Recently a blind schoolteacher who is battling with a rare genetic condition Hermansky-Pudlak Syndrome (HPS) underwent successful double-lung transplantation at the Apollo Hospitals. It is the first case in India and second in the world where the double-lung transplantation for this genetic condition has been practiced successfully. Dr Paul Ramesh Thangaraj, senior consultant Cardiothoracic and transplant surgeon at Apollo Hospital revealed the first successful transplant was done in the US in 2005. HPS is a rare condition to be found in one in every five lakh to one in Million.
This severe health condition results from the mutations in one of the seven different genes. The doctor explained further “The 26-year-old patient was extremely malnourished. Also, this causes absence of pigment in the skin, blindness, bleeding tendencies and the slow degeneration of the lungs, causing the exchange of oxygen and carbon dioxide to be severely impaired (as happens in end-stage lung disease). The condition called idiopathic pulmonary fibrosis made the patient bedridden, dependent on oxygen 24 hours a day,” During the press conference the doctors further reported that since the patient’s heart was functioning well his two lungs were removed and replaced by lungs donated by a victim of a road accident. While talking to the media persons Dr Thangaraj said The patient was 5’6 inch tall but he weighed only 36 kg.
So, matching lungs that suited his size was very difficult as the lungs should not be too large or too small. Fortunately, we received the lungs and the transplantation was performed five weeks ago, The patient was admitted in the hospital prior to the transplant operation and his health was critical since he was unable to remove the oxygen even for a second. According to transplant surgeon As he stays somewhere in Nellore, we kept him in the hospital for more than five weeks. He is well now and will be discharged soon. It will take some three to six months for him to gain weight.