Her lungs have been mysteriously closed. How could that happen?

The 21-year-old woman gasped as she read the caption: “The 16-year-old girl who walks and eats tacos while on life support.” She skimmed the article about a girl suffering from a mysterious illness that was destroying her lungs and who now needed a breathing machine. “I have to do something,” she said to herself after finishing the article. She thought she knew what was killing this young girl because the story could have been her own six years earlier.

At that time she was a high school graduate in the starting line-up of the girls’ volleyball team. Just a few days after the start of the new school year, she developed a fever of 43 degrees and a sore throat. Her doctor in tiny Thief River Falls, Minnesota, suspected she had some kind of viral infection and predicted she would feel better after a few days of rest. He was wrong. The fever vanished, but was replaced by the deepest tiredness the girl had ever known. Just getting out of bed took her breath away. Her mother took her to the nearest emergency room, 25 miles away.

When the nurse checked the young woman’s vital signs, she looked worried. The patient’s oxygen saturation, which would normally be well over 90 percent, was dangerously low in the ’60s. The nurse put an oxygen mask over her nose and mouth and turned to the doctor in charge. A chest X-ray showed a gray cloud entering her lungs. Minutes later, she was in an ambulance headed to Sanford Medical Center in Fargo, ND, the closest hospital with a pediatric intensive care unit.

In Fargo, she was started on several broad-spectrum antibiotics. Doctors there didn’t know what pathogen was causing this pneumonia, but until they did, they thought these antibiotics were supposed to protect them. But she continued to deteriorate and had to be put on a ventilator within days.

When even that wasn’t enough, Sanford’s doctors contacted the Mayo Clinic in Rochester, Minnesota. Eight days after she came to the emergency room, the patient’s lungs were barely functioning. The next step was an artificial heart-lung machine known as ECMO – short for extracorporeal membrane oxygenation. This device, about the size of a refrigerator, acts as a lung to remove the carbon dioxide waste product from the blood and replace it with oxygen, and then as a heart to circulate the oxygenated blood back through the body. The Mayo Clinic ECMO team flew their plane to Fargo, attached the young woman to the device and flew her back to the Mayo Clinic hospital. This machine breathed for her for the next 116 days.

Like the girl in the article, she had left while connected to the giant machine. She, too, had eaten at the machine, but no tacos. The first thing that came out of her lips was a communion wafer when she finally felt fit enough to walk at least part of the way to the hospital chapel, surrounded by a squad of doctors, nurses and technicians. They never found out why their lungs failed. She spent months on the transplant list waiting for a new heart and lungs to replace those her doctors thought would never recover. But they did. And finally, after seven months in the hospital, she was able to go home.

After that, she returned to Mayo every six months for a few years for check-ups. During these visits, she always stopped by the pediatric intensive care unit to see the nurses who had become a second family to her during the months she was near death. On a visit, two years after her own time in the hospital, several nurses told her about a child whose illness was remarkably similar to her own.

Hours later, she and her parents met the child’s parents, who shared the story of their daughter, who was just 12 years old and whose lungs had simply stopped working after what appeared to be a viral illness. The families compared notes to see if there were any similarities between the two children’s lives and exposures. They lived in different environments – one rural, one urban – in different parts of the state. Nothing seemed to add up until the child’s parents finally reported that their daughter had been taking an antibiotic in the weeks leading up to hospital admission: trimethoprim-sulfamethoxazole (TMP-SMX), known by the brand name Bactrim. The young woman gasped. She had been taking this antibiotic (in her case to treat acne) – up until the day she went to the emergency room

Since then, another family has reached out to her with a familiar story: A healthy, active youth becomes desperately ill, his lungs so damaged that he needs life support. She asked these parents if their son was taking TMP-SMX when he became ill. Yes, came the astonished answer. There were three cases in total. Maybe she had found a real connection.

And now there was this young woman on the news. Her name was Zei Uwadia. The article named Dr. Jenna Miller as the pediatric intensive care unit specialist at Children’s Mercy Hospital in Kansas City, Missouri who cared for Uwadia. The young woman found the doctor’s email address and immediately sent her a message. “I started taking Bactrim for acne about 3-4 weeks ago [my] acute respiratory failure,” she wrote. “This has happened to at least 3 children between the ages of 12 and 20 [old]. … The similarities between our cases are uncanny.” She asked if Uwadia had also taken TMP-SMX.

Mueller was amazed. In fact, the girl was taking TMP-SMX when she got sick. Could there be a link? Miller turned to a friend, Dr. Jennifer Goldman, a pediatrician trained in infectious diseases and clinical pharmacology. She spent years researching side effects of this drug. TMP-SMX is an effective, safe, and inexpensive drug, making it the sixth most prescribed antibiotic in the country. It could be a coincidence that these four people, a tiny fraction of the millions taking this drug on any given day, got sick. Despite this, the doctors agreed that they should examine it. The two pediatricians collected the medical records of the patient who sent the email and the other cases she found. All were healthy young people who suffered a devastating lung injury after a brief flu-like illness, often with a fever, sore throat or cough. And all had taken TMP-SMX.

What convinced doctors there was a connection were the biopsies of the affected lungs. Each showed the same unusual pattern of concentrated destruction: the only cells within the lungs affected were those where carbon dioxide was taken in and oxygen delivered—the cells that do most of the work of breathing. Two, including the patient who first noticed the link between their disease and the drug, eventually grew back these essential cells, allowing them to breathe on their own again. Others, whose lung tissue did not recover, required a lung transplant. Of those first cases, two died: the 12-year-old the young woman met in Mayo, and Uwadia, the girl on the news.

In the four years since Miller received the patient’s email, she and Goldman have identified a total of 19 patients, most under the age of 20, who had this reaction after treatment with TMP-SMX. Six died. It’s still unclear how the antibiotic triggers this rare but devastating destruction. Goldman thinks it’s probably some kind of allergic reaction. But they still can’t predict who is at risk or why.

As a doctor in the intensive care unit, Miller says she uses this drug frequently. And while these cases are rare, the devastation caused is horrific. “Most of these people,” she says, referring to the 19 cases, “were not being treated for a life-threatening illness, and yet they were given this common drug — and it ended their lives or changed them forever.”

This original patient shares Miller’s mixed feelings. She is now 26 and a nurse who takes care of patients who have just had heart and lung transplants. She regularly gives TMP-SMX to her patients. And they need it – to treat diseases they have and prevent diseases they might get. But she knows her lungs will never be the same because of her reaction to this drug. She can play a friendly game of volleyball, but gets tired after climbing stairs. Despite this, she has a good life. And she’s proud to have made a contribution to science that she hopes won’t happen to anyone else one day.

Lisa Sanders, MD, is a contributing writer for the magazine. Her latest book is Diagnosis: Solving the Most Baffling Medical Mysteries. If you have a solved case you want to share, write to her at Lisa.Sandersmdnyt@gmail.com.

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