Re: [pyrnet] GPCA -- and dwarfs

[Kshoffman@aol.com]

In a message dated 10/12/2000 7:35:47 AM Eastern Daylight Time, 
JGentzel@aol.com writes:

> Yes, Kelly a lot of misinformation out there.

I disagree. I don't feel there is a lot of misinformation out there, at least 
not in the way I believe you are suggesting there is.  It seems to me you are 
suggesting the educational information that has been or is being disseminated 
to club-affiliated breeders is somehow attempting to pull the wool over their 
eyes, and I believe you are dead wrong about that.  They've been studying and 
working on this project for how many years now, I'm thinking going on 10 at 
least.  How long have you been seriously studying and working on the project?

I believe there is a wide range of variation in level of knowledge and 
understanding of the information that is out there, hence a wide range of 
variation in how people *interpret* and *apply* the information that is out 
there.  I also feel even the alleged experts on this condition have changed 
their tunes with regard to interpretation of just how severe or concerning it 
is relative to quality of life issues over the past several years.

Much of the range of variation in interpretation can be the result of folks 
own personal biases, opinions, beliefs, and it often boils down to WHICH 
information and which so-called experts they choose to put more stock in.  
Even scientific evidence generally can't be considered to be exact, precise, 
and absolute. Science involves a lot of trial and error. There are almost 
always differing opinions in the scientific community and research academia 
as to how any derived data should be interpreted, how much confidence should 
be placed in the findings, and more importantly, how it should be *applied* 
in the decision making process.

I started seriously studying and scrutinizing issues related to dwarfism in 
the breed around 1991-1992 or so.  I have made it a point to look at the 
whole picture and ensemble, not just parts and pieces, not just one 
particular viewpoint.  While I admittedly have my own biases relative to the 
matter, I can unequivocally say my perhaps biased opinions have been arrived 
at after thoroughly studying ALL points of view on the matter.  I have not 
formulated my opinions nor drawn my conclusions based on only the part of the 
picture I might prefer to see.

> Patric  posted a Literature search on September 28.  Go and review
> that and you will see some scientific evidence of radiographical
> abnormalities in the vertebrae.   I will quote just a short portion from
> only one, having to do with Pyrs directly:
>  
> "Radiographic abnormalities in chondrodysplastic Great Pyrenees
> are restricted to the metaphyses of long bones and vertebrae."

Yes, I recall the list of journal abstracts/citations Patric posted, and I 
have read these abstracts before, but I have not read all the full text 
articles, have you?  I did refresh my memory and read the abstracts again.  
Here's where we get into an interpretation issue, and certainly we can't 
fully correctly interpret the researchers findings in proper context only by 
the abstract, we'd need the full text of the articles, and we'd need someone 
skilled in reading journalese to do some "translating" for us.

Aside from that, I asked you to cite what "debilitating spinal deformities" 
had been documented in Great Pyrenees, DEBILITATING being the operative word 
here.  These abstracts don't give me that information.  The abstract you 
refer to addresses radiographic findings which may or may not be 
consequential as to implications with regard to functionality, these findings 
do not address functionality nor quality of life issues.  For that 
information and assessment, I must also consider what you are calling 
anecdotal data and my own personal experience and that of others.
  
>  DR Padgett classifies it as follows:
>  
>  Severe Traits
>  
>            Disorders that disfigure, maim or otherwise render an animal 
>  nonfunctional (i.e. cataracts, retinal dysplasia and
>       detachment, chondrodystrophy). 

Well, it might interest you to know that apparently Dr. Padgett has revised 
this, because he presents the information differently in his book.  In his 
book he states the following (Page 161):

"2. Disorders that disfigure, maim, or otherwise render an animal 
nonfunctional
Example: English Pointer dwarfism, cataracts, retinal dysplasia and 
detachment, Malamute chondrodystrophy, PRA, deafness."

A couple of my own comments/opinions on the above:

1. He does not *specifically* mention dwarfism in Great Pyrenees nor dwarfism 
in several other breeds even though he lists Pyrs and *several* other breeds 
in the back of his book as having dwarfism (chondrodystrophy) occurrences.  
Somehow, believe it or not, it would appear he has differentiated in the 
various expressions and types of dwarfism and the degree of severity and 
quality of life issues as such in his priority scheme.

2. I disagree with categorically throwing cataracts, retinal dysplasia (folds 
type), PRA and even deafness in this category:

A. Cataracts is a VERY BROAD catchall category, and the majority of the 
cataracts that are identified in many breeds NEVER cause blindness, and some 
(many) don't even cause vision impairment until old age if at all.  That's 
not to say we should breed affected dogs, not AT ALL; it's an argument 
against his broad assessment of what is maiming, disfiguring, or 
nonfunctional.

B. Same for retinal dysplasia-folds type.  Rarely does this conditions result 
in blindness, or even much vision impairment.

C. PRA - once again I can't categorically agree with this.  Researchers are 
finding a wide variation in expression of PRA (and different mutations 
responsible in different breeds, even at different gene loci), and some PRA 
dogs get along just fine and dandy, retain plenty enough vision to be 
perfectly functional with it.  Not to mention PRA is most commonly a 
late-onset disorder in most breeds, and since it is also a progressive 
disorder, a dog that first presents at the average age of  6 yrs often still 
has good enough vision to see pretty well until 10+ years of age.  We are 
seeing this in Belgian Tervuren, and we have an ongoing research project in 
progress, this is not something I dreamed up.  The type of PRA we have in 
Tervs is "stumping" the experts at MSU because it's not progressing like they 
"expect" it to based on what prior scientific research dictates -- dogs 
diagnosed at 6-8 yrs of age are still navigating complex mazes at 10-11 years 
of age.  Aguirre's team at Cornell are seeing the same thing in Basenjis, 
variable expression of the condition, little of it resulting in total 
blindness and virtually none of it rendering animals nonfunctional.

D. Whether or not deafness causes an animal to be nonfunctional is a matter 
of interpretation.  I think those who have lived with and successfully 
trained unilateral or bilateral deaf dogs are in a much better position to 
determine if their partial or fully hearing deaf dogs are "nonfunctional".

>  There are others references.  If we rely on our information from
>  antidotal information we will leave ourselves open to very possibly
>  dealing with rumors, interpretations, spin, lies, mistakes, and the
>  whole spectrum of  misleading information.

But you can't rely *solely* on medical journal citations either!  First off, 
have you even read the full text of these articles, or are you just focusing 
on the abstracts?  Do you really know what the researchers are presenting 
her, and precisely what they are saying in context?  Secondly, virtually all 
scientific findings are debatable and open to further scrutiny and testing 
and hypothesizing by other scientists.  And thirdly, research journal 
articles that focus primarily on histiological or pathological or etiological 
findings don't and can't effectively address quality of life issues.  If that 
were true, I could have just written off three members of my immediate family 
as having no quality of life and being nonfunctional a long time ago!

>  Does it surprise you that
>  you do not hear a lot about the culled dogs from a litter?

Of course it doesn't surprise me, but it doesn't tell me they were culled due 
to being maimed, disfigured, and nonfunctional.  They are often culled 
because there is a perception by some that this condition is something far 
more grave than it is with regard to quality of life issues, and there is 
also the desire to hide the condition because of the unwarranted stigma 
associated with it. Aside from that, the dwarfs that are culled in litters 
are culled LONG BEFORE anyone has had a chance to assess quality of life 
issues. They aren't culled for health reasons, so cut me a break.  I think 
most of us know these dogs are culled primarily for esthetic reasons and the 
desire not to deal with them, simple as that.  Personally I think culling or 
not is a choice each breeder has to make, I'm not making judgments on 
culling, but let's not pretend it's done for reasons other than why it is 
generally done.

>  The survey was antidotal.  Nothing wrong 
>  with that as long as we understand what that means.  It does not
>  mean that it represents any degree of  accuracy from science standards.

So what?  I don't believe that's what it was intended to be. What it DOES 
provide is a glimpse of what those who LIVE WITH a dozen plus or so dwarf 
Great Pyrenees have evaluated as these dogs quality of life.  None indicated 
the dogs were in pain, none felt the dogs were maimed, none felt the dogs 
were nonfunctional.  Are you saying you think they were all lying?  Why would 
they do that?

>  Lets look at this differently.  Lets suppose we accept Dwarfs as some
>  cute harmless aberration of our dogs.

Why does the word "cute" always have to get thrown into this point in 
context?  Why can't we just say a comparatively harmless aberration to other 
genetic health issues that afflict the breed.  If we accept there are worse 
things a breeder might have to deal with, that doesn't mean we think it is 
cute nor that it's okay to breed two carriers together on purpose.

>  And yes people are getting rich breading 
>  them, which is how this thread started anyway, 

Joe, once again, the majority of them are STERILE and can't be bred.  Dr. 
Sande I believe tried two or three times to do a dwarf to dwarf breeding to 
test the mode of inheritance, and those experiments failed because the 
majority of dwarfs are sterile and hence incapable of reproducing.  No one is 
or ever will be "getting rich" breeding for dwarf Great Pyrenees.  I doubt 
anyone will EVER successfully breed two dwarfs together.  I've heard the 
concerns that breeders (I'm going to assume here breeders outside of GPCA) 
might purposefully breed carriers together to get dwarfs, and I've heard 
there have been inquiries to the health committee from prospective pet owners 
looking to acquire a dwarf, so what does that tell you with regard to how 
severe this condition must be viewed in general?  Do you think people are 
calling the health committee asking where they can get an epileptic Pyr or a 
dysplastic Pyr or one with bilateral luxating patellas, or one with subaortic 
stenosis who might drop dead at a year of age, or one with cardiomyopathy who 
might do the same?  Tell me Joe, which of any of those conditions do you 
honestly feel is less concerning and less of a threat to the breed than 
dwarfism?  I agree dwarfism is wholly misunderstood by whomever these idiots 
are that call the health committee wanting one, but don't you figure they 
must have met one somewhere down the line, and didn't see anything overtly 
sickly about it, and talked to the owners who also didn't have any major 
issues with the dogs quality of life?  Otherwise, where would they get the 
(wrong) idea it's some cute harmless aberration?  We have a hard enough time 
on the "inside" getting information about these dogs, yet I'm supposed to 
believe that information flows quite freely about the existence of dwarfs in 
circles/communities outside those of club affiliates?  No where has dwarfism 
been glorified in educational materials, so how do these inquirers even know 
about them?  My cousin has a pet store Golden that's a dwarf (quite healthy 
by the way, age 6 or 7 yrs).  I imagine the same thing happens in Pyrs, that 
somebody's neighbor or friend or relative happened to get one and that person 
saw it and wanted one too.  No reason to believe the estimated 1% affected 
rate doesn't apply across the board to all breeding cultures and that there 
are pet owner folks out there, here and there, who've acquired dwarfs from 
farm breeders or backyard breeders or pet stores.  Yes, they misunderstand 
the condition, but apparently they are seeing seemingly health dogs, that is 
my point.  It seems to me the overwhelming evidence is it's not a serious 
quality of life issue in the majority of the cases.

>  i.e. the use of the marker 
>  study to identify Dwarf carriers thereby enabling those who breed
>  them to succeed better.

Joe, do you realize IF we get a DNA marker test, either a linkage or direct 
gene test, what the cost is likely to be per dog?  Probably somewhere in the 
neighborhood of $150-200.  I am quite positive no GPCA breeders are 
purposefully going to breed two carriers for dwarfs to "get rich", and as for 
those outside the club, most of them won't pay for a stinking hip ray on the 
parents, or for more than one (if any) set of shots for puppies, or for 
wormings, or for anything else that might cut into the profit, and they shove 
the puppies out the door at 4-6 wks of age to save more money, and you think 
they are going to shell out $150-200 a pop on a bunch of breeding stock to 
locate dwarf carriers so they can breed them together and produce ~25% dwarfs 
in the litter and get rich?  Let's get real here.  This is not something I 
fear in the least.  It's completely irrational and goes against everything we 
know about these types of breeders and what type of efforts, monetary or 
otherwise, they are willing to make when breeding a litter.

>  My remark that wait until they get the
>  deformed dogs, with the response, said then and repeated many
>  times, "What deformities."  Now that we have identified the
>  "deformities" we are now at a point that "oh, its not so much."

Joe, those abstracts merely "identify" some deformities by words that mean 
little or nothing to 99.9% of the people on this list.  Are you 100% 
absolutely clear and positive as to what meaning those words have in the big 
picture?  What are the IMPLICATIONS of these findings?  Don't you think that 
is important information to gather?  Don't you think perhaps it would be 
better either: a) for the author to explain in layman's terms what this means 
for practical purposes; or b) for some other orthopedist and/or radiologist 
to explain what all this really means, what the practical implications are?

>   So lets tell everyone how much is OK to tolerate in the
>  "Maimed and disfigured" dogs (not my words but DR Padgett's).
>   1% seems to be within the tolerance level of some from prior posts.

Yes, 1% of affected animals breedwide with a condition that the majority of 
time does not seriously negatively impact on quality of life issues is 
"within my tolerance level", especially when my scope also happens to include 
all the other genetic health defects mentioned elsewhere that are likely at a 
frequency higher than 1%, and that in my opinion are more debilitating, have 
more impact on quality of life issues, and are a much bigger threat to the 
breed as a whole.
  
>  So some of you tell us what percentage is tolerable as we go forward
>  with these cute little dogs being bred that are really a lot like Corgis,
>  Bassetts, etc. 

What exactly is your point here?  NO ONE has suggested breeding dwarfs 
together, not one single soul.  No one has suggested breeding two known 
carriers together to get dwarfs by design. So what do you mean by "go forward 
with these cute little dogs"?  Please clarify because I don't understand what 
it is you want or are concerned about.  Are you saying we should eliminate 
all potential (suspect) carriers from the gene pool?  Are you saying we 
should make a unilateral recommendation to do that?  What exactly ARE you 
saying and/or accusing folks (seemingly folks like me) of?

Kelley Hoffman
kshoffman@aol.com