Florence Ndimubakunzi during a screening for rheumatic heart disease in Kigali, Rwanda Image Credit: NYT

Neighbours whisper that she is pregnant, a disgrace for a young, unmarried woman. The rumours 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 wealthy 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.

Elina Mukagasigwa during a screening for rheumatic heart disease at the University Teaching Hospital of Kigali in Rwanda. NYT

Worldwide, 33.4 million people had rheumatic heart disease in 2015, and at least 319,400 died from it, according to estimates published in 2017 — 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 35 kg.

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,” 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,” Come said. Would her big belly go away? Florence asked. A drug, Lasix, that helps rid the body of excess fluid might help, Come said.

‘Entry Point Is Heart Failure’

Experts say programmes 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 Organisation, calls rheumatic fever and the heart damage it causes “neglected diseases of marginalised 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 younger than 25 by 2025. The group also called for a strep vaccine.

Innocent Nsabimana, 16, with two other patients before their heart valve replacement surgeries at King Faisal Hospital in Kigali. NYT

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 HIV — 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 paediatric 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.

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 travelling 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, 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, anaesthesiologists, experts in cardiac ultrasound, biomedical technicians, pharmacists, support staff and perfusionists who run the heart-lung machine that keeps patients alive during surgery.

Well Enough to Survive Surgery

A week before the operations were to begin, at the King Faisal Hospital in Kigali, Team Heart members visited other medical centres to screen patients. 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,” Come said. “Then this year, he was too late.”

One year, a patient was turned down because she was pregnant, 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,” 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 haemorrhaging in the mother, and birth defects.

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 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.

Window Is Closing for Patients

At another screening site, Dr Patrick Hohl, a cardiologist from Portland, Maine, 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 he 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 haemorrhage during surgery. Rwanda does not have an extensive supply of blood for transfusions.

Even so, 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, 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, Hohl told the team: “This is the window. It’s closing. If he lives till next year, it might be too late.”

Many Candidates, 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 Bolman, three other heart surgeons, and several cardiologists and anaesthesiologists. 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 agonised over some patients, particularly Gaudence, a 14-year-old girl who weighed just 29 kg — barely big enough for the available valves. She was high-risk, but Come, 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 onto others. Innocent was chosen. 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,” 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.

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, 8 feet-by-8 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 neighbours.

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 a car and sped off to the hospital.

A Short Operation Is the Key

After three days of treatment with drugs to stabilise 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. Bolman, not scheduled to operate, had scrubbed in anyway, to help speed the surgery and minimise her time under anaesthesia.

“The key on this lady is a short operation,” Leavitt said.

Seeing that Elina was still short of breath, the anaesthesiologist, 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, Muehlschlegel said: “She’s teetering. She’s not in a good state. There’s a fine line between too risky and nick of time.”

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 have moved to a larger home in Kigali, with two rooms.

Elina now has a job in Kigali from 6.30 am to 5pm, 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.

A Cardiac Centre for Rwanda?

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 per cent 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,” 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,” 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 rheumatic heart disease was a real concern of the government and that programmes to treat strep throat and rheumatic fever were essential. But for advanced cases, “surgery is the solution,” she said. “We definitely need this centre.”

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.

–New York Times News Service