A 60-year-old lady suffering from a rare lung disorder – ‘Pulmonary alveolar proteinosis (PAP)’ – has been recently treated successfully by the Pulmonology Department of KRIMS Hospital, Nagpur.
What is Pulmonary alveolar proteinosis?
Pulmonary alveolar proteinosis is a rare lung disease. Its incidence rate is one case per 50 lakh people. It is caused due to the accumulation of abnormal protein material called ‘surfactant’ inside the lungs. This leads to reduced oxygen gas exchange inside the lungs which ultimately leads to severe breathlessness.
How is it diagnosed and treated?
PAP is diagnosed through a variety of tests including x-rays, lung function test, CT scan and lung biopsy. Till today, there is no curative medicine which can reduce the protein deposition inside the lung. It is important to remove the protein from the lungs so that the patient can breathe normally. The process of removal of deposited protein from the lung is known as Whole Lung Lavage (WLL) and is the current standard treatment for PAP. This procedure is tedious and sometimes can be life threatening.
The case at KRIMS Hospital
When the 60-year-old lady at KRIMS hospital went through a CT scan, the doctors were suspicious of PAP. On doing the open lung biopsy, she was proven to be a case of PAP. At the time of diagnosis, she was unable to maintain oxygen saturation and her lung functions were very low. The team of doctors from KRIMS Hospital including Pulmonologists, Anesthetists and Physiotherapists then planned to perform WLL on this patient.
On September 30, she underwent WLL for left lung while her right lung was mechanically ventilated. A total of two liters of the proteinaceous fluid was successfully removed. The whole lung lavage procedure took three hours. The patient was discharged with improvement in her health and her oxygen requirement was dramatically reduced.
A repeat lavage was performed on November 4, after a month. This time the right lung was lavaged while the left lung was mechanically ventilated. Approximately seven litres of water was instilled with a return of six and a half litres. The procedure took four hours to complete. There is drastic change in the patient’s health and CT scans now. She is now able to walk on her own without oxygen support and her quality of life has improved.
The team of doctors involved in the second successful lavage included Pulmonologist Dr Ashok Arbat, Dr Sameer Arbat, Dr Mitesh Dave, Anesthetist Dr Daksha Mandhaniya, Dr Vaishali Shelgaokar along with Physiotherapists Dr Prajakta Borkar and Dr Pallavi Salodkar.