PRA – Progressive Retinal Atrophy

Progressive Rod-Cone Degeneration (PRCD / PRA-prcd)

General

PRA has multiple variants which effect different breeds. Research to date shows that Cocker Spaniels are effected by the prcd variant, it is commonly abbreviated as PRCD or prcd-PRA.

Progressive rod-cone degeneration (PRCD) is an inherited form of late-onset progressive retinal atrophy (PRA) an inherited eye disease affecting English Cocker Spaniels. PRCD affects the photoreceptor cells in the eye involved in both night and day vision

The age of onset varies between individual Cocker Spaniels with PRCD. Evidence of retinal disease can first be seen on an Electroretinogram in dogs older than 2.5 years of age, but most affected dogs will not show signs of vision loss until 8 to 12 years of age or later.

PRA has several different forms, but progressive rod cone degeneration (PRCD) is the most widespread, and occurs in many other breeds.

In most forms of PRA, Symptoms often start with vision problems in dim light (night blindness) and loss of peripheral vision. The dog first becomes uncertain and fearful in the dark. This is due to the atrophy of retinal rod cells, which are important for night vision. Over time affected dogs continue to lose night vision and begin to show visual deficits in bright light. The dog becomes completely blind as the retinal cone cells atrophy. The pupil is wide and the increased reflection of the fundus can be seen especially clearly when the light is focused on the enlarged pupil.

Although there can be individual variation in the age of onset and the rate of disease progression, the disease eventually progresses to complete blindness in most dogs. There are other eye disorders that can appear clinically similar to PRCD. Genetic testing will confirm this.

There is no treatment for PRA or PRCD at this time. So its important that you purchase your cocker spaniel puppy from a reputable breeder undertaking DNA health testing.

DNA Testing is not done at your local vets.

Mode of Inheritance
Autosomal Recessive
This is a heredity condition passed on from the parents. If you're getting a puppy make sure that at least one parent has been DNA tested Clear, to ensure you're puppy wont be affected by this condition.
Onset
Late Onset (7+)
The medium is 7+yrs. But clinical symptoms can develop as early as 3yrs, And some never display symptoms.
Testing Available
DNA
DNA testing is available from international laboratories. (It can not be done at your local vets).  
Clinical Diagnosis
Specialist Veterinary Ophthalmologists can test your cocker spaniel for clinical signs of eye disease including PRCD, but only once the symptoms develop. Your local vet may be able to offer initial advice and make a referral if required.
Breeding Strategy
1 clear parent
To ensure progeny are not affected by this condition at least 1 parent must be "clear"
Veterinary Ophthalmologists
Pet Eye Care: Waikato
Website
Dr Kellam Bayley & Dr Petra Price
Hamilton
Auckland Animal Eye Centre
Website
Peter Collinson
Auckland
Mcmaster & Heap
Website
Dr Steve Heap
Christchurch & Dunedin Clinics
Vets with Special Interest
Barrowman Goodman Vets
Website
Dr Anna Goodman
Rotorua Clinic
Studies in ophthalmology at Massey, some training with Craig Irving. Eyes and all conditions relating to them are Anna's special interest, the clinic has advanced diagnostic equipment.

My Research Notes on PRA-PRCD

My thoughts on developing minimum breed standard testing. As PRA can be scientifically determined using DNA, it should be required in any programme. There is no reason to undertake clinical evaluations for PRA . * See other Eye Conditions.

Reliable genetic testing for determining breeding strategies is important so the gene diversity is retained and a depressed gene pool does not create other problems within the breed

Cocker Spaniels that don't carry the mutation have no risk of having PRCD affected pups. And successful breeding strategies have breed clear to carrier/affected slowly reducing the incident of the disease.

Because symptoms do not appear until adulthood, genetic testing should be performed before breeding. At least one parent should be confirmed clear of the mutation in order to eliminate this from breeding lines and to avoid the potential of producing affected pups, breeding of known carriers to each other is not recommended.

However, please remember because there are multiple types of PRA caused by mutations in other genes, a normal result in PRCD does not exclude the possibility of other types of PRA being identified in the future. Its important to keep learning.

Eye exams can detect the early clinical signs of different forms PRA, and cataracts which often develop concurrently.

Check Out The Progress Made In Reducing PRA-Prcd

PRA-graph

Huge progress made in reducing PRA-prcd by Cocker Spaniel Breeders This graph is part of a 15yr time-trend study, to evaluate how test results changed over time.
Genotype and allele frequencies in the top five tested breeds for prcd-PRA (2004–2019)
(A) prcd-PRA homozygous variant genotype
(B) prcd-PRA heterozygous genotype
(C) prcd-PRA disease-associated variant allele

This progress in reducing PRA-prcd in Cocker Spaniels over 15years has been achieved by breeders understanding and implementing breeding strategies based on DNA Testing.
Just imagine what those results would be now in 2023!

PRA-Prcd Articles

PRA Around The World

The Global Frequency Analyses of Canine Progressive Rod-Cone Study shows that genetic testing informed breeding decisions to produce fewer affected dogs.

However, the presence of dogs homozygous for the disease variant, especially for prcd-pra, was still observed fourteen years after test availability, potentially due to crosses of unknown carriers.

The genetic testing has proven to be a invaluable tool and an intrinsic part of breeding healthy cockers and reducing the likelihood of disease.

informal data collected from RKC & CSDB
Other databases had insufficient validation of data to be included, and some of these dogs will be on the CSDB
Data lasted updated February 2024

% are more important when considering these statistics rather than the numbers recorded above, as some dogs results may have been duplicated in different databases