Washing away the 'sins' of the lung, the SGPGI way

Lucknow: In an ultra-rare medical condition called Pulmonary Alveolar Proteinosis (PAP), tiny air sacs of the lung trigger the formation of a frothy or foamy build-up. Over time, this scum engulfs the lung, causing patients to suffocate and gasp for every single breath. The 'soapy' action of PAP (read sins because it reminds of the Hindi word paap meaning the same) has just one solution—whole lung lavage (WLL), which simply means washing of the lungs. Two patients with this severe disease, which is seen in less than 30 persons per 10 lakh population, were successfully treated at SGPGI's pulmonary medicine department recently. What makes SGPGI's feat bigger is that it undertook bilateral lung lavage (washing of both the lungs in one go)—a rarity at most centres.

Director Prof RK Dhiman congratulated the entire team on this achievement.

WASHING THE LUNGS: Team leader, Dr Mansi Gupta—supervised by Prof Ajmal Khan and helped by Dr Prasanth AP and Dr Yash Jagdhari—informed that diagnosis begins with bronchoscopy (lung scan) and a blood work-up. "Once confirmed, each lung is gently washed with a large quantity of lukewarm saline under anaesthesia to remove the build-up," she said, adding that while pulmonologists lead the procedure, cardiac anaesthetists (Dr Amit Rastogi, Dr Pallav Singh, Dr Aarib Sheikh, and Prof Prabhat Tewari) and cardiothoracic and vascular surgeons (Prof SK Mishra) are crucial to its success and bail the patient out in case their heart or lung starts to slip into failure.

"The two women treated at SGPGI needed about 60 litres of oxygen when they came. Now they are largely off the support. While they have reclaimed activities of daily life, oxygen is needed only upon exertion," said Dr Gupta, stating that treatment helps in 85% of patients, though many patients may need repeat sessions.

AWARENESS MATTERS: Noting that 'PAP often hides in plain sight, causing harm with its presence, but slipping under the radar until it becomes severe,' head Prof Alok Nath said: "PAP as a rare disease can easily be mistaken for asthma or common infections like viral illnesses, tuberculosis, etc., causing dangerous delays. Early suspicion and timely referral to expert centres can be game changers for the patient, flipping the script from a crippling life on long-term oxygen support to a normal, active life. Our team at SGPGI can deal with it in the best possible way."

WHO IS AT RISK? In 90% of patients, PAP is an autoimmune condition—where one's own body gets into the self-harming mode. The lungs of a PAP-inflicted patient block their own cleaning system. Risk factors include smoking, certain infections, blood cancers, and occupational exposures such as dust (silica) or fumes. Secondary causes like TB account for the remaining 10%. More men are affected. However, both patients attending at SGPGI were women in their 40s. Hailing from rural backgrounds, they were referred with advanced respiratory failure. One of them was supported through the CM-Relief Fund.