The 21-year-old woman gasped as she read the headline: “The 16-Year-Old Girl Who Walks and Eats Tacos While Alive.” He scanned the article about a girl who had a mysterious disease that had destroyed her lungs and who now needed a breathing machine for her. “I have to do something,” she told herself as she finished the article. She thought she knew what was killing this girl, because the story could have been about her six years before her.
At the time, she was a young high school girl in the starting lineup of the women’s volleyball team. Within days of the start of the new school year, she developed a fever of 103 degrees and a sore throat. Her doctor in tiny Thief River Falls, Minnesota, thought she had some kind of viral infection and predicted she would feel better after a few days off. Hey she was wrong. The fever cleared but was replaced by the deepest exhaustion she the girl she had ever known. Just getting out of her bed left her breathless. Her mother took her to the nearest ER, 25 miles away.
As the nurse checked the young woman’s vital signs, she looked alarmed. The patient’s oxygen saturation, which would normally be well over 90 percent, was dangerously low in the 1960s. The nurse put an oxygen mask on her nose and mouth and contacted the doctor in charge. A chest X-ray of her showed a gray cloud invading her lungs. A few minutes later she was directed to an ambulance for Sanford Medical Center in Fargo, ND, the nearest hospital with a pediatric intensive care unit.
In Fargo she was started with several broad-spectrum antibiotics. The doctors didn’t know which insect was causing this pneumonia, but until they did they thought these antibiotics should protect her. But it continued to get worse and within a few days it was necessary to put on a fan.
When even that wasn’t enough, Sanford doctors contacted the Mayo Clinic in Rochester, Minnesota. Eight days after she entered the emergency room, the patient’s lungs were barely functioning at all. The next step was an artificial heart and lung machine known as ECMO, short for extracorporeal membrane oxygenation. This refrigerator-sized device acts as a lung to remove the carbon dioxide waste product from the blood and replace it with oxygen and then the heart to recirculate oxygenated blood through the body. The Mayo Clinic ECMO team flew to Fargo in her car, attached the young woman to the device, and returned with her to Mayo Clinic Hospital. That machine breathed for her for the next 116 days.
Months in the transplant list
Like the girl in the article, she too had walked while connected to the huge car. She too had eaten while she was on the car, even though she didn’t have tacos. The first thing she passed out of her mouth was a communion wafer when she finally felt well enough to walk at least partially to the hospital chapel surrounded by a team of doctors, nurses and technicians. They never understood why her lungs failed. She spent months on the transplant list, waiting for a new heart and lungs to replace the ones her doctors thought would never recover. But they did. And finally, after seven months in the hospital, she was able to go home.
For a few years later she returned to Mayo every six months for a checkup. During those visits she always stopped at the pediatric intensive care unit to see the nurses who had become a second family to her in the months she had been hanging in the balance near her death. On a visit from her two years after her time in the hospital, several nurses told her about a child whose illness looked very similar to hers.
Hours later she and her parents met this little girl’s parents, who told the story of their daughter, just 12, whose lungs had simply stopped working after what appeared to be a viral disease. The families compared the 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 match, until the girl’s parents reported that in the weeks before her arrival at the hospital, their daughter had taken an antibiotic: trimethoprim-sulfamethoxazole (TMP-SMX), known by the brand name Bactrim. The young woman gasped. She had taken this antibiotic (in her case of hers to treat acne) – until the day she went to the emergency room
Since then, another family has contacted her with a family history: a healthy, active teenager becomes desperately ill, with lungs so damaged that he needs life support. She asked these parents if their son was taking TMP-SMX when he got sick. Yes, the astonished answer came. This resulted in a total of three cases. Maybe he had found a real connection.
And now there was this young woman in the news. Her name was Zei Uwadia. The article titled Dr. Jenna Miller as a Pediatric ICU Specialist at Children’s Mercy Hospital in Kansas City, Mo., who cared for Uwadia. The young woman found an email address for the doctor and immediately sent her a message. “I started taking Bactrim for acne about 3-4 weeks earlier [my] acute pulmonary insufficiency, “he wrote.” This happened to at least 3 children between 12 and 20 years old [old]. … The similarities between our cases are disturbing. ”She asked if Uwadia had also taken TMP-SMX.
The same unusual model
Miller was flabbergasted. In fact, the girl was taking TMP-SMX when she fell ill. Could there be a link? Miller contacted a friend, Dr. Jennifer Goldman, a pediatrician trained in infectious disease and clinical pharmacology. She has been researching adverse reactions to this drug for years. TMP-SMX is an effective, safe and inexpensive drug and, for this reason, is the sixth most prescribed antibiotic in the country. It could be a coincidence that these four people, a small fraction of the millions who take this medicine on any given day, have fallen ill. However, the doctors agreed they should investigate. The two pediatricians collected the patient’s medical record that she sent the email and the other cases she had encountered. All were healthy young men who developed devastating lung injury after a brief flu-like illness often with fever, sore throat or cough. And they all had taken TMP-SMX.
What convinced doctors there was a link was biopsies of the affected lungs. Each exhibited the same unusual pattern of targeted destruction: the only cells within the lung that were affected were those in which carbon dioxide was absorbed and oxygen supplied, the cells that do the most important work of breathing. In two, including the patient who first noticed the connection between her disease and the drug, these essential cells eventually regrown, allowing them to breathe on their own once again. Others whose lung tissue did not recover needed a lung transplant. Of those first cases, two died: the 12-year-old that the young woman met in Mayo and Uwadia, the girl from 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 have experienced this reaction after being treated with TMP- SMX. Six are dead. It is still unclear how the antibiotic triggers this rare but devastating destruction. Goldman thinks it’s probably some kind of allergic reaction. But they can’t yet predict who is at risk, or why.
As an ICU doctor, Miller tells me, he often uses this drug. And although these cases are rare, the devastation caused is terrible. “Most of these people,” he says, referring to the 19 cases, “were not being treated for a life-threatening disease, yet they were given this ordinary drug, which ended their life or changed it for always”.
This original patient shares Miller’s mixed feelings. She is now 26 years old and is a nurse who cares for patients who have just undergone a heart and lung transplant. She regularly gives her patients TMP-SMX. And they need it to cure the diseases they have and to prevent the diseases they might get. Yet she knows that due to her reaction to that drug, her lungs will never be the same again. She can play a friendly game of volleyball but she feels breathless after climbing a couple of flights of stairs. She still has a good life. She and she are proud to have made a contribution to science that she hopes will one day prevent this from happening to anyone else.
Lisa Sanders, MD, is a contributing writer for the magazine. Her latest book is “Diagnosis: Solving the Most Baffling Medical Mysteries”. If you have solved a case to share, write here to Lisa.Sandersmdnyt@gmail.com.