A breather for pulmonary fibrosis patients

The scenario for patients suffering with Pulmonary Fibrosis has become brighter with guideline approved drugs available at affordable cost and more than 5,000 doctors actively treating through the IPF therapy. Pulmonary fibrosis is more serious than just having a scar in your lung. In PF, the scar tissue builds up in the walls of the air sacs of the lungs, and eventually the scar tissue makes it hard for oxygen to get into your blood.

Also, pulmonary fibrosis is a family of more than 200 different lung diseases that all look very much alike. Before 2010 no targeted therapy was available in India for IPF. Even after diagnosis of IPF there was no treatment available. Dr N Ravindra, MD Global Hospital says, “Early, non-specific symptoms are commonly mistaken for ageing, cardiac disease, emphysema, bronchitis, asthma or COPD.

Such misdiagnosis, or ignoring the early symptoms entirely, may explain why IPF is rarely diagnosed immediately. Often a correct diagnosis may take several months or even years.” Explaining the symptoms, he says, “A common sign of IPF is bibasilar aspiratory ‘Velcro crackles’ on lung auscultation.

These occur in more than 80 per cent of patients and can be detected when a physician listens to the sounds with a stethoscope made by the lungs during inspiration. The crackles appear firstly in the basal areas of the lung, where the disease initiates, with further progression to the upper zones of the lungs.”

Another symptom of IPF is finger clubbing, where the fingertips and fingernails spread out and become rounder. Finger clubbing occurs in 25 to 50 per cent of patients. Other extra pulmonary symptoms are rare but weight loss, malaise and fatigue may be seen in some patients. Symptoms, especially dyspnea and cough, can cause significant impairment to overall quality of life for patients.

Symptoms are also likely to get worse over time, with patients in the later stages of disease noting that dyspnea curtails physical activity and is extremely distressing. Dr N Ravindra states, “Cough has been described by patients as being dry and non-productive and occurring when they talk for long periods with several even mentioning a nagging desire to cough constantly and never feeling relieved after coughing.”

IPF can be diagnosed through a method of exclusion only thus it is very important that all clinicians actively look for IPF in general respiratory patients and thus increasing the index of suspicion must be increased among physicians. Early diagnosis and aggressive treatment is essential for better patient care. While doctors and care providers will help a patient to manage the disease, the patient is himself the most important advocate.

Continued monitoring of pulmonary fibrosis is a central part of maintaining health. Some patients remain in stable condition for extended periods of time; others may experience a rapid progression of symptoms; while another group may experience a stepwise deterioration over time, fluctuating between periods of stability and worsening symptoms. Accordingly, pulmonary fibrosis treatment strategies are highly individualised based upon one’s medical history and other important conditions.