The symptoms of IPF are mostly not recognized in the beginning. The similarity with aging problems, other health problems and sometimes a history of heavy smoking lead physicians sometimes in wrong directions.

The most common signs and symptoms are:

• A dry, chronic irritating cough
• Shortness of breath (SOB).
• Crackle sound in the lungs (heard through a stethoscope - sounds like Velcro)
• Rounding of the fingernails, a condition called clubbing
• Fatigue and a general feeling of not being well
• Unintended weight loss / loss of appetite

Physicians who find pulmonary fibrosis or even IPF in a very early stage usually identify it during a yearly physical by the light coughing and the light crackling noise the lungs make.  In a more advanced stage of IPF, most people complain of persistent coughing.  However, many of the symptoms are similar to those of other diseases, such as asthma, chronic obstructive pulmonary disease (COPD), and congestive heart failure.  The first step will be to eliminate these other diseases and to confirm the pulmonary fibrosis. 

In order to confirm the specific diagnosis of IPF, physicians will have to rule out all known causes of fibrotic lung disease; therefore, it may take some time to explore and eliminate other possible diagnoses.

FAMILY PHYSICIAN

Physical Exam and your history:

A detailed history will show if there were any environmental , e.g. asbestos,  occupational, e.g. coal mining,  or other medical conditions that could have contributed or predisposed a person to the development of the disease.  If other members of your family have had it there may be a genetic origin to your disease. When listening to the lungs, your physician hears ‘crackles’ or Velcro-like sounds with the stethoscope. Widening of the fingertips (clubbing) due to a lack of oxygen in the blood may be another symptom. However this is also a symptom in other lung disorders and heart disease, or you can be born with it. Most likely your physician will order an X-Ray of your lungs.

Chest X-Ray:

A routine chest x-ray may be used as a screening test but more often is used to rule out IPF. Because many patients with significant scarring have a normal chest x-ray your physician may order a CT-scan to confirm the diagnosis.

CT-scan

CT-scanning combines special x-ray equipment with sophisticated computers to produce cross-sectional images (slices) of the inside of the lungs. The sharper and more detailed images can be examined on a computer monitor or easily sent to specialists for interpretation. CT scans of your lungs provide greater clarity and reveal more details than regular x-ray exams and in IPF, a radiologist may see a pattern refered to as “honeycombing” that suggests lung scarring and damage to the air sacs. There may also be a “ground-glass opacity”, which refers to the hazy appearance of lung tissue that is most associated with inflammation. (CT-scan will show the progress of IPF over the years)

PULMONOLOGIST (Lung Specialist) 

Once your family physician has identified the underlying disease as IPF he will direct you to a lung specialist. A pulmonologist, or pulmonary (lung) disease specialist, is a physician who possesses specialized knowledge and skill in the diagnosis and treatment of pulmonary (lung) conditions and diseases. With the knowledge of your X-Ray and CT-scan the pulmonologist will order a PFT to determine your current lung status and establish a baseline. It will give him the information needed to track the progress of the IPF over time by comparing your yearly numbers for lung capacity, flow, diffusion etc.    

Pulmonary Function Tests (PFT) (Every 6 to 12 months):

These are breathing tests that measure the lungs’ ability to exchange oxygen and carbon dioxide. These tests were formerly done in a hospital or clinical laboratory but a majority of pulmonologists now do them “in-house”. You will be asked to breathe into a spirometer which is basically a multitasking measuring device connected to a computer. The PFT can also be  done in a “body box” which looks like a glass telephone booth. There are two important components to a Pulmonary Function Test:  

• Spirometry, which measure inspired and expired lung volumes and the rate at which this occurs, and
• Diffusion capacity, or DLco, which measures the ability of oxygen to diffuse into the blood stream. (Gasses will be added to the air-mixture to get a clearer  picture)

Keep in mind that the numbers are indicators and that you (like any healthy person) can also have a bad day. Fluctuation is normal, it's the long-term trend that's important.

More and complete information on a PFT and the separate tests: 

http://www.grmc.org/documents/pulmonary-function-tests.pdf

or

https://www.hopkinsmedicine.org/health/treatment-tests-and-therapies/pulmonary-function-tests

and

https://www.youtube.com/watch?v=teqX9U2BzYc

Comparable % numbers for "normal" people. https://www.aafp.org/afp/2004/0301/p1107.html 

Six Minute Walk Test (Every 6 to 12 months):

To see how your lungs perform under stress you will perform a six-minute walk test (6MWT). Measuring your blood pressure, heart rate, SpO2 (Pulse Oximeter) and the distance walked in 6 minutes, exercise testing is used to evaluate how well the lungs respond to exertion. It also establishes a baseline for future comparison. 6 Minutes walk tests can sometimes be done with Oxygen and can differ in different hospital locations.

https://pulmonaryrehab.com.au/patient-assessment/assessing-exercise-capacity/six-minute-walk-test/

Pulse Oximeter:

This is a screening test which indicates the amount of oxygen in the blood. It’s an indicator of how well your lungs transfer oxygen from the air to your blood. A meter is placed on one of your fingers or on your head using a headband when you are exercising. The oximeter transmits light at different wavelengths through small blood vessels. Normal ranges are 95-100% on room air. Keep in mind that every time you are in a hospital and see a heart rate monitor, there is very often a measurement of the oxygen in the blood of the patient as well. Pulse oximetry does not measure carbon dioxide levels so a blood gas level measurement may be necessary in some patients. You can buy a Pulse Oximeter at your pharmacy or online for about $45.

Bronchoscopy:

This involves an examination of the lungs using of a small, flexible tube called a bronchoscope. A brochoscopy is usually done in a hospital. It helps to evaluate your IPF and your lungs in general and it also gives the doctor the opportunity to take sample tissue or fluids. The lung tissue samples and fluids obtained through a bronchoscopy will be sent to a lab. Unfortunately, too often the samples are small and are usually inadequate for definitive diagnoses but it gives one more indication of your disease.

Arterial Blood Gas (ABG):

A direct measurement of arterial pH, oxygen, and carbon dioxide is obtained by taking it directly from an artery. Unlike other times when blood has been drawn from your vein, blood will be drawn from an artery in your wrist.  Arterial blood is rich in oxygen because it comes straight from the lungs and it shows how much oxygen is available to the body. Blood from a vein has a lower oxygen concentration and indicates how much oxygen has been used in the body while it’s on the way back to the lungs to be oxygenated again.

VATS - Surgical Lung Biopsy:

The most definitive diagnosis comes from a surgical lung biopsy. The video assisted thoracoscopic surgery is performed by a chest surgeon in an operating room while you are under general anesthesia. Small incisions are made on the side of your chest, and then small samples of lung tissue (after being identified with a CT-scan) are being removed and sent to a laboratory. It is important to get a correct diagnosis because there are many diseases that look like IPF and have similar symptoms. Many of those diseases have different outcomes and there are many different ways to treat them. The results from the lab are the most reliable way to identify IPF. If you have IPF, the surgery will also help determine how far it has progressed. VATS is usually well tolerated, but it may not be recommended for everyone.

Second Opinion:

Even with all this information it's sometimes wise to get a second opinion. There are specialised ILD centers where specialists in insterstitial lung diseases can verify the outcome of the tests and give you a second opinion.

A list of these centers can be found at:

Pulmonary Fibrosis Foundation - Research Centers



INSPIRATION

Take the first step in faith. You don't have to see the whole staircase, just take the first step.

Dr. Martin Luther King Jr.

HEROES

“A hero is an ordinary individual who finds the strength to persevere and endure in spite of overwhelming obstacles.”

Christopher Reeve