World Health Organization defines rare disease as a debilitating lifelong disease or disorder with a prevalence of 1 or less, per 1000 population. As per 2011 census, over 7.2 crore Indians suffer from rare diseases. Idiopathic pulmonary fibrosis (IPF), is a chronic progressive interstitial lung disease which lacks of recognition at many different levels which leads to decades of neglect and underdiagnoses1. Traditionally, the overwhelming burden of tuberculosis during the last century has tended to dominate the respiratory landscape. The fact that tuberculosis can cause fibrosis that mimics some of the ILDs, especially sarcoidosis and hypersensitivity pneumonitis (HP), has also undoubtedly contributed to delayed and inaccurate diagnoses. According to the American Journal of Respiratory and critical care medicine in 2015, there were 2.2 million Indian tuberculosis cases out of a global incidence of 9.6 million cases.
Idiopathic pulmonary fibrosis is a form of lung disease that leads to scarring of lung tissue that occurs due to unknown reasons. The major cause of occurrence could be related to environmental causes such as exposure to dust or chemicals, smoking, familial history of IPF, and viral infections. It results in damage and scarring (fibrosis) of the lungs over time making it hard for the patient to breathe by depriving the supply of oxygen to the body which eventually hampers the day to day activities.
Dr. Raja Dhar and Dr. Pawan Agarwal addressed the issue of increasing IPF trend in Kolkata at a media briefing with an aim to increase awareness of the growing IPF cases, its distinct symptoms and the importance of early diagnosis.
Talking about IPF incidence in Kolkata, Dr. Raja Dhar, Director, Department of Pulmonology at Fortis Hospital Anandpur said, “In the last 10 years, the number of IPF cases have increased significantly; due to better diagnosis. The national registry data shows around 14% IPF amongst all ILD patients. Other studies have shown upto 21% incidence of IPF. Though the symptoms vary in each individuals, the most common symptoms include chronic dry cough, breathlessness, loss of weight/ appetite, and clubbing of fingers. IPF is more common amongst males, whereas we see a ratio of 3:1 amongst male and female (Indian ILD Registry data). Hence, it needs to be given due attention at the onset of signs and symptoms by a specialist. In general terms, there’s no absolute cure as the viability of the lung tissue after scarring would be lost which cannot be fully recovered”.
The symptoms and treatment for IPF can have significant physical, psychological and emotional effects on patients as well as their families. As the disease progresses over some time it can also hamper the patient personally by impeding their performance in basic day to day activities and professional life.
Highlighting the diagnosis and management of IPF, Dr. Pawan Agarwal, Consultant at Calcutta Medical Research Institute said, “The diagnosis of the condition is done with the aid of blood tests, radiologic scans, and lung biopsies. The procedure to manage the disease can be discussed with the patient. Individuals should consult an expert for a regular checkup at-least once a year. If there is family history, it is compulsory to get one. If tested regularly and adhere to the advised treatment, IPF can be managed. Patients are advised to quit smoking, exercise, consume healthy diets. Due to better diagnosis currently, the number of IPF patients have risen. Hence it is extremely important that IPF gets diagnosed at an early stage which will help reduce and control the progression”.
Early diagnosis and treatments could help prolong the life expectancy of the patient. If there is a family history it is compulsory to get oneself tested regularly and adhere to the advised treatment.