Legendary tabla virtuoso Zakir Hussain died at age 73 in San Francisco, of idiopathic pulmonary fibrosis, a severe and progressive lung disease. His death has brought attention to the chronic illness, which otherwise remains under the radar for many. Below is a detailed look at what idiopathic pulmonary fibrosis is, its causes, symptoms, and available treatments.
Idiopathic pulmonary fibrosis, as classified by the U.S. National Heart, Lung, and Blood Institute (NIH), is a serious, chronic lung disease affecting the tissues around the air sacs, or alveoli, in the lungs. This disorder gradually makes the lung tissue stiff and thick, leading to permanent scarring, which is also known as fibrosis. The ability to breathe and get oxygen to the bloodstream becomes significantly impaired as the disease advances.
The precise causes of idiopathic pulmonary fibrosis are not well understood. However, the condition is believed to be the result of repeated cycles of damage and healing in the lungs. Over time, this repeated damage causes the formation of scar tissue, making it difficult for the lungs to function properly.
Although the direct cause has remained unknown, there are certain factors by which the risk of getting such IPF is known. For example, there can be a history of smoking; age is usually much more advanced; and then sometimes, there may even be a family history with it. In some other instances, genetic mutations could contribute to the cause.
Many factors contribute to developing an idiopathic pulmonary fibrosis:
Age: IPF is often found in patients aged 60 to 70 years. The risk factor grows with age.
Lifestyle: Smoking is the foremost risk factor for the condition of IPF.
Sex: Males are said to be more prone than females to suffer from this disease.
Genetics: Family history of IPF or genetic mutations like mutations in the MUC5B gene may raise a person’s risk for having the disease.
The MUC5B gene mutation leads to abnormal mucus production, which assists in clearing harmful particles from the lungs, and this mutation is often present in individuals with IPF.
Individuals with idiopathic pulmonary fibrosis can present with many symptoms, including:
Shortness of breath: At first, noticeable during exercise, the shortness of breath progresses over time, even when a person is resting.
Chronic dry cough: An unrelenting cough, dry and non-improving with treatment
Muscle and joint aches: Aching in muscles and joints
Excessive fatigue: Tiredness or weakness that accompanies everyday activities
Unexplained weight loss: IPF can cause slow, unwanted weight loss
If individuals have these symptoms, medical help should be consulted to give a proper diagnosis and begin timely treatment.
Currently, there is no known cure for idiopathic pulmonary fibrosis. However, a combination of medicines and treatments can help slow the progression of the disease and improve the quality of life for patients.
Medications: Drugs like Nintedanib and Pirfenidone can help improve lung function and reduce the risk of acute exacerbations, or sudden worsening of symptoms. Other treatments may involve antacids to prevent stomach acid from worsening the condition.
Supplementary oxygen therapy can help reduce difficult breathing and increase exercise performance.
Ventilator: For patients with severe inability to breathe, ventilatory support can be provided. This is usually done where the patient is unable to breathe properly.
Lung Transplant: In this case, a lung transplantation may be performed.With this, there are infections and organ rejection. They always have to take immunosuppressive drugs for life if this medical transplant is done.
Additional lifestyle changes that may improve a patient’s management of the disease include quitting smoking, eating healthy, exercising regularly, and obtaining mental health support.
Idiopathic pulmonary fibrosis is a debilitating and progressive disease, having an enormous impact on quality of life. Even though it has no cure, treatment and lifestyle modifications continue to be undertaken in the hopes of slowing down the disease’s progression. The death of Zakir Hussain reminds one to continue spreading awareness for conditions like IPF, which, although not always apparent, may have a strong influence on people suffering from the condition.
