All our dogs have undergone the relevant health tests. As from December 11th 2011 Our springer spaniels are tested for Fucosidosis, PRA. Gonioscopy KC/BVA/ISDS. All our cocker spaniels are tested for KC/BVA/ISDS, FN (Familial Nephropathy), PRA.
Information about some of these diseases is below.
Fucosidosis
What is it
- Canine Fucosidosis is a disease which is severe, progressive and ultimately fatal.
- It is characterised by deteriorating signs of the nervous system that progress over a period of several months, sometimes from an early age. Signs include inco-ordination and ataxia (loss of control of movement), change in temperament, loss of learned behaviour, loss of balance, apparent deafness, visual impairment and varying degrees of depression. The inco-ordination and ataxia affects all four legs and is mostly evident when affected animals are walking on slippery surfaces or attempt more complicated movements such as turning. In addition, affected dogs lose weight and may suffer from swallowing difficulties and sometimes regurgitation of food.
- The disease, which affects young adults, usually between 18 months and 4 years of age, is caused by the absence of an enzyme called alpha-L-fucosidase. This enzyme is one of many required to break down complex compounds into simple molecules that the body can use. When this enzyme is absent, the pathway is blocked and the more complex compounds build up in the cells of the affected animal, accumulating in the lymph nodes, liver, pancreas, kidney, lungs and bone marrow. However, it is the accumulation in the brain and peripheral nerves that is most important, since it interferes with normal function, giving rise to the clinical signs described, eventually resulting in death.
- Canine Fucosidosis can affect all English Springer Spaniels, whether they are of ‘field trial’, ‘working’, ‘show’ or ‘pet’ origin. Don’t let anyone tell you otherwise!
- The condition is inherited through an autosomal recessive trait and has been reported by ESS owners/breeders from all over the world.
BVA/KC Eye Scheme
The BVA/KC Eye Scheme offers breeders the possibility of eye testing to screen for inherited eye disease in certain breeds. By screening breeding stock for these diseases, breeders can use the information to eliminate or reduce the frequency of eye disease being passed on to puppies. Although any breed can be examined for eye disease, currently only the results of those breeds that appear on Schedule A of the Eye Scheme are sent to the Kennel Club for inclusion on computer records and printing in the Breed Records Supplement. In general, the best age for eye testing is before a dog has reached one year old and thereafter on an annual basis. However, in some breeds, it is necessary to test them as young puppies (usually between six and twelve weeks of age) and so details of litter screening are also included in the literature although results of litter screening are not published.
Although any breed can be examined for eye disease, currently only the results of those breeds that appear on Schedule A of the Eye Scheme are sent to the Kennel Club for inclusion on computer records and printing in the Breed Records Supplement.
There is a list available, from either the BVA or the Kennel Club, of appointed eye panellists who can issue certificates under the scheme and owners can make an appointment with one of the panellists directly or through their own veterinary surgeon. Often, breed clubs will arrange for a BVA panellist to attend their shows. This allows many dogs to be examined on one occasion and may save time and money.
FN – Familial Nephropathy
Familial Nephropathy (FN) is a recessively inherited renal disease that has been recognized in the English Cocker for more than 50 years . FN is a form of “hereditary nephritis” which refers to a group of glomerular diseases that are linked to genetic collagen defects.
Onset of renal failure due to FN typically occurs between six and 24 months of age . Clinical signs may include polydipsia (drinks more), polyuria (urinates more), weight loss, lack of appetite, vomiting, or diarrhea. These symptoms are commonly associated with any type of renal failure.
Structure/Function
The kidney is an organ made up of hundreds of thousands of tiny structures called nephrons. Each nephron consists of a glomerulus and a tubule. Blood flowing through the kidney is filtered by the glomerulus, with the fluid that is filtered out of the blood subsequently passing down the length of the tubule. Cells that line the inner surface of the tubule process the fluid as it flows along, reabsorbing certain components of the fluid and excreting others. The fluid leaving the tubule at the end of this process is urine, which is a combination of water and waste products.
Dogs affected with FN have a genetic defect within the glomerulus. This defective glomerulus lacks a certain type of collagen that helps to hold the structure of the filter together. As a result of this collagen defect, a chain reaction of events takes place. Once the glomerulus begins to loose its ability to function properly, blood proteins leak through the defective filter into the urine. The glomerular abnormality also leads to subsequent tubular damage, and the chain of events eventually destroys the entire nephron. Nephrons that are severely damaged or destroyed can’t be replaced.
Since the kidney serves as the main waste-disposal system in the body, it is a master at compensation. When one nephron dies, another takes over its work. Over the course of time and with continual compensation the number of functioning nephrons is greatly reduced. Once at least 75 percent of the nephron population is destroyed, end-stage renal failure occurs. Since the disease is gradual and progressive, affected dogs do not appear sick until very late in the course of disease.
Gloucoma – Gonioscopy
Glaucoma is defined as an increase in pressure within the eye. The increased pressure is the result of a buildup of the intraocular fluid which is known as aqueous humor. In a healthy animal, aqueous humor primarily drains out through a circular filter at the junction of the clear cornea and white sclera, called the iridocorneal angle. Animals with glaucoma have an abnormality in the filter which obstructs outflow, resulting in a buildup of fluid within the eye. An analogy would be a kitchen sink – if the drain is open and the water is running, the sink is operating normally. However, the drain becomes clogged for some reason and the water continues to flow, then the sink fills up with water and overflows!
There are various causes of a defective filter. Dogs of some breeds are often born with abnormal filters and are therefore prone to getting inherited (genetic or primary) glaucoma in both eyes. Other breeds have a genetic predisposition to developing displaced (luxated) lenses, which block the filters, obstructing the flow of fluid. In both dogs and cats, the filters can be clogged with inflammatory cells if inflammation inside the eye (uveitis) occurs. Intraocular tumors can also lead to glaucoma.
Prcd-PRA
The genetic disorder, prcd-PRA , causes cells in the retina at the back of the eye to degenerate and die, even though the cells seem to develop normally early in life. The “rod” cells operate in low light levels and are the first to lose normal function. Night blindness results. Then the “cone” cells gradually lose their normal function in full light situations. Most affected dogs will eventually be blind. Typically, the clinical disease is recognized first in early adolescence or early adulthood. Since age at onset of disease varies among breeds, you should read specific information for your dog. Diagnosis of retinal disease can be difficult. Conditions that seem to be prcd-PRA might instead be another disease and might not be inherited. OptiGen’s genetic test assists in making the diagnosis. It’s important to remember that not all retinal disease is PRA and not all PRA is the prcd form of PRA. Annual eye exams by a veterinary ophthalmologist will build a history of eye health that will help to diagnose disease.
Unfortunately, at this time there is no treatment or cure for PRA.
Inheritance
Prcd-PRA is inherited as a recessive trait. This means a disease gene must be inherited from each parent in order to cause disease in an offspring. Parents were either “carrier” or affected. A carrier has one disease gene and one normal gene, and is termed “heterozygous” for the disease. A normal dog has no disease gene and is termed “homozygous normal” – both copies of the gene are the same. And a dog with two disease genes is termed “homozygous affected” – both copies of the gene are abnormal.
It’s been proven that all breeds being tested for prcd-PRA have the same disease caused by the same mutated gene. This is so, even though the disease might develop at different ages or with differing severity from one breed to another.
Although prcd-PRA is inherited, it can be avoided in future generations by testing dogs before breeding. Identification of dogs that do not carry disease genes is the key. These “clear” dogs can be bred to any mate – even to a prcd-affected dog which may be a desirable breeding prospect for other reasons. The chance of producing affected pups from such breedings depends on the certainty of test results.