BURDEN OF DISEASE

Acute rheumatic fever and rheumatic heart disease were once common across the world, including in Australia, Europe, Great Britain, and North America. Rates of disease declined in many countries during the mid-20th century, primarily due to improved socioeconomic conditions, better access to healthcare, and the wide use of penicillin to treat streptococcal (Strep A) infections. In Australia, rheumatic fever and rheumatic heart disease continue to cause significant ill-health and premature death at high rates among First Nations peoples.

CULTURE AT THE CENTRE OF CARE

Many First Nations peoples have a holistic view of health that does not align with the western biomedical model of healthcare. For these people health is not just the physical wellbeing of an individual, but refers to the social, emotional, and cultural wellbeing of the whole community. Cultural and structural competencies in healthcare are necessary to close the evidence-practice gap.

PRIMORDIAL PREVENTION

Primordial prevention of rheumatic fever aims to reduce the opportunity for Strep A infections and prevent initial episodes of rheumatic fever. Historically, healthy housing and environments, access to adequate sanitation and washing facilities, and access to timely healthcare have been associated with reducing rheumatic fever and rheumatic heart disease in the population.

PRIMARY PREVENTION

Primary prevention of rheumatic fever aims to interrupt the link between Strep A infection and the abnormal immune response to the infection (which is rheumatic fever). Primary prevention focuses on identification and treatment of Strep A skin and throat infections. Recommended prevention (treatment) is either one intramuscular benzathine benzylpenicillin injection or a course of antibiotic tablets or syrup.

DIAGNOSIS AND MANAGEMENT OF ACUTE RHEUMATIC FEVER

Acute rheumatic fever is an abnormal inflammatory response which can occur following an untreated Strep A infection. It is a self-limiting illness that can affect the joints, skin, heart and brain. Involvement of the heart during rheumatic fever is called carditis. There is no single diagnostic test available. Correct diagnosis is based on clinical assessment of the symptoms and signs. Severity of symptoms range from very mild, to severe where the person may be bed-bound due to joint pain or heart failure. Rheumatic fever does not present the same way for everyone, and symptoms do not necessarily all occur together or may be subtle. A smartphone Application can support clinicians with rheumatic fever diagnosis in the Australian context. Strep A infections and rheumatic fever are most common in children and adolescents.

Medical management is based on confirming the diagnosis, treating the Strep A infection, determining whether the heart is involved using an echocardiogram (ultrasound of the heart) and relieving symptoms. Hospital care includes close monitoring, rest, pain management, culturally appropriate education, and developing a plan for longer-term care.

DIAGNOSIS AND MANAGEMENT OF RHEUMATIC HEART DISEASE

Rheumatic heart disease is damage to the structure and function of one or more heart valves which can occur following acute rheumatic fever carditis. The valves remain stiff and scarred, and do not open properly or do not close properly. This interrupts normal blood flow through the heart; however, symptoms may not be noticed for many years. Complications of rheumatic heart disease cause progressive disability, reduce quality of life, and can lead to premature death in young adults. Many people have one or more heart murmurs which can be heard through a stethoscope. Rheumatic heart disease is diagnosed using an echocardiogram (ultrasound) machine.

Medical management focuses on relieving symptoms and preventing or reducing the impact of complications. Heart valve surgery can help manage symptoms and improve length and quality of life but does not provide a cure.

SECONDARY PREVENTION OF ACUTE RHEUMATIC FEVER

People living with acute rheumatic fever and rheumatic heart disease generally require regular, long-term antibiotic prophylaxis to prevent further Strep A infections and recurrent rheumatic fever. Antibiotics are most effectively delivered as intramuscular benzathine benzylpenicillin injections at least every 28 days during the period of highest risk, which for most people continues into adulthood. Tablet alternatives are available for people with a documented penicillin allergy, although this may be less effective in preventing rheumatic fever and requires careful monitoring. The duration of treatment depends on several factors including age at diagnosis, recurrent rheumatic fever illnesses, severity of rheumatic heart disease if it is present, and the ongoing risk of further Strep A infections. An echocardiogram and medical specialist review are required before treatment can be ceased.

Receiving and providing injections and other medical care over many years can be difficult for both patients and health services. Health services can support people who experience difficulties with injections by providing competent and patient-centred care and using strategies to minimise injection pain and distress.

SCREENING FOR RHEUMATIC HEART DISEASE

Echocardiographic screening to detect rheumatic heart disease in high-risk populations has been widely used in Australia and internationally. Screening procedures have evolved over time, using different technologies and involving operators with varying levels of expertise. Population-based screening using a stethoscope to identify heart murmurs is not accurate for detecting rheumatic heart disease.

WOMEN AND GIRLS WITH RHEUMATIC HEART DISEASE

Rhematic heart disease is twice as common in women as in men. In Australia, First Nations women, Māori and other Pacific Islander women, and women from other countries with high rates of disease are at high risk for rheumatic heart disease. Medical and social care includes managing rheumatic fever and rheumatic heart disease within the context of reproduction. Preparing for heart valve surgery and timing of pregnancies should start early for young women who are diagnosed with rheumatic fever and rheumatic heart disease.

More information is available in the Australian guideline for prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease (3rd edition).