Interstitial Lung Disease – Pulmonary Fibrosis

The lungs are involved in around 80% of all patients with diffuse scleroderma, a chronic connective tissue disease that compromises the majority of the internal organs. Pulmonary fibrosis is a type of Interstitial Lung Disease. Lung involvement in all its forms has emerged to be the leading cause of death and disability.

The key elements of scleroderma lung disease involve:

  • Inflammation
  • Lung scarring
  • Blood vessel injury


What is interstitial lung disease?

Inflammation and scarring of the lung tissue is called interstitial lung disease or ILD. It is often assessed through a stethoscope examination and a pulmonary function test. These detailed tests measure lung volumes, breathing mechanics and other features of how your lungs are functioning. The forced vital capacity (FVC) and diffusing capacity (DLCO) are the two key tests examined. The FVC measures lung volume, which is reduced if the lungs are stiffened by scar tissue. The DLCO measures how easily oxygen makes it to the air sacs of the lung into the blood stream, which is reduced if the air sacs are inflamed or thickened by car tissue AND if there is injury to the lunge blood vessels. A CT scan of the lungs can also be used to determine how much inflammation is present.

What is pulmonary fibrosis?

Pulmonary fibrosis, a type of Interstitial Lung Disease (ILD), is a build up of scar tissue in the lungs that progresses to a point that a patient’s breathing is affected. The scarring blocks the lungs ability to transfer oxygen into the blood stream. Individuals with pulmonary fibrosis have repeated damage to the inner sacs of the lungs known as alveoli. Every time alveoli must rebuild from this damage, scar tissue forms around the sacs. This reduces the expansion of the lungs during normal breathing.

There are currently no proven medications to reverse lung changes once they have occurred. It is important, therefore, to MANAGE the condition and PREVENT further damage to the lungs.

Pulmonary fibrosis is characterised by progressive pulmonary restriction, ventilatory inefficiency, dyspnoea, impaired gas exchange and hypoxaemia, which all lead to diminished exercise capacity. Patients with pulmonary fibrosis are also more breathless and tend to be less physically active to avoid these symptoms. All of these manifestations have a significant negative impact on the functional capacity and quality of life of patients, emphasising the importance of appropriate exercise.

Essential tips to manage pulmonary fibrosis

  • Stay on top of symptoms. If you feel there is a change in breathing, inform your doctor.
  • QUIT smoking and/or AVOID passive smoke
  • Avoid over exposure to air pollutants
  • Stay physically active
  • Participate in deep breathing exercises and a graduated aerobic exercise program


Exercise as a means of disease management

Exercise training has been widely considered as a safe, effective and powerful behavioural treatment in prevention and rehabilitation medicine. It is, however, recommended to avoid exercising in extreme hot or cold conditions and to make sure that the air is relatively clear when exercising outdoors. It is important to pace yourself with exercise and read your symptoms carefully.

  • Aerobic exercise: Walking, cycling, swimming. Remember that it is okay to take it slow if needed. Monitor HR if possible.
  • Mobility exercises: Thoracic mobility is a great way to open the chest and lungs. Examples: Foam rolling on the upper back, side-lying rotation twists, lying face down back extensions.
  • Resistance exercise: Especially important for good bone and muscle health due to the common side effects of many of the drugs such as corticosteroids. Examples: resistance bands, free weights, body weight exercises.
  • Deep breathing exercises: Breathing exercises are great methods for improving lung capacity. Some good types of exercises include:


Diaphragmatic breathing:

This technique will help strengthen the often-weakened diaphragm of people with respiratory illnesses. When you have pulmonary disease, air often becomes trapped in the lungs, pushing down on the diaphragm. The neck and chest muscles must then assume an increased share of the work of breathing. This can leave the diaphragm weakened and flattened, causing it to work less efficiently.

  1. Lie on your back with knees bent (option to have pillow under your head for support)
  2. Place one hand on your chest and the other hand just below the ribcage (on their belly).
  3. Inhale through the nose expanding the lungs as much as possible, feeling pressure under the hand on the belly. ‘Belly rise’. There should be no movement under the hand on the chest.
  4. Exhale through the nose and pull the stomach muscles toward the back, deflating the belly. ‘Belly fall”. Again no movement should be felt under the hand on the chest.

At first, this exercise can be practiced for 5-10 minutes about 3-4 times per day, person dependant. The amount of time spent doing this exercise can gradually increase, and perhaps even the effort can be increased placing a book on the abdomen. This exercise can also be done sitting on a chair once it becomes easier.


Pursed lip breathing

This is an exercise that will help those who experiences shortness of breath due to pulmonary fibrosis.

  1. Sit on a chair with your back straight, relaxing neck and shoulders.
  2. Inhale slowly through the nose (not forceful or too deep) for a count of two
  3. Exhale by puckering the lips (as if they are about to blow a whistle) and blow out the air slowly for four counts.
  4. Continue for approximately 5 mins or however long is tolerated and then continue breathing normally.


Forced Coughing (If mucus build up is a common issue)

Forced coughing is a technique to remove excess mucus from the airways.

  1. Sit on a chair with back straight and feet pressed against the floor.
  2. Inhale deeply and feel the diaphragm expand.
  3. Hold breath for three counts.
  4. Open the mouth and cough twice (This may bring up excess mucus so get them to expel the mucus onto a tissue and discard)
  5. Repeat until airways feel clear of mucus.


In collaboration with a health care team, your accredited exercise physiologist will be able to safely provide an individualised exercise program that will assist you in both your recovery & management of the disease, help prevent relapses and progression of the disease from occurring and maximise your quality of life.