What Is Idiopathic Pulmonary Fibrosis, Lung Disorder That Claimed Tabla Virtuoso Zakir Hussain's Life?

Tabla virtuoso Zakir Hussain passed away in San Francisco at the age of 73 due to idiopathic pulmonary fibrosis (IPF), a severe chronic lung disorder.

His death has raised awareness and questions regarding this illness. Here's everything you need to know about it.

What is idiopathic pulmonary fibrosis?

According to the US National Heart, Lung and Blood Institute (NIH), IPF is a serious chronic lung disease that affects the tissue surrounding the air sacs, or alveoli, in the lungs. The condition develops when, for reasons unknown, the lung tissue becomes thickened and stiff.

Over time, these changes can lead to permanent scarring of the lungs, known as fibrosis, making it increasingly difficult for a person to breathe.

Individuals with a smoking habit or a family history of IPF are at higher risk of developing the condition, and the risk increases with age.

Causes of IPF

The scarring of lung tissue seems to result from a cycle of damage and healing within the lungs. Once the healing process stops, scar tissue forms. However, the exact causes of these changes remain unknown, according to the NIH.

The scarring impairs the lungs' ability to function properly, making it harder for oxygen to be transferred from the lungs to the rest of the body.

In a healthy lung, oxygen easily passes through the walls of the alveoli and into the capillaries, eventually entering the bloodstream. In contrast, with IPF, the walls of the alveoli thicken, making it difficult for oxygen to pass into the blood.

Risk factors for IPF

Several factors increase an individual's risk of developing IPF:

- Age: The risk of IPF increases with age, and most people are diagnosed in their 60s or 70s.

- Lifestyle: Smoking is the most common risk factor for IPF.

- Gender: IPF is more common in men than women, according to the NIH.

- Family history and genes: A family history of IPF, especially among close relatives like parents or siblings, increases the likelihood of developing the condition. Certain genetic mutations can also make a person more susceptible to IPF, particularly mutations in the MUC5B gene, which is involved in the production of mucus and helps protect the lungs. A mutated version of this gene increases the risk of developing IPF.

Symptoms of IPF

Common symptoms of idiopathic pulmonary fibrosis include:

- Shortness of breath: Initially, it may be hard to breathe during physical activity, but over time, breathing difficulties worsen, even at rest.

- Prolonged dry cough: A persistent, dry cough that doesn't improve is another symptom. This cough may worsen over time and be difficult to control.

- Joint and muscle pain: Ache in the joints and muscles may seem normal but is also a symptom of IPF.

- Fatigue or weakness: Feeling unusually tired or weak is a common symptom of many illnesses, including IPF.

- Weight loss: Unexplained weight loss over time may also occur in people with IPF.

If an individual experiences these symptoms, it is advised to seek medical advice.

Treatment for IPF

Currently, there is no cure for idiopathic pulmonary fibrosis, but treatments can help slow the progression of lung damage and improve quality of life.

- Medicines: Drugs like Nintedanib or Pirfenidone can help the lungs function better, prevent acute exacerbations, and increase survival chances. Antacids may also help reduce the risk of stomach acid entering the lungs and worsening the condition.

- Other treatments: Oxygen therapy can ease shortness of breath and improve exercise capacity. Ventilator support may be required to assist with breathing.

- Surgery: A lung transplant may be an option for some individuals with advanced IPF, but it carries risks such as infection or organ rejection. People who undergo a transplant must take medication for life to prevent rejection.

Adopting a healthier lifestyle, such as quitting smoking, eating a balanced diet, and exercising regularly, can also help manage IPF. Counselling and therapy may also be beneficial for managing stress and anxiety.