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Genetic Diseases & Your Dog

Syringomyelia (SM):
     Syringomyelia (SM) is a frightening medical word, however the definition is far more terrifying for those of us who are Cavalier King Charles Spaniel owners.  SM is a genetic disease diagnosed in a large number of Cavalier King Charles Spaniels.
     A simple description of the condition is fluid fills cavities within the spinal cord near the brain causing great pressure and pain to the dog.
     Other pure-bred dogs are also diagnosed with the disease, however one study in England estimated that over 50% of all Cavalier King Charles Spaniels have the condition, but may not be symptomatic.
     In 2009, 1 of my 3 Cavalier King Charles Spaniels, C.C. Belle, was diagnosed with SM.  She was 6 years old at the time.
     Had I been more educated, and aware of the details about the disease, I would have recognized her symptoms when she was a puppy.
     She had tenderness around her head, neck, and especially her ears.  She had a hypersensitivity to pain.  She also frequently scratched her ears, which I interpreted as an ear infection.  The veterinarian never found evidence of an infection.
     In 2009, my alarm bell went off when I was taking C.C. Belle, and her brother Riece, on a walk around the block.
     I did not see her symptom, but rather I heard it.  As she walked I heard a slight dragging sound, as if her paws were scraping over the pavement.
     I looked down and noticed that she was dragging both of her back paws as she walked.  This did not slow her down or even cause her pain, but I made a mental note to continue to observe her as we took our walks.
     I did not think much of this symptom at first, but after the third day of her dragging her back paws, I decided to take her to the veterinarian.
     During the initial examination they took an x-ray of her spine and legs.The vet explained a condition called syringomyelia, and said that it is prevalent in pure-breed dogs, especially Cavalier King Charles Spaniels.
     The vet suggested I consult a specialist.
     I went home and began my research on the internet about SM, and the more I discovered the more I wept.
     SM is a degenerative disease, non-curable, and possibly manageable through medication and/or surgery.
     The images on the web of the surgery were heart wrenching.
     The surgeon cuts into the dog’s skull to relieve the pressure of the cranial fluid.  They place a mesh over the brain and reattach the skull.  The dog has a long scar down the center of its head parallel to its ears.
     While there has been success with this procedure, it has never been a long-term solution because some of the symptoms return.
     I was quite frightened that C.C. Belle would have the disease and then undergo surgery.
     One of the leading specialists in the field of SM is located in England – not surprising as the country has many Cavalier King Charles Spaniels - but since I live in New York City, I was lucky enough to have access to one of the best specialist in the U.S. - Dr. Levitin, Veterinary Specialist, located at Blue Pearl Vet NYC (see link below).
     After an initial examination, Dr. Levitin, his team and I, sat on the floor of the examining room so that we could get a better view of how C.C. Belle moved.
     We got her to walk so that the doctor could evaluate the extent of the condition, and make a recommendation.
     C.C. Belle is an alpha female dog at home and on the street, but at the vet’s office she is shy, demure, and frightened.  She wanted to sit in my lap and was shaking when anyone else touched her.
     We coaxed her out of her fear, and using their experience, Dr. Levitin and his staff, were able to entice her to walk, sit and lay down.  I was impressed.
     The next day I received a detailed e-mail from Dr. Levitin summarizing the condition, as well as, his recommendations for diagnosis and treatment.
     One of the unfortunate aspects of diagnosing this disease is that it can only be officially confirmed through an MRI and lumbar puncture.
     Both are extremely expensive, especially in New York City.  In 2009 it cost about $5,000.00.
     I decided that the MRI and lumbar puncture were essential for C.C. Belle’s health.
     C.C. Belle had to be put under anesthesia to make sure that she would be still for the procedure.
     After the test results were back, Dr. Levitin called me to confirm that C.C. Belle did have SM.
     Again I wept.
     The course of treatment for this particular genetic condition can vary depending on the progression of the disease.
     For C.C. Belle, Dr. Levitin prescribed a cocktail of antibiotics and steroids.  He did not think that surgery was necessary, but if her condition deteriorated, and the pain worsened we would have to reevaluate.
     C.C. Belle was put on the steroid prednisone, as well as a 21 day dose of antibiotics.
     The prednisone was given in high doses for the first few months, and then slowly tapered off to a manageable dose.
     However, the first week or two were very bad.  The prednisone caused her to urinate frequently, so much so that I had to carry her down the steps of my building and often she would urinate in my arms.
     She would have accidents in the house, and while the urine was mostly water and there was no smell, it certainly was a lot of work to change the bedding, wash the rugs, and make sure that she was clean.
     Her back legs had begun to deteriorate, which made her wobble and fall.  I have hard wood floors so this is a problem.
     I tried various carpets and throw rugs, however Riece, my boy dog, would urinate on them, and carpeting the whole apartment was not an option since I have terrible allergies.
     To stabilize her back legs, I tried two little, rubber booties for her back paws, however they never stayed on, or they were too tight.
     The other side effect of prednisone is a ravenous appetite, and an aggressive behavior.  C.C. Belle has always had both, but the medication magnified the side effect.
     Since 2009 the vet has reduced the prednisone to half a tablet per day plus an antacid.
     The side effects on her little body are that the muscles in her legs are deteriorating, and will continue to do so.  Her back legs are thin while her belly is swollen from the medication.  She has not gained much weight, which is also a side effect, because she has a lot of energy and almost seems to refuse the limitations imposed on her by the condition of her body.
     I find her determination quite inspiring.
     Because of the long term use of the steroids, her fur thinned, and her beautiful plum-like tail lost its feathery look.
     The drug also forced her reproductive system into pre-mature menopause, so her heat cycles have stopped.
     The prednisone has compromised her immune system.  I have to be very careful when I walk her on the sidewalks to steer her way from garbage, debris, urine and feces, in an attempt to reduce exposure to any bacteria.  Even with these precautions, she has had frequent urinary and bladder infections which have required multiple doses of different antibiotics.
     The veterinarian has informed me that long-term use of prednisone will eventually compromise her liver functions, and I should not expect her to live a long life.
     Even so, I feel very lucky because in the three years since she was diagnosed she still is mobile, playful, and cuddly.
     She reminds me that power of the mind is far greater than the power of pain.  She reminds me that no matter how much pain she suffers, life still holds great pleasure – a yummy treat, a nice walk, and a million kisses.I am grateful for the lessons she continues to teach me.

What is Syringomyelia (SM)?

     Syringomyelia (SM) is a genetic condition where fluid fills cavities within the spinal cord near the brain.  This disease is also called the “neck scratcher’s disease” because a common symptom is for the dog to attempt to scratch its neck with one of its hind paws, only to have the paw circle in the air near the neck.
     The condition is caused by the fact that most Cavalier King Charles Spaniels’ skulls are too small to accommodate the cerebellum.  Thus the cerebellum squeezes through the foramen magnum (the hole at the back of the skull), and partially blocks the flow of cerebrospinal fluid (CSF) down the spinal cord.
     The pressure created by this abnormal blockage is believed to create the cavities in the spinal cord.  The pain can be so great that specialists have likened it to having the worst migraine headache.
     The spread of SM appears to worsen with each new generation of Cavalier King Charles Spaniels.  The reasons for this vary, and some experts believe the condition began during World War II when the Cavalier King Charles Spaniel breed almost became extinct.  During this time a few wealthy patrons saved the breed from extinction, however they had a small gene pool for breeding.
     Currently experts suspect that the disease continues because of unethical breeders, and puppy mills that breed as many dogs as possible regardless of the parent’s health.
     Along with the Cavalier King Charles Spaniels other breeds are also known to be affected, however to a lesser extent.  These include the Bichon Frisé, Boston Terrier, Brussels Griffon (Griffon Bruxellois), Bull Terrier, Chihuahua, French Bulldog, Havanese, King Charles Spaniel (the English toy spaniel), Maltese Terrier, Miniature Dachshunds, miniature and toy Poodles, Papillon, Pomeranian, Pugs, Shih Tzu, Staffordshire Bull Terrier, and the Yorkshire Terrier.

The Primary Symptoms of Syringomyelia (SM):

 Excessive scratching (especially while on the lead/leash, and often 'air scratching' where the dog scratches in mid-air, which may cause the dog to hop while walking).
 Ongoing tenderness around the neck, head, shoulders, and hind limbs; or weakness, stiffness and pain in limbs.
 Hypersensitivity including yelping, whining or whimpering as if in pain, but for no apparent reason.  Episodes can disappear then return at varied intervals.  In some dogs the barometric pressure changes in the weather, such as an approaching storm or the onset of cold weather, may induce episodes of pain.

The Secondarvy Symptoms of Syringomyelia (SM):

 Seeking cool areas to sleep.
 Restlessness where the dog will constantly shift, pace and walk back and forth rather than find a cozy place to sleep.
 Head shaking where the dog shakes its head and ears.
 Excessive yawning and licking its lips in an attempt to reduce the pressure it feels in its head.
 Excessive head rubbing from side to side on the floor, furniture or bedding.
 Pushing or digging into furniture pillows or bed pillows.  Often a dog will run the length of the sofa or bed while pushing its head into the soft pillows.  This is a frantic behavior, unlike the normal behavior of a dog.
 Stiffness, nerve damage, and seizures all can affect the dog in many ways from loss of feeling, hearing, and muscular movement to neurological issues with its eyes.  Unfortunately, nerve damage appears to be progressive with this disease.  Some dogs develop stiffness in their necks making it difficult for them to drink or eat from bowls that sit on the floor.  Some dogs also develop weakness in their legs and paws, often wobbling while walking or suddenly falling because their back legs give out.  Seizures also happen to some dogs with this condition.

Syringomyelia and Chiari Malformation Symptoms in Dogshttp://www.youtube.com/watch?v=fDjxvgfUVRU (April 15, 2012)
www.ckcsc.org/ckcsc/ckcsc_inc.nsf/founded-1954/syringomyelia2.html (April 15, 2012)
www.newyork.bluepearlvet.com/ (April 15, 2012)



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