Short of Breath: Pulmonary Fibrosis

We have all felt short of breath after a hard workout at the gym, but imagine having this feeling while blow-drying your hair or walking to the mailbox down the street.  Approximately 20,000 Canadians suffer from pulmonary fibrosis, a chronic disease that causes swelling and scarring of the alveoli (air sacs) and cell tissue of the lungs. 

While in most cases, pulmonary fibrosis is a mild disease with few symptoms, it can also be severe and possibly fatal.  Approximately 4,000 Canadians die each year of pulmonary fibrosis, with another 5,000 diagnosed each year. While the progression of this disease is usually very gradual and can take place over the period of several years; in some rare cases, the disease can progress very quickly leading to death from respiratory failure in as short a period as a few weeks.

Over a period of time, the lung tissue becomes thickened, stiff and scarred.  The development of this scar tissue is called fibrosis.  The scar tissue slowly replaces healthy tissue and causes inflammation and a stiffening of the lungs.  As the lung tissue becomes scarred and thicker, breathing becomes more difficult and the lungs lose the ability to transfer oxygen into the bloodstream, meaning the brain and other organs don’t get the oxygen they need.  

Causes:

Despite its prevalence in Canadian and American society, this is a disease that has stumped doctors.  Limited awareness of the causes and progression of the disease is a concern in the medical community.  There are over 140 known causes associated with pulmonary fibrosis. 

While the nature of the initial disease is not always known, the most common cause is sarcoidosis – a type of fibrosis associated with occupational hazards.  Those who work with asbestos, ground stone or metal dust often inhale small particles of these materials, which can damage the alveoli and cause fibrosis. 

Miners are at an increased risk of developing silicosis – another disease that is known to cause pulmonary fibrosis.  Those performing such tasks as sandstone grinding, tunnelling, sandblasting, concrete breaking and granite carving are at an increased risk of encountering silica.  While large silica particles are stopped in the upper airways, the tiniest particles can be carried down into the alveoli where they can lead to pulmonary fibrosis.  Improving these workplace environments through improved ventilation and wearing masks can reduce the occupational risk to these workers. 

There are many more causes of pulmonary fibrosis, most of which are unknown.  While cigarette smokers are more likely to develop pulmonary fibrosis than non-smokers, the disease is also thought to be linked to an infectious or allergic condition.   

Symptoms:

The most common symptom of pulmonary fibrosis is a shortness of breath, often described as “breathlessness”.  Most patients will first notice this breathlessness while exercising and often ignore these early symptoms, attributing it to “getting older” or being “out of shape”.  If left untreated, the condition can progress and the damage to the lungs can become much more severe, causing breathlessness to occur with minor physical activities such as showering, cooking dinner or getting dressed.  Simple, everyday activities such as speaking on the phone or eating dinner can become difficult, and those with further progression of pulmonary fibrosis can experience this feeling of breathlessness even while resting.

A chronic dry cough, discomfort in the chest, loss of appetite and rapid weight loss are other symptoms associated with pulmonary fibrosis.

Treatment:

There is no known cure for pulmonary fibrosis.  Most often, treatment is limited to teaching patients to breathe more efficiently and taking drugs to reduce inflammation in the lungs.

Corticosteroid drugs are often prescribed to stop the inflammation.  Stopping inflammation can prevent the growth of scar tissue (fibrosis) in the lungs and therefore slow down the progression of the disease.  Studies demonstrate that these drugs given early on in the disease can help to improve the condition.

Rehabilitation therapy can help to teach patients how to breathe more efficiently, using as little energy as possible, helping them to perform daily living activities and maintaining their quality of life while avoiding the feeling of breathlessness.  Managing the condition is key.  Here are a few maintenance tips for those suffering from pulmonary fibrosis:

1 - Stay in Shape

While patients with chronic respiratory problems often limit their physical activities to avoid shortness of breath and discomfort, exercise is very important when managing a pulmonary condition.  The restriction of exercise can actually cause a more rapid progression of the disease.  Physical inactivity causes muscles to weaken and become less efficient.  With regular exercise, muscles become stronger and more resistant to fatigue. 

2 – Eat Right

A healthy diet is important for everyone, but especially for someone suffering from a pulmonary condition.  A full stomach can increase the feeling of breathlessness.  Eating smaller, more frequent meals can relieve stomach fullness, making it easier to breathe.

3 – Rest up

Getting at least 8 hours of sleep each night can boost the immune system and well-being.

4 - Control Your Environment

Avoid environmental irritants like cigarette smoke.  Second hand smoke can be as harmful to someone with a lung condition as though they were smoking themselves.

5 - Practice Relaxation Techniques

Relaxation techniques can help to help manage the panic that accompanies shortness of breath.  Anxiety and depression are common in people with chronic breathing disorders and these feelings can aggravate the underlying disease.

6 – Be Positive

The most important way to manage any chronic condition is to keep a positive attitude.

Sources:

Canadian Lung Foundation.  www.lung.ca/diseases-maladies/a-z/pfibrosis.../index_e.php

Canadian Pulmonary Fibrosis Foundation.  www.canadianpulmonaryfibrosis.ca

Pulmonary Fibrosis Foundation.  www.pulmonaryfibrosis.org