Vintage medical textbooks are filled with diagnoses unfamiliar to contemporary doctors – wandering wombs, blackwater fever, biliousness and other historic curios. Acute rheumatic fever has become one of these anachronisms in developed countries but Australia has a shameful secret.
Improvements in living standards, reduction in overcrowding and access to health care have made the acute rheumatic fever rare in most of the country. Yet, in low-resource settings, including in Aboriginal and Torres Strait Island communities, the illness remains painfully real.
How it works
Acute rheumatic fever is an abnormal immune response to throat and skin infections from the bacteria group A streptococcus. Usually, the bacteria are harmless colonisers of the nose and mouth, but an active infection causes people develop a “strep throat”.
This prompts the body’s immune system to respond. In most cases, the immune response is appropriately targeted to kill bacteria and the infection resolves.
In some cases, the body mistakenly targets normal tissues in the body, including the heart, skin and joints. This causes the joint pain and fevers characteristic of acute rheumatic fever.
Approximately 3% of children are susceptible to acute rheumatic fever after a strep throat infection. The determinants of this susceptibility are a combination of environmental factors (overcrowding, for instance, or inadequate housing), genetics, and bacterial characteristics (including the subtype of the bacteria). The way these factors interact to cause disease remains unclear.
Young people who develop the illness experience symptoms, such as fevers, painful joints, skin and movement changes, two or three weeks after infection. Most will also experience inflammation of the heart membrane, muscle and valves, which is known as carditis.
The fever and joint pains that typify acute rheumatic fever tend to resolve over a period of weeks, but damage to the heart valves generally persists.
Those who’ve had one episode of the illness are much more likely to have recurrent episodes because of factors that made them susceptible in the first place. Each recurrence causes further heart damage.
Eventually, the valves of the heart become scarred. This chronic phase of the disease is called rheumatic heart disease. Over time, it increases the risk of heart rhythm disturbances, stroke and heart valve infections, and culminates in heart failure.
Once common worldwide, both illnesses have declined due to economic development and improved access to health care, antibiotics and treatment of the causal “strep throat”.
Today, more than 80% of people living with rheumatic heart disease worldwide are from low and middle-income countries, reflecting poverty, overcrowding, under-nutrition and limited access to health care.
Rheumatic fever and rheumatic heart disease are also endemic in Australia’s Indigenous communities. Indigenous Australians are 122 times more likely to live with rheumatic heart disease than their non-Indigenous peers.
Statistics too readily sanitise the human reality of rheumatic heart disease, which contributes tremendous personal, social, economic and community harm to young people in the prime of life. In the Kimberly, the average age of death from the illness is 41 years.
The greatest tragedy is that it’s possible to reduce the burden of rheumatic heart disease and control the development of new cases.
Preventing the first bout of the illness requires timely diagnosis of strep throat infections and prompt delivery of antibiotics. The goal is to prevent the abnormal immune response that causes acute rheumatic fever.
Antibiotics can prevent almost all cases but require medical evaluation of all sore throats. The problem is that in Indigenous Australian populations, sore throats are comparatively rarely seen to by doctors. The fact that sore throats may be mild makes it difficult to diagnose and treat infections.
People who have a demonstrated susceptibility to acute rheumatic fever require regular antibiotics, with the goal of preventing new strep throat infections. Young people who’ve had the disease, or who are known to have rheumatic heart disease, need penicillin for at least a decade – until the highest risk of recurrence has passed.
The most reliable form of penicillin for this purpose is a long-acting injection delivered into muscle every four weeks. But the health system struggles to contact people in remote settings who move frequently or don’t have reliable telecommunications to remind them about injections.
Sending a nurse to give the injection or arranging for people to come to the clinic can be complicated. And, the injections are painful, inconvenient and frequent for people living with rheumatic heart disease.
Although there are early signs of progress in disease control, both illnesses remain persistent and present an indefensible disparity between Indigenous and non-Indigenous Australians.
The functional disappearance of acute rheumatic fever is a triumph, but the continued burden of disease in Indigenous Australia precludes celebration. Greater and better targeted efforts are needed to make acute rheumatic fever and rheumatic heart disease anachronisms across all of Australia.