By Dr. Mahavir Modi
The demise of renowned tabla maestro Ustad Zakir Hussain on December 15 brought to light a previously lesser-known health condition that affects your lungs. Known as idiopathic pulmonary fibrosis (IPF), it is a long-term, gradually worsening disease that results in lung tissue becoming gradually fibrotic, and it becomes difficult for a person to breathe as well as for the oxygen to be effectively transported to every body tissue. This scaring is, however, permanent, and as the disease progresses, it affects a person’s quality of life significantly.
What Is Idiopathic Pulmonary Fibrosis And How Is This Disease Diagnosed?
IPF is a terminal illness in which the patient’s lungs degenerate gradually and are replaced with scar tissues thus making them to thicken. In turn, the lungs are unable to expand as they used to, and this hinders the transfer of oxygen from the air sacs known as alveoli into the blood stream. This leads to constant breathlessness, exhaustion and more such like symptoms. But what makes IPF a rather serious condition is the fact that the cause remains unidentified most of the times, and this is why it is known as idiopathic pulmonary fibrosis. But there are predisposing factors that cause it, through genetics, environment, or certain infections.
IPF is more commonly found in men than in women, as well as in White people compared with Afro-Caribbean or South Asian people.
As it is often the case with IPF, the subjects most often affected are those in the age of over 50, but males are slightly more endangered than females.
There are several risk factors that increase susceptibility to IPF, including:
- Family History: It may also be due to the family history predisposing to pulmonary fibrosis.
- Environmental Exposures: Long exposure to such particles that are toxic like silica, asbestos or mold can damaging affect the lungs.
- Smoking: Tobacco has a direct toxic effect on the lungs and may worsen the patient’s condition suffering from IPF.
- Chronic GERD: Persistent aspiration of stomach acid in to the lungs by chronic acid reflux could result to lung injury.
Though older adults are most commonly affected, younger individuals with genetic predispositions may also develop IPF, often manifesting in familial forms of the disease.
While IPF is commonly observed in persons older than fifty, young people can also be grossly affected especially in regards to genetic mutation induced diseases. Even some familial types of pulmonary fibrosis, while relatively rare, may be seen in young adults and anyone who experiences chronic males, shortness of breath, or a dry cough should see their doctor.
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How Is IPF Managed?
Managing IPF involves a comprehensive approach, as there is currently no cure for the disease. However, with early diagnosis and appropriate intervention, the progression of IPF can be slowed, and symptoms can be managed to improve quality of life.
Some of the key aspects of managing IPF include:
Medications: Antifibrotic drugs like pirfenidone and nintedanib are used to slow the progression
of fibrosis in the lungs.
Pulmonary Rehabilitation: Supervised exercises, breathing techniques, and emotional support
are integral to improving lung function and enhancing the patient’s quality of life.
Lifestyle Modifications:
- Quit smoking to prevent further lung damage.
- Avoid environmental triggers, including dust, mold, and other pollutants.
- Treat underlying conditions such as GERD to prevent further lung irritation.
Vaccinations: Flu and pneumonia that also cause respiratory infections must be addressed, and their vaccines can offer some protection to those who develop IPF.
Advanced Options: In the last stages, there might be an option to perform the lung transplantation for nonsurgical candidates. Also, the patient get to engage in clinical research touching on the new treatments such as regenerative medicine with the hope of trying to stop the progression of the disease or gain better lung function.
Dr. Mahavir Modi is Pulmonologist & Sleep Specialist at Ruby Hall Clinic, Pune.
[Disclaimer: The information provided in the article, including treatment suggestions shared by doctors, is intended for general informational purposes only. It is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified healthcare provider with any questions you may have regarding a medical condition.]
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