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Understanding Pulmonary Fibrosis

Pulmonary fibrosis, a lung disease whereby lung tissue becomes damaged and scarred, forms a thickened stiff tissue that makes it tough for the lungs to work properly. As the condition worsens, the person becomes progressively more short of breath, according to the Mayo Clinic. While the resulting lung damage cannot be repaired, medications and therapies may ease symptoms and enhance the quality of life. For some, a lung transplant may be an option. We see this condition a lot in hospice care in San Mateo and elsewhere, and understand how to treat it in our patients. But we thought we would go into more detail on pulmonary fibrosis to keep you informed, as part of Global Pulmonary Fibrosis Awareness Month.


Signs of pulmonary fibrosis include:

  • Shortness of breath
  • Dry cough
  • Fatigue
  • Weight loss
  • Widening and rounding of the tips of the toes or fingers, AKA clubbing
  • Aching joints and muscles

The severity of symptoms may vary quite a bit from one person to the next. Some people fall ill really quickly with severe disease, while others only have moderate or mild symptoms that get slowly get worse over the course of months or years. Some people suffer from a rapid worsening of symptoms, such as breath shortness, that can last for many days or weeks. In that case, patients may be placed on a mechanical ventilator. They may also be prescribed antibiotics or corticosteroid medications.


Pulmonary fibrosis thickens the tissue around and in between alveoli, or air sacs, in your lungs, making it more difficult for oxygen to pass through into your bloodstream. The damage may be caused by a variety of factors, certain medical conditions, long-term exposure, radiation therapy, and certain medications.

Occupational and Environmental Factors

Long-term exposure to pollutants and toxins can cause damage to the lungs, including:

  • Asbestos fibers
  • Silica dust
  • Grain dust
  • Hard metal dust
  • Coal dust
  • Bird/animal droppings

Radiation Treatments

Those who have received radiation therapy for cancers of the lung or breast may show signs of lung damage many months or even years after treatment. The damage severity will depend on:

  • How much of the lung has been exposed to the radiation
  • The total amount of radiation administered
  • If chemotherapy was also used
  • Underlying lung disease


Drugs can also damage the lungs, especially:

  • Chemotherapy drugs
  • Heart medications
  • Some antibiotics
  • Anti-inflammatory drugs

Medical Conditions

Lung damage may result from the following conditions:

  • Mixed connective tissue disease
  • Dermatomyositis
  • Polymyositis
  • Rheumatoid arthritis
  • Systemic lupus erythematosus
  • Scleroderma
  • Sarcoidosis
  • Pneumonia

Is it Hereditary?

The short answer is yes, but in most cases, it’s not as straightforward as it is to inherit green eyes or red hair. According to the Pulmonary Fibrosis Foundation, there are three scenarios that can play out:

Do genes inherited from your parents contribute to PF even if you are the only one in your family with PF?

Yes. There have been many identified genes appearing to increase the risk of developing PF in the case of an abnormal form of the gene being inherited.

Is it possible for PF to run in my family?

Yes. Between three and 20 percent of those with PF have another family member who also has it. This, however, does not mean up to 20 percent of your family members will develop pulmonary fibrosis. In general, the chance that one of your family members develops PF is quite low.

Are there specific types of pulmonary fibrosis where the whole disease is due to just one gene?

Yes. There are some rare genetic forms of PF that may impact both children and adults when just one gene is inherited in an abnormal form from both parents. Hermansky-Pudlak syndrome is an example. Those who have it have a very light skin pigmentation because of the lower levels of melanin contained in their skin, as well as bleeding problems stemming from platelets in their blood that don’t function well. Dyskeratosis congenita would be another example, featuring poor-growing fingernails, increased risk of bone marrow problems, and changes in skin pigmentation.

How Do You Know You Have It?

Physicians can recognize and diagnose pulmonary fibrosis in three main ways:

  • Stiff lungs: Scar tissue and inflammation stiffen the lungs, which makes them hard to stretch. The muscles responsible for breathing have to work overtime just to get enough air, which your brain senses and then triggers shortness of breath when you exert yourself. Stiff lungs also can’t hold as much air as healthy lungs, and it’s this “shrinking” that doctors use to diagnose and track PF.
  • Low blood oxygen: Scar tissue will block oxygen’s movement from the inside of the air sacs into the bloodstream. Sufferers will experience a big drop in oxygen levels during exertion. You may be prescribed oxygen through a nasal cannula or facemask during sleep and times of exertion.
  • Lung sounds: Your doctor may hear “crackles” in your lungs, also known as “rales.” Any condition that affects the air sacs will cause crackles.

Once your doctor suspects you have pulmonary fibrosis, he or she will attempt to diagnose the specific kind of PF you suffer from. There are more than 200!

Contact Pathways Home Health and Hospice

As we said above, our staff has a lot of experience handling hospice patients suffering from pulmonary fibrosis. To learn more about our level of care, please contact us at 888-978-1306.