Progressive Retinal Atrophy

I have been asked by this person to remove her photo and all of my references to her.


I recently received this letter by snail-mail from my good Icelandic friend (I have been asked to remove this person's name from my blogs).


Dear James L. Hansen,

In the course of an eye examination by Finn Boserup on 29th November 2009 Icelandic Sheepdog Fljóta Táta, IS08170/04 was diagnosed with suspected Generalized PRA.
The veterinary surgeon recommends that she is not bred from until she is re-examined after 8-10 months.

In the Icelandic Kennel Club's regulations regarding pedigree registration it states that when a dog is diagnosed with PRA the dog itself, any progeny it might have, both parents and all previous progeny from the parents are banned from breeding.

As your dog Thórdunu Kría has the same sire as Fljóta Táta she is marked as banned for breeding with the Icelandic Kennel Club until Fljóta Táta is re-examined.

The Icelandic Kennel Club recommend that you have Thórdunu Kría eye-tested as soon as possible.

With kindest regards,
(Helga Andresd.)

Copy to DIF, the Icelandic Sheepdog breed Club in Iceland
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(I have been asked to remove this person's name from my blog.)

Here are some excerpts from my response.

Hello (I have been asked to remove this person's name from my blog),

It was nice to receive your letter today even though it contained extremely sad news about finding a suspected case of Progressive Retinal Atrophy, PRA, in one of your dogs in Iceland, Fljóta Táta (IS08170/04).

What a horrible thing to happen so close to Christmas.

That is truly devastating news.

Of course Thórdunu Kría, Leiru-Thórshamar Týri x Thórdunu Eyja, is tested annually for possible eye problems and has so far been OK. She will be six years old this year.

I will schedule an appointment for Kría as soon as possible. When I take her this year, I will be sure to ask her veterinary ophthalmologist to look very closely for signs of PRA. It should go without saying that I will not breed her this year because of this suspected case of PRA in her half-sibling, same father, different mother (Leiru-Thórshamar Týri and Galtanes Röskva).
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1. Do you have a list of all of the affected dogs besides Kría? Could I please have a copy?

2. Have there been any other cases or even suspected cases of PRA or premature unexplained blindnesses in Iceland?

3. Do you have any idea which ancestors, besides Leiru-Thórshamar Týri and Galtanes Röskva, might have been carriers of this gene?

4. There is a university in the state of Pennsylvania (Actually it's in New York.)that will test for the presence of the possible genes for the different kinds of PRA.

It might be worth sending them DNA samples from Fljóta Táta and her parents Leiru-Thórshamar Týri and Galtanes Röskva.

There is, as you know, more than one kind of PRA. I'm absolutely sure you know there are several genes that could be the cause of the problem in Icelandics.
http://www.optigen.com/opt9_test.html

Here is their email address: - genetest@optigen.com

There is also the possibility for further research into our problem at that University: -
http://www.optigen.com/opt9_research.html

5. Would it be possible for you to contact them directly?

6. Have there been any cases of PRA in Europe?

7. If so, do we know the suspected carrier ancestors in Europe?

Perhaps the worst part of all of this for all of us is simply not knowing what to do, isn't that right?

In the past we have found that a lack of direct communication has exacerbated the problems we have had. It would be nice to be able to avoid that happening this time.

It would be great if this proves to be a false alarm and the suspected case of PRA is not what we all fear.

If it indeed turns out to be one of the several forms of PRA, then it would be wonderful if we could find the correct gene and the gene test so we could test all of our breeding age dogs and remove all the carriers from the gene pool or, using them very carefully, breed carriers to known PRA-gene-free dogs and screen their offspring to retain as much genetic diversity as possible for the future. (We should all be able to agree that we do not need another genetic bottleneck at this point in time.)

Fortunately, from what I have read, it seems that the inheritance is not complicated like it appears to be for hip dysplasia. Simple autosomal recessives with genetic tests are fairly easy to eliminate from the gene pool with careful controlled breedings.

Alienating well meaning conscientious people from this project would be counterproductive and, in the long run, very detrimental for the breed. I hope we can avoid that.

8. Might it be possible to either:
a. actually ship Fljóta Táta to the states for examination by several veterinary ophthalmologists?
or
b. send an expert ophalmologist from the states to Iceland to examine Fljóta Táta?

It seems that time is of the essence here. Having to wait an additional eight to ten months for an update seems like a long time.

The sooner we can prove that the suspected affected dog either has PRA or does not have PRA, the faster we can eliminate the gene.

= = = = =

Best regards,

James L. Hansen

I did not get a response to my email yet.

(As of March 2, 2010 I have still not received a response to my questions. I do not think they were inappropriate.)

I did take Kria to two certified Veterinary Ophthalmologists recently. She has healthy eyes. She will be six years old this spring (2010) and should show signs of PRA by now if she has the condition.

Both of the Ophthalmologists explained PRA and saw no reason not to breed her.