What is pulmonary fibrosis?
Pulmonary fibrosis is a condition where there is increased scarring of the lung tissue. As discussed early, scar tissue may be the body’s way to “cut its’ losses” when a healthy repair cannot be accomplished. However, this is an excessive and often progressive continuation of that process. Instead of cutting losses, this leads to the destruction of the lung with decreased lung function and increased shortness of breath. In some people, the cause of the lung injury and resultant inflammation, immunological changes and scarring can be found. For example, acid reflux of stomach contents into the lung, or the aspiration of oil-based material from ointments placed in the nose which melt and drip into the lung, can both cause permanent and irreversible damage and scarring. When there is active inflammation, sometimes medications such as steroids can prevent the formation of scar tissue and reverse some of the lung damage. Active inflammation can sometimes be diagnosed on CT scan when “ground glass” changes are seen. In general, however, the chronic use of steroids in patients with fibrosis and no measurable inflammatory component, has not had a significant impact on scarring. Sometimes, the cause of the fibrosis cannot be determined. These patients are diagnosed as having “idiopathic pulmonary fibrosis” or IPF. In all of these people, a crackling sound is often heard in the chest from the hardened and scarred lung tissue.
How is pulmonary fibrosis treated?
The goal of the management of fibrosis is the prevention of further damage to the lung. Active inflammation should be aggressively treated. Infections should be treated within the first 1-2 days of illness to prevent further scarring and damage. Acid reflux with either heartburn or silent heartburn should be aggressively treated. Silent heartburn occurs when material leaves the stomach and regurgitates back into the throat, but the person doesn’t feel or notice it because the material is not irritating to the throat (however, it can be severely irritating to the lung). When treated for “silent heartburn,” some people may notice a decrease in cough, chest tightness or throat clearing. It is uncommon for them to refer to the sensation as “heartburn.” Treatment can be accomplished with medications that block acid or help food move through the digestive tract. If this is inadequate, anti-reflux endoscopic (minimally invasive) or surgical procedures (laparoscopic) can be considered.
People should also consider having a formal evaluation of their swallowing. This is traditionally accomplished with a modified barium swallow. Material is given to you to swallow while x-rays are performed to see where this liquid contrast goes. Material may be seen to go directly from the throat and into the lung, this may be either liquid material or solid material. It may all go into the stomach, and then can be watched to see if it leaves the stomach and refluxes back up into the throat and then falls into the lungs (termed reflux or heartburn). Swallowing problems, or dysphagia, can be treated with special swallowing techniques which are taught by Speech Therapists. Mechanic problems with swallowing (excessive tissue in a ring or web around the throat) can be treated with endoscopy (camera) by Gastroenterologists. If swallowing problems are severe, some patients may want to consider alternative feeding, with feedings placed directly into the stomach via a feeding tube to avoid material moving “down the wrong pipe” from the mouth. While it is true that some material placed through a feeding tube can still reflux up from the stomach into the throat and be aspirated into the lungs, this is usually a smaller amount and less irritating to the lung then table foods.
How fast does pulmonary fibrosis progress?
Most patients have a very slow course which lasts for decades. Other patients have a rapidly progressing course, which can progress over five years of less. There are rare forms of accelerated fibrosis which can progress to fatal fibrosis over a matter of days to weeks. Your doctor can likely tell you which group they feel you belong to
What happens to my lungs over time with fibrosis?
As fibrosis progresses over time, many patients will require supplemental oxygen. Oxygen is usually delivered via plastic tubing that is placed into the nose (nasal cannula) or directly into the trachea (tracheostomy or transtracheal oxygen). The nasal route is the most common and is used for low amounts of oxygen. The tracheal route is usually used for higher amounts of oxygen. Tracheostomies are plastic tubes that connect the oxygen supply through the skin of the neck directly into the airway (trachea). This route is usually necessary for high flow oxygen; however, it can also provide a route for the direct suctioning of secretions (mucus) from the airway. People can eat and talk with tracheostomies. They can usually perform all their usual daily functions with it in and can wear a plastic cap on it to allow for speech. They cannot swim because water would enter through this same hole and they would drown. People with tracheostomies need to learn how to care for them and clean them. The tubing periodically must be changed by a physician, but this easily slides in and out, and there is minimal discomfort. The oxygen supplied to a tracheostomy has to have humidification (water vapor). Humidification is required because bypassing the nose and mouth and passing directly from the skin into the airway, this pathway avoids the only means the body must warm and moisten the air.
Oxygen supplies come in two main forms. First, there is an oxygen concentrator which is placed in the home. This pulls oxygen from the air and concentrates it into your nasal cannula. It is usually plugged into an electric outlet, and you have extension tubing so that you can be anywhere in your home with it on. The second form is a portable oxygen tank. These tanks can be small and worn with a backpack, or larger and pulled in a handcart. Sometimes, patients can have oxygen-conserving devices added, which deliver oxygen only when breathing in (instead of continuously) and can make the tank last longer. Tanks can be filled with either gaseous or liquid oxygen. Remember that oxygen supports combustion and must be kept away from heat sources and open flames. Also remember that metal tanks can be magnetic and must be kept away from MRI machines and other large magnets.
What are other complications of pulmonary fibrosis?
In addition to the direct injury to the lung from fibrosis, there is one additional problem that can develop, pulmonary hypertension. The lung is filled with air, which must get into the bloodstream. The lung, therefore, is a highly vascular organ, with lots and lots of blood vessels. Anything that affects the lung, can in turn affect these blood vessels. Because the increased injury to the lung, the blood pressure in the lung can elevate and patients get what we call “pulmonary hypertension.” This is different from the high blood pressure that the doctor measures when they place a cuff on your arm. This elevated blood pressure is occurring in the heart and lungs only. It leads to increased shortness of breath and is a sign that someone is entering into one of the more advanced stages of pulmonary fibrosis. The good news is that there are many new treatments for pulmonary hypertension, including oxygen, anticoagulants, and vascular-specific medications. Many times, people will have a significant improvement in their shortness of breath when beginning these therapies and can extend a good quality of life.
Can I reverse the fibrosis?
Currently, there is no way to reverse scar tissue in the lung. However, you can start treatments that can improve your shortness of breath and try to prevent future damage.
What are the common tests done to evaluate someone with pulmonary fibrosis?
Common tests include routine blood work, a CAT scan of the chest, lung function testing, a 6 minute walk test, pulse oximetry, arterial blood oxygen level, bronchoscopy, echocardiogram, and open lung biopsy (surgical lung biopsy).
Self-Check for Management of Pulmonary Fibrosis
- I have had a thorough evaluation to exclude known common causes of fibrosis, including esophageal reflux, swallowing disorders, airway irritants, and infections.
- I have had the blood pressure in my lungs checked (pulmonary hypertension).
- I have had all the necessary vaccinations needed to protect my lungs from infection.
- I regularly have my oxygen level checked.
- I have participated in a pulmonary rehabilitation program and continue to exercise.
Managing Doctors
- Primary Care
- Pulmonary
- Cardiology
- Rheumatology
- Surgery