Shillong, Aug 18: Doctors at the North Eastern Indira Gandhi Regional Institute of Health and Medical Sciences (NEIGRIHMS) have, for the first time in the state and probably the North Eastern region, performed a rare procedure on a patient suffering from a very rare lung disorder.

The patient, a young adult male, had been suffering from progressively worsening breathlessness for over 8 months. He had been treated outside the state as a case of Interstitial Lung Disease. However, his condition did not improve.

The patient then reported to NEIGRIHMS and was evaluated by the team of doctors from the Department of Tuberculosis and Respiratory Diseases, NEIGRIHMS led by Dr. Vijay Noel Nongpiur, Assistant Professor, Dr John Muchahary, Senior Resident and Dr Gideon Thangkhiew, Junior Resident. On evaluation, the patient was found to be suffering from a very rare disease called Pulmonary Alveolar Proteinosis.

Pulmonary Alveolar Proteinosis is a rare respiratory disorder with incidence of only 6.87 per million population. It is characterized by the accumulation of surfactant lipoprotein (protective lung protein), which is produced in abnormally high amounts and get deposited inside the lung, leading to impaired exchange of gases and difficulty in breathing. In the untreated cases, the respiratory failure gradually progresses which may even lead to death.

The treatment of Pulmonary Alveolar Proteinosis involves a technique called Whole Lung Lavage, in which the lungs are completely filled with saline and then washed out to remove the excess protein.

The Whole Lung Lavage was performed on the patient under general anaesthesia at NEIGRIHMS, where as much as 7 litres of saline was used to wash each lung. Following the successful treatment, the patient showed near immediate improvement of his oxygen levels. The patient’s symptoms also markedly improved and the patient has now been discharged and is on follow-up.

The procedure was performed by a team of doctors, and technical staff from the Departments of Tuberculosis and Respiratory Diseases, Anaesthesia and CTVS OT. Dr. Priyanka Dev, Associate Professor and Dr. Dasara Nongbet, Senior Resident and their team from the Department of Anaesthesiology provided anaesthesia support during the procedure. Dr. Reuben Kynta, Associate Professor, Department of CTVS provided surgical cover in the operation theatre.

This is a major breakthrough for the treatment of Pulmonary Alveolar Proteinosis in the NE region. The successful performance of this procedure at NEIGRIHMS will now make it possible for more patients with this rare disease to receive the treatment they need.

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