Double Barrel Bulldogs

 Ceroid lipofuscinosis is a broad term describing a group of incompletely
understood diseases caused by an abnormal accumulation of metabolic
by-products within cellular lysosomes of neural and visceral organs. The
disease is reported as an inherited cause of progressive nervous system
dysfunction in humans, nonhuman primates, dogs, cats, cattle, sheep, and
goats.1–11 Clinical signs of disease include impairment of motor function,
conscious proprioceptive deficits, ataxia, progressive blindness, behavior
changes, hypersensitivity to stimuli, and seizures. The disease is variable
in rate of progression, irreversible, and fatal. The report link below is 
graphic and contains a case history, including autopsy  details of the case study.

This report describes the
clinical, computed tomographic, biochemical, microscopic, and ultrastructural
features of ceroid lipofuscinosis in a dog.

Many vets, just seeing the dog in the early stages, will guess that it has hip dysplasia.  While others havn't a clue what infact NCL is.  Within the next 1 to 3 years, the dog won’t even be able to keep from falling.. when walking the dog will tend to stagger and move randomly, especially down a flight of stairs or over uneven ground. When running, the rear will show very little control.  Normally, by the time the dog is 5 years old, the dog will be euthanized....just standing will be very difficult, much less trying to walk. With hip dysplasia, on average a dog can live a reasonably normal life, even if it has to be on pain killers. Also, except for severely dysplastic dogs, most dogs won’t even show signs of a problem until later in life.

 
NCL Description for American Bulldogs
Age of onset of clinical signs: 0.9 to 3 years
Age of death or euthanasia: 3.5 to 5.5 years
Abnormalities often observed by the owner:
Mental changes: Physical symptoms may appear to worsen during times of stress.
Affected dogs do not indicate they are in any pain as coordination decreases.
Changes in gait and posture: Initially, uncoordinated movement in the rear is noted. As
the disease progresses, affected dogs develop a wide-based stance in the rear, and
eventually involving all four legs. Affected dogs may exhibit muscle twitching, especially
when sleeping. The dogs remain well-muscled through the course of the disease.
Visual abnormalities: None reported
Seizures/convulsions: None reported
Other changes: None reported

CLEAR                 CARRIER            AFFECTED

Basically a clear dog bred to a carrier will, on average, produce 50% clear pups and 50% carriers, with no affected pups. 2 carriers bred together will produce 50% carriers, 25% affected and 25% clear pups, on average.  With a little thought you can see how just a few breedings, even of carrier to clear, will produce a lot of potential problems for the breed, especially since symptoms may not manifest until the dog is up to 3 years of age. Breeding an affected dog with a carrier, produces 50% carrier, 50% affected.

TESTING 

 In Matt Boyd’s line Moleque and High Jumping Mikie were the most influential dogs that were carriers, even Greg Souza has said that Koa and Matias are both carriers.  But anything from Moleque Country Boy x Snowbird, Cross Eyed Mary, Outback's Diesel, or anything that originated with Hines should be tested.  Its also be known that some Johnson lines have been found to be carriers of the disease too.

One of the littermates of Rob Boyd’s “Hammer” was a carrier so he and any of his littermates have a 50% chance of being carriers. All dogs with any of these dogs in their pedigree need to be tested before breeding!!
This does not mean that a carrier cannot be bred, it must not be bred to another carrier, and that if bred to a clear dog, the disease can be easily controlled with careful breeding. And the breeder has a responsibility to test the puppies to determine which are carriers and which are clear before placing or selling them however in my opinion, they should not be bred thus breeding out the disease all together.

Hip Dysplasia is a terrible genetic disease because of the various degrees of arthritis (also called degenerative joint disease, arthrosis, osteoarthrosis) it can eventually produce, leading to pain and debilitation.

The very first step in the development of arthritis is articular cartilage (the type of cartilage lining the joint) damage due to the inherited bad biomechanics of an abnormally developed hip joint. Traumatic articular fracture through the joint surface is another way cartilage is damaged. With cartilage damage, lots of degradative enzymes are released into the joint. These enzymes degrade and decrease the synthesis of important constituent molecules that form hyaline cartilage called proteoglycans. This causes the cartilage to lose its thickness and elasticity, which are important in absorbing mechanical loads placed across the joint during movement. Eventually, more debris and enzymes spill into the joint fluid and destroy molecules called glycosaminoglycan and hyaluronate which are important precursors that form the cartilage proteoglycans. The joint's lubrication and ability to block inflammatory cells are lost and the debris-tainted joint fluid loses its ability to properly nourish the cartilage through impairment of nutrient-waste exchange across the joint cartilage cells. The damage then spreads to the synovial membrane lining the joint capsule and more degradative enzymes and inflammatory cells stream into the joint. Full thickness loss of cartilage allows the synovial fluid to contact nerve endings in the subchondral bone, resulting in pain. In an attempt to stabilize the joint to decrease the pain, the animal's body produces new bone at the edges of the joint surface, joint capsule, ligament and muscle attachments (bone spurs). The joint capsule also eventually thickens and the joint's range of motion decreases.

BREEDING PROSPECT vs EVALUATION RESULTS

Do not ignore the dog with a fair hip evaluation. The dog is still within normal limits. For example; a dog with fair hips but with a strong hip background and over 75% of its brothers and sisters being normal is a good breeding prospect. A dog with excellent hips, but with a weak family background and less than 75% of its brothers and sisters being normal is a poor breeding prospect.

Although there is a genetic disposition for this disease, the variables as described on the OFA and Penn Hip websites clearly summarize that research should be done prior to breeding to avoid producing displastic offspring. Since it is rare that  every dog in any given pedigree  has been screened, there is a chance regardless. The more consistent background a puppy has regarding hip scores, the better . The OFA has a data base you can look up any dog that has been read and obtain the results of their hip evaluation. The University of Pennsylvania (Penn Hip) is in the process of doing the same.  Below is information regarding the difference between Penn Hip, and OFA hip evaluations.

THE BOTTOM LINE 

 There is much controversy over which method more accurately predicts the possibility of Displastic Joints, the reason being; One looks at laxity and the other at total hip conformation so techically you can have a dog that is OFA good with deep sockets, smooth lines and nice looking hips but they have a lot of laxity which means that may not stay good into adulthood because there is more room for movement and thus changes. To the reverse, a dog's DI (distraction index) can be favorable but still displaystic due to shallow hip sockets. Hopefully this helps you understand why both are necessary to determine the breeding worthiness of the dog.

No one can predict when or even if a dysplastic dog will start showing clinical signs of lameness due to pain. There are multiple environmental factors such as caloric intake, level of exercise, and weather that can affect the severity of clinical signs and phenotypic expression (radiographic changes). There is no rhyme or reason to the severity of radiographic changes correlated with the clinical findings. There are a number of dysplastic dogs with severe arthritis that run, jump, and play as if nothing is wrong and some dogs with barely any arthritic radiographic changes that are severely lame.

 SUMMARY

The Penn Hip can be done as early as 6 months of age where as OFA  requires the animal to be at least 24 months old. The laxity does not change, however the bones can.  A dog should have minimal laxity-the closer to 0.30 the better and the closer you get to 0.70 raises the probability of problems regardless of the socket depth and shape of the bones. 

Deep socket covering 50% or more of the femur head in conjunction with round femur head (and low laxity) results in a lesser probability. Shallow sockets, and a less round femur head  CAN  be a higher probability IF the degree of laxity is 0.60 or higher. The most accurate probability is to have an evaluation using both methods utilize the information using common sense.

There are many skin and health issues that can be resolved by change of diet or supplementing to replace the deficiancy that has created the symptons/issue. The most common is allergy symptons. You can spend a ton of money at the vet and be prescribed  medications that are actually harmful and detrimental to your dogs well being. Over abundance of yeast in a dogs system in my opinion is the most common issue confused with allergies. The dog scratches, has a rash or redness of skin, rust colored areas under the tail, front legs next to the chest  same as in people, it can manifest into a variety of symptons different in each dog. Antibiotics and or prednisone treatment resolve the issue temporarily only to return in full force shortly after the treatment is stopped. These medications actually help the yeast grow by killing the good bacteria necessary to keep things in balance. If it is yeast related there is a simple way to stop the problems and avoid recurrance with very little trouble. Follow the basics and I promise you and your pet will be happier for it. The dogs system gets out of balance and allows the yeast to overproduce. Yeast is present in everyone. When it overproduces, it causes a variety of problems internal and external. It can even affect internal organs. Changing the ph in the dogs digestive tract and over all system will inhibit over production putting the dog back in balance resolving the issue. It takes several weeks to see improvement and a few months to get completely under control. Persistence is the key, and it does work.

Feed food with NO corn. Avoid food with wheat and  use rice based dog food.

Feeding a probiotic supplement may be another alternative that produces results. In theory it should be a quicker way to get there, however I dont have any experience there fore unable to provide an opinion.

Add a few tablespoons of dried cilantro to the dogs food each day. This absorbs the trace metals in the digestive system. An over abundance can be present in tap water, ground water etc. Yeast needs this so removing it controls the yeast.

Feed 1/3 to 1/2 cup of plain yogurt every day. This helps put good bacteria back in the dogs digestive system and helps control the yeast that has taken over.

Add  a few tablespoons of ACV (apple cider vinegar)  for each gallon to your dogs watering dish, bucket etc. Replace the ACV each time you change. This will also control tear stains, which is yeast growing in the dogs fur.

I highly recommend only giving your dog filtered water if your water quality is sub par in general. We actually installed a filtering system and swear by the results.

 Please feel free to call and ask any questions you may have. It is difficult to cover everything and it may be something new to you that is common knowledge to most so I dont think to post it. Visit the CONTACT US portion of the website. I am always happy to talk dogs and do my best to answer any questions you may have.