIAL DISEASE BUT NO INHERITANCE
| INHERITANCE PATTERN PATTERN DEFINED
| ESTABLISHED (try to arrange matings
| to test the mode of
| inheritance proposed)
| ---------------------------------
| | |
PROVIDE BREEDING ADVICE PUBLISH SCIENTIFIC
TO BREEDER AND CLUBS PAPER
SET UP SYSTEM FOR
FUTURE RECORDING OF
DEFECT
Scheme for investigating a possible inherited defect.
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