Compact Staffords
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L-2-HGA (L-2-hydroxyglutaric aciduria)

L-2-HGA (L-2-hydroxyglutaric aciduria) in Staffordshire Bull Terriers is a neurometabolic disorder characterised by elevated levels of L-2-hydroxyglutaric acid in urine, plasma and cerebrospinal fluid.

L-2-HGA affects the central nervous system, with clinical signs usually apparent between 6 months and one year (although they can appear later). Symptoms include epileptic seizures, "wobbly" gait, tremors, muscle stiffness as a result of exercise or excitement and altered behaviour.

The mutation, or change to the structure of the gene, probably occurred spontaneously in a single dog but once in the population has been inherited from generation to generation like any other gene. The disorder shows an autosomal recessive mode of inheritance: two copies of the defective gene (one inherited from each parent) have to be present for a dog to be affected by the disease. Individuals with one copy of the defective gene and one copy of the normal gene - called carriers - show no symptoms but can pass the defective gene onto their offspring. When two apparently healthy carriers are crossed, 25% (on average) of the offspring will be affected by the disease, 25% will be clear and the remaining 50% will themselves be carriers

The mutation responsible for the disease has recently been identified at the Animal Health Trust. Using the information from this research, we have developed a DNA test for the disease. This test not only diagnoses dogs affected with this disease but can also detect those dogs which are carriers, displaying no symptoms of the disease but able to produce affected pups. Carriers could not be detected by the tests previously available which involved either a blood or urine test detecting elevated levels of L-2-hydroxyglutarate or magnetic resonance imaging. Under most circumstances, there will be a much greater number of carriers than affected animals in a population. It is important to eliminate such carriers from a breeding population since they represent a hidden reservoir of the disease that can produce affected dogs at any time.

The test is available now and information on submitting samples is given below.

Breeders will be sent results identifying their dog as belonging to one of three categories:

CLEAR/NORMAL:
the dog has 2 copies of the normal gene and will neither develop L-2-HGA, nor pass a copy of the L-2-HGA gene to any of its offspring.
CARRIER: the dog has one copy of the normal gene and one copy of the mutant gene that causes L-2-HGA. It will not develop L-2-HGA but will pass on the L-2-HGA gene to 50% (on average) of its offspring.
AFFECTED: the dog has two copies of the L-2-HGA mutation and is affected with L-2-HGA. It will develop L-2-HGA at some stage during its lifetime, assuming it lives to an appropriate age. Carriers can still be bred to clear dogs. On average, 50% of such a litter will be clear and 50% carriers; there can be no affecteds produced from such a mating. Pups which will be used for breeding can themselves be DNA tested to determine whether they are clear or carrier.
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Hereditary Cataract

Hereditary Cataractin Staffordshire Bull Terriers has been recognised as an inherited condition since the late 1970's. Affected dogs develop cataracts in both eyes at an early age. The condition is not congenital, so the lenses are normal at birth but cataracts appear at a few weeks to months in age, progressing to total cataract (and resulting blindness) by 2 to 3 years of age.

The mutation, or change to the structure of the gene, probably occurred spontaneously in a single dog but once in the population has been inherited from generation to generation like any other gene. The disorder shows an autosomal recessive mode of inheritance: two copies of the defective gene (one inherited from each parent) have to be present for a dog to be affected by the disease. Individuals with one copy of the defective gene and one copy of the normal gene - called carriers - show no symptoms but can pass the defective gene onto their offspring. When two apparently healthy carriers are crossed, 25% (on average) of the offspring will be affected by the disease, 25% will be clear and the remaining 50% will themselves be carriers

The mutation responsible for the disease has recently been identified at the Animal Health Trust. Using the information from this research, we have developed a DNA test for the disease. This test not only diagnoses dogs affected with the disease but can also detect those dogs which are carriers, displaying no symptoms of the disease but able to produce affected pups. Under most circumstances, there will be a much greater number of carriers than affected animals in a population. It is important to eliminate such carriers from a breeding population since they represent a hidden reservoir of the disease that can produce affected dogs at any time.

The test is available now and information on submitting samples is given below.

Breeders will be sent results identifying their dog as belonging to one of three categories:

CLEAR/NORMAL: the dog has 2 copies of the normal gene and will neither develop Hereditary Cataract, nor pass a copy of the Hereditary Cataract gene to any of its offspring.
CARRIER: the dog has one copy of the normal gene and one copy of the mutant gene that causes Hereditary Cataract. It will not develop Hereditary Cataract but will pass on the Hereditary Cataract gene to 50% (on average) of its offspring.
AFFECTED: the dog has two copies of the Hereditary Cataract mutation and is affected with Hereditary Cataract. It will develop Hereditary Cataract at some stage during its lifetime, assuming it lives to an appropriate age.

Carriers can still be bred to clear dogs. On average, 50% of such a litter will be clear and 50% carriers; there can be no affecteds produced from such a mating. Pups which will be used for breeding can themselves be DNA tested to determine whether they are clear or carrier.

Persistent Hyperplastic Primary Vitreous (PHPV)

This was first described in Staffords in the 1980's although there are earlier reports of it in Dobermanns in Holland . PHPV is the failure of the blood vessels supplying the developing eye of the puppy in the womb to wither and disappear completely by the time it is born. The amount of material remaining is variable. In mild cases there may just be some spots, or possibly a small encapsulated cataract, behind the lens while more severe cases will have greater quantities of residue from the foetal blood vessels. In Staffords, mildly affected dogs are unlikely to have any further problems and will go through life as if nothing was amiss. However more severe cases have a high risk of problems, such as the development of secondary cataracts, which may lead to loss of vision. Both eyes are usually affected, as with HC, but unilateral cases with only one eye involved are not uncommon with PHPV, especially if mild. There is little doubt that PHPV in an inherited condition. There have been several suggestions for its mode of transmission but none have been substantiated so for the moment we have to accept, We simply do not know.

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Hip dysplasia

Hip Dysplasia: is a crippling condition in which laxness in the hip joint causes premature breakdown of the cartilage lining, leading to arthritis. The tendency to hip dysplasia can be passed on from one generation to the next; researchers believe that it is a polygenic-based disorder, having more than one causative gene. Symptoms may include difficulty jumping or using stairs; unusual stiffness after exercise and a peculiar bunny-hopping, gait. While there is a strong hereditary component to this affliction, many believe environmental factors, such as overfeeding leading to too-rapid growth in puppies, can cause the condition to progress much more rapidly if a genetic tendency is already present. Often, the effects of mild hip dysplasia are not seen until the dog is middle-age or older, and can be managed by weight control and anti-inflammatory medications. Occasionally, however, puppies and young dogs can show symptoms of hip dysplasia which are rapidly progressive and crippling; special surgical techniques have been developed for these dogs to help them lead a more normal and pain-free life. According to the latest OFA statistics (2006), with 379 Staffords having been evaluated using hip x-rays, 16.2% are rated abnormal (dysplastic), and 80.2% have both hips graded normal. Since this is at least partially a hereditary condition, careful breeding will reduce its incidence in a breed over time. If both parents have normal hips, there is a lessened possibility for one or more puppies in a litter to be later rated dysplastic.

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Elbow Dysplasia

Elbow Dysplasia: The term elbow dysplasia is used to describe a degenerative disorder of the elbow joint caused by improper development of a portion of a particular bone in this joint (anconeal process of the ulna). Young dogs that have this condition often exhibit pain when jumping or turning quickly, or may show variable foreleg lameness. It is thought to be hereditary in nature, and tends to run in canine families. However, exogenous causes, such as over feeing a puppy into too-rapid growth, may be partially to blame as well, especially if a genetic tendency is present. Injury to the joint might also be a cause. Depending upon the age of the dog, the actual cause, and the severity of the symptoms, treatment can include modalities ranging from anti-inflammatories medications up to surgery. According to the latest OFA statistics (2006), with 105 Staffords having been evaluated using elbow x-rays, 14.3% of these Staffords have elbow dysplasia, while 84.8% are rated as having normal elbows.

Knee
Patellar Luxation

Patellar luxation is a condition in which the patella, or kneecap, of the dog's stifle joint frequently luxates or pops out of place. Depending upon the severity, symptoms can be intermittent and mild throughout the dog's life, with the only evidence of the condition an occasional funny kick of the dog's hind leg, or it may eventually lead to severe, permanent degenerative changes in the joint including arthritis. Infrequently, onset may appear to be sudden, leaving the dog unable to stand on the affected hindleg(s). Patellar luxation is believed to be hereditary, as well, although the exact mode of transmission is uncertain. According to the latest OFA statistics (2006), with 21 Staffords having been evaluated radiographically (using x-rays), 100% were evaluated as normal. However, these statistics are considered misleading, not only because of the small sample size evaluated, but also because patellar luxation is known for certain to occasionally be found in this breed.

Atopic
Atopic dermatitis (skin allergy)

Atopic dermatitis (skin allergy): Certain Staffords are prone to various skin allergies, causing them to bite, scratch, and lick their itchy places continually until the source of the problem is corrected by the owner or their vet. An afflicted dog may lose huge patches of hair or develop large lick sore in the process. Secondary infections may then occur in the raw, exposed skin. A flea bite allergy often plays a considerable role in this condition; aggressive flea control measures (both on the dog and in the premises where the dog resides) throughout the year will often by itself ameliorate this condition in milder cases. Contact allergies to pollens, dusts, molds, and other environmental allergens may be to blame. (One indoor allergen responsible for much atopic dermatitis in dogs, the feces of the ubiquitous house dust mite, is a major allergen for humans, as well.) Less frequently, food intolerances may play a role, and the dog must be relegated by the veterinarian to a strict dietary protocol. For milder cases, some dog owners and vets have seen improvement using fish oil supplements; the omega-3 fatty acids contained in fish oils act as a natural anti-inflammatory and help to relieve the itching. Severe cases of atopic dermatitis can be difficult to manage, and the advice and help of a veterinarian are needed to bring the afflicted dog relief. Often treatment to control the itching (such as antihistamines or even prednisone), identification and removal of the source of the allergen from the dogs environment and treatment of the secondary skin infections are all necessary in order to control the condition in especially severe cases. While no studies have been done specifically on Staffords, research with dogs in general show this condition to have, at least in part, a genetic mode of transmission.

demodex
Demodectic mange (demodicosis)

Demodicosis is a skin condition caused by the tiny mite Demodex canis. Nearly all dogs carry this tiny skin parasite, but most adult dogs immune systems are able to keep the mite in check, and no symptoms are observed. (Even most humans carry this mite in limited numbers in their skin!) Frequently in puppyhood and adolescence (4 months to 18 months of age is most typical), puppies of many breeds (including Staffords) may develop a few quarter-sized bald patches on their face and chest; these usually resolve in time without treatment. Unfortunately, certain dogs are not so lucky; the condition becomes generalized and wide-spread over the entire body. In these cases, veterinarians must be consulted for treatment, and the problem is often not easy to permanently resolve. Previously, only rather toxic dips, usually containing the compound amitraz, would be used to control the mites in dogs with generalized demodicosis. More recently, the heartworm preventive ivermectin has been successfully used to treat demodicosis; however, it is not yet approved by the FDA for this purpose, although it is licensed for use as a heartworm preventive in the dog. Ivermectin is highly toxic to many individuals of herding breeds, but most Staffords appear to tolerate it well. Certain lines of Staffords appear to be particularly prone to generalized demodicosis. Whether or not a Stafford with generalized demodicosis should be used in a breeding program is a controversial subject at the moment, however, the tendency towards the condition is thought be many to be an inherited one.

Brachycephalic Syndrome

The full name of this disorder is brachycephalic airway obstruction syndrome (BAOS). Brachycephalics are those breeds which have a comparatively short head. Because of their anatomy, virtually all dogs of these breeds have some degree of increased work associated with breathing from the time they are born. Many have varying degrees of obstruction to their airways, which causes signs ranging from noisy breathing to collapse. The most common anatomical features that lead to the respiratory difficulties typical of these breeds, include an elongated and fleshy soft palate, and narrowed nostrils. Many affected dogs also have changes to the larynx (everted laryngeal saccules) and a relatively small trachea.

How is brachycephalic syndrome inherited?
Selection for exaggerated features has resulted in the respiratory difficulties in these breeds. For example breed standards for the English bulldog specify that the face should be very short, as should the distance between the tip of the nose and where it is set deep between the eyes. It is hardly surprising that this leaves little room for the structures involved in normal breathing.
 
What breeds are affected by brachycephalic syndrome?
These problems are generally most common and severe in the English bulldog. Other brachycephalic breeds in which this syndrome is found include: American Pit Bull Terrier, Bichon Frisé, Boston Terrier, Boxer, Brussels Griffon, Bulldog, Bull Mastiff, Cavalier King Charles Spaniel, Dogue de Bordeaux, English Mastiff, English Toy Spaniel, French Bulldog, Japanese Chin, Lhasa Apso, Neapolitan Mastiff, Pekingese, Pomeranian, Pug, Rottweiler, Shih Tzu, Shar-Pei and Staffordshire Terrier
 
What does brachycephalic syndrome mean to your dog & you?
Problems associated with this syndrome range in severity, with most brachycephalic dogs snuffling and snorting to some degree. Some will have no further difficulties, but many will have problems such as increasingly noisy breathing, coughing and gagging, fainting or collapsing episodes, and a decreased tolerance for exercise (ie. they tire easily). Over the long term, this also puts an increased strain on the heart. Some dogs, such as English bulldogs, may have frequent episodes of sleep-disordered breathing. Overheating is especially dangerous in these breeds, because increased panting (the normal mechanism for cooling in dogs) can cause further swelling and narrowing of the already constricted airways, which will increase your dog's anxiety. Excitement, exercise, or warm weather (and especially a combination of these factors) can trigger this vicious cycle. These dogs can also have gastrointestinal problems, because of difficulties coordinating swallowing when they are working so hard at breathing. This can result in vomiting ar gagging because of swallowing so much air, or aspiration pneumonia, because of breathing in saliva or food particles. All dogs of these breeds have an increased risk associated with sedation and anesthesia, for which your veterinarian will take extra precautions.
How is brachycephalic syndrome diagnosed?
These problems are usually evident from a young age. If your dog has respiratory difficulties, your veterinarian may discuss this syndrome with you as part of a regular visit, or you may bring your dog in because of an episode such as collapsing after exercise.Because some changes in anatomy are common to all dogs of these breeds, diagnosis is really a question of the degree of abnormality. The overlong soft palate is best examined under general anesthesia, and so, because of the associated risks, your vet will most likely ask your permission in advance to surgically correct it at the same time if necessary. Neutering can often be performed at the same time.
How is brachycephalic syndrome treated?
Medical treatment (oxygen therapy, corticosteroids) can be used for short term relief of airway inflammation. Surgery is required where severe anatomic faults interfere with breathing. Most commonly this involves removal of some of the excess fleshy soft palate, and widening of air passages at the nostrils. It is important to keep your dog from becoming overweight, as this will worsen his or her respiratory difficulties in the long run.
For the veterinarian:
In mild episodes of obstruction, short-acting steroids, oxygen therapy, and cooling the dog while it calms down may be sufficient. Bear in mind that sedation without intubation will relax upper airway muscles and may increase obstruction, and that hyperthermia may develop in an oygen tent or cage and exacerbate the problem.
These dogs, particularly the English bulldog and other Bully breeds, have an increased risk of aspiration pneumonia following surgery to correct airway problems.

Congenital Epilepsy

Idiopathic epilepsy is a specific term referring to a seizure disorder that has no identifiable cause. It is also referred to as genetic or congenital epilepsy. The terms epilepsy, seizure, fit or convulsion all mean the same thing, the physical manifestation of a sudden, excessive electrical discharge of neurons in the brain that results in a series of involuntary contractions of the voluntary muscles, abnormal sensations, abnormal behaviors, or some combination of these events. In your pet, the physical manifestation can vary between a far-away look or twitching in one part of the face to your pet falling on his side, barking, gnashing his teeth, urinating, defecating and paddling his limbs. Seizures usually appear suddenly and end spontaneously, and can last from seconds to minutes. Idiopathic epilepsy can occur in all pedigree breeds as well as mixed-breed dogs. In some breeds, idiopathic epilepsy has been proven to be genetic. These breeds include German shepherd dogs, keeshonds, Belgian tervurens, beagles, Irish setters, Saint Bernards, poodles, wirehaired fox terriers, cocker spaniels, Labrador retrievers and golden retrievers. Because idiopathic epilepsy or a predisposition to epilepsy might be inherited, neither epileptic animals nor their first-degree relative should be used for breeding.
Components of a Seizure
There are three components of a seizure:
1. Aura. Certain signs of an impending seizure may be evident, such as restlessness, whining, shaking, salivation, affection, wandering or hiding. These signs may persist from seconds to days in duration and may or may not be apparent to you.
2. Ictus. During ictus, the seizure occurs. The attack may last seconds or minutes. Your dog may fall on his side and may look like he is kicking or paddling. He will salivate, lose control of his bladder, and be unaware of his surroundings.
3. Postictial stage. This stage occurs immediately after the seizure. Your dog will appear confused and disoriented and may wander or pace. He may still exhibit salivation and may be unresponsive to you. Or he may come to you for comfort. The period may be short or it may last for days.
Diagnosis:
By definition, idiopathic epilepsy is a seizure disorder with no known cause, however it is important for your veterinarian to determine your pet's general health and make sure there is no underlying disease that may be causing the seizures. Your veterinarian will take a detailed history and perform a complete physical and neurological examination. Recommended blood tests may include a CBC, serum biochemistry panel, toxin screen, urinalysis and fecal examination.
Treatment:
The goal of treatment is to reduce the severity and frequency of the seizures while avoiding unacceptable side-effects. It is rare to completely eliminate all seizures in dogs with idiopathic epilepsy. your veterinarian may choose to treat the disorder with anticonvulsant medications
Drug Therapy:
1. Phenobarbital is usually the drug of first choice for idiopathic epilepsy. It is given two to three times a day by mouth at an initial dose of 1 mg per pound twice a day.
2. The other common anti-convulsant used in dogs is oral diazepam or valium.
3. Bromide is the active ingredient in potassium bromide and sodium bromide and is another anticonvulsant that can be used in addition to phenobarbital or as an initial monotherapy drug. Many dogs that do not initially respond to phenobarbital alone will have a dramatic decrease in seizure frequency and severity with the addition of bromide.
Home Care:
At home, follow your veterinarian's recommendations regarding drug administration and monitoring, and maintain a complete seizure log with information regarding the seizures, any medication change, veterinary visits and illnesses. Haphazard drug administration or abrupt changes in medication may be worse than no treatment at all and may cause status epilepticus, a condition characterized by persistent seizure activity for a period of more than 30 minutes or repeated episodes of seizure activity without recovery in between. Blood tests will be required to monitor your dog's response to therapy and guard against toxic effects from the seizures as well as the anti-convulsants. The diagnosis of idiopathic epilepsy is not a death-warrant; epilepsy is a chronic disease that can be managed in the vast majority of cases. There is help for you and your dog. Work with a veterinarian with whom you feel a good rapport. Educate yourself on seizures and their treatment.

Epilepsy is a condition characterized by recurrent seizures. Approximately 2 to 3 percent of dogs are epileptic and the age at which dogs with idiopathic epilepsy have their first seizure is usually between 1 and 5 years of age. Many pets can have one seizure without ever having a second. The seizures can be generalized or partial. Partial or focal seizures indicate activation of a limited number or group of neurons. Generalized seizures indicate a synchronous discharge of a large number of neurons in both sides of the brain. The majority of dogs (50 to 60 percent) with idiopathic epilepsy have generalized seizures. Seizures usually appear suddenly and end spontaneously. Seizures can last from seconds to minutes.

What to Do If Your Pet Has a Seizure:
1. Do not panic. If your pet is having a seizure, he is unconscious and he is not suffering. Your pet may seem like he is not breathing, but he is.
2. Time the seizure. Actually look at a clock or watch and note the time; although it may seem like forever, it may only be 30 seconds.
3. Keep your pet from hurting himself by moving furniture away from the immediate area. Also protect him from water, stairs, and other sharp objects. If possible, place a pillow under his head to prevent head trauma.
4. Note what type of muscular activity or abnormal behavior does your pet exhibits during the seizures? Your veterinarian may want you to keep a record of the date and length of time of each seizure.
5. If the seizure lasts more than 5 minutes, call your veterinarian or veterinary emergency clinic immediately.
6. Pets do not swallow their tongues. Do not put your hand in your dog's mouth � you may get bit. Do not put spoons or any other object into your pet's mouth.
7. Keep children and other pets away from your seizing animal.
8. Remain by your pet's side; stroke and comfort your animal so when he comes out of the seizure you are there to calm him.
After the Seizure:
1. Observe your pet's post-seizure behavior. Do not allow your pet access to the stairs until he is fully recovered. Offer water if he wishes to drink.
2. Be prepared for vocalization and stumbling after the seizure ends. You need to be strong and offer support and comfort to your pet. He will be confused and may feel as though he did something wrong. Speak softly and with a soothing voice.
3. If your pet has not fully recovered within 30 minutes, contact your veterinarian or local emergency facility.
Signs That Require Emergency Veterinary Attention:
1. Seizures that last longer than 10 minutes
2. Seizures that occur more than 2 times in a 24 hour time period
3. Seizures that begin before your pet has completely recovered from the previous seizure


Distachiasis

Dogs with this defect have one or more eyelashes that grow toward the cornea, rather than away from it. The inward-growing hair can abrade the cornea. This is painful and, if not treated, can damage vision. Surgical correction is available but can be expensive. The mode of inheritance is unknown. Affected animals can be identified before they reach breeding age and should not be bred. Unaffected individuals who produce it repeatedly, especially with multiple mates, should be pulled from breeding.

Canine Melanoma

Melanoma is a type of cancer that occurs commonly in dogs with pigmented (dark) skin. Melanomas can occur in areas of haired skin, where they usually form small, dark (brown to black) lumps, but can also appear as large, flat, wrinkled masses. Melanomas also can occur in the mouth, toes, or behind the eye. In general, skin melanomas tend to be benign, and those in the mouth, toes, or eyes tend to be malignant. However, there are many exceptions to this rule. The term cancer refers to a large number of diseases whose only common feature is uncontrolled cell growth and proliferation (multiplication). This loss of cell growth control results from an accumulation of mutations (errors introduced into the DNA code) in genes that control cell division and cell survival. The most common mechanism that introduces mutations into the DNA of somatic cells (non-reproductive cells) is the inherent error that occurs during normal cell division. In mammalian cells, there is an error rate of about 1 in 1,000,000 to 1 in 10,000,000 bases during each round of replication. The genome consists of many millions of base pairs, so each daughter cell is likely to carry at least a few mutations in its DNA. Most of these mutations are silent; that is, they do not present any problems to the cell�s ability to function. However, others can disable tumor suppressor genes or activate oncogenes that respectively inhibit or promote cell division and survival. Given the fact that cell division is responsible for most mutations, it is not surprising that the most common cancers arise from cells that divide frequently in the performance of their function. Fortunately, the body has many built-in safeguards that mandate that abnormal cells be destroyed. For that reason, before cancer can take hold, a malignant cell must eliminate or evade these safeguards. The reliability of these systems is evident in the fact that our pets are not �walking tumors�. Still, cancer is the most frequent cause of death in dogs. Melanoma arises from melanocytes, cells that impart pigment or coloration to the skin. In humans, melanoma arises due to mutations induced by repeated, intense exposure to ultraviolet light (for example, frequent tanning or working outdoors). This does not seem to be a major factor in dogs, as in most breeds the hair coat affords them protection from sunlight. However, pigment cells divide every time there is injury to the skin, or if there is constant trauma (for example, areas where dogs constantly scratch or lick). Nevertheless, risk factors for canine melanoma are not well established. Mutations that contribute to cancer can also be inherited. An inherited mutation in a single gene that is important in cell growth control will increase the risk of that individual to develop cancer. This can be due to reducing the overall number of acquired mutations that must accumulate before a cell becomes cancerous, or it can be due to disabling a critical safeguard gene that normally prevents cells from becoming tumors. Specific genes that are responsible for familial melanoma have been identified in humans and in mice. In dogs, there appears to be a predisposition among certain breeds or families to develop specific types of cancer, suggesting that a hereditary component may be important in the development or progression of the disease.

The laboratory of the AMC Cancer Center has an ongoing program to define the role of genetics in canine cancer. With support from the Canine Health Foundation of the American Kennel Club and specific breed clubs, Doctors are evaluating the role of various target genes in canine melanoma. For additional information on this project, or to inquire if your breed can be included in Doctors study we encourage you to visit the CHF web site at www.akcchf.org, or contact your breed club�s health representative



Staffords are, overall, much healthier than many other breeds, and their joie de vivre makes them a real pleasure to own. Knowledge about the health conditions occasionally found in the breed, combined with your veterinarians skill and knowledge, will help to ensure your Stafford a long, happy, and active life in your company.







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