Pulmonary fibrosis: a progressive disease
Current treatment aims to improve symptoms (quality of life) and slower progression of the disease, the usefulness of steroids and immunosuppressants is under discussion.
Dr. Araceli Carrillo Muñoz
Pulmonary fibrosis (PF) is a condition in which the tissue inside or between the alveoli heals and becomes thicker and stiffer, this type of scarring is called fibrosis. This hinders the passage of oxygen through the alveoli into the bloodstream making it difficult for the person to breathe. With this, vital organs, such as the heart and brain, do not receive enough oxygen to function normally.
Causes
There is no certainty as to the exact cause of this abnormal repair of lung tissue, some include environmental contaminants, dust, gas or fumes, chemotherapy or radiotherapy, infection, genetics, some medications or connective tissue diseases; and in most cases, the origin is not found and this is called idiopathic pulmonary fibrosis.
Symptoms and diagnosis of pulmonary fibrosis
The most common symptoms are shortness of breath or shortness of breath, dry cough that does not improve, fatigue and weight loss for no apparent reason.
Other discomforts may be pains in the muscles and joints, acropaquia or fingers in a drum stick (the tips of the fingers and toes are enlarged and the nails take a curved shape).
Because pulmonary fibrosis has symptoms and a pattern of scarring similar to other lung changes, it can be difficult to diagnose. In fact, in some cases it is defined after discarding other diseases.
In this regard, an adequate medical history, complete and careful physical examination, chest X-ray should be performed initially; and most of the time, a high resolution chest tomography is required, and they should always have pulmonary function tests (spirometry, plethysmography, DLCO, gasometry, 6-minute walk).
Treatment of fibrosis
Current treatment aims to improve symptoms (quality of life) and slower progression of the disease, the usefulness of steroids and immunosuppressants is under discussion. One promising treatment that is already approved for some of the very specific forms of pulmonary fibrosis is pirfenidone. Some other treatments are in the research period. Pulmonary transplantation is another option.
Do you have a cure?
The disease is progressive, and as time passes, it causes an increase in pulmonary scarring and worsening of symptoms. Unfortunately it is finally disabling. Until now there is no cure for the disease and in some cases can become deadly.
Some recommendations
- It is essential to carry a complete treatment, including oxygen if required, in the form prescribed by your doctor.
- If you smoke, it is very important that you stop. There are programs and products that can help you quit smoking, talk to your doctor.
- Perform moderate exercise like walking and stationary cycling. It is recommended that you talk to your doctor before starting a new exercise program.
- If you already exercise, continue with it. This will help maintain better lung function. Patients who require it should use oxygen during exercise.
- Join a pulmonary rehabilitation program to help increase your stamina by teaching you breathing techniques that will be very helpful to you.