An 18-year-old male had been experiencing progressively worse breathing difficulties, with doctors outside Shillong unable to offer any cure.
However, it was at NEIGRIHMS that the young man was diagnosed with having a very rare disease, known as pulmonary alveolar proteinosis, and a procedure never before performed in the state (if not the North East as a whole) has given him hope of leading a normal life once again.
Over eight months, the patient had been suffering from increasingly worsening breathlessness. He had been treated outside Meghalaya as a case of interstitial lung disease but his condition failed to improve.
On arrival at NEIGRIHMS, the patient was evaluated by a team of doctors from the Department of Tuberculosis and Respiratory Diseases, led by Dr Vijay Noel Nongpiur, Dr John Muchahary and Dr Gideon Thangkhiew.
On evaluation, the patient was found to be suffering from pulmonary alveolar proteinosis (PAP), a rare respiratory disorder with an incidence of only 6.87 per million population. It is characterised by the accumulation of surfactant lipoprotein (protective lung protein), which is produced in abnormally high amounts and gets deposited inside the lung, leading to impaired exchange of gases and difficulty in breathing. In untreated cases, respiratory failure gradually progresses, which may even lead to death.
The treatment of PAP 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 procedure 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 he 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 and Dr Dasara Nongbet and their team from the Department of Anaesthesiology provided anaesthesia support during the procedure. Dr Reuben Kynta of the Department of CTVS provided surgical cover in the operation theatre.
This is a major breakthrough for the treatment of PAP in the North East 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.
