This is the second article in a series addressing
Primary Ciliary Dyskinesia in the Bichon Frise

This article and information contained herein are not to be used as a substitute for proper veterinary care. It is critical that you get a VETERINARY diagnosis of PCD prior to using and discussing the following treatments with your vet.

Primary Ciliary Dyskinesia

Primary Ciliary Dyskinesia, or PCD, is a disease of the respiratory tract that occurs in humans, dogs and other species. It is genetically transferred; the mode of inheritance is currently believed to be autosomal recessive. PCD affects the cilia, or hair-like structures, on the lining of the mucus membranes in the nose, trachea and lungs. 

Normal cilia move together in a wave-like fashion to move fluids through the system and protect the respiratory tract from inhaled pathogens. In a respiratory tract afflicted with PCD, the cilia are incorrectly formed and can’t move in unison. Consequently, fluids in the respiratory tract collect along with pathogens creating respiratory ailments and infections.

Because the symptoms mirror other more common respiratory ailments, cases of PCD have been misdiagnosed as bronchitis, bronchiectasis, bronchopneumonia and even canine distemper viral infection. Microorganisms that have been isolated from animals with PCD include streptococcus, staphylococcus, pseudomonas and mycoplasma.

In addition to the respiratory tract, PCD can affect other parts of the body:

-         Otitis media which causes infections and inflammation of the ears;

-         Renal fibrosis

-         Hydrocephalus which is a build-up of fluid inside the skull causing brain swelling; and

-         Bone abnormality, including abnormal sternum, vertebrae, and ribs.

Proper Diagnosis of PCD

PCD usually appears in the neonatal period or prior to two weeks and up to 12 weeks of age in a dog. A majority of puppies affected have a rattling, raspy or snorkeling sound coming from their noses. A breeder may incorrectly believe the amniotic fluid has not been cleared from the puppy’s airway or that it is not nursing properly. Nasal discharge is also a common symptom of PCD.

It is harder to identify an adult dog with the disease. Generally, the adult dog affected with PCD will have repeated bouts of sinus infections, upper respiratory problems and pneumonia. If the dog has repeated chest or nasal infections, PDC should be suspected.

Unfortunately, a firm diagnosis can only be made by examining the cilia with an electron microscope or by a Gama x-ray. Puppies are not physically big enough for samples to be taken until they are about five weeks old. It is, therefore, important for your veterinarian to be familiar with the disease and to rule out other, similar health issues, such as bronchitis, bronchopneumonia and even canine distemper.

Fortunately, treating PCD is similar to treating other respiratory ailments. Also, adjusting the dog’s environment to remove dust, pathogens and other irritants will help your Bichon to live as comfortably as possible. 

Treating the Bichon with PCD

The good news is that many Bichons with PCD live happy, healthy and otherwise normal lives. Adjustments in the living environment, diet and exercise will help reduce how often or how severe your Bichon’s infections and ailments will be.

Sinus and Ear Infections

Bichons with PCD will present different symptoms, but the typical one is a nasal discharge that is yellow or green, indicating infection. Bichons may also have a clear runny nasal discharge. Changes in thickness or in color may be the first indication that there is an infection. Increased ‘snorkeling’ or a wheezing sound, coughing or reverse sneezing are also signs of an infection.  Please note that your Bichon may still be active and playful at the beginning of an infection.  

Early treatment of infections is essential. If left untreated, they can become more serious and even progress into pneumonia.  Signs of pneumonia include lethargy, arching of the back, loss of appetite, labored breathing, increased coughing and fever.

The ears can also become infected. It’s important to treat these infections early, as well. Chronic ear infections can lead to loss of hearing. Early diagnosis and therapeutic interventions allow us to try and prevent the possible long-term complications of this condition. 

The proper course of antibiotics and length of treatment is critical to insure infections are gone and a relapse is unlikely. Additionally, proper treatment limits the resistance to antibiotics that results from too much use. It is important to have a good relationship with your vet and to have an emergency plan in effect in case your Bichon becomes sick over a weekend at night or on a holiday.

The Lungs

 

The Bichon with PCD will have trouble clearing its lungs of mucus and particles from the air, such as dust and pollen, causing him to cough, wheeze, sneeze and potentially develop an infection. There are some techniques you can use on a daily basis that will help your Bichon clear his lungs. It is important that dogs with PCD never be given steroids or cough suppressants because coughing is the only way of clearing the lungs and nasal passages.

Steaming or Nebulizing and Coupage

Steaming loosens the mucus in the lungs and coupage aids the dog in coughing it out. Steaming can be done in a small bathroom by running hot water in the shower. You can also use a steamer such as a facial steamer designed for home use found in beauty supply stores and. Holding your dog or putting him in a crate will allow you to direct the steam toward his face. It’s important that you keep the steam at least five inches from the dog’s face so that you do not burn him. After ten minutes of steam, you should use a technique called coupage that uses body positioning and percussion to move drainage from the lungs. It is important you ask your veterinarian to show you the proper way to perform coupage. After two minutes of coupage, exercise will help the Bichon to cough out the mucus, keeping his lungs clear and healthy. Playing indoors reduces the risk of the dog being exposed to dust and outdoor allergens. Up to three sessions a day is suggested.

A nebulizer is a device used to administer medication or moisture to people or dogs in forms of a liquid mist to the airways. It can be used in lieu of steam and takes less time. Your veterinarian can suggest the proper nebulizer for your dog. As with steaming, follow nebulizing with coupage and exercise. 

                        A Suggested Daily Routine to Maintain Healthy Lungs

Consult with your veterinarian before using the suggested daily routine listed below.

If your dog is wheezing, coughing or has nasal discharge:

Morning:   10 minute Steam, 2 minute Coupage, exercise.
Afternoon:  Quick Coupage & Exercise.
Evening:  10 minute Steam, 2 minute Coupage, Exercise.
Note that with Coupage, you may NOT get an active cough

1 dose of Metacam to help with inflammation.
2 drops of SinoFresh in each nostril once every 24 to 48 hours

 

If your dog has PCD but is not experiencing any symptoms of PCD, it is suggested you follow this routine once or twice weekly. 

Veterinary Care and Treatment Log

Your veterinarian may not be familiar with PCD. It’s a good idea to consult with him about the disease and the symptoms your dog has. You may even consider taking this article with you when you first suspect your dog has PCD.

As mentioned before, every Bichon will present different symptoms and of varying severity. Keep a close eye on your dog so that you recognize when he shows signs of an infection or problem. It is important to see your veterinarian as soon as they start so that treatment can begin.

Should your dog need to go to the hospital, be sure to inform the vet that care must be taken before using oxygen on a dog with PCD. Oxygen is dehydrating and dogs with pneumonia tend to already be dehydrated. Dehydration will make it harder to release mucus from the respiratory tract.

Keep a log of when your Bichon gets sick, the signs that led up to the illness, the course of treatment and how long it took for him to return to good health. This will also help you to prepare an emergency kit, along with your vet’s approval, in the event your dog becomes sick over the weekend, during a holiday or while on vacation.

Some possible items in the kit include: antibiotics, Metacam (a nonsteroid anti-inflammatory drug), SinoFresh Nose drops (antiseptic for the nasal passages) and Guaifenesin (an expectorant). Dosage and instructions recommended by your veterinarian should be written down and kept with the kit. Be sure to check the expiration dates and replace the items when necessary. You may also want to include a nebulizer and have homemade chicken broth in the freezer to add to the dog’s water to encourage him to drink water and remain hydrated.

Creating a Healthy Environment

Dogs with PCD have trouble clearing their lungs and sinus passages because the cilia do not move correctly. This increases the risk of infection in the respiratory tract. Providing an environment free from irritants will help to keep your Bichon healthy.

The American Lung Association has great tips on how to create a healthy home for those with lung or respiratory disorders, including dogs.  You can visit its website at http://www.lungusa.org/atf/cf/%7B7A8D42C2-FCCA-4604-8ADE-7F5D5E762256%7D/CREATING_GUIDEHH.PDF. Some of the tips are listed below.

Air Cleaners

Air cleaners can help reduce the dust levels in your home. If you have furnaces or air conditioning, be sure to keep the air filters clean.  Cleaning the ductwork also cuts down on irritants. 

A Hepa air cleaner with a pre-filter may give a marked improvement in PCD symptoms, such as wheezing and snorkeling. The most important factor when choosing an air cleaner is to be sure it is not an ionic air purifier. Over time, ionic units leave a fine, white dust on the floors, walls and in furniture which exacerbates the dog’s condition. Ionic products are discussed on the lungusa.org website.

Home Humidity Levels

The correct home humidity level will assist your Bichon in clearing their lungs and nasal passages from mucus. Both low and high humidity allow more particles to get into the airways.  Mid-range levels, 35% - 45%, are the best. If you are unsure of your home humidity levels you can purchase a digital hygrometer at most hardware stores.

If you live in a dry climate, a room humidifier can help with increasing the humidity level in your home.  Cool-mist humidifiers are less apt to promote mold and bacteria growth.   Again, be sure your humidifier are neither ionic or ultrasonic because they create a fine white dust which over time it collects on floors and walls.

If you have high humidity levels you should consider purchasing a dehumidifier. High humidity produces mold, dust mite proliferation and allergies. Whether you have a humidifier or dehumidifier, it is important to keep it clean so that you are not creating more problems than you had. 

Air-cooling

Air-conditioning is recommended over evaporative swamp coolers when trying to cool the air in warm, dry areas. Swamp coolers add humidity and increase mold and bacteria in the environment. The proper humidity level is an essential part of providing a healthy environment for your Bichon.

Cleaning Products

Microfiber - Microfiber cloths and mops reportedly are able to clean with just water as well as rags with disinfectant.  Microfiber items also have a static charge that attract dust, making it much better for dusting than ostrich feather dusters or ordinary cloths that just stir the dust around.

Steam Cleaners - Steam cleaners disinfect as they clean without using chemicals so there is nothing to irritate the lungs or nose. Moreover, these cleaners are small and can be carried around the house to steam clean upholstery, floors, or anything else you want to deep clean.

Disinfectants and Cleaners – Most chemical cleaners are irritants and can provoke flares of PCD.  Arm & Hammer Baking Sodaâ is one of the best cleaning products and one of the mildest. Consider switching from scouring powders, bleaches and other harsh chemicals to plain baking soda. Arm & Hammer Baking Sodaâ now sells baking soda in a convenient shaker bottle just for cleaning uses.

The Great Outdoors

Dust, pollen and dry air are some of the worst conditions for a dog with PCD to encounter. If it is windy outside, it’s a good idea keep your dog inside and keep doors and windows closed. It is a good idea to avoid kennels, doggie day care, dog parks and pet stores, as well. 

Before traveling with your Bichon with PCD, be sure to talk with your vet about potential health risks where you are going and how to avoid them. Remember to take your emergency kit along with you.

 

Diet and Exercise

Exercise

Exercise is vital to a Bichon with PCD.  Because the cilia do not work properly, your dog needs to be trained to use other body functions to help move and rid mucus secretions from his body. Exercise is an excellent way for your dog to keep his lungs healthy. Two to three good exercise times a day help get the coughing reflexes started. 

 

Feeding Stations

A feeding station elevates feeding and watering bowls, reducing the risk of your dog inhaling the water or food into its lungs. The raised bowls are better for long-term health in all dogs, not just the dog with PCD.

Diet

Good health includes a good diet and staying hydrated. Some Bichons with PCD have a reduced sense of smell and need more aromatic food choices to keep them interested in eating and drinking. The following homemade, cooked food recipe may well give your dog a healthy appetite.  Of course, make certain your dog is not allergic to any of the ingredients or cannot otherwise tolerate them. You should also check with your veterinarian before changing your dog’s diet.

Studies in humans suggest that apples are good for the lungs, possibly because they provide certain flavanoids that benefit the lungs.  For this reason, a small amount of fresh apple is given daily. You can feed more, keeping in mind that over time this will increase calories and might cause weight gain. Applesauce should not be substituted due to the increased sugar. 

All weights given refer to the food after it has been cooked, except for the apple. The amount is per day unless otherwise noted.

2.5 oz ground beef (20% fat)

1 oz beef heart

4.25 oz potato

2 eggs per week

1 Multi Mineral Complex every 10 days, such as that found at www.Monicasegal.com

1 oz beef liver every 5 weeks

1.25 tsp bone meal, such as that found at www.Monicasegal.com

5 mg magnesium citrate*

1 tsp kelp per month, such as that found at www.Monicasegal.com

3 cod liver oil capsules per week, such as that found at such as that found at www.Monicasegal.com

1/2 oz fresh apple (with skin, never feed the seeds)

*Magnesium citrate will be in health food stores and/or pharmacies. Most are available as 150 mg capsules. You need only 5 mg daily so that means 1 capsule will last one month. The best bet is to open the capsule and sprinkle ¼  of the contents in a meal one time weekly. Put the capsule back together and use again as needed.

Water

Water is essential to good health. As noted, some dogs with PCD have lost interest in food and water, due in part to a lack of smell. It is imperative that your Bichon stay hydrated. You should contact your vet immediately if your dog becomes dehydrated. One way to encourage your dog to drink water is to add about 2 tablespoons of chicken broth to each cup of water. Do not allow water with chicken broth to sit out too long.

 

These are the signs of dehydration:

1. The skin loses elasticity. This can be somewhat misleading since younger and fatter dogs will have more elasticity than older, thinner dogs. It is important to have an idea of what your dog's skin looks and feels like on a normal basis. Pinch a little skin between your thumb and forefinger on your dog's back. When you release it, it should pop back into place immediately. (You can try this on the back of your own hand as an example) As the tissue under the skin loses moisture, the skin moves back more slowly. In extreme cases, the skin doesn't pop back.
2. The eyes appear sunken and lack moisture.
3.The mouth as well as the gums and nose appear dry.
4. Place your index finger firmly against the gums so that they appear white. Remove your finger and see how quickly the blood returns to the gums (they will become pink in that area again). This is called capillary refill time. If you do this when everything is normal, you will have a basis upon which to compare. The gums of a normal dog refill immediately, the gums of a dehydrated dog could take 3 seconds or so to return to their pink state.

Conclusion

Proper care and love are the best medicine for a Bichon with PCD. It is essential you consult your veterinarian to make a diagnosis of PCD. As always, follow the vet’s instructions.

With time and research, PCD will become a disease of the past. We remember all the Bichons that have suffered from PCD and are grateful for the knowledge and information they and their breeders have provided to the medical and scientific communities. Without them we would not be closer to eradicating this disease. 

Reference Websites and Information:

Primary ciliary dyskinesia (Siewert's / Kartagener's Syndrome): Respiratory symptoms and psycho-social impact
I Christopher McManus¹  , Hannah M Mitchison²  , Eddie MK Chung²  , Georgina F Stubbings³  and Naomi Martin^3 
1Department of Psychology, University College London, Gower Street, London WC1E 6BT, UK
²Department of Paediatrics and Child Health, University College London, Gower Street, London WC1E 6BT, UK
³Department of Psychology, University College London, Gower Street, London WC1E 6BT, UK
BMC Pulmonary Medicine 2003, 3:4     doi:10.1186/1471-2466-3-4

Products for the Lungs and Respiratory Tract

http://www.lungusa.org/site/apps/s/content.asp?c=dvLUK9O0E&b=34706&ct=67517

The Mabis Nebulizer, from Asthma Freedom.  Speak with Jean, 716-688-5922 or visit their website http://www.asthmafreedom.com/

The Kaz Facial Steamer at Cache Beauty Supply 1-800-643-0333 or visit the website at: http://www.cachebeauty.com

Sinofresh www.sinofresh.com

Chemical Sensitive Disinfectants and Cleaners

http://www.ecohousewares.com/docs/other_es_ecotru.shtml?gclid=CMfcw4aTlIICFU9YCwodck4iIg

http://www.safetyplus.net/ecotru.html#56ECO22

Micro fiber Mops    http://www.eriecotton.com/view-category.asp?sub_category_id=20&aiid=1032

http://www.seventhgeneration.com