There are 2 forms of eye disease that Staffordshire Bull Terriers are known to suffer from these being Hereditary Cataracts (HC) & Persistent Hyper Plastic Vitreous (PHPV) the article below was written by Juanita Hobbs of Crossbow Staffordshire Bull Terriers South Africa and she has kindly allowed me to use this article here, but for further information and a much more in depth look at these diseases please visit her website on this Link: http://www.staffords.50megs.com

The following article explains what WE as responsible owners and breeders of this wonderful creature need to do in order to stop these terrible diseases spiralling out of control, HOW WOULD YOU FEEL IF YOUR BEAUTIFUL NEW PUPPY WAS BLIND BY THE TIME IT REACHED 2 YEARS OLD??

Hereditary Cataracts (also called Juvenile Cataracts) is an inherited condition, a cataract being opacity of the lens or its capsule. The age of onset has been most commonly seen from 8 to 10 weeks of age up to about 18 months, but has been seen as late as 3 years. It is usually bilateral and symmetrical. Once the cataract becomes total the dog is completely blind. This cataract is presumed to follow an autosomal (not sex linked) recessive mode of inheritance.

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UNDERSTANDING HEREDITARY CATARACTS (HC)
by Juanita Hobbs "Crossbow" (South Africa)

INTRODUCTION

A well-known English stud dog, UK Ch Jodel's Mr Cool, has recently produced an HC-affected puppy, so there has once again been an uproar amongst Staffordshire Bull Terrier fanciers around the world.

Accusations and vile gossip has become the order of the day, and, inaccurate statements and assumptions are being accepted as the "gospel truth".

The most frightening truth which has come to light is that an amazing number of "breeders" do not understand the most basic facts about Hereditary Cataracts.

This new section of our website is presented to provide as much factual information as possible about Hereditary Cataracts in order to aid in defeating the problem that exists in our Breed. We CAN eradicate this dreaded genetic disease, and we CAN do it in an educated, effective, ethical and efficient manner.

In order to offer a broad spectrum of opinion you will find a wide variation of information on this section of our website:

(1) This particular article is written in layman's terms - but there are also several expert articles available to read via the HC specific links page.

(2) There is a direct link to the SBT Breed Council's webpage, as well as a page with the updates on the HC DNA Project.

(3) There is The 1971 List of names of carriers.

(4) There are some of the pedigrees of the 1971 List of carriers.

(5) There are various facts and figures.

(6) There are details and pedigrees of the four publicly acknowledged 1990's carriers - UK Ch Jodel's Mr Cool & Jodel's Xavier and Jackstaff Rustic Prince & UK Ch Jackstaff Heaven Sent.

PLEASE, if you own or have bred a carrier, and you are prepared to make the facts public and offer full details - e-mail us here. It doesn't matter what country you live in, the information is relevant to us all. Tell all your Stafford friends about it too - maybe they don't have access to the Internet, and everyone needs to know.

It is our hope that with the dissemination of such information we, as breeders, will have the knowledge and the bravery to stand up and face the problem head on.

Additionally, we appeal to everyone to contribute anything they can to these pages - whether it be pedigrees that we are missing, whether it be educational articles, whether it be links to other relevant sites, or whether it be names and pedigrees of carriers. The latter takes a great deal of courage to contribute - but, we appeal to you to do so - our Breed needs and deserves YOUR courage.

There is no way to accurately state how prevalent the problem is in our Breed. But, because most breeders do not regularly test their stock's eyes, coupled with the fact that most breeders tend to sweep such things as hereditary diseases under the carpet, I fear that there may be more of it around than we'd like to admit. After all, many of the expert articles call HC "common" or "prevalent" in the Staffordshire Bull Terrier.

BASIC GENETICS & HOW H.C. IS PASSED ON

The basic unit of life is the cell. Inside the cell there are structures called chromosomes. On the chromosomes there are a series of genes. Genes are the units of inheritance.

Chromosomes occur in pairs and each species has a constant number of pairs. The dog has 39 pairs. Of these 38 are identical pairs and are known as homologous chromosomes (autosomes). The 39th pair is the sex chromosomes and they are not identical. The female possesses two X chromosomes and the male has one X and one Y chromosome.

As I already said, all the chromosomes other than the sex chromosomes are known as autosomes and so a gene carried on an autosome is known as an autosomal gene. Conversely, a gene carried only on the sex chromosomes is called a sex-linked gene.

Each parent contributes one of each pair of chromosomes to the puppy and so it is luck of the draw which one of each pair of chromosomes is transmitted. This is why the individual puppies in the same litter are not identical.

This is a very simplified scenario, but let us say these two animals are mated:

Sire Dam
Aa Aa

There are four possible combinations in the puppies produced:

AA Aa Aa* aa
Pup 1 Pup 2 Pup 3 Pup 4
"A" from sire "A" from sire "a" from sire "a" from sire
"A" from dam "a" from dam "A" from dam "a" from dam

* Note: It is expressed "Aa" rather than "aA" because geneticists write the dominant symbol first.

In the same way that chromosomes are paired, so are the genes which they carry. In some pairs of genes, one is capable of suppressing the effect of the other one. In this case, the first gene is known as a DOMINANT gene, and the other one is known as a RECESSIVE gene.

Let us say that in the above example the dogs are Labradors and A is the gene for black and a is the gene for chocolate. In Labradors black is dominant and chocolate is recessive. Therefore Pup 1 will be black, Pup 2 will be black, Pup 3 will be black and Pup 4 will be chocolate.

Pup 1 is black because he inherited black from both parents. Pups 2 and 3 each inherited one black gene, and one chocolate gene - but, because black is dominant over chocolate they will both have black coats themselves, BUT they are still CARRIERS of the chocolate gene - in other words they both possess the chocolate gene. Pup 4 is chocolate because he inherited chocolate from both parents - there is no black gene present in him to suppress his chocolate colour.

To break it down into likely percentages:

25% of the offspring will be black and carrying only black genes ("AA");
50% of the offspring will be black and carrying chocolate genes also ("Aa");
25% of the offspring will be chocolate and carrying only chocolate genes ("aa").

Any progeny produced by Pup 1 will only inherit the black gene from him because he has only the black gene ("AA") to pass on. Likewise, Pup 4 will only pass on to his progeny the chocolate gene because he has only the chocolate gene ("aa"). Pups 2 and 3 are capable of passing on either black or chocolate genes to their progeny because they themselves are carrying both genes ("Aa").

Therefore the only way to guarantee that chocolate puppies will be produced is to mate two chocolates together - because you know that the parents can only be carrying chocolate genes. It is impossible to get black puppies if two chocolates are mated together.

In our example above, both parents are black coated but because they are both carrying the chocolate gene, they are capable of producing chocolate puppies. Just because you can't see the chocolate colour in the parents doesn't mean that the chocolate gene is not present in them.

Remember that :

Can't see doesn't mean not present.

This is the problem with dominant genes - you can't see what genes they are suppressing or hiding. Unless, of course, there is a DNA test available for those specific genes.

So, let's get back to Hereditary Cataracts and UK Ch Jodel's Mr Cool.

UK Ch Jodel's Mr Cool is a carrier of HC. He himself is NOT affected - in other words, he DOES NOT have cataracts himself. In fact, he has been TESTED CLEAR himself. However, he has produced an affected (blind) puppy - and that is what makes him a carrier of the recessive HC gene.

In HC the "bad" gene is recessive. So "A" would be normal eyes (dominant) and "a" would be hereditary cataracts (recessive).

So in our above example, Pup 1 ("AA") will have good eyes and he is not carrying the HC gene, Pups 2 and 3 (both "Aa") will have good eyes but they are both carrying the HC gene - and you can't tell that by a simple eye test, you could only tell that by a DNA test for HC. And, Pup 4 ("aa") will be affected (blind with HC), because he only has the HC gene.

Pup 1 is not blind (he will get an "eyes clear" certificate) because he inherited good genes from both parents.

Pups 2 and 3 each inherited one good gene, and one bad gene. But because the good gene is dominant over the bad gene they will not have HC (not be blind) themselves (and, they will both get "eyes clear" certificates). BUT they are still carriers of the bad HC gene.

Pup 4 is blind (affected) because he inherited bad genes from both parents - there is no good gene present in him to suppress his HC gene - and he so will not get an "eyes clear" certificate.

To make the point absolutely clear:

Because the HC gene is recessive ("a") this means that a blind puppy must have inherited "a" from BOTH parents.

An affected (blind) puppy is "aa" - so both his parents MUST be "Aa" if they themselves were not blind.

Therefore UK Ch Jodel's Mr Cool is "Aa".

Now, ONLY if UK Ch Jodel's Mr Cool was mated to a bitch which was also "Aa" can he have produced a blind puppy. This is exactly what happened.

The problem is if you have a dog which is "Aa" and he is only ever mated to "AA" bitches (i.e., bitches which are not carriers) then it is not possible for a blind puppy ever to be produced by him. BUT puppies which are carriers can be produced by him.

In fact, even if both parents are carriers, it is possible that no puppies will be blind - but they could all be carriers - it depends on the fall of the dice, as does all genetics.

In this way you could go on breeding for generations a whole long line of carriers without ever producing a single blind puppy.

And then one day, the proverbial hits the fan!

WHO TAKES THE BLAME?

NO BLAME CAN BE ATTACHED to anyone for this problem. Laureen & Eddie Williams, the breeders of UK Ch Jodel's Mr Cool have eye tested their stock for six generations - and all their stock has been certified HC clear up until this point. The Williams' have been completely responsible throughout their breeding to test their stock. And now, they have been completely responsible by bringing to public attention what has happened so that everyone can have the chance to pay vigilant attention to the pedigrees involved, and breed (or not breed) accordingly.

This is a quote from a Breed Lecture given by the Williams' in 1997 at the Border Staffordshire Bull Terrier Club, East London, South Africa:

"Because we are aware of the eye problems in our breed, we always have our dogs tested before we breed from them. If we cannot have our puppies eye tested before he or she leaves our home we ask the new owners to have them tested as soon as they can, or most definitely before they breed from him or her. It is most irresponsible not to have his or her eyes tested, and, yes, we do know a lot of breeders don't test.

Don't think a litter from your bitch will do her good, it won't. If you don't want to breed from her, don't - have her spayed, then you won't have the problems with seasons. If you do want to breed we most strongly recommend you wait until the bitch is at least 18 months old. At that time she can be eye tested before she is mated."

There have been people in the past who have bred dogs that have turned out to be carriers of HC - and some of those people have been responsible in the way the Williams' have been, and brought it out in the open so that everyone can benefit.

And, some have not been so responsible.

There is a breeder in England who some years ago hid the fact that his dog was a carrier, and KNOWINGLY allowed the dog to continue to stand at stud. The consequences of this person's action are unimaginable and horrifying.

It is however, up to the people who have the bloodlines concerned to be very careful when breeding. If they are not careful then they will have to accept the consequences if something goes wrong for them in the future. Does this include YOU?

THE ONLY PEOPLE TO BLAME are the unscrupulous and irresponsible breeders that there have been from time to time, who FAILED to do the right thing. Probably these people were motivated by money or "international" puppy sales - that is, "big bucks rules", and "ethics are thrown out the window". These people were perfectly well aware of the problem and they grossly neglected their responsibility to the Breed by not telling the truth.

YOU MUST UNDERSTAND that it is completely possible that no other stock from Jodel's are carriers of the HC gene - and so there may be NOTHING further to worry about as far as their bloodlines are concerned.

BUT until the DNA test for HC in Staffords is developed and we can therefore have all our Staffords DNA tested, we MUST always keep in the back of our minds that any Stafford MAY be a carrier of the HC gene.

WHILE WE WAIT FOR THE DNA TEST TO BE DEVELOPED, WHAT CAN WE DO ABOUT IT?

The most important thing YOU can do RIGHT NOW is:

Make a financial contribution towards to DNA test's development. Click here for further details. Or, e-mail Ivor Keyes with your pledge. Remember every penny counts - so add a few of your own for the love of Staffords.

And, it is VITALLY IMPORTANT to understand that it is IMPERATIVE that dogs of virtue are RETAINED in our breeding programs.

Once the DNA test for HC in our Breed is developed, we CAN completely eliminate Hereditary Cataracts in our Breed in ONE generation.

For example, a litter could be bred from UK Ch Jodel's Mr Cool once the test is available. Virtually as soon as the puppies are born, a small blood sample would be taken from each of them, and tested. It would then be known without a doubt which puppies carried the "bad" gene, and which didn't. The pups with the "bad" gene would then immediately be eliminated from Breed's gene pool. The pups that are thus proven not to carry the HC gene would then be available to carry forward the very important virtues required for the positive progress of our Breed as a whole.

Therefore, it is we must take immediate and positive steps to ensure that we do not lose virtuous, and thus invaluable, dogs such as UK Ch Jodel's Mr Cool to our Breed. Whilst they may have withdrawn their stud dogs from use, owners of any such dogs which are carriers, or suspected carriers, must be proactively encouraged to take, freeze and store semen from these dogs. As soon as the DNA test is available, the semen can be used with absolute safety by simply doing the DNA test on the pups produced.

This is exactly what the Williams' have done, and they have been "hauled over the coals" by the rumour mongers for storing Mr Cool's semen - WHY???? Having faced such a tragedy with tremendous courage and honesty, and then still to have managed to think about and plan for the long term future, is more than admirable. Critics of their actions are nothing less than short-sighted and ignorant.

Of course, as an extra bonus to the development of the DNA test, puppy buyers of the future will be able to insist on the results of their prospective puppy's DNA test being produced by the breeder prior to purchase.

If this method is followed to the letter once the DNA test is available - Hereditary Cataracts will be eradicated from our Breed virtually overnight.

Otherwise, as far as breeding in the meantime is concerned, if your stock comes from one or more carrier lines, you have two choices:

(1) Never breed from the stock again and have all progeny produced so far neutered or spayed. And start again.

(2) Be extremely circumspect in your breeding and breed away from the known carriers in our Breed as quickly as possible.

Obviously, choice (2) sounds far more agreeable!

If you choose this route, you will have to learn to read pedigrees and make sure that you don't breed your dogs to stock that descends from any carriers of HC. Read the other pages of this section for names and pedigrees of dogs which are carriers. UNDERSTAND before you breed your Stafford.

It is also vital to understand that IT IS NOT THE ANSWER to simply look for the presence of one of these named dogs in the pedigree.

These dogs are carriers of HC therefore they inherited the bad gene from one of their parents. The gene DID NOT just suddenly appear in any of these dogs from the middle of nowhere!

Therefore either the mother AND / OR the father of any one of these dogs HAVE TO BE CARRIERS of the gene. But, there is no way of telling which one of them was responsible for the gene in their particular progeny.

In addition, whichever one of them is responsible will have inherited it from one or both of his/her parents. So, this same principal must therefore be applied the whole way back through the entire pedigree. As you can see that means that there are an enormous number of dogs which COULD BE CARRIERS of the HC gene. Be aware that you must take all such dogs into consideration when planning a mating or when agreeing to a mating.

To illustrate the need for care, in the five generation pedigree of UK Ch Jodel's Mr Cool there has to be a minimum of five Hereditary Cataract carriers besides himself - one on each generation.

This mind-boggling permutation is the place to start when considering a mating with your stock.

CONCLUSION

It is very important to understand when discussing UK Ch Jodel's Mr Cool that:

(1) He is NOT the only dog to blame.

(2) All his other progeny MAY be completely HC-free - and they may NOT.

(3) It is completely possible that all his other progeny are NOT even carriers of the HC gene - and they MAY be.

The point is that it is unwise to ASSUME that your stock is HC-free, regardless of the dogs in your pedigrees. That is what ALMOST EVERYONE ELSE has done.

Other breeders have acted irresponsibly by allowing their dogs to be bred from indiscriminately. The fact that they may not have been aware of the consequences is no excuse - it is every breeder's duty to understand exactly what is present in their pedigrees and to act accordingly. The Breed's extremely bad state of affairs worldwide is due to a very few breeders acting out of self-interest and without any thought to the future effects. THERE IS ABSOLUTELY NO EXCUSE FOR THESE BREEDERS' ACTIONS.

The fact is that the Breed may never recover from what has been done to it by a small minority.

It is up to us to try and make things right - and this discussion is only one of the many steps which need to be taken to put us back on the right track.

If we wish to claim to be "responsible and ethical breeders" then it is up to us to take these steps to preserve our Breed in the long term. Tomorrow's show wins are nothing when compared to the long term future of the Breed.

Our ever-diminishing gene pool means that unless we act responsibly, our Breed will be riddled with various hereditary diseases such as Hereditary Cataracts in a very short time - after all one generation in dogs is only 18 months. When that happens it will be far more important and significant to be a breeder of sound, healthy Staffords - than just another breeder who sacrificed their stock's health for the sake of a pile of worthless cards and rosettes.

Look, learn - and, do the right thing - FOR THE LOVE OF STAFFORDS.