Adrenal medullary hypoplasia

Doug Lindsay was 21 and starting his senior year at Rockhurst University, a Jesuit college in Kansas City, Missouri, when his world imploded.

After his first day of classes, the biology major collapsed at home on the dining room table, the room spinning around him.

It was 1999. The symptoms soon became intense and untreatable. His heart would race, he felt weak and he frequently got dizzy. Lindsay could walk only about 50 feet at a time and couldn't stand for more than a few minutes.

"Even lying on the floor didn't feel like it was low enough," he said.

The former high school track athlete had dreamed of becoming a biochemistry professor or maybe a writer for "The Simpsons."

Instead, he would spend the next 11 years mostly confined to a hospital bed in his living room in St. Louis, hamstrung by a mysterious ailment.

Doctors were baffled. Treatments didn't help. And Lindsay eventually realized that if he wanted his life back, he would have to do it himself…

Whatever was wrong with him ran in the family.

By the time Lindsay was 18 months old, his mother was so weak she could no longer pick him up.

By the time he was 4 she could no longer walk. She did manage to pick him up one more time that year, when he was choking on a jawbreaker. She saved his life.

Otherwise, she was too frail. She lived for decades, mostly bedridden with the same condition that stole her son's twenties. After years of tests, she determined her condition was related to her thyroid, but she was too sick to travel to the Mayo Clinic to get more specialized care, Lindsay said.

Lindsay's aunt also developed the same ailment, growing so feeble she couldn't tie her own shoes…

From the fall of 1999 onward, Lindsay was bedridden about 22 hours a day.

"If I was up, it was because I was eating or going to the bathroom," he said.

Lindsay immersed himself in medical research, determined to find a way out. He saw specialists from endocrinology, neurology, internal medicine and other specialties. When one doctor was out of ideas, he referred Lindsay to a psychiatrist.

That's when Lindsay he realized he'd have to figure his predicament out on his own…

He zeroed in on his adrenal glands, which sit atop the kidneys on either side of the lower abdomen…

Lindsay suspected his body was producing too much adrenaline. He knew of a drug called Levophed, which is approved by the US Food and Drug Administration to raise blood pressure in some critically ill patients. Levophed is basically an injection of noradrenaline, which counters the symptoms created by excess adrenaline.

Dr. H. Cecil Coghlan, a medical professor at the University of Alabama-Birmingham, approached Lindsay after his presentation. Coghlan said he thought Lindsay was on to something.

At last, Lindsay had a medical ally.

It hadn't been done before, but Lindsay convinced Coghlan to repurpose the drug so he could live on a 24/7 noradrenaline drip for the next six years.

Lindsay spent "every second of every day" hooked up to an IV. It stabilized his condition and allowed him to be active for short periods of time around the house.

"I was no longer at risk of losing everything," Lindsay said…

Still, other than doctors' visits, a high school reunion and a few weddings, Lindsay's autonomic dysfunction kept him mostly confined to the house he grew up in well beyond his twenties.

Why was he so sick, he wondered? Something was dumping way too much adrenaline into his blood.

Coghlan told him he might have an adrenal tumor. But three scans of his adrenal glands all came back negative…

A fourth scan in 2006 showed his adrenals "glowing brightly," Lindsay said, an abnormality consistent with his new theory.

Coghlan called Lindsay and said, "We found it!" The diagnosis: bilateral adrenal medullary hyperplasia…

And he fixed on what seemed like a simple solution: If he could cut out the medullas of his adrenal glands -- sort of like slicing into a hard-boiled egg and removing the yolk -- his health would improve…

Eventually he recruited a surgeon from the University of Alabama-Birmingham. In September 2010 Lindsday went to the university hospital, where the doctor successfully extracted one of his adrenal medullas.

Three weeks after the procedure, Lindsay could sit upright for three hours. By Christmas Eve, he had the strength to walk a mile to church.

As he stood in the back of the church during midnight Mass, it finally felt like hope was winning.

But progress was slow. In 2012, he underwent a second surgery at Washington University in St. Louis to remove the medulla from his remaining adrenal gland.

A year later, he was well enough to fly with friends to the Bahamas. It was the first time in his life the Midwesterner had seen the ocean.

By early 2014, he was coming off some of his meds…

Today he still lives in his childhood home in St. Louis. He needs to take nine medications per day, and his health is far from perfect, but he has his life back.

He's not exactly the biology professor he dreamed of being at 21, but he's not far off the mark. He's leveraging his experience into a new career as a medical consultant.

https://www.cnn.com/2019/07/27/health/doug-lindsay-invented-surgery-trnd/index.html?no-st=1564288495

Courtesy of a colleague