The National Department of Health (NDOH) South Africa declared the first week of August Rheumatic Fever Week more than 25 years ago, bringing long overdue recognition to this preventable condition which disproportionately affects women and children in lower income countries such as Uganda and middle-income countries, such as South Africa. This year, Rheumatic Fever Week is being observed from 1st through 7th August with the intention of raising awareness of the harm that rheumatic fever (RF) can cause. Moreover, the Heart and Stroke Foundation South Africa (HSFSA) and the Children’s Heart Disease Research Unit (CHDRU) would like to play a role in disseminating more information and knowledge regarding the relationship between RF and rheumatic heart disease (RHD).
What is Rheumatic Fever?
Rheumatic fever is an abnormal immune reaction to a common bacterium called Group A Streptococcus. Patients typically experience a range of symptoms that can include joint pain, fever above 38°C, generally feeling unwell, shortness of breath, a skin rash and, on rarer occasions, uncontrolled body movements. Rheumatic fever is preceded two or three weeks earlier by a bacterial throat infection, commonly called strep throat.
A single episode or repeated episodes of rheumatic fever can cause damage to the heart valves, resulting in rheumatic heart disease. Left untreated, rheumatic heart disease will eventually lead to further heart valve damage, stroke, heart failure, and even premature death. The disease requires life-long medication, medical surveillance and often heart valve replacement surgery. Significantly however, rheumatic fever can be completely prevented by the oldest antibiotic available, namely, penicillin. Key to this preventive step is an early diagnosis.
Recent Scientific Advances and Current Research on RHD
The pathophysiologic characteristics and genetics of rheumatic heart disease are not fully understood. Funding for more research is needed to fill this gap as well as to investigate what the best practice treatment strategies are until such time as effective vaccines against strep throat are developed. Quote: Professor Liesl Zuhlke, paediatric cardiologist and director of the Children’s Heart Disease Research unit reminds us that”A sore throat can damage your heart”
RHDGen Study
A pan-African study led by researchers at the University of Cape Town has identified the genetic determinants of rheumatic heart disease risk in black Africans. The study, published in June 2021, is the largest of its kind to provide insights into the genetic determinants of rheumatic heart disease in Africa.
Researchers found a single genetic risk locus associated with rheumatic heart disease at genome-wide significance in black African individuals but not in other groups. What this means is that there is an important polygenic or heritable component to rheumatic heart disease risk in black African individuals which goes beyond a shared environment. In other words, Africans have certain genes specific to people from the region, as well as genes found in other populations, which makes them more likely to have the disease including its severe cases.
These findings are important because they help to fill the gaps which remain in our understanding of individual host susceptibility to rheumatic heart disease after Group A streptococcal infection and provide a clear basis to work from going forward. The RHDGen study findings could aid in the development of vaccines and treatments but further investigation is still needed. This finding also highlights the fact that there are potentially other factors that influence the onset of RHD.
GOAL Trial
Findings of a clinical trial published in November 2021 shed new light on the efficacy of secondary antibiotic prophylactic penicillin in preventing the progression of latent rheumatic heart disease in children and adolescents. Authors found that (Ugandan) patients who received prophylactic penicillin were significantly less likely to experience disease progression compared to those who did not receive treatment – an important finding for future treatment recommendations and further research.
A follow up study, ‘GOAL Post’, will monitor patients for a further five years to determine the durability of prophylaxis in preventing adverse outcomes as well as whether it is safe for children to stop antibiotic prophylaxis once their heart returns to normal. Another study looking at oral versus intramuscular penicillin, which potentially has more added practical value in low-resource settings, is also planned.
Proteomics Study:=
Scientists have identified six proteins implicated in rheumatic heart disease which could revolutionise the way in which the condition is treated and diagnosed in the developing world. Published in March 2022, findings from the collaborative study between research teams at the universities of Manchester (UoM) and Cape Town (UCT) offers hope to the 40 million of the world’s population disabled by this devastating disease.
The study – which used state of the art technology called proteomics to discover the disease’s biomarker signature – revealed for the first-time ongoing inflammation activity in 215 patients with severe rheumatic heart disease from 10 African countries.
The discovery of the biomarker signature could one day enable the development of simple test kits which could be used in the home and clinic. Although more research is needed, the team argues that existing anti-inflammatory treatments could now potentially be repurposed, so that they are taken prophylactically to prevent disease progression.
WHA Resolution Scorecard Project
A team from the University of Cape Town and the South African Medical Research Council are conducting a study to develop a scorecard for monitoring country-level implementation of the 71st World Health Assembly resolution on rheumatic fever and rheumatic heart disease in Africa. The scorecard will provide a simple and reliable tool for tracking progress over time and establishing accountability mechanisms for RF and RHD in Africa.
The study is designed to allow RHD stakeholders to be at the forefront of developing the scorecard by sharing their knowledge and experience of RHD in African settings. Thirty-one RHD stakeholders have participated through individual in-depth interviews and group discussions. The stakeholders included RHD patients, healthcare workers, researchers, health administrators, policymakers, donors, and primary school personnel from nine African countries, Cameroon, Malawi, Namibia, Rwanda, South Africa, Sudan, Tanzania, Uganda, and Zambia; and three countries outside Africa, Fiji, Geneva, and the United States of America. The data collected from these RHD stakeholder engagements informs the development of the scorecard. This scorecard will be validated in two African countries before disseminating for broader use throughout Africa.
What Do These Findings Mean for RHD Prevention advocates and the Public?
While these scientific advances and ongoing research are steps in the right direction, it takes time for findings to be put into practice. Continued prevention efforts at the community level are therefore very much recommended. These could be focussed on raising community awareness of Group A streptococcal throat infection and the link with rheumatic fever and rheumatic heart disease. Likewise, improving living conditions, hygiene and access to primary health care should continue to be prioritised.
Moreover, both advocates and those affected by RHF need to mobilize and constructively work together to ensure the correct access to essential care for RF and RHD, particularly in the public sector. Prof Pamela Naidoo, CEO of the HSFSA states that the organization plays an important role in education and knowledge dissemination for RF, RHD , as well heart disease in general. Please contact the Foundation for more information and to also work with us to reduce the RHD burden. In addition, If you’d like to raise awareness, consider sharing this useful infographic with schoolteachers and care givers in your community! If you are interested in keeping up to date with RF/RHD research and activities from around the world, feel free to subscribe to the quarterly RHD Pulse newsletter. You can find previous editions released this year here.
