Renowned musician Zakir Hussain, who weaved magic on tabla, passed away on Monday morning, after being admitted in a San Francisco hospital due to deteriorating health condition. As per the family statement, ‘idiopathic pulmonary fibrosis’ is stated as the cause of death.
Pulmonary fibrosis, or idiopathic pulmonary fibrosis, is a chronic, progressive lung disease that can severely affect the respiratory functions of the body. Mid-day.com speaks to Dr Kamlesh Pandey, Consultant Chest Physician, Wockhardt Hospitals, Mira Road and Dr Sheetal Chaurasia, Consultant – Pulmonary Medicine, Manipal Hospital, Whitefield, who explain the symptoms and causes of the disease, also stressing on why early diagnosis is essential to slow down its progression.
What is pulmonary fibrosis?
Pandey: Pulmonary fibrosis is a term used when there is a progression of fibrosis in the lungs. It is a diffuse lung disease. Lung is an elastic organ. During pulmonary fibrosis, the lung function declines over a period of time i.e., elasticity of the lungs goes down. Over time, the oxygen levels also go down, causing difficulty in breathing.
Chaurasia: Pulmonary fibrosis is scarring of the lungs due to some injury. It is an umbrella term that covers multiple diseases that by different mechanisms cause lung injury and initiate a cellular and inflammatory reaction – the end result of which is permanent scarring of the lung known as fibrosis.
Pulmonary fibrosis causes stiffening of the lungs by replacing normal airway cells with scar tissues. This hampers the gas exchange as well as decreases the compliance of the lungs. In simple terms, the lungs become thick, stiff and shrunken, and patients will have to put in more effort to breathe normally.
What are the causes of this respiratory condition? Are there any risk factors?
Pandey: There could be multiple causes, falling into categories like autoimmune connective tissue diseases, chronic hypersensitivity pneumonitis, certain infections like COVID-19, etc. Smoking is a major risk factor.
Chaurasia: There are over 200 causes of pulmonary fibrosis, with the most common ones being environmental, drug-induced, toxin-related, autoimmune diseases, occupational and idiopathic.
Irrespective of the cause of pulmonary fibrosis, the majority of these conditions are progressive in nature and the speed of progression is variable and unpredictable.
What is idiopathic pulmonary fibrosis?
Pandey: A lot of times, no particular cause is found for the disease. This is called idiopathic pulmonary fibrosis, and is more prevalent from what I have observed in my practice.
Chaurasia: In a great number of cases, the cause of pulmonary fibrosis couldn’t be ascertained and these are called idiopathic.
What are some symptoms one should look out for in order to ensure early diagnosis?
Pandey: Some common symptoms include persistent cough, breathlessness that is progressive over a period of time, frequent infections during seasonal changes that exacerbate the condition, etc.
Chaurasia: Clinical features are non-specific with the majority of patients presenting with chronic dry cough in the early stage and slowly progressive breathlessness at a more advanced stage. The diagnosis may be difficult at the early stage because symptoms are often non-specific and chest x-ray may not identify the disease at the early stage. Thus, in the early stage, it is often misdiagnosed as allergic bronchitis or asthma.
Is there a treatment for the condition?
Pandey: Pulmonary fibrosis is irreversible. There’s no cure. The treatment offered is only to delay the progression of the disease.
Chaurasia: The main goal of the treatment is preventing or slowing the progression of the disease. Pulmonary fibrosis is an end outcome of a variety of insults to the lung and is irreversible, hence early diagnosis is paramount.
Once the disease is significantly progressed, it can cause respiratory failure and subsequently right heart failure. The only treatment available at this stage is a lung transplant or heart-lung transplant depending on the severity. However, if diagnosed earlier, the progression of the disease could be slowed down or checked by certain anti-inflammatory and anti-fibrotic medications. So, a chronic cough and breathlessness not responding to regular treatment should be evaluated at an early stage by a pulmonologist.
Can pulmonary fibrosis be prevented?
Pandey: There is no particular way to prevent it. But if the cause is identified, the exposure to those elements can be avoided. Smoking should be avoided. If someone in the family has suffered from pulmonary fibrosis, people should be more careful and go for active screenings.
What are some things that pulmonary fibrosis patients should keep in mind?
Pandey: Patients should be vaccinated against common infections like flu and pneumonia. Certain exercises and rehabilitation practices, as advised by doctors, can aid in preserving lung function and improving quality of life.
Disclaimer: This is for informational purposes only and does not replace professional medical advice. Kindly consult a qualified medical expert for personalised guidance.
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