Diagnosis

It’s a different path for each individual which is why pulmonary fibrosis is so difficult to diagnose. It takes two years on average for patients to receive their PF diagnosis.

Obtaining a Diagnosis

Patients with PF experience symptoms that are often subtle and similar to other lung disorders. To diagnose PF, a doctor will take a complete medical history and perform a thorough physical examination. During this examination, the doctor will listen to the patient’s chest to determine if their lungs are producing any abnormal sounds while breathing.

Types of pulmonary fibrosis

Once a doctor suspects pulmonary fibrosis, the next step is to try to diagnose the specific type of PF— there are more than 200 different kinds. Doctors typically start by asking questions, performing a careful physical examination, and ordering several blood tests.

Diagnostic Tests or Procedures

Chest Imaging

Helps the clinician view lung structures, look for scar tissue, and assess patterns of scarring.

Radiologic machines are used to take pictures of your lungs, such as x-ray or High-Resolution Computer Tomography (HRCT).

Pulmonary Function Test

Measures the degree of impairment in lung function using a device with a mouthpiece to measure a patient’s breathing capacity.

Exercise Test or Desaturation Study

Measures how well the lungs and heart respond to physical activity and evaluate oxygen levels with exertion by monitoring the patient while using a treadmill or stationary bicycle.

Six Minute Walk Test (SMWT)

Measures the distance a person can walk as well as lung function during the walk. During this test the patient walks on a flat surface as far as possible in six minutes.

Lung Biopsy

A procedure in which a lung tissue sample is obtained through a bronchoscope or by means of a small surgical incision (VATS – video-assisted thoracic surgery) between the ribs (open-lung biopsy) for direct examination

Symptoms of Pulmonary Fibrosis

Symptoms usually have a gradual onset and may include:

Shortness of breath, particularly during or after physical activity
Spasmodic, dry cough
Gradual, untended weight loss
Fatigue and weakness
Chest discomfort
Clubbing, or enlargement of the ends of fingers (or sometimes the toes) due to a buildup of tissue

Three Consequences of PF

Scar tissue and inflammation makes it harder to breathe. It takes a greater effort to inhale, causing a feeling of breathlessness, or shortness of breath, especially with exertion. The more scar tissue present in the lungs, the less air they can hold.

Scar tissue inhibits the transfer of oxygen from the lung’s air sacs into the blood. For many people living with pulmonary fibrosis, oxygen levels are only slightly reduced while in a resting state, but drops significantly more with exertion. Doctors often prescribe supplemental oxygen. As pulmonary fibrosis progresses, oxygen may be needed 24/7 and the flow rate may need to increase.

The doctor may indicate that “crackles” were heard in the lungs, similar to Velcro being pulled apart. This is very common, although not everyone has them. Crackles can be heard in many lung diseases including PF, pneumonia, or a buildup of fluid in the lungs from heart failure.

More on Diagnosis

Genetics & ILD – Dr. Grant-Orser & Dr. Johannson

ILD respirologists Dr. Amanda Grant-Orser and Dr. Kerri Johannson discuss how genetics can be an important tool for patients with ILD, shared the results of a recent national patient survey, and explained genetic testing in Canada.

Dr. Matthew Binnie

Navigating a New Pulmonary Fibrosis Diagnosis

In this webinar, Dr. Matthew Binnie discusses how to navigate a new pulmonary fibrosis diagnosis . Dr. Binnie is an Assistant Professor at the University of Toronto and Staff Physician at the Toronto Lung Transplant Program.


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