Forgotten and Fatal

Rheumatic Heart Disease in Developing Countries. An article by Adaugo Amajuoyi, Perspectives Editor.

It is a commonly known fact that conditions like HIV, Malaria and Tuberculosis claim the lives of millions of people around the world with the majority of that population living in developing countries in Africa and Asia. While these problems dominate the media it is rare to find similar kind of attention drawn to the serious issue of Rheumatic Fever (RF) and Rheumatic Heart Disease (RHD). RHD is still a major cause of morbidity and is the most common form of acquired cardiovascular disease in children. It accounts for 60% of all heart disease in children and young adults. There are 15.6 million people with RHD and 470,000 new cases of Rheumatic Fever reported each year. There are 350,000 deaths each year attributed to RHD/RF. What’s more is that these figures may not truly reflect the overwhelming burden of RF, as the statistics on these figures are often unavailable.

RHD is an autoimmune disease that starts off with a common throat infection caused by group A streptococcal bacteria (GAS).The antigens on the surface of the bacteria provoke an abnormal immune reaction that triggers the production of cytokines and antibodies aimed to prime streptococcal carbohydrates and myosin (a component of heart tissue) to be destroyed by the individuals immune system. This immune reaction, Rheumatic fever, damages and weakens the heart valves. The individual may appear healthy but further streptococcal infections can result in further damage of the heart valves. Poor living conditions, overcrowding and poor access to health care means that re-infection with GAS is inevitable.

The condition develops insidiously until the damage to the heart valves becomes far too extensive before the problem is detected. At this point the child requires heart surgery which is unlikely to be available and if so very expensive. Cost is not the only unfortunate result of RHD. In developing countries ravaged by the disease, the majority of the workforce are young adults. This disabling condition could indirectly hinder the productivity of the country and slow down economic development.

Thing of the past

RHD was a major problem for the industrialised nations in the Mid-twentieth century. Cararpetis explained that children with RF filled the Paediatric wards at the time. Coincidently the vast number of papers published on the topic of RF at the time exceeded other urgent issues like Stroke and other cardiovascular disorders. However towards the later part of the last century, the prevalence of RF receded and with that, the amount of research on the disease also decreased.

Unfortunately RHD is still major problem for developing nations whose health systems are already stretched with the demands of infectious diseases like HIV, Malaria, Tuberculosis. The decline in interest and research in RHD will only further hinder awareness to the growing problem of RHD. But why does RHD once a disease of the both the industrialised world and the developing still affect the developing countries. The answer is that RHD is a disease of the poor.

In the latter half of the last century, wealthy nations like the UK, increased availability and widespread use of antibiotics like Penicillin for treatment of acute pharyngitis has helped significantly to prevent repeated infections of the bacteria and the development of Rheumatic Fever. In addition the standard of living has improved since the first half of the 20th century when RF and RHD was a major concern. Better living conditions and improved quality of hygiene contributed to the reduction in the transmission of bacteria including group A streptococcal bacteria involved in pathogenesis of Rheumatic Fever. Since the 1950s, these improvements including better access to medical services and awareness of the condition, Rheumatic Fever and RHD is rarely diagnosed in children of most industrialized countries.

Similar improvements cannot be said of developing nations. There is still a high incidence and prevalence of RF and RHD in non-industrialised nations as well as the indigenous populations of countries like Australia and New Zealand. In the WHO report on RF and RHD, the annual incidence of RF in developed countries is now 1.0 per 100,000. However, incidence rates in French Polynesia was recorded to be 72.2 per 100,000 while that in Sudan was 100 per 100,000. Unfortunately similar figures where found for most African and South Asian countries. The report also showed that the prevalence of RHD of school aged children also varies widely from 0.2 per 1000 in Havana Cuba to 77.8 per 1000 in Samoa. These figures support the concept that persisting prevalence of RF and RHD are strongly influenced by the socioeconomic and environmental factors.

Barriers to eradicating RF and RHD

There are several problems that contribute to the high incidence of RF and RHD in developing countries. These include the following:-

• Poor living conditions and poor hygiene and under nutrition.
• Overcrowding, which leads to increased transmission and increased risk of the development of RF from the streptococcal throat infections contributes to the virulence of strep strains
• Limited or no access to health care
• Poor availability of antibiotics and poor adherence to secondary prophylaxis.
• A shortage of Resources for providing quality health care
• Inadequate expertise of Health care providers
• Low level awareness of the disease in the community and by health care providers.
• Shortage of an anti-streptococcal vaccine

These problems are common to most if not all developing countries and hinder the elimination of RF and RHD.

Limited and in most cases no access to quality healthcare is a major obstacle in the primary prevention of RF which involves detection of the sore throat infection. Although this has been proved successful in well supported settings, it is impractical partly due to the nature of the disease, the low level of awareness and in part due to the strain put on health care systems by other disease such as HIV infection and Malaria.

Poor availability of Antibiotics like Penicillin makes it difficult to achieve the secondary measure of preventing the development of RHD. Children with RF are recommended to be given monthly penicillin injections to prevent further damage of the heart valves. However such recommendations are difficult to be sustained in the long term.

Developing countries are not the only nations are struggling to deal with RF and RHD. The indigenous populations of countries like Australia, New Zealand and Hawaii have some of the highest incidence of RF and RHD while the disease appears to be eradicated in the non-indigenous population. The Aboriginals in the Northern Territory of Australia have the highest documented incidence of RF in the world, 305 cases per 100,000 per year and up to 650 cases per 100,000 per year in remote regions. In addition the standard death rate for RF/RHD is 30.2 per 100,000, 30 times the death rate for non-aboriginals in Australia. The reasons for the high incidence of RF and RHD among the indigenous communities are similar to those in developing countries but also include the following:-

• Lack of access and trust in the health services
• Population mobility – makes it difficult to keep a register of those with RF
• Language differences
• The availability of water for washing is a problem
• Educational limits

These additional factors only exacerbate and facilitate the spread and development of RF and RHD among the indigenous communities of industrialised countries like Australia.

What needs to be done

RF and RHD present its self as a significant cost to both the individual and to health services of the community. In some countries in the Pacific, 15% of their total health budget is spent on sending children with RHD abroad for surgery. Such high costs could have been easily avoided by increasing awareness of the RF and RHD to communities and improving access of antibiotics.

As catching RF early on is crucial for the prognosis, diagnosis must be improved. This will involve improving the expertise of health care professionals and also involving more sensitive equipment during diagnosis, such as echocardiography. A systematic review by Marijion et al 2007 involving children in both Cambodia and Mozambique, indicated that echocardiography screening detected a significantly greater number of children with RHD than the clinical diagnosis alone. Although expensive, this start-up cost would be nothing in comparison to the future costs of heart valve repair surgery. Losing out on the use of echocardiographic screening would miss out on a significant proportion of children with RF and leave them at risk of severely disabling heart conditions and increased risk of morbidity. In addition the recent availability of high quality portable ultrasound equipment1 makes it possible to screen large number of children at a time, a desirable feature to facilitate health care planning.

Increased awareness of RF and RHD among the community and health care workers is crucial to elimination of the disease. There was marked decline in RF and RHD in Cuba between 1986 and 1996 as discussed by Nordet and colleagues. The findings suggested that prevention and control of RF/RHD is feasible and affordable in developing countries and involved training of health care personnel, posters and educational material for the population, and working with teaching institutions and policy makers to gain their support in targeting those susceptible to RF, school children. The study show that the prevalence of RF and RHD fell from 2.27 patients per 1000 children in 1986 to 0.24 per 1000 in 1996. In addition the implementation of the programmed did not incur much additional cost for the healthcare system. Such encouraging results are inspiring and give a good example to other countries what can be achieved.

Cuba however has many luxuries other developing countries can dream of. There is a well structured health system with free and easy access to medical treatment for the whole population; the same cannot be said for the majority of developing countries struggling to meet the basic health care needs of their population.

There are organisations that are committed to helping eradicate RHD such as RHDnet, part of the World Heart Federation. They provide resources for health professionals including best practice tools - sample databases, management, guidelines, and staff training resources as well as links to other programme resources.

Action is being taken, The Pan Africa Society for Cardiology (PASCAR) in partnership with the World Health Federation started a project to achieve the following:-

1. Increased awareness of RF/RHD among the Public and Health Professionals
2. Establish surveillance systems
3. Advocate for increased resources for treatment in African nations

With such measures in place, it is hoped that this preventable disease will be eliminated left in the past.

There is an old Igbo lamentation that begins with the singer asking God for help saying ‘I am at your feet God, for there are so many in your hands, don’t forget to look at your feet.’ Although Rheumatic Heart disease may not be in the limelight, it is such a tragic and disabling condition, the true tragedy would be if such an easily treatable condition goes unnoticed and forgotten.

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Adaugo Amajuoyi is a second year Medical Student at UCL and the 2009/10 Chief Editor or Perspectives.

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