How the war against rheumatic heart disease was – and wasn't – won

If you grew up in the United States after the middle of the past century, you've probably never thought much about rheumatic fever. And for good reason. In that era, the disease seemed to become a thing of the past.

But in the first part of the 20th century, it was ever-present, untreatable and devastating.

"About 100 years ago, there were more pediatric hospital beds occupied by children with rheumatic fever and rheumatic heart disease than all other infectious diseases combined," said Dr. Stanford T. Shulman, a professor emeritus of pediatrics at Northwestern University Feinberg School of Medicine in Chicago who has written about the history of such diseases. "That just gives you a handle on how really common this illness used to be."

In the 1920s, rheumatic fever was a leading cause of death for people between the ages of 5 and 20. A 1947 medical journal article called it "childhood's greatest enemy."

The fact that it touches few U.S. children today is one of the great scientific successes of the century. But that does not make rheumatic fever a historical footnote.

"It is largely forgotten, but it's certainly not gone," said cardiovascular pathophysiologist Dr. Kathryn A. Taubert, an adjunct professor at UT Southwestern Medical School in Dallas. She co-wrote an editorial about the history of rheumatic fever in Circulation in 2021.

The course of the illness starts with a sore throat from an infection with group A streptococcal bacteria – strep throat. Left untreated, strep can develop into rheumatic fever, said Taubert, who is based in Interlaken, Switzerland, and has spent decades working for and with organizations, including the American Heart Association, to fight the disease.

Children are more at risk than adults. "Partly it's just that they haven't built up all the immunity that they will build up," Taubert said, and partly it's because they're around other children and more likely to be exposed.

The fever follows one to five weeks after the infection, according to the Centers for Disease Control and Prevention. Initially, the fever might be accompanied by obvious symptoms such as achy joints. But silently, if rheumatic fever is not treated quickly, the disease can attack the heart through an overactive immune response, leading to rheumatic heart disease.

Heart valves are frequently affected. Other problems can include a swollen heart muscle (myocarditis), heart rhythm issues (such as atrial fibrillation) or heart failure (where the heart doesn't pump properly).

Every subsequent case of rheumatic fever makes problems worse, Taubert said. But a family might not notice problems until much later.

A 4-year-old who has the illness might not show signs of a problem until they start playing sports in school and struggle to keep up, Taubert said. Girls might not know about heart damage until adulthood and pregnancy, she said, when the strain of giving birth could become dangerous or even fatal.

Doctors started to piece together the links between sore throat, fever and heart problems in the 1880s, said Shulman, who also worked at Northwestern's Ann and Robert H. Lurie Children's Hospital. That institution got its start in 1882 as a memorial to a boy who died of rheumatic fever at 13.

Rheumatic fever played a significant role in the founding and growth of many children's hospitals – and in the development of pediatric cardiology itself, said Shulman.

In Baltimore, the Harriet Lane Home for Invalid Children was opened in 1912 by a couple whose sons had died of rheumatic fever. Linked to Johns Hopkins Medical School, the home cared almost exclusively for children with rheumatic heart disease.

Such centers attracted doctors with an interest in children's hearts. One of them was Dr. Helen Taussig, who started her pediatric work at Harriet Lane and became its director in 1930. Taussig would go on to do pioneering work in children born with congenital heart defects and is known as the mother of pediatric cardiology.

Rheumatic fever also helped drive the establishment of the AHA in 1924. Dr. Paul Dudley White, one of its co-founders, met his wife, social worker Ina Reid, while the two were researching rheumatic fever. At the time, White said the "tremendous crippling of the young people" from rheumatic heart disease was an argument for founding an organization that would lead the fight against it.

Because young people with rheumatic fever often needed months to recover, many cities had convalescent homes for them. Caring for those children was a broad public health concern. Money from the then-new Social Security program was earmarked for their care and for cardiac research in the late 1930s.

The financial burden on a family could be overwhelming – and children suffered in many ways.

"It has been pointed out that many of these children lose the continuity of education and are later handicapped to the extent where they are completely discouraged and give up the attempt of regaining what has been lost," a 1951 article in the Journal of Educational Sociology stated. "Furthermore, it must be admitted that many of these will develop irreversible cardiac damage which will impose great limitations upon their physical abilities."

An article in the American Journal of Nursing in 1945 gives a sense of what children endured. A nurse, the author wrote, "may be able to provide hours of pleasure and lessen the irksomeness of enforced inactivity by enabling her patient to listen to quiet musical programs on the radio or to Victrola records, to watch a plant grow and blossom, or to observe the antics of a small turtle or goldfish in an aquarium." Gradually, the child could move on to more strenuous pursuits, such as "pasting scrapbooks, painting or drawing, modeling in clay, or carving soap."

Nurses were warned that failure on their part could lead to a patient becoming "a permanently willing invalid and a psychological cripple," the article said.

World War II made rheumatic fever an issue of national security, as troops, living in crowded training camps, were afflicted. In its 1953 annual report, the AHA noted that 210,000 young men had been rejected for military duty because of rheumatic heart disease.

Several factors led to the end of the rheumatic fever era in the United States. The big one, Taubert said, was the development of antibiotics, specifically penicillin.

Suddenly, strep throat could be treated. "And if you treat strep throat, it's less likely to develop into rheumatic fever," she said.

But even before penicillin was widely adopted to treat strep in the 1950s, rheumatic fever had been declining. Improved living conditions helped, Taubert said. "Overcrowding is a prime reason for the spread of strep, and therefore the possible development of rheumatic fever."

Other factors might also have been involved, said Shulman, but the bottom line is that "antibiotics were crucial to the effective prevention of rheumatic heart disease. There's no doubt about that."

But the story does not end there.

For people who'd had rheumatic fever, the damage had been done. Famously, Bobby Darin, singer of "Splish Splash" and "Mack the Knife," had rheumatic fever when he was 8 and was told he would not live to age 16; he died during heart surgery in 1973 at age 37. Actor Bill Paxton had rheumatic fever as a 13-year-old and died of a stroke after heart surgery in 2017 at age 61.

Early in her career, Taubert worked at a Veterans Administration hospital that routinely saw World War II-era patients who'd had rheumatic fever and were suffering from related heart problems more than 30 years later.

And in parts of the world, the disease never went away.

"It is a sad reality that although eminently preventable … rheumatic heart disease remains the most common cause of cardiovascular morbidity and early mortality in young people worldwide," according to a 2023 review article in the Journal of the American College of Cardiology.

AHA statistics show that rheumatic fever or rheumatic heart disease were responsible for more than 3,900 deaths in the U.S. in 2021, with the highest rates clustered in Alaska, Mississippi, Alabama, Kentucky and Utah. Worldwide that year, rheumatic heart disease killed an estimated 380,000 people. The highest rates were in South Asia and Oceania.

Poverty is a factor, Taubert said, as are "lack of money to do the research, lack of money to have adequate supplies of penicillin, lack of money to be able to do echocardiograms (ultrasounds of the heart)."

Progress is being made toward a strep vaccine, Taubert said. "That would clearly be a huge step forward. Huge."

So while parts of the century-long story of rheumatic heart disease can be celebrated, that's not the case everywhere. "Because you wouldn't have 55 million people walking around with it if it had been a complete success," Taubert said.

Shulman agreed. "There are groups around the world that have begun to make some very significant inroads into this," he said. "But they still have a long way to go. A long way to go."