Idiopathic Pulmonary Fibrosis otherwise known as Westie Lung Disease, to put in simple terms, is a disease which slowly over a period of 12 – 18 months increasingly prevents the lungs from functioning as they should, restricting their movement and therefore their ability to absorb oxygen into the body.

What this means is that the lungs have to work harder and harder to take in oxygen that the body desperately needs, compared to healthy lungs. In so doing the heart has to work harder, to get the oxygen around the body putting extra strain on the heart & lungs.

We possess countless books on Westies, some very general books but most are detailed insights into the breed covering all aspects of bringing up your puppy, feeding it, training, grooming, common ailments etc. In all but one book, the disease is completely ignored. They only mention the standard complaints that most dogs suffer from. In the one book it is mentioned, it is given a short paragraph but merely mentions it in passing, focusing more on the above issues. It is as if the author felt the need to flag it up, but didn’t want to draw attention to it. It is an all too common disease.

This disease is real. It is very real.

There are several sites on the internet providing very detailed scientific information but this link below we found to be a good general over-view:

http://www.barfplaats.nl/forum/index.php?topic=68042.0

Another very good site has been developed by Professor Cécile Clercx (small animal internal medicine) and her team at the University of Liège in Belgium and they are highly concerned with Idiopathic Pulmonary Fibrosis. She is the coordinator of a European project to better understand this disease; they have several veterinary partners in the UK also (Dr Sheena Warman from Bristol University is the local coordinator). Their website can be found by pointing your browser at:

http://www.caninepulmonaryfibrosis.ulg.ac.be.

Whilst the disease has no known cause, hence the name Idiopathic, it is commonly found in Westies, suggesting that it may have a genetic background and therefore could be reduced with careful and better breeding. Research is ongoing but some sources suggest there are links between the fibrosis, the immune system and allergies. Nothing has seemingly been proven for sure.

There is no cure for this disease and the hard facts are that your precious Westie will succumb to the condition and your only hope is that you can catch the disease as early as you can to delay the onset of the symptoms so that you can spend as much quality time with your pet.

A lot is known about what the disease actually is but there is precious little information out there as to how you help your dog live and try to cope with it 24/7 and so we hope that reading Austin and Harry’s story will help you feel that you’re certainly not on your own. There are plenty of people out there going through the very same experiences as you. Vets can certainly give you an overview of the condition but they don’t live with the pets they treat and often can’t provide you with the vital practical assistance to help you on a day to day basis.

Our advice purely from experience to anyone getting a new Westie puppy would be to get your vet to listen to their lungs very closely at the very least every 6 months once they reach about 6 years of age, so that you give yourself the best opportunity to help your precious pet.

FIBROSIS OF THE LUNGS

As mentioned above, there is no known cause. One theory is that it is a result of repeated injury by a variety of unknown agents, maybe microscopic organisms, pollutants and infectious bodies in the air which are inhaled. As a result of the “injury”, the body tries to protect itself by developing stiff scar tissue in the place of elasticated or stretchy cells. In so doing the once flexible lungs become a lot stiffer, and less able to function properly. The scarring is very like a scar on the skin, it is a replacement for what was once there but is never quite the same as the original. So repeated scarring in the lungs slowly inhibits the lungs from expanding like they used to thereby preventing them from taking in oxygen as well as they used to, and also stops the lungs getting rid of waste gases.

Symptoms

The classic early symptom of Westie Lung Disease is the sound of crackling in the lungs when your Westie breathes. This is the noise the “stiff” scarred tissue makes as it tries to expand and contract. It is very faint and can only be heard by putting your ear very close to your dog’s mouth in a quiet room. It sounds like the noise children’s Moondust powder made when they put it on their tongue. The sound of the crackling can be heard within the narrative of Frazer’s Story but if you click on the link below and listen closely, you will hear the sound the lungs typically make:

It becomes more apparent the further the disease progresses. So the earlier you can detect the onset of this noise, the better chance you have of trying to keep the fibrosis or scarring from building up in the lungs. Sadly its onset is inevitable, but at least you have a chance of trying to fight it, and thereby prolonging the life of your beloved Westie.

As a result of the fibrosis, your dog will slowly get more tired because of its inability to take in air like it used to. Quite often because of the lungs beginning to fail, there is an increased coughing when doing something exertive, like running around, when their collar presses on the windpipe when out on a walk or even just having a good scratch.

The short video below shows how too much exertion with Westie Lung Disease can bring on coughing very easily:

With the makeup of the lungs changing, your Westie will often change the way they sit or lie down because they don’t want to press on their lungs. We found that Austin & Harry wanted to lie on their sides more or even upside down in their beds thereby giving the lungs as much freedom to move.

As time progresses, more towards the latter stages of Westie Lung Disease, your Westie may well display a lot of the following symptoms:

  • The neck is elongated to get more air into the lungs
  • A lack of desire to run or even walk quickly
  • An increased obsession with sniffing on walks
  • Walks become slower and shorter owing to their lack of energy and breathing capacity. Below is a short video of Harry  a month before he died still able to run around, but getting short of breath quicker:

  • Insecurity when on a walk, always looking back for fear of other dogs, not having the ability to run off if needs be. This was taken a week before Harry died. Note the slow walking and looking back on the short clip below:

  • Chest & Lungs will move in & out deeper & quicker. The clip below, taken three days after the above clip shows Harry breathing heavier whilst sleeping. Despite tiring quicker, he was still relatively active and still enjoyed his food. He passed away 4 days later. It can therefore be noted how quickly the fibrosis takes effect within a short space of time once it takes hold. Note that his head is on a pillow, which we found helped him settle:

  • A dry coughing/hacking up of phlegm aggravated by pulling on the lead or when scratching the neck and chest area, or when pressure is put on the chest
  • They often eat more slowly because of a shortage of oxygen when eating
  • More mucous generated by the lungs, and a more regular build up of “sleep” in the corner of their eyes
  • Wanting to sleep more owing to a lack of energy, sleeping very deeply and becoming harder to wake up

It should be made clear that whilst the disease is taking hold and progressing, your dog will rarely panic but merely take their condition in their stride. They don’t know that they are ill and try to make to best of their life. They don’t really understand illness.

Basic Treatment and Prevention

Basic treatments prescribed by vets are;

  • steroids (sometimes known as corticosteroids) to prevent the build up of the scarring in the lungs. These are typically taken orally, or more recently an inhaled version.  We have had experience of both types, as can be read on Harry and Austin’s pages.
  • Lazer therapy, details of which can be found on Frazer’s Story page.
  • diuretics to relieve or remove excess fluids which build up in the lungs as a result of the scarring and inefficiency of the heart,
  • bronchodilator drugs to open up the airways providing a better flow of air intake to the lungs.
  • heart tablets to assist with increasing the flow of blood around the lungs and body thereby improving the distribution of oxygen around the body.
  • cough suppressants may be prescribed simply to reduce the potential damage caused by coughing to the lungs and also to the stress on the heart.

Alternative Options

Some people have used alternative remedies like the use of enzymes. One of our visitors, Fiona Green, had good results with her Westie using enzymes, avoiding the use of steroids, with the blessing of her vet. Her story and details of how this can be achieved can be seen on our visitors page, dated 6 February 2013. Though not tried and tested like normal prescription drugs, they appear to have a positive impact on the fibrin in the lungs, and provided Fiona with an extra one year and ten months with her beloved Jodie. Also, Ann Robertson, who originally sent us a post on 18 August 2013, has been using enzymes with her Molly, with good results so far. We have posted some information she has kindly provided in terms of her dosage of enzymes with Molly.

Making Life More Comfortable

Living with a Westie from diagnosis to its final days can be very upsetting for an owner especially if you don’t know what is about to come. Knowledge of any disease will always help you care for your dog and is one of the inspirations for sharing our experiences. One thing that must be underlined, is that throughout your dogs’ illness you should always try to let your dog live its life to the full and do what it wants to do given the constraints of the disease as you may never know when it will be the last time your dog does that particular thing, let them make the choice to stop. What would be the point in not letting it enjoy its life in the short time you have left with it? However there are little things that you can do to make their lives a little more comfortable so that the symptoms have as little impact as possible.

  • You might find that when lifting your little Westie, that you will need to cradle their bottom with your hand and let their front paws rest on your other arm, to avoid unnecessary pressure on their lungs and rib cage. Don’t lift them up like you would a child with both hands around their chest.
  • During the warmer months, the use of cool air fans blowing air on to them will certainly help their breathing and air intake. At night, if they sleep upstairs, see if you can keep a couple of windows open to keep the air fresh rather than all closed and stuffy, because the air quality generally deteriorates at night. If your central heating is on, by keeping the temperature levels down to say 16-17 degrees Celsius it will keep their panting to a minimum.
  • Use of portable air conditioning units to keep the temperature down.
  • Using air de-humidifiers to try and keep the humidity down to around no more than 50-55%.
  • During colder months when the atmosphere outside tends to be cold and damp try to take them out for a walk when it isn’t so chilly or wet as this often takes their breath away making breathing difficult with the “thin” air. We found that when damp or frosty, the air made our Westies actually visibly shiver, something they never did when in better health. If they do get wet on a walk (very likely in our climate!) we found it better to towel them dry as much as possible on their return and keep them warm.
  • Minimise use of air fresheners and aerosol sprays around the house again to maintain better air quality.
  • When eating and drinking, they will appreciate having their food and water bowls being raised up to help keep the neck in a better position to take in air.
  • When out in the car (see below), if it is safe to do so, let them travel for short periods with their head out of the window taking advantage of the cool air blowing quickly thereby taking in more oxygen.
  • If you take your Westie for a lead walk, as opposed to an off-lead walk in a park etc., the use of a harness may help avoid unnecessary stress and pressure on their windpipe if they pull on the lead and collar. This helps prevent coughing.

Breeding

As you will read on Harry’s page our dogs were very closely related by having the same dogs in each breeding line. In our view, with the benefit of hindsight, this strengthens our suspicions that Westie Lung Disease is strongly linked to a genetic disorder and is therefore preventable. There are more and more people innocently breeding their dogs with little thought for the future consequences of their actions. It would seem to be more prudent to look for a well proven breeder with dogs who have lived to a ripe old age and who knows the history of each of their dogs and their offspring. It is our aim to collate together and populate a database on this site which hopefully gives some kind of source where the genes are being passed down from.

 When it gets too much to bear

If you have been able to catch the disease in its earliest stages, there will come a time after 12 to 18 months (if you’re very fortunate) when the positive impact of steriods will have run their course. The fibrosis will have taken a real hold of your Westies’ lungs and they will have been fighting it off for as long as they can. The three clips of Harry above illustrate this perfectly. Normally by this stage it will be becoming too much for your beloved pet to bear not to mention yourself having to see your pet go through this. They will be starting to gasp for air at this stage and their tongue and gums will be more blue than the rich pink colour you’ve been used to seeing.

Long trips in the car with their head hanging out of the window will certainly help but its impact is only for a short period. This serves to get more air into their lungs thereby oxygenating the blood temporarily. Once your dog has enjoyed this session of relief, the normal symptoms will soon return with the effects of poorer quality air intake. Some vets have suggested use of oxygen by sitting in an oxygen tent or nebuliser, as this significantly improves a dogs blood oxygen levels, but it is only temporary. It shouldn’t really be used as an ongoing solution for the quality of life of your dog.

The impact of fibrosis is unrelenting and it is sadly a battle your pet will not win. The treatment you give your pet will ultimately have no further effect once the scarring reaches a certain level. All you can do is your best for your pet. There will come a time that you may have to be faced with the awful decision to let your pet go, as it will be the kindest thing to do, to avoid prolonging any suffering. You will know in your heart when the time comes and your vet will also advise you as well.

We were approaching the stage where we would be faced with this potential decision to have to make with our first two boys but we didn’t reach the very final stages of the condition. With Austin and Harry the fibrosis took hold very quickly in the latter stages and other factors took over. It is often the case that the side effects of fibrosis, most notably the stress and strain on the heart, will have the final say as was the case with both our boys Austin & Harry, and can be read on their pages. Heart-breakingly, with Frazer, his condition worsened so rapidly following a spell of experiencing dreadful air quality, we had to make the decision to say good bye to him to save him any further suffering, such was the way it took over his body.