Improving care for acute rheumatic fever and rheumatic heart disease

Acute rheumatic fever and rheumatic heart disease

Acute rheumatic fever (ARF) and rheumatic heart disease (RHD) are preventable conditions associated globally with poverty, crowded housing and poor living conditions.1 ARF is caused by an autoimmune response to infection with streptococcal bacteria that are often found in skin sores and sore throats. It can cause inflammation of the heart, often resulting in permanent damage to the heart valves, usually because of recurrences. RHD is the long-term condition caused by permanent damage to one or more of the heart valves following ARF. The heart valve or valves may become narrowed, obstructing the flow of blood or failing to close properly or both. This can lead to heart failure and the need for cardiac surgery, or stroke, and often leads to premature death.

RHD is a serious public health problem in low- and middle-income countries and among marginalised communities in high-income countries, including Indigenous populations. In 2018, some 30 million people were thought to be affected by RHD globally. RHD disproportionately affects girls and women; where endemic, it is a significant cause of poor maternal health outcomes.2

People affected by ARF and RHD need to have ongoing active engagement with the healthcare system for many years. This means the role of PHC practitioners in the prevention, screening, diagnosis, management and referral for ARF and RHD is crucial. Where RHD control programs are in place, practitioners may also be responsible for ARF and RHD notifications.

ARF/RHD and recommended care for Aboriginal and Torres Strait Islander peoples

RHD data show one of the greatest inequities in disease rates between Indigenous and non-Indigenous Australians, with rates of ARF and RHD in some Aboriginal and Torres Strait Islander communities among the highest documented globally.3 A recent study found the age standardised ARF incidence (for people younger than 45 years) was 124 times higher and RHD prevalence (for those younger than 55 years) was 61 times higher among Aboriginal and Torres Strait Islander people compared with non-Indigenous Australians, with substantially higher rates in northern, remote Australian regions.4 The number and rate of ARF notifications increased between 2016 and 2022, and almost half (46 per cent) of diagnoses were children aged 5 to 14 years.5 These data provide compelling evidence of the need to improve living environments, disease awareness and management.

Recommended prevention and treatment

ARF can be prevented through improving living conditions and prompt antibiotic treatment for streptococcal infections. The recommended treatment of ARF and RHD is as follows:

• People diagnosed with ARF or RHD are assigned a RHD priority classification (1–4) based on disease severity. The classification guides recommended disease management.
• People who have had ARF need benzathine benzylpenicillin G injections every 4 weeks for a minimum of 5 years after the last episode or until age 21 (whichever occurs later), to avoid recurrences and prevent RHD progression.
• Continued secondary prevention (prophylaxis) to age 35 is recommended for moderate RHD and to age 40 or lifelong for severe cases, especially in people requiring heart valve surgery.
• Administration of more than 80 per cent of a client’s prescribed benzathine benzylpenicillin G injections substantially reduces the risk of ARF recurrence, thereby reducing the risk of early death from RHD.6

Comprehensive PHC advice, resources and clinical guidelines are available on the RHD Australia website.7 The World Heart Federation guidelines for diagnosis of RHD were updated in 2023.8

Findings: quality of acute rheumatic fever and rheumatic heart disease prevention and care

The data presented below on the quality of ARF/RHD care come mainly from six research papers and reports from the ABCD research program. The studies collectively analysed 5,209 client records from PHC centres, which were audited between 2008 and 2014. Most PHC centres were in geographically remote locations in northern Australia. Stakeholders participated in data interpretation to identify priority evidence-to-practice gaps and factors influencing improvement. Information on the application of CQI for ARF and RHD care and on clinical audit processes and audit findings are reported elsewhere.9 We provide a brief overview of key findings below.

Across the studies, patterns and priorities for improvement could be identified.

• Secondary prevention: the majority of clients did not receive levels of secondary prevention known to offer best protection from ARF recurrence. From 2008 to 2014, the proportion of clients receiving more than 80 per cent of their scheduled benzathine benzylpenicillin G injections did not improve. PHC centres with high scores for “systematic processes of follow-up” were significantly better at administering the injections at recommended levels.10
• Management plans and education: some 70 to 80 per cent of clients had a disease management plan in place. However, clients’ RHD priority classification, the provision of education about secondary prevention and follow-up action plans for clients with low rates of benzathine benzylpenicillin G injections were not consistently recorded. Only 12 per cent of clients with poor adherence to benzathine benzylpenicillin G injections had action plans. Provision of health education appeared low, even among people younger than 25 years, for whom secondary prevention has the most potential to improve outcomes. These findings indicate a need for better systems to translate secondary prevention guidelines into quality care.11
• Overall delivery of ARF/RHD prevention and care: there were improvements in overall service delivery. PHC centre mean delivery of care went from 42 per cent in 2008 to 66 per cent in 2012, but the upward trend was not sustained. There was large variation in service delivery between health centres, and this variation did not reduce over time, despite sustained CQI activities12 (see Figure 17.1)
• Client age: children younger than 15 years received a higher level of ARF/RHD care compared with adults in all age groups. Most clients younger than 25 years (88.5 per cent) had RHD (as opposed to ARF), and 20 per cent had RHD without a documented history of ARF, indicating earlier missed diagnosis or asymptomatic cases of ARF. Many older people were found to have been prescribed penicillin, in contradiction to guidelines.13

Figure 17.1 shows the trend over the audit years for service delivery for up to nine best-practice indicators for rheumatic heart disease care (present in the rheumatic heart disease audit tool): a record of the rheumatic heart disease classification in the health summary; a record of current and of complete acute rheumatic fever and rheumatic heart disease management plans; a record of planned frequency of injections (if the client had been prescribed injections); whether 80 per cent or more of injections had been received (if the client had been prescribed regular injections and there was a record of planned frequency); a record of active recall if less than 80 per cent of injections had been received; timely doctor and specialist review, and echocardiogram (according to recommended schedule based on the rheumatic heart disease classification); and record of client education (provided within the last 12 months).

Figure 17.1 Trends in overall RHD care. Source: J. Bailie, Matthews et al. 2016.

Improving ARF/RHD care in Aboriginal and Torres Strait Islander PHC

Our studies show that systems are inadequate to translate secondary prevention guidelines into quality care. In addition, the low number of clients with a recorded RHD priority classification (30 per cent of 1,081 audits in one study) suggests that this classification is rarely used to guide management.14

ARF and RHD occur mostly amongst people living in remote locations, where poor housing infrastructure and crowded living contribute to the transmission of infections.15 PHC centres are typically understaffed and have a high turnover of staff. A workforce study in the Northern Territory found that only 20 per cent of nurses and Aboriginal healthcare providers remained working at surveyed remote clinics 12 months after commencing. Half left within four months.16 Embedding change in care delivery systems, providing staff training and maintaining knowledge in ARF/RHD management and use of information systems is very challenging but even more critical under these circumstances.17 The trial of a multi-strategy RHD intervention in 10 remote communities found that long-term PHC system–strengthening strategies, with more linkages between PHC teams and communities, are essential to improve adherence to secondary prevention.18 

Early prevention should include comprehensive skin and throat programs in high-risk communities.19 Transitioning children with ARF safely to adulthood without rheumatic heart damage requires tailored care. Engaging and valuing local navigators to address language and cultural barriers has been recommended as a sustainable alternative to transition coordinators in mainstream programs.20

Cultural competence of healthcare teams, including appropriate communication styles that empower clients and their families with knowledge about their health conditions and treatment,21 is critically important when tackling diseases of disparity such as RHD. Clinicians’ competency in communicating influences health outcomes for Aboriginal and Torres Strait Islander people,22 and multiple Australian studies of CQI in ARF and RHD care have highlighted the importance of communicating with clients in local languages and using culturally appropriate educational resources for improving RHD prevention and management.23 Culturally competent and well-prepared PHC teams, strong community engagement and Aboriginal and Torres Strait Islander leadership are key to improvement.24

References