Uncoordinated Cilia Function in Dogs

Cilia are the small hairs that remove foreign substances from certain areas of the body, such as the respiratory tract, ears, brain, spine, uterus, and testes. When these cilia do not function properly, as they do in primary ciliary dyskinesia, those foreign substances cause infection and other problems. Dogs with primary ciliary dyskinesia tend to have a lot of breathing issues because the incoordination of the cilia functioning slows the mucus removal as well as not filtering out the foreign substances. This can cause chronic respiratory problems such as bronchopneumonia (inflammation of the lungs and bronchi), rhinitis (swelling of the nostrils), bronchiectasis (dilation of the small airways from too much coughing) and bronchitis (swelling of the bronchioles), as well as infections of the sinuses and ears from chronic inflammation and obstruction of airways. There is also a possibility of bronchiectasis, (the development of lesions caused by constant swelling and blockage of the airways), hydrocephalus (swelling of the brain) and dilation of the tubules of the kidneys.

Uncoordinated cilia function (primary ciliary dyskinesia) is a serious congenital disorder that affects the cilia, which are the small hairs that move unwanted substances out of the dog’s airway. These cilia line the entire respiratory tract and do not work the way they are supposed to and causes susceptibility to respiratory infections in the affected animal.

Symptoms of primary ciliary dyskinesia generally show up as respiratory and ear infections when your dog is young. This can happen when your dog is about three to six weeks old and since it is a lifelong illness, you should be prepared to spend a lot of time and money for veterinarian visits and medications.

• Coughing
• Nasal discharge (with a lot of mucus)
• Wheezing
• Labored breathing
• Extreme sleepiness
• Difficulty exercising
• High body temperature
• Difficulty hearing
• Weight loss
• Infertility
• Shortness of breath
• Depression
• Blue skin and mucous membranes (cyanosis)

Primary ciliary dyskinesia is caused by genes that have mutated, and no longer encode proteins in the cilia and sperm. Unfortunately, some males that are used as breeders (studs) can have the mutated gene without ever showing any symptoms at all. Because of this, the disease is passed on to many other animals without the owner or breeder’s knowledge. To prevent this disease, a simple DNA test of the stud and bitch can determine whether they are a carrier of primary ciliary dyskinesia. The breeds known to be susceptible to this mutated gene disorder are:

• Bichon Frise
• Border Collie
• Bullmastiff
• Chihuahuas
• Chow Chow
• Cocker Spaniel
• Dalmatian
• Doberman Pinscher
• English Setter
• Golden Retriever
• Gordon Setter
• Long-Haired Dachshund
• Miniature Poodle
• Old English Sheepdog
• Newfoundland
• Rottweiler
• Shar-Pei
• Springer Spaniel
• Pointer
• Staffordshire Bull Terrier

Even if your dog is one of the many breeds known to carry this mutated gene disorder, your veterinarian will need to know your dog’s complete medical history and as much information as you have about the symptoms and when they started. The veterinarian will also do a thorough physical examination including blood pressure, heart rate, and body temperature.  He will also need to run some tests to rule out any other causes for the primary ciliary dyskinesia symptoms. Some of the tests that will need to be done are:

• Complete blood count (CBC)
• Urinalysis
• Digital radiographs (x-rays) which may reveal under developed nasal sinuses
• CT scan
• MRI
• Fine needle biopsy
• Nasal swab
• Semen samples (if your dog is a male) as most dogs with this condition are infertile
• Bronchoscopy which could show an accumulation of pus-like material in the trachea
• Endoscopy
• Culture of cilia cells
• Biopsy (nasal, tracheal or bronchial mucosa)
• Bronchoalveolar lavage
• Cilia function tests
• Electron microscopy of cilia

Unfortunately, at this time, there is no proven treatment or cure for primary ciliary dyskinesia. The only thing the veterinarian can do is to treat the infections that come up from time to time, such as with pneumonia, ear infection, and sinus infections. Pulmonary rehabilitation (physical therapy) can be extremely helpful in preventing the buildup of mucus and infections. Pulmonary rehabilitation includes the forced cough for expiration of the lungs, postural drainage, massage, and vibration.

It is also important to note that DNA testing can prevent this disease for those who are raising these certain breeds that are susceptible to primary ciliary dyskinesia.

Although there is no cure for primary ciliary dyskinesia, your dog can live for many years with this disease if you are vigilant about getting treatment for every sign of infection. In addition, you should provide a warm and dry environment for your dog and plenty of ventilation. Avoid extreme temperature changes (both hot and cold), and dusty environments. Be sure to provide your dog with a healthy diet and consult your veterinarian if there are any changes in your dog’s health or behavior.