Where a Sore Throat Becomes a Death SentenceSeptember 16, 2018
CreditCreditAndrew Renneisen for The New York Times
KIGALI, Rwanda — Neighbors whisper that she is pregnant, a disgrace for a young, unmarried woman. The rumors mortify her. She hates her swollen belly.
But Florence Ndimubakunzi is not pregnant. Her heart is failing. It pumps so poorly that blood backs up in her veins, bloating her liver and spleen, and filling her abdomen with fluid. She is only 18.
For millions like her in poorer parts of Africa, Asia and other regions, this devastating heart disease began insidiously. During childhood, they contracted strep throat — an infection caused by streptococcal bacteria.
In the United States and other rich countries, children with sore throats are routinely tested for strep and quickly cured with penicillin or other cheap antibiotics.
But in poor countries, strep throat often goes undiagnosed and can become a long, slow death sentence. Without treatment, it can lead to rheumatic fever and rheumatic heart disease, in which the immune system attacks the heart valves — intricate flaps of tissue that must open and shut properly 100,000 times a day for the heart to work normally.
As the valves deteriorate, the heart struggles and gradually wears out. Patients become weak, short of breath and unable to attend school or work. Many die before they reach 30. Women with the illness who become pregnant can suffer severe and sometimes fatal complications.
Worldwide, 33.4 million people had rheumatic heart disease in 2015, and at least 319,400 died from it, according to estimates published last year — a public health disaster caused by a preventable disease that has been largely wiped out in the United States and Western Europe.
Earlier this year, hoping to beat the odds, Florence and her mother consulted doctors from a humanitarian group, Team Heart, that flies in from the United States and Canada once a year to perform valve-replacement surgery.
About 100 people showed up to be screened for the lifesaving operation. The team could operate on only 16.
Lying on an examining table, eyes huge in her gaunt face, Florence looked impossibly fragile, her arms thin as broomsticks, her shoulders jutting up like a skeleton’s. She had wasted away to 78 pounds; five were fluid.
Dr. Pat Come, a Harvard cardiologist, pressed a stethoscope to Florence’s chest, back and neck, and palpated her belly. A sonographer, Marilyn Riley, from Beth Israel Deaconess Hospital in Boston, ran an ultrasound probe over Florence’s chest, showing her heart valves in motion and measuring the pressure gradients across them, the blood flow through her heart and the size of its chambers.
“She has significant disease of two valves,” Dr. Come finally said. “But the operative mortality is likely too high. Medical therapy is the best option.”
A translator explained in Kinyarwanda that Florence was too sick for surgery. Florence asked if the medicines would cure her. No, but they could keep her “on an even keel,” Dr. Come said. Would her big belly go away? Florence asked. A drug, Lasix, that helps rid the body of excess fluid might help, Dr. Come said.
As Florence sat up, buttoning her dress, Ms. Riley said, “She’s so tiny I’m afraid she’ll fracture getting off the table.”
A nurse helped her down.
Outside the examining room, Florence said she’d had to stop attending school because she was too weak to get there. She was eager to go back. She hoped to be a pediatrician.
She has been ill since she was about 8. Doctors warned that only surgery could cure her. Now, they were saying it was impossible.
“It’s a disappointment,” Florence said.
‘The entry point is heart failure’
Experts say programs to educate people about sore throats and strep, and to distribute penicillin widely to local clinics, could help greatly to prevent rheumatic heart disease in poor regions. But even those efforts probably would not wipe it out completely, because not everyone with strep seeks medical attention.
The World Heart Federation, which works with the World Health Organization, calls rheumatic fever and the heart damage it causes “neglected diseases of marginalized communities.” Poverty, crowded living conditions and lack of medical care create breeding grounds for strep.
In 2013, the federation set a goal of decreasing deaths from the condition by 25 percent in people under 25 by 2025. The group also called for a strep vaccine.
Little is spent on studying the illness, less than $1 million globally in 2013. From 500 to 1,000 times as much research money is spent on tuberculosis, malaria and H.I.V. — which each kill three to five times as many people as strep, according to an editorial in The New England Journal of Medicine.
If rheumatic fever is detected early, long-term treatment with penicillin can prevent valve damage. But many cases are already advanced.
“Unfortunately, the entry point is heart failure,” said Dr. Joseph Mucumbitsi, a pediatric cardiologist at the King Faisal Hospital in Kigali, and a consultant to Team Heart. “We have many rheumatic heart disease patients below 17. We have some as young as 5.”
He estimated that there might be as many as 20,000 people with advanced disease who need surgery.
Though prevention would be ideal, the rapid tests widely used in the United States to diagnose strep are too expensive in Rwanda, and throat cultures are not widely available.
Some doctors advocate skipping the tests and just giving penicillin shots to all children with sore throats. Others worry that antibiotic resistance and penicillin allergies would result.
The genocide that killed a million people in Rwanda in 1994 also destroyed its health care system, and the country has had to rebuild it. H.I.V., malaria, tuberculosis and rotavirus have been major priorities — not heart disease.
Rwanda has only five cardiologists and no heart surgeons or hospitals equipped to perform heart surgery — for a population of 12 million.
Since 2008, Team Heart has been traveling to Rwanda. The group was founded by Cecilia Patton-Bolman, an intensive-care nurse, who had seen a ward full of teenagers dying from rheumatic heart disease when she visited the country in 2006, and her husband, Dr. R. Morton Bolman III, who was the chief of cardiac surgery at Brigham and Women’s Hospital in Boston. (He later moved to the University of Vermont, and recently retired.)
Once a year, 40 to 60 volunteers fly to Kigali: heart surgeons, cardiologists, nurses, anesthesiologists, experts in cardiac ultrasound, biomedical technicians, pharmacists, support staff and perfusionists who run the heart-lung machine that keeps patients alive during surgery.
They come from the University of Vermont, Harvard-affiliated hospitals and other medical centers. They pay their own airfare, and Rwanda’s Ministry of Health covers some hotel rooms and meals. Most use their vacation time, and this year Team Heart asked each volunteer also to raise $500 in donations to help cover costs.
The doctors and nurses became so close to the patients that they routinely referred to them by first names in their private interactions and group deliberations, a practice reflected in the first-name references to the patients in this article.
Sick enough to need surgery — but well enough to survive it
A week before the operations were to begin, at the King Faisal Hospital in Kigali, Team Heart members visited other medical centers to screen patients.
Ms. Riley, the sonographer, said, “The first year I came, it felt like ‘Who gets the life jacket in a sinking boat?’”
The ideal candidates are sick enough to die in a year without replacement valves — but well enough to survive the operation. The screeners also rule out those who seem strong enough to wait another year.
“Last year, we deferred someone who was too early,” Dr. Come said. “Then this year, he was too late.”
One year, a patient was turned down because she was pregnant, Dr. Come said. She returned a few days later — after an abortion. She underwent the surgery and did well.
Elina Mukagasigwa, 26, a tiny woman who gasped for air if she tried to walk uphill, and who coughed up blood in her sleep, was among the many hoping for help.
Dr. Samvit Tandan, a cardiologist from the University of Vermont, told Elina through a translator that she had a diseased mitral valve, which controls blood flow between the chambers on the left side of the heart.
“We can fix the mitral valve,” Dr. Tandan said. Elina gave him a wry grin and clapped softly.
But then he explained that the surgery would make it dangerous for her to become pregnant, so she should plan on never having children.
Pregnancy is discouraged because people with mechanical valves need lifelong treatment with the drug warfarin to prevent clots from forming on the valves. The drug can cause hemorrhaging in the mother, and birth defects.
“Do I have to have the surgery?” Elina asked.
“That’s your decision, but the valve will not get better on its own,” Dr. Tandan said.
“It’s not possible to have a baby?” she asked.
“It’s possible, but there is very high risk,” Dr. Tandan said, and explained it again.
She thought it over, and said, “The good decision is to not think about babies, so I can save my life a little bit longer.”
“It is more than a little bit longer,” Dr. Tandan said.
Replacement valves made from cow tissue do not require warfarin, but Team Heart rarely uses them, because they wear out quickly in young people. Mechanical valves can last for decades.
The prospect of not having children has led some patients to decline surgery. In other cases, women have said they would not conceive and then had babies anyway. How they survived is not clear — some combination of luck and good care by their local doctors seems to be the best guess.
After Dr. Tandan left, Elina said she had hoped to have a child and, “until a few minutes ago,” to marry her boyfriend. But he would leave her, she said, if she could not have children.
The window is closing for these patients
At another screening site, Dr. Patrick Hohl, a cardiologist from Portland, Me., examined Innocent Nsabimana, 16, a quiet boy with a shy smile. His face, legs and chest had swelled, his eyes had turned yellow, he had developed a cough and could no longer ride his bike. Sick for a year, Innocent was taking five heart medications. He tried to hide his illness from friends, fearing they would reject him.
Two of his heart valves turned out to be severely damaged. And his liver was slightly enlarged — a warning sign, because the liver helps control blood-clotting, and liver trouble increases the risk of hemorrhage during surgery. Rwanda does not have an extensive supply of blood for transfusions.
Even so, Dr. Hohl thought Innocent was a good candidate.
“I’ll advocate heavily for him,” he told Innocent’s uncle, Tuyisenge Chan Kamoso, 30, a graphic designer.
Turning to Innocent, Dr. Hohl said: “It’s a big surgery, but our hope would be that you’d get much better. Your breathing would improve, you’d get your appetite back. We have to review your case with our head surgeons. What do you think about all this?”
“No problem,” Innocent said.
Later, Dr. Hohl told the team: “This is the window. It’s closing. If he lives till next year, it might be too late.”
So many candidates for too few slots
Once the screening was completed, the team of about 60 people gathered in a hospital classroom to select those who would get the surgery. They had 39 good candidates — for 16 spots.
In the first row were the cardiologists, nurses and sonographers who had examined the patients and drawn up a spreadsheet, with the best cases first.
In the second row were Dr. Bolman, three other heart surgeons, and several cardiologists and anesthesiologists. The surgeons would ultimately say yes or no, after viewing PowerPoint data on each patient and clips from the echocardiogram. The setup resembled “America’s Got Talent” — except that these choices would mean life or death.
The group agonized over some patients, particularly Gaudence, a 14-year-old girl who weighed just 66 pounds — barely big enough for the available valves. She was high-risk, but Dr. Come, Ms. Riley and Julie Carragher, a cardiology nurse practitioner, made a special appeal.
Eyeing her test results, Dr. Bruce Leavitt, a surgeon from the University of Vermont, said, “She seems very on the verge of being too ill for what we do here.”
Reluctantly, the surgeons agreed to examine Gaudence.
They moved on to others. Innocent was chosen. Dr. Tandan urged his colleagues to consider Elina, whose name was much farther down the list.
By the time they got to her, all 16 slots were filled. “I think she needs to be on the list,” Dr. Bolman said. “I think she’s sicker than we thought.”
Coughing up blood indicated advanced disease; Elina might not be able to wait a year, he cautioned.
Another patient was made an alternate, giving Elina one of the last slots.
The meeting ended on a mix of emotions: relief and happiness for patients who made the list; anguish and frustration for the rest.
“I’m sad,” Dr. Mucumbitsi said about a patient who was rejected.
“Last year he wasn’t sick enough,” Dr. Mucumbitsi said. “This year he’s too sick. That’s not fair. I always say that when they come, they should start with the patients they left behind. You cannot operate only on simple cases.”
Dr. Come and Ms. Carragher went straight to the hospital to examine Gaudence yet again, with the surgeons. Dr. Come, a grandmother, said she had lain awake the night before, thinking of the girl. Gaudence was slim and bright-eyed, with closely shaved hair, a bright red hoodie and a hacking cough. The doctors listened to her heart and lungs, and walked her up a flight of steps with an oxygen monitor on her finger, as her mother watched every move, desperation plain in her face.
Ultimately, the surgeons said no. She was too ill.
Later that day, she sat with a young girl who was accepted for surgery, reading a booklet about the operation.
Gaudence would be one of the patients sent home with some medicines.
One life lost, another saved
Months earlier, Elina’s sister Charlotte, a security guard in Kigali, had moved her from the countryside to the city to get her medical help.
They shared Charlotte’s home — one room, eight feet by eight feet, with rough concrete walls, a tin roof and a dirt floor. It was crammed into a warren of dwellings on the side of a hill, down a steep flight of uneven stone steps from the road. The sisters slept together in a bed that took up most of the room. They cooked outside, and shared an outdoor water faucet and toilets with neighbors.
Three days after Elina’s screening, as I was interviewing them at home, Charlotte’s phone rang. Elina’s operation was being moved up, because another patient had become too ill for surgery. Elina threw some things into a bag, and she, Charlotte, the photographer, our driver, translator and I all squeezed into the car hired by The New York Times and sped off to the hospital.
There, Elina waded through a seemingly endless admission process. Doctors had gone home. The X-ray department was closed. A clerk forgot to order a test. A form was missing. The admitting nurses were busy. Go here, go there, go back again. Wait.
After almost four hours of dragging herself around the hospital — barely able to catch her breath — Elina joined three other Team Heart patients in a room. She changed into a pair of blue hospital pajamas and sank into bed.
The next day, Elina was worse — feverish and teary, her heart racing. During the night, she’d had trouble breathing and began coughing up blood. At the same time she’d begun gasping for air, the patient whose place she took, Emmanuel, 22, was dying in the ward next door. The nurses were spread thin. The mother of another patient, seeing Elina’s distress, had summoned them and called Charlotte back to the hospital.
Dr. Tandan said Elina might now be too sick for surgery. The fever could signal an infection. Worse, she had missed a week’s worth of her heart medications, which had run out ahead of her scheduled hospital appointment.
For someone with heart failure, skipping just a pill or two can bring on a crisis. Elina had gained six pounds of fluid, which was backing up into her lungs. The team poured medications into her, but knew they might have to scratch her off the list.
Emmanuel’s misfortune had probably saved her life. Had she not been called to the hospital ahead of schedule, she could easily have died at home that night.
Is the aftercare sustainable?
One surgeon who joined Team Heart for the first time, Dr. Thoralf M. Sundt III, the chief of cardiac surgery at Massachusetts General Hospital, had concerns about the program.
“It raises more questions than it answers,” he said. “We can create problems for governments. We create a pool of patients with big needs. Is this sustainable?”
Patients with mechanical heart valves have a permanent need for regular lab tests and the blood-thinning drug warfarin. A recent study found that rural district hospitals in Rwanda were providing good follow-up care. Ms. Patton-Bolman said, however, that she had struggled to obtain the drug for past surgical patients, who have sometimes gone perilous weeks without it.
But Dr. Sundt also acknowledged that with Team Heart’s program, “Lives are saved.”
A short operation is the key
After three days of treatment with drugs to stabilize her heart and dry out her lungs, Elina was wheeled into the operating room.
She had requested Christian music, so “Amazing Grace” played softly on one of the doctor’s phones.
Dr. Bolman, not scheduled to operate, had scrubbed in anyway, to help speed the surgery and minimize her time under anesthesia.
“The key on this lady is a short operation,” Dr. Leavitt said.
Seeing that Elina was still short of breath, the anesthesiologist, Dr. Jochen D. Muehlschlegel, from Brigham and Women’s Hospital in Boston, kept the head of the operating table elevated while she was prepared for surgery. As she was being sedated, the playlist switched to “Rescue Me.”
Once Elina was unconscious, Dr. Muehlschlegel said: “She’s teetering. She’s not in a good state. There’s a fine line between too risky and nick of time.”
He and the surgeons watched her heartbeat on an echocardiogram monitor. Her diseased mitral valve was limiting the flow of blood to her left ventricle, which is normally the heart’s biggest, strongest pumping chamber. As a result, it had shrunk, and the chamber above it — the left atrium — had become massive. The right side of her heart was also abnormally large. But her left ventricle was pumping well — a good sign.
The surgeons cut open her chest, sawed through her breastbone and cranked open her rib cage with a retractor.
“Her heart is huge,” one said.
The next step required diverting Elina’s circulation through a heart-lung machine, which would pump and oxygenate her blood, so that the surgeons could stop her heart, slice it open, cut out the diseased mitral valve and sew in a mechanical one.
“This is the worst mitral valve I’ve seen,” Dr. Leavitt said as he pulled it out of her chest and dropped it into a steel basin. What should have been smooth and translucent appeared white, crusty and malevolent looking, with spiky, calcified cusps. The opening for blood flow was a small fraction of what it should have been.
Minutes later, Dr. Leavitt had sewn in a new, mechanical valve. On the echocardiogram, it opened and closed briskly, letting the blood flow freely.
“We love to see that,” Dr. Bolman said.
Her surgery went quickly, as the doctors had hoped, lasting about three hours.
The doctors predicted a rocky recovery. Elina regained strength slowly, needed oxygen for nearly a week, longer than most patients, and appeared pained and discouraged. Two weeks after surgery, she went home with Charlotte.
But the real test of the surgery, and of the very idea of providing it in a place like Rwanda, comes months down the road. Are patients better than they were before the operation? Can they live independently, work, go to school, enjoy life?
Five months after her surgery, Elina said in an interview: “I’m happier and healthier. I’m now enjoying the life that I almost missed.” A few months ago, she and Charlotte returned to their home village for a feast with family and friends, to celebrate Elina’s “regaining of life,” Charlotte said. And they’ve moved to a larger home in Kigali, with two rooms.
Elina now has a job in Kigali from 6:30 a.m. to 5 p.m., at the company where Charlotte works. She cleans the office and makes tea and coffee for the staff. She hopes to gain experience and move on to better jobs, to help Charlotte and their mother.
Innocent has been less fortunate. He returned to school shortly after the surgery, earned high grades and even resumed playing soccer. But he spent much of August in the hospital, with fever and chest pains — and no clear diagnosis. “I don’t know what to do,” his uncle said.
Florence’s health has continued to decline.
Will Rwanda get a cardiac center of its own?
Since it began working in Rwanda, Team Heart has operated on 165 patients. An article in a medical journal earlier this year, based on the first 149 cases, reported a survival rate of 95 percent in the first month after surgery.
The Bolmans said it was always their intention not just to fly in once a year, but also to train Rwandan doctors and nurses, and ultimately to build a cardiac hospital.
“We envision putting ourselves out of business,” Ms. Patton-Bolman said.
“We thought that in seven to 10 years, we’d have a hospital staffed by Rwandans, and we’d turn the keys over,” Dr. Bolman said. “We thought we could start a prevention project in a few years. But it’s more like a generation.”
He estimated that it would cost $10 million to build a hospital and $10 million a year to run it.
Rwanda’s minister of health, Dr. Diane Gashumba, said in an interview that rheumatic heart disease was a real concern of the government and that programs to treat strep throat and rheumatic fever were essential.
But for advanced cases, “surgery is the solution,” she said. “We definitely need this center.”
The team has lined up prospective donors. But despite years of discussion, the government has not been able to commit to paying for patient care.
In recent months, the Bolmans have reined in their ambitions. Instead of building a new hospital, they now hope to create a cardiac center at the King Faisal Hospital, which is owned by an Angola-based company, Oshen.
The center could perform all types of heart surgery, not just valve replacement, on about 100 adults and 30 to 50 children a year, Dr. Bolman said. The government would cover the poorest patients, and the profit from insured or paying patients would subsidize those who did not quality for government help but still could not pay, he said.
“We’re scaling back, but not giving up,” Dr. Bolman said. “We’re very excited.”
The team expects to make its annual surgery trip next year.
Hyppolite Ntigurirwa contributed reporting from Kigali, Rwanda to this article.
Denise Grady has been a science reporter for The Times since 1998. She wrote “Deadly Invaders,” a book about emerging viruses. @nytDeniseGrady