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" FACTS ABOUT PROGRESSIVE RETINAL ATROPHY "
( PRA )
~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~ By Leona
Domino ~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~ |
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PRA is a general term for a number of eye diseases. These diseases have included certain features. One is that the diseases begin at a very specific time in each breed. The diseases progress at a very predictable rate. The dogs that are affected with the disease become blind at an age very
specific to the breed. Some become blind early, some late. The
end result is blindness. There are a number of different kinds of PRA. PRA can be divided into either dysplastic disease, where the cells develop abnormally, or degenerative disease, where the cells develop normally but then undergo a damaging change. Usually, dogs affected with PRA first experience difficulty seeing at night or in dim light. As the disease progresses, the dog is finally blind both day and night. Papillons are affected with a slowly progressive degeneration that causes blindness at seven to
eight years old. At present there is no cure and no treatment to
arrest the course of degeneration in the affected dogs. PRA IS NOT CONTAGIOUS. It is an inherited disorder, passed along through the generations by a simple autosomal recessive gene. Simple autosomal recessive inheritance means that if a dog is PRA affected ( is either blind or going blind ) BOTH of the parents must possess the PRA gene. The dog has inherited the PRA gene from BOTH sire and dam, giving him a double-dose of the gene. Only when a dog has inherited TWO PRA genes, one from each parents, can he be
actively affected with PRA blindness. PRA gene. They are referred to as CARRIERS. A carrier shows NO physical signs of possessing the PRA gene. A carrier has no outward signs that it carries the PRA gene. The way a PRA carrier reveals itself as a carrier is by
producing a PRA AFFECTED ( blind ) offspring.
parent donates ˝ of its genetic material to the new puppy. Therefore you will note that a carrier will not have signs of PRA but can continue the disease 50% of the time. If bred to a bitch with the same gene then an affected puppy can be
produced. It takes 2 donated genes to produce affected. Since carriers appear normal outwardly, the problem becomes complicated. The situation can be compared to an iceberg. Blind animals make up the tip of the iceberg that shows, but underneath the surface are the carriers. They cannot be easily detected as of this date. It is possible for carriers to remain “hidden” in breeding programs before they finally, if ever, produce an affected puppy. Each time the carrier is used for breeding, a percentage of more "little-carriers" are added to the breeding population. As time goes on the more carriers there are within a breed, the greater chances are that
affected will ultimately be produced by breeding two "hidden"
carriers together. That is impossible to answer. Since there is no reliable way to identify the carrier status until it reveals itself by
producing a blind puppy, many carriers will never be identified.
Research is being conducted at Michigan State University ( Michigan, USA ) to find the marker on the gene that would result in breeders having a test that would tell them the genetic state of the breeding dog. Dr. Simon Petersen-Jones is the scientist working on this project. The Papillon Club of America ( PCA ) is assisting with getting a colony of affected dogs available to the doctor to use in his research.
CERF and
stay vigilant regarding your dog’s vision. Keep in mind that
night blindness or dim night vision is the first sign.
( See : Night Blindness Test below )
Do not breed to a dog that has produced an affected puppy or that has not been cleared by an eye exam. Eye exams by
a V.O. should start by 2 years and be yearly until 8-9 years of
age. vision. This may not be visible to the V.O. doctor until 2 to 3 years of age, do the night test on all your puppies.
3-4 month old puppies may show night vision hesitancy. This
would be a serious indication of vision problems to come. See test procedure here. It is so simple.
You only need a
flashlight and red tissue paper.
THE "NIGHT-BLINDNESS TEST"
An Aid In Detecting PRA
Update
of the previous article :
Home Testing for PRA
_____________________________________________________
A feasible way for most
everyone to check for the
possibility of Progressive
Retinal Atrophy in their
Papillons is a
method referred to as
"night-blindness test".
The properly done
electroretinograph is the
most definitive method of
detecting retinal
deficiencies but
a
"night-blindness test" will
give you an indication of a
problem long before an ophthalmoscopic
examination
will find signs of disease.
I have begun to use this
test on my puppies prior to
ERG to prove the
correlation. I suggest it
to be
performed at 12, 14,
and 16 weeks.
WE use 2 flashlights using
“D” batteries. ( make sure
that the batteries are new )
Two thicknesses of
red
tissue paper are held in
place over the light with a
rubber band. The
flashlights are placed at
least
5 feet above the floor
directing the beam of light
to opposite corners of the
ceiling.
The room must be totally
darkened … it’s necessary to
cover the windows. The
flashlights should give
just
enough light so that you can
observe the dog ( a
normal-eyed dog can see well
in red light )
therefore,
give your eyes time to
adjust to the dim light,
before proceeding with the
test.
Prior to turning off the
regular lightning,
set-up an obstruction in the
center of the room, such as
;
~
2
chairs placed on
their sides,
~
a grooming table
on its side,
~
2 waste baskets
side by side, or
a large carton
Crate the dog to be tested
in a corner of the room.
One person will release the
dog about 6 feet in front of
the obstruction while
another person ( well known
by the dog ) calls the dog
from behind it. My
experience has been that a
normal-eyes dog will around
the obstruction. Animals
affected with a moderate
loss of sight will walk up
to the obstruction and then
walk around it, while a
severely affected dog will
walk into the obstruction,
usually bumping it solidly.
Young puppies ( 12 to 16
weeks ) tend to inquisitive
and if they are normal-eyed
will boldly walk up to
the
obstruction and check it
out, before going around.
They will also, move about
the room seemingly
unaware
of the lack of the light.
Affected pups prefer to
remain where you placed
them. One puppy did
come to
me after I stepped to the
side of the obstruction,
continentally talking to her
… she crawled into
my feet
and sat there crying.
The size and color of the
testing area will determine
the amount of light needed
to observe the dogs
behavior, therefore it may
be necessary to add more or
larger flashlights.
Naturally, a light colored
ceiling will reflect the
light more readily than a
dark ceiling.
All humans present must
remain in front of the or
directly behind the
obstruction, not beside it.
The obstruction can vary in
width from 18 inches to 3
feet with at least 2 feet of
space on each side.
Be awe
that the dog is not
following a wall.
You must be be sure that the
dog being tested will
respond in a positive manner
to the person calling him.
No other words, the dog must
know and like the person
calling and know how to
respond to the word
or words
used, such as “come”.
Also you may wonder if you
are using the proper
procedure but, after going
through it several times you
will gain confidence.
Do not take the dogs
personality into
consideration when you
judge the results ...
there is no such room for
rationalization.
Instead, have the dogs with
inadequate performances
examined by
an ophthalmologist and a
"proper" ERG, if possible.
OPEN THE
"NIGHT BLINDNESS TEST" IN A OWN PAGE INCLUDING A PRINTABLE VERSION (
WORD DOC )
of the body cells are being plotted for many human and dog diseases. Many dog and human problems have been solved
and the PCA is working in this direction with Michigan State to
find the PRA gene. a problem that we can work to eradicate it. Personal ego’s need to defer to scientific discovery. The affected animals are very valuable and should NOT be hidden. Any breeder admitting to having an affected should be praised and be recognized for his/her integrity. These people are the role models for our breed.
They are the ones that will help solve the problem and perhaps
be the first to eliminate problems from their lines. The day will come when we can all have our dogs genetic background available.
RECESSIVE GENE INHERITANCE PATTERN
GENETIC CHART
Applicable to GM-1 and PRA
_________________________________________________________________________________________________________________
-represent
genetic
: NORMAL
-represent
genetic
:
-represent
genetic
:
AFFECTED
These are only six possibilities for
mating.
Each of our dogs is the product of one of
these six
breeding
combinations. Both
GM-1* and PRA, Progressive Retinal Atrophy,
are recessive
genes. This
chart can be applied to the
genetic
inheritance pattern
of each disease.
_________________________________________________________________________________________________________________
EXPECTED
AVERAGE RESULT, BASED ON A
MINIMUM OF 16 PUPPIES
PARENTS
OFFSPRING
Both parents are NORMAL, not
carrying the recessive gene.
100
% of the puppies will be
NORMAL.
PARENTS
OFFSPRING
One
parent is NORMAL, one parent
is CARRIER of the recessive
gene ... 50 % of the
offspring will be NORMAL, 50
% will be
CARRIERS.
In
"Storage" disease Carriers
can be detected by a blood
assay.
analysis
is critical
PARENTS
OFFSPRING
An
AFFECTED parent will always
produce CARRIER offspring.
In
this situation, only 50 % of
the offspring will be
AFFECTED,
but
the other 50 % will still be
CARRIERS.
AFFECTED individuals used
for breeding automatically
identify
PARENTS
OFFSPRING
CARRIER to CARRIER breedings
will produce 25 % AFFECTED
offspring ; 25 % NORMAL
offspring ; and 50 %
CARRIERS.
In
"Storage" disease, the
process of genetic
identification has
different
offspring's genetic make-up
is extremely complex.
PARENTS
OFFSPRING
An
AFFECTED parent will always
produce 100 % CARRIER
offspring.
AFFECTED parent breed to
NORMAL parent will not
produce
any
AFFECTED offspring, but they
will all be CARRIERS.
PARENTS
OFFSPRING
AFFECTED parent bred to
AFFECTED parent will always
produce all AFFECTED
offspring.
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