Idiopathic Pulmonary Fibrosis: A lung disorder without cure, for now

Pulmonary fibrosis is a lung condition that results in the formation of scar tissue within the lung tissue, leading to difficulty in breathing and decreased lung function over a period of time. It is a chronic and progressive disease that is characterized by thickening and stiffening of the lung tissue, making it increasingly difficult for the lungs to transfer oxygen into the bloodstream. This can result in shortness of breath, fatigue, and a persistent cough, among other symptoms.

There are several forms of pulmonary fibrosis, including idiopathic pulmonary fibrosis (IPF), which occurs without a known cause, and familial pulmonary fibrosis (FPF), which is a genetic disorder form of the disease. Other forms of pulmonary fibrosis are associated with underlying medical conditions such as sarcoidosis, rheumatoid arthritis, and lupus.

Pulmonary fibrosis can develop slowly over a period of time, and its onset of the disease is often gradual. Early symptoms may include fatigue, shortness of breath, and a persistent cough. As the disease progresses, these symptoms may become more severe and patients may experience weight loss, chest pain, and increased difficulty in breathing.

Diagnosing pulmonary fibrosis can be challenging, as its symptoms are often similar to those of other lung conditions. A thorough medical evaluation, including a complete medical history, physical examination, and lung function tests, is necessary to determine the underlying cause of the symptoms. In some cases, a biopsy of lung tissue may be necessary to make a definitive diagnosis.

In some cases, doctors can find out what is causing the medical condition, but in most cases, physicians cannot find a valid reason. That is why it is called idiopathic pulmonary fibrosis (IPF). IPF varies from person to person. In some patients, the lung tissue quickly becomes thick and stiff. In other cases, the process is much slower. In some others, the condition stays the same for years.

There is currently no cure for pulmonary fibrosis, and the goal of treatment is to slow the progression of the disease and alleviate symptoms. Treatment options may include oxygen therapy, medications to reduce inflammation, and lung transplantation for severe cases.

Oxygen therapy is a common form of treatment for pulmonary fibrosis. It involves the use of supplemental oxygen to increase the amount of oxygen available in the bloodstream. This can help alleviate symptoms of shortness of breath and fatigue and improve quality of life for patients.

Medications that reduce inflammation, such as corticosteroids, may also be used to slow the progression of pulmonary fibrosis. Other medications, such as immunosuppressant, may be used to reduce the severity of symptoms and slow the progression of the disease.

In severe cases of pulmonary fibrosis, lung transplantation may be recommended. This is a complex and invasive procedure that involves removing the damaged lung tissue and replacing it with healthy lung tissue from a donor. Although lung transplantation can be life-saving for patients with severe pulmonary fibrosis, it is associated with a number of risks and complications.

To conclude, there is currently no definitive cure for IPF. However, new medication can mitigate the disease burden among patients and improve their quality of life significantly. Indeed, there is an unmet need for accelerated research into IPF mechanisms so that progress can be made in therapeutics toward the goals of increasing life expectancy, alleviating symptoms, and improving well‐being. Pray the medical fraternity and public health policy makers realise this and act expressly.

(Dr Amit P Jose is a Consultant Pulmonologist at Lourdes Hospital, Kochi)