
A first at KGMU: WLL performed to treat rare lung disease
Patient Anirudh Maurya, a resident of Basti, showed severe respiratory distress and was later diagnosed with PAP, a rare lung disorder in which proteinaceous material accumulates within the lung alveoli, KGMU authorities in a presser on Thursday.
He was admitted to unit 2 with breathlessness, dry cough and high oxygen requirement of nearly 15 litres per minute under the care of Dr SK Verma, Dr Rajiv Garg and Dr Anand Srivastava of the department. The patient's occupational history revealed several years of exposure to cement and stone-blasting environments, a potential risk factor for certain lung conditions.
The procedure was cashless and covered under the government of India's Ayushman Bharat scheme.
Initial treatment was started with high flow nasal oxygenation (HFNO) and supportive care, while a comprehensive diagnostic workup was initiated. Routine blood investigations and a chest X-ray revealed diffuse bilateral lung involvement. To further investigate, a high-resolution CT (HRCT) chest scan was done, which showed 'ground-glass opacities' with a classic 'crazy-paving' pattern, raising suspicion of PAP, said authorities.
The patient underwent a bronchoalveolar lavage (BAL), which validated the presence of PAP. Given the rarity and severity of the disease, a multidisciplinary team was constituted to perform WLL, currently the only established treatment for this condition. The major challenge for the team was to adequately ventilate the lung during the procedure as both the lungs of the patient were extensively involved with the disease. The procedure was conducted in two stages, right lung lavage on June 13 and left lung lavage on July 7.
The procedure was performed by a joint team comprising, Dr Rajiv Garg and Dr Anand Srivastava, along with their resident doctors. Dr Shefali Gautham from the department of anaesthesia and her team members provided vital support.
The patient responded well to both procedures and was taken off the ventilator within 12 hours during both the settings. The patient is now stable and off oxygen support, marking a remarkable recovery, they added..
Vice-chancellor Soniya Nityanand congratulated the team for their outstanding achievement and commitment to patient care, calling it 'a proud moment for the university and a new chapter in KGMU's excellence in tertiary medical care.'
WHAT IS PAP?
* Pulmonary Alveolar Proteinosis (PAP) is a rare lung disease in which a thick, sticky substance made of proteins and fats builds up inside the tiny air sacs (alveoli) of the lungs.
* This makes it difficult for oxygen to pass into the blood, leading to breathlessness, cough, and low oxygen levels.
* The exact cause is often unknown, but it can sometimes be linked to workplace exposure to dust or chemicals. PAP is uncommon and requires specialized diagnosis and treatment, such as Whole Lung Lavage, a procedure that 'washes out' the lungs to remove the build-up.
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