Patient with reverse organs recovers after complicated surgery

NT Correspondent

A 25-year-old patient with an extremely rare disease called Kartagener’s syndrome, where the organs develop on the opposite side of the body recently underwent a lung transplant surgery at a hospital in Bengaluru.

This is an extremely rare condition that causes the mirror image reversal of in- ternal organs and is said to occur in about 1 in 30,000 of the population. Also called primary ciliary dyskinesia or situs inversus totalis, pa- tients experience breathing problems from birth and suffer from repeated respi- ratory tract infections. People with this disorder often suffer from lung infections such as bronchiectasis, recurrent sinusitis, and have year round nasal congestion and a chronic cough.

“Shivam (name changed) had developed bronchiecta- sis in both lungs which af- fected the left more than the right with complete destruc- tion of the left lung. This also put extensive pressure on his right heart. In the last month, the patient got admitted to the hospital for breathing difficulties which took a severe turn, due to which he had to be put on a ventilator. A lung transplan- tation surgery would be life- saving and the only option for the patient,” explained Dr Sandeep Attawar, Chair & Director of Thoracic or- gan transplants and Assist devices at KIMS Heart & Lung Transplant Institute.

Surgical challenges

Because of lower weight and poor nutritional status of the patient, there were surgical challenges such as poor healing and dehis- cence and the patient need- ed meticulous handling. As the left lung was badly ad- hered to the chest wall, re- leasing and retrieving the damaged lung took a long while with risk of higher bleeding and injuries to very important nerves.

Even though the heart was supposed to be towards the right chest in his case, due to a collapsed left lung it was pushed towards the left due to hyperinflation of the right lung. Mobilisa- tion of the distorted anato- my and implantation of the new lungs was a great sur- gical challenge which was managed by highly skilled and experienced team.

Smooth recovery

Hailing from Shivamogga, Shivam was lucky enough to find a very good match of donor lungs right the day after he registered on the organ transplant list for donor lungs. The sur- gery was conducted for a period of eight hours dur- ing the first week of March and went smoothly without any complications. The patient responded to the donor lungs very well and showed optimal signs of recovery within just three days of the surgery.

“While people with this disorder are largely able to live a normal life, those with lung involvement in the disease are unable to effectively clear fluid and mucus from the airways, leading to build-up and consequent accumulation of bacteria and making them more prone to infec- tion. Repeated infections lead to poor quality of life, lower appetite, weight loss and repeated hospital admissions. They become oxygen dependent with re- duced mobility and develop multiple comorbidities like osteoporosis, muscle weak- ness, depression etc. Early detection of this disorder will help such patients take measures to preserve their lung function, however in certain cases it is a com- plicated disorder that can worsen and even turn life threatening. In such cases lung transplant may be the only hope,” said Dr Pavan Yadav, Lead Consultant – Interventional Pulmonol- ogy and Lung Transplan- tation, Aster RV Hospital.

Kartagener’s disease is a genetic disorder that is caused due to a mutated gene which is present in both parents. Shivam’s sister also suffered from the same disease but un- fortunately succumbed to it. “It has been difficult liv- ing with this disorder as I had multiple complications throughout my life and breathing was always an issue. I was not able to put on weight, struggled with sports or activities and was always worried about my health. I am grateful to the doctors and the donor family for giving me the chance for a new life. I’m looking forward to getting back to my normal life and pursuing my career,” said Shivam.

As a student, his stud- ies used to get interrupted because of his illness and repeated hospital visits and admissions.

Lung transplantation surgery is usually conduct- ed through deceased organ donation, making it a chal- lenge for those in need. There is a lack of aware- ness about organ donation in our country.