Ustad Zakir Hussain died of idiopathic pulmonary fibrosis. What is this lung condition? Premium
Idiopathic pulmonary fibrosis (IPF) is a disease that causes scarring of the lungs. This scarring causes breathing to become progressively difficult. Pulmonary fibrosis is an interstitial lung disease. Interstitial lung diseases are a group of conditions characterised by inflammation or scarring or both that damage the lungs.
‘Idiopathic’ means that it has no known cause. IPF is the most common form of pulmonary fibrosis and is a serious, chronic condition.
Our lungs are made up of millions of air sacs known as alveoli. It is through these tiny air sacs that the exchange of oxygen and carbon-di-oxide is carried out. In IPF, these alveoli and the tissues around them, become thick and stiff, losing their elasticity. This thickening makes it harder for the alveoli to do their job of sending oxygen to the rest of the body. This scarring, or fibrosis, worsens over time, and since stiff lung tissue does not expand as well as it should, this makes breathing difficult.
While there is no known cause, there are some risk factors that may increase chances of a diagnosis of IPF. These include:
Pulmonary fibrosis has also been linked to exposure to certain types of dust, such as metal or wood dust, viral infections and gastro-oesophageal reflux.
Early during the course of the disease, many people may have no symptoms. As the disease progresses, patients may experience shortness of breath, a persistent dry cough, fatigue, loss of appetite and unexplained weight loss. Due to a lack of oxygen in the blood, some may experience rounded and swollen fingertips (clubbed fingers), a thickening of the flesh under the fingernails. Eventually, patients may become breathless while carrying out routine, everyday activities.
The six classic symptoms of respiratory disease, says R. P. Ilangho, a Chennai-based pulmonologist, are cough, phlegm, bleeding while coughing, chest pain, breathlessness and wheezing. Patients with IPF usually experience cough and breathlessness. It is important to see a doctor if any of these symptoms do not subside in two weeks, he says. “Think of the lung as a balloon. IPF hardens the lung tissue, making its elasticity go away. This means the lung is not able to open fully, making it hard to breathe,” he says.
A doctor may collect information about your medical and work history as well as details of your personal environment. A CT scan may be prescribed as well as blood tests to rule out other conditions. Doctors will also monitor the progression of the disease with lung function tests, carbon monoxide diffusion testing and six-minute walk tests. In rare cases, doctors may perform a lung biopsy, which involves taking a small piece of lung tissue for analysis.
There is no specific treatment for IPF, says Kaushik Muthuraja, professor, pulmonology, Sri Ramachandra Medical College. However, there are medications available that slow down the fibrosis, he says. “The rate of progression of the disease is not predictable as it varies from person to person. Depending on how much the disease has progressed, patients may need supplemental oxygen. Some patients, who are eligible, may be recommended for a lung transplant. For others, pulmonary rehabilitation (exercises to help make breathing easier during day-today-activities) are crucial,” he says. Treatment for gastro-oesophageal reflux may also be prescribed. At present, estimates indicate that the five-year survival for patients of IPF is between 50 and 60%, he adds.
Published - December 17, 2024 06:00 pm IST
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