Medical detectives raced to save a man from a rare, ‘universally lethal’ disease

The problems started after Erich Burger returned from an unforgettable safari in Botswana and Zambia last month.

The Baltimore software engineer got sick the day after he got home. He headed to a community hospital after about a week of fever and chills, thinking he might have malaria given his recent travel to Africa.

Clinicians took a detailed travel history, admitted him to the intensive care unit as his blood pressure plummeted and ordered blood tests. A sharp-eyed hematology technician discovered when she looked in her microscope that he was suffering from a disease so rare in the United States that it has been seen only 40 times in the past 50 years. The disease is one of the few “universally lethal” infections: It always kills unless it is treated, and it kills quickly.

It is often what went wrong that dictates the course of stories about exotic diseases or the challenge of diagnosis. But in this case, the opposite was true: At every step, Burger encountered health-care workers who did the right thing at the right time, with little precedent for their decisions, while everyone raced the clock.

Burger, 48, spent about four weeks this fall in Botswana and Zambia with his wife, Bernadette, his brother, Dorsey, a wildlife biologist, and friend, Patrick Sosnowski. It was a reunion of a safari trip they had taken together two decades earlier. They roamed game parks without guides, driving 30 to 60 miles a day in rented Toyota Land Cruisers, spotting wildlife that included lions, elephants, hyenas, even a python.

Burger and his wife returned to Baltimore on Nov. 19. When he developed fever, aches and chills the next day, he thought he had picked up a minor bug — and that the long flights were catching up with him. He got progressively worse and had to skip Thanksgiving at a relative’s house.

On Monday, Nov. 28, Burger went to his primary care doctor. Initial blood tests showed some troubling indicators, such as elevated readings of liver enzymes. The doctor suggested over-the-counter medication to control the fever. But by Dec. 1, Burger had nausea and was having bad night sweats and difficulty getting out of bed. His wife drove him to the emergency room at Greater Baltimore Medical Center in Towson, Md., the hospital where his doctor is affiliated.

Burger could barely stand. His blood pressure had fallen to 70 over 52 millimeters of mercury; a typical reading is 120 over 80. For several hours, Burger received intravenous fluids to stabilize his blood pressure before being admitted to the intensive care unit.

“He was very sick. Once that top number is much below 100, it’s often associated with a patient who is in shock,” said David Vitberg, the ICU doctor at GBMC who oversaw Burger's care.

The ER team took his travel history, including the specific countries he had visited. Like Burger, doctors thought malaria was the most likely suspect, even though he had taken anti-malarial medication. The ICU team started Burger on medication to treat malaria and sent his blood to the lab for a positive diagnosis.

A panic alert

The order for a blood parasite test was at the top of Gail Wilson’s to-do list when she arrived for work at 7 a.m. Friday, Dec. 2. She has worked for 22 years as a medical technologist, the last two at GBMC. She knew the procedure would take several hours. The blood smears — one thin and one thick — needed to dry for three hours first before a stain could be applied that would highlight any parasites.

Looking at his blood cells under a microscope, she realized he didn't have malaria. Instead, she found a rare and lethal parasite most clinicians have only seen in textbooks. Wilson had to look really hard because “there were just a few on my slide and could have easily been missed,” she said. But there they were, some purplish-blue parasites that looked “like a wavy kind of ribbon with a dot in the middle,” she said. Wilson recognized the distinctive shape from competency exams she is required to take every year.

“When I first saw it, I was like, ‘Oh, no,' ” recalled Wilson. “I knew it was serious and that the patient could die.”

After conferring with colleagues and supervisors, she notified Burger’s nurse that the blood parasite screen was positive for trypanosome parasites. The notification was a panic alert reserved for lab tests that show extreme or unusual results. Wilson realized that Burger had African trypanosomiasis, or sleeping sickness — a deadly disease spread by the tsetse fly.

It was 10:55 a.m.

Among infections, African trypanosomiasis is in a special category.

“Unlike almost any other infection, this is a death sentence if it’s not treated,” said Theresa Shapiro, a clinical pharmacology professor at Johns Hopkins University School of Medicine, who has spent much of her career studying the disease. Along with HIV and rabies, sleeping sickness is one of the few “universally lethal” diseases: they are always fatal without treatment.

The disease has two forms, both transmitted by the tsetse fly, which lives only in rural Africa. The parasites for both forms look identical.

West African trypanosomiasis accounts for virtually all reported cases of sleeping sickness, according to the World Health Organization. A person can be infected for months or years without major signs or symptoms of the disease.

Initial symptoms include fever, headache, and muscle and joint aches. But left untreated, the disease progresses to the central nervous system after one to two years, leading to personality changes, daytime sleepiness and progressive confusion, according to the CDC. Death usually occurs in about three years.

East African trypanosomiasis is more rare but kills within months. Nearly all cases are reported in parts of eastern and southeastern Africa, including Uganda, Tanzania, Malawi and Zambia. People at greatest risk are tourists, hunters and others working in or visiting game parks, where hoofed animals are the main reservoirs for the parasite that causes the disease and tsetse flies are common.

Over the past century, the disease has caused several epidemics in Africa, and those hardest hit live in the most remote parts of the continent. In the United States, most cases of sleeping sickness have been in travelers such as Burger, on safari in East Africa.

Within seconds of Wilson's panic alert, the information popped up in Burger’s electronic medical record. Vitberg and the rest of Burger’s medical team happened to be in his room during their daily rounds.

“I think I said, ‘Holy cow, I’ve never seen that before,’ ” Vitberg recalled. He knew that making a diagnosis of African trypanosomiasis in the United States was “probably a once-in-a-medical career experience.” He wanted to make absolutely sure. On the laptop, he immediately pulled up an online medical reference, an industry standard known as UpToDate, and rifled through the sections on the disease.

He asked Burger whether he remembered being bitten by tsetse flies, “and he was immediately able to tell me he was bitten when he was in Africa,” Vitberg recalled.

Burger suspects he was bitten by tsetse flies while walking through game parks. “They’re sizable and they hurt and they tend to land on you and they stay,” Burger said. Once, he had a dozen flies on his back. It was too hot to wear anything but shorts and T-shirts, he said. Insect repellent is not particularly effective against tsetse flies, which can bite through lightweight clothing.

Immediately, the diagnosis shifted from a consideration to near 100 percent confidence.

What was worse, Burger's medical team knew he must have the fast-acting East African form of the disease because of his travel history and because he got sick so quickly after infection.

But no one knew which stage of the disease he had, and the treatments are different — and dangerous.

Within minutes, the hospital’s infectious disease doctor, Alina Sanda, started making calls, including to the CDC’s 24-hour parasitic diseases hotline, to ask for help. The drugs to treat the disease are extremely toxic, and only the CDC has a supply. Sanda, with seven years as an infectious disease doctor, had only learned about sleeping sickness in theory.

“I never took care of a patient with it, so that made me anxious and worried,” she said.

Unable to reach someone at the CDC right away — she left a message on a recording — Sanda contacted an infectious disease colleague at Hopkins for help. Robin McKenzie, an infectious disease specialist, had never treated African trypanosomiasis, either. But she was able to reach an epidemiologist at CDC’s parasitics diseases branch.

It was just before 2 p.m.

'The stakes are very high'

At the CDC, epidemiologist Eugene Liu spoke with both infectious disease experts, consulted with another CDC expert and began putting the wheels in motion for the agency to get the medicine and transport it to Baltimore. His background is also infectious disease, but he had also never cared for a patient with African trypanosomiasis.

“It is very rare, even for the CDC,” he said.

GBMC sent electronic images of the blood smear to Atlanta, and the CDC lab confirmed it showed the lethal parasites.

But now a critical question loomed.

Which medicines to send? The medications differ depending on the form of the disease and how far the infection has spread. Suramin, discovered in 1920, is used to treat patients with the East African form if the parasites are only in blood. But if the parasites have reached the central nervous system, a much more toxic medication is the only treatment. Called melarsoprol, it is basically arsenic.

“Between five and 10 percent of people die from the treatment because it’s so toxic,” said Shapiro, the Hopkins sleeping sickness expert. Untreated, the patients always die. “So the stakes are very high,” she said.

The CDC, which maintains a stockpile of therapeutics to treat rare, serious or life-threatening diseases, decided to send both medicines, just in case. By then, the agency’s regular drug service was closed. Liu had to get the medicines from an emergency supply at the Parasitic Diseases Branch.

He wrapped 10 ampuls of the melarsoprol and five vials of suramin in bubble wrap and put them in a package marked with a red-and-white “emergency medical supply” label. The agency’s Emergency Operations Center arranged for the medicine to be flown on a Delta Air Lines flight leaving Atlanta at 10 p.m. for Baltimore-Washington International Airport. Liu waited outside the agency’s security gate for the courier. There would be no charge for the medicine or the emergency transportation.

The only way to tell whether Burger had parasites in his central nervous system was by performing a spinal tap to collect the clear fluid that circulates in the space surrounding the spine and brain. But there was concern about when to do the spinal tap. If the infection had not yet spread to the central nervous system, injecting a needle into Burger's back could make things worse.

“Often when you do a spinal tap, as you push the needle through the skin and muscle, you drag in red blood cells from those tissues,” Vitberg said. That could introduce parasites from the blood into the spinal column.

By then, it became clear that Hopkins, an academic medical center with more resources and expertise, would be better equipped to treat Burger than GBMC, a community hospital. Burger arrived by ambulance at Hopkins on Friday evening. The medicines from the CDC arrived a few hours later, at 1:30 a.m. Saturday, Dec. 3.

Burger got his first dose of suramin on Saturday, an intravenous treatment that lasted four hours. That allowed the drug to reduce the level of parasites in his blood so doctors could do a spinal tap the next day to check for parasites or evidence of infection in his spinal fluid.

The results showed no parasites and no sign of infection. “That was a big relief for me,” Burger said.

Within days, he was feeling better. On Dec. 8, he was discharged, one week after he showed up in the emergency room.

He still needs one more treatment, on Friday. And doctors must monitor him closely because the drug can be toxic to the kidneys and liver. He has developed an itchy rash on his arms, legs and chest.

But he feels lucky and grateful to the CDC and the personnel at both hospitals, especially to technician Wilson who first spotted the parasite. She, more than anyone, saved his life. He’s thinking of framing the electronic image of the parasite from her blood test, but he may hold off. He still needs to have a spinal tap every six months for the next two years to make sure the parasites aren't lurking in his nervous system. And he doesn’t want to press his luck.

And yet, he would not hesitate to travel to Africa again.

“Really, my answer has always been, of course I'll go back to Africa,” said Burger, who has made several trips, starting when he was 15. “I don't want to go through life too frightened of the risks to actually live and enjoy. I'm not going to stop traveling because a fly bit me.”

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