CLEVELAND – At just 15 years old, Ajit Tolani noticed one day while showering that his hand was turning blue. Soon after, doctors near his home in India diagnosed him with scleroderma, a rare autoimmune disease that can cause skin and other parts of the body to thicken and harden.
Despite the diagnosis, his condition remained stable for years. Tolani was in good health overall, despite some acid reflux, for which he took medication. He stayed active and built a successful career as an international tax lawyer.
Fast-forward 20 years later, in 2014, when his wife, Shruti Tolani, noticed a strange cough whenever Tolani would walk or run.
“Within a few months, I started getting fevers and sometimes pneumonia,” said Tolani.
In July 2016, doctors in India told Tolani he was fine — that his lungs were healing and that he’d be back to normal soon. But just two months later, his condition worsened, and a doctor told him he had only a few months to live.
A friend connected Tolani with the family of a lung specialist who worked in the U.S., Atul Mehta, M.D., of Cleveland Clinic’s Department of Pulmonary Medicine. Dr. Mehta was visiting in India and agreed to meet with Tolani.
“He came to me with all his charts, X-rays and CT scans and asked for my advice,” said Dr. Mehta. “It was obvious that he was suffering with scleroderma and that his lungs were heavily affected. He was already at the end stage of pulmonary fibrosis, scarring in the lungs that makes it hard to breathe.”
Dr. Mehta recognized Tolani’s esophagus also was affected by scleroderma, which was causing his acid reflux. As he’d regurgitate food and liquid, he’d sometimes inhale it into his lungs, causing regular bouts of pneumonia, which led to pulmonary hypertension.
Knowing treatment options were limited elsewhere, Dr. Mehta urged Tolani and his family to come to Cleveland Clinic as soon as possible.
By December, the family was in the United States, but Tolani’s condition had worsened. He was on continuous oxygen, and lung transplantation was his only remaining treatment option.
“He had some of the worst esophageal disease we have ever seen in our lung transplant program,” said Dr. Mehta.
If the esophagus isn’t working, reflux can damage new lungs, which could have hurt Tolani’s chances at a transplant, but his team had a plan.
“We take a lot of pride in offering this therapy to patients with advanced diseases,” said Cleveland Clinic lung transplant surgeon Kenneth McCurry, M.D. “About 15 to 20 percent of the patients that we transplant have been declined by other transplant centers.”
After some setbacks with an infection, Tolani eventually improved. Three days after his infection disappeared, his medical team was able to relist him for lung transplant.
One week later, Tolani received his new lungs — but his battle was only half over.
While he was breathing on his own a few days after transplant, he was still the “sickest guy in the hospital,” said Cleveland Clinic thoracic surgeon Sudish Murthy, MD, PhD. “It’s not just his lungs that were the problem. His whole body was profoundly deconditioned.”
Tolani was fed through a tube in his abdomen, and could not eat or drink by mouth for five months. Eventually, he healed from the transplant and regained enough strength for a second laparoscopic antireflux surgery to tighten the bottom of his esophagus and reduce acid reflux.
Today, he is living in Northeast Ohio with his wife and 11-year-old daughter. He will take immunosuppression medications for life due to his lung transplant, but also to keep his scleroderma under control.
“My scleroderma has become much better because of these meds,” said Tolani. “The flexibility in my fingers is full.”
He now stays active at the gym, doing yoga and gymnastics, and has started working again. And he can eat anything he likes.
“His resolve before the transplant was inspiring and that resolve has been surpassed only by his resolve after the transplant to get better,” said Dr. McCurry. “He’s done a spectacular job and worked quite hard.”
“I see life actually better than it was,” said Tolani. “Two years ago, I was just focusing on work. But now I focus on life.”