Epidemiology of rheumatic heart disease in India and challenges to its prevention and control

Transcription

1 Epidemiology of rheumatic heart disease in India and challenges to its prevention and control Anita Saxena, MD, DM Department of Cardiology, Cardiothoracic Centre, All India Institute of Medical Sciences, New Delhi, India Abstract Rheumatic heart disease (RHD) has virtually disappeared from the western world; however it continues to be a public health problem in India and several other developing countries. It is a disease of poverty and is associated with overcrowding, substandard living conditions, poor sanitation and inadequate access to healthcare. The disease affects children and young adolescents causing progressive damage to cardiac valves. It is the commonest cause of heart failure in young population. Estimates of RHD based on school surveys primarily suggest a decline of its prevalence in some states of India which have a better per capital domestic product. Data from lesser developed states of India is not available. The control of RHD is challenging, especially due to marked religious, cultural and linguistic diversity of India. Other factors contributing to its continued prevalence include absence of a national policy, lack of awareness about the disease, inconsistent availability of injectable penicillin and lack of RHD registries in India. In addition, the medical fraternity in India is losing interest in RHD as most like to believe that RHD prevalence is declining. A multipronged approach is required for control of RF and RHD. Firstly, we need to define its burden in all parts of India. Register based control programs should be instituted and penicillin made widely and freely available. The rates of secondary prophylaxis are likely to improve with these measures. In addition, advocacy with government is also an important component, if we want to eradicate RHD from India. Key Words Rheumatic heart disease (RHD) Rheumatic fever (RF) Group A beta hemolytic streptococci (GAS) Introduction Rheumatic heart disease (RHD) results from valvular damage caused by acute rheumatic fever (RF) which is an autoimmune response to group A beta hemolytic streptococci (GAS). The exact pathogenesis is not well understood and the epidemiologic triad of RF includes GAS, a susceptible host and an opportune environment. RF involves multiple systems of the human body, including joints, skin and brain but apart from cardiac sequel all other effects are reversible. Cardiac valve damage follows either a single serious episode or multiple episodes of RF. Acute RF and RHD have virtually disappeared from the western world, but RHD continues to be a major public health problem in developing countries, including India. Since, most of children vulnerable to RHD live in developing countries, it is estimated that decline in RF and RHD has 1 occurred for <20% of the world population. India contributes to about 25 50% of newly diagnosed cases of 2 RHD. The disease affects young children and adolescents, mostly from poor families living in unhygienic conditions associated with overcrowding. RF occurs at a relatively younger age in India, affecting children as young as 3-years. The incidence of carditis is also higher during an episode of RF. Further, the disease progresses very rapidly with a malignant course and may produce severe mitral 3,4 stenosis at a very young age. The reasons for this accelerated course of the disease are not very clear and may be either due to a genetic predisposition or due to recurrent attacks of RF as secondary prophylaxis is generally very inadequate. Girls and women are more severely affected than men. Received: ; Revised: ; Accepted: Disclosures: This article has not received any funding and has no vested commercial interest Acknowledgements: None A decline in prevalence of RF and RHD has been reported from some parts of India; however the data from more populous states is not widely available. Unfortunately with an upsurge in coronary artery disease, RF and RHD have 256

2 Epidemiology of rheumatic heart disease in India become an orphan disease with little interest to the physicians or the policy makers. It neither falls into the category of communicable diseases, nor in the noncommunicable disease group. Recently, however, RHD was discussed as one of the priority diseases at the UN summit on non-communicable diseases. In this article epidemiology of RF and RHD in India will be discussed in detail and barriers to its prevention and control will be listed. Finally, some remedial measures will also be outlined. Measured estimates of burden of RF and RHD The first article on RF/ RHD from India was published in Since then several reports have been published highlighting the magnitude of this problem. These articles may be grouped into three categories: 1. Population surveys: As one would imagine, population based surveys are likely to be most representative of the problem, these are difficult to conduct. One such study published in 1993 defined a 6 prevalence of 0.9/1000 in the community. Another community survey from Kanpur, UP, conducted in 4326 villages and published in 2000 reported a much higher prevalence of 4.58/1000. The incidence of RF 7 was 0.4/1000 in population above 15-years of age. These prevalence data have been used to extrapolate the burden of RF and RHD in India presuming it to be 8 uniform all over, which is unlikely to be true. 2. School based studies: Much more robust data is available for school children, aged 5 16-years. The prevalence of RHD has varied from 1.8 to 11/1000 children (average 6/1000) in the 1970s and 1980s. Studies conducted in the 1990s showed a relatively 9,10 lower prevalence of 1 to 3.9/1000 children. Prevalence of 0.5/1000 children was reported from 11 Gorakhpur (UP) in 2007 and 0.67/1000 from Vellore 12,13 and Bikaner. Indian Council of Medical Research (ICMR) has also sponsored school surveys in several different parts of India at three time periods more than a decade apart. The data obtained in these studies also 9,14,15 shows a decline in prevalence of RHD. However, the regions chosen in ICMR studies are not uniform so it is difficult to be sure of a decline. Some of these school based studies, especially the ones in the last years, have used echocardiography for validation of suspected cases. This makes sense as clinical assessment remains less sensitive and specific as compared to echocardiography. Studies which have used echocardiography have reported a lower prevalence rates as some of these children had either benign murmurs or murmurs due to congenital heart 11,12 disease. In one study the prevalence of RHD reduced from 16.7/1000 (clinical assessment) to /1000 after echocardiographic validation. It is also likely that if echocardiography was employed for all cases a higher prevalence will be picked up. Besides, the technique used there are other confounders like: methodological issues, type of population surveyed, clinical skills of the physician assessing these children, rates of absentees and so on. Recently, several reports have appeared wherein echocardiography has been used as a screening tool to diagnose subclinical cases of RHD in asymptomatic school children. These studies are from Africa, Pacific islands, Cambodia, Nicaragua, Fiji and India. All these studies have shown over 10 times higher prevalence of RHD as compared to clinical prevalence. Our data showed a prevalence of 20.4/1000 as 21 compared to 0.8/1000 by clinical evaluation alone. It is not clear whether this high prevalence represents true RHD as the echocardiographic criteria for detection of subclinical RHD are still evolving. A consensus statement on echocardiographic criteria has been 22 recently published; these needs to be validated. Currently, the significant of echo diagnosed RHD is not well understood and the natural history of children diagnosed to have RHD by echo alone is also not known. 3. Hospital statistics: In 1980s and 1990s nearly half to one third of total cardiac admissions were due to RHD 8,10 in teaching hospitals of India. A more recent survey has reported this figure to be between %. This decline in percentage of all cardiac admissions may not be true decline for several reasons. The proportion of RHD related admissions to a given hospital depends on the socio-economic status of the population served by that hospital. We are aware that admissions for coronary artery disease and congenital heart disease are on the rise at centres with advanced facilities for their treatment. Further several of the state-of-the-art hospitals may be privately owned charging a substantial amount of fee for treatment. Since, RHD affects mostly poor patients, affordability becomes a major issue. Hospital statistics may become more useful if derived from the same hospital over different period of time. This type of data is published from a hospital in Cuttack, Orissa where authors reported that RF and RHD contribute to 45% of all cardiac admissions with 24 no decline from 1991 to Government funded hospitals with facilities for cardiac care, including 257

3 Saxena A valve surgery continues to see a large number of patients with RF and RHD coming mostly from underprivileged regions of India. At All India Institute of Medical Sciences, New Delhi RHD remains the 25 commonest cause of heart failure. Estimated RHD burden in India Data on RHD prevalence is lacking from many lesser developed states of India, e.g., Bihar, Jharkhand, these states are likely to have the highest burden of RHD. Similarly, rural areas may have a high prevalence not only due to poverty and unhygienic living conditions but also due to lack of access to effective health facilities. In a study published in 2009, continued high prevalence of RHD was reported from tribal population of Chhattisgarh, 26 Maharashtra, and even from some pockets in Kerala. Hence, RHD prevalence may have declined in some regions, it is unlikely to be a uniform phenomenon all over India. As per WHO estimates 133,000 deaths occur annually in 2 Southeast Asia, which are attributable to RF or RHD. This is in sharp contrast to 10,000 deaths in Americas and 30,000 deaths in Europe. Considering an average prevalence of 0.5/1000 children in age group of years, currently there are 3.6 million patients in India who have RHD (estimated from 2011 census). Since, majority of these patients are in their most productive phase of life, RHD is severely affecting the workforce of emerging nation like India. Barriers to prevention and control As is evident from the data presented above, RHD has declined in some parts of India. The determinants of this decline could be improved living conditions, better awareness about the disease and prompt access to healthcare. Most states of India where RHD prevalence has shown a decline have the best per capita domestic product, e.g., Kerala and Tamil Nadu. States where per capital domestic product remains low (e.g., Orissa, Uttar Pradesh), RHD prevalence continues to be high. These inequalities in economic growth, literacy levels, basic sanitation and hygiene, healthcare availability, overcrowding etc. result in dual epidemiology of RHD in India. Other parameters like infant and maternal mortality rates tend to be lowest in regions with low RHD prevalence. These data show that RHD has a complex epidemiology and needs to be dealt with at various levels. India The land of striking contradiction India is a country which can be said to be living in multiple 258 time zones. It has marked religious and language diversity. Over 30 languages are spoken by a million people each. The literacy levels vary from as low as 30% to as high as 91% in different states. The female literacy rates are lower. About 70% of population lives in rural areas. What is more relevant to RHD is that 24% of a total of 1.2 billion population lives in slums. These slums are located in the midst of urban areas, including in metro cities. The residents in these slums have often migrated from rural areas of states not doing too well economically. Overcrowding, unhygienic living conditions are rampant, as is poverty. Unfortunately, there has not been a major improvement in the situation overall. The percentage of population living below poverty line is still high at 37.2% 27 as per 2010 figures. It is estimated that 77% of Indians live 28 on less than half a US dollar per day. In addition health issues such as malnutrition and infectious diseases are very common and get priority in national programs. State of healthcare in India The basic units of healthcare, i.e., primary health centres are understaffed and ill equipped. Paradoxically some of the best, state-of-the-art, world class health facilities are available in many metro cities of India, catering to even international patients. These advanced facilities centres are generally clustered in big cities and are out of reach for a common man who is poor and lives far from a big city. Treatment of patients with RHD is not on their priority list as it is a commercially non-viable venture. Majority of patients treated at such cardiac centres have coronary artery disease. Very few surgeons have the desired skill to repair a rheumatic mitral valve, majority will replace it with a prosthetic valve which is an inferior treatment due to prosthetic valve related morbidity and mortality. There is lack of political will also as no effective auditing or regulatory body exists for medical (mal) practice. The government spends only 3.5% of total government 29 spending on healthcare. This is in comparison to 10% spent by China and South Africa. The funding is prioritized towards infectious diseases and malnutrition. To add to the woes of a common man, corruption is rampant and the meager funds meant for healthcare hardly reach the beneficiary. Problems specific to RHD RHD is a disease where environment seems to play a major role in its causation. Overcrowding, poor sanitation coupled with high level of illiteracy are congenial to the development of RF and hence RHD. RHD is not a notifiable disease and there are no national programs for RHD in India.

4 Epidemiology of rheumatic heart disease in India Lack of awareness In most parts of India, very little knowledge exists for RF and RHD in community. The link between a streptococcal sore throat and development of RF and consequently RHD is not known to the majority of even literate people. Children with sore throat are often treated by indigenous methods like ginger, honey, etc. by the local elders. Although most sore throats are viral in etiology, throat cultures are hardly ever done to rule out a bacterial pathology. Those who visit a physician for sore throat are also treated empirically with an antibiotic. In fact most of primary health centres do not have facilities for throat swab and culture. Impediments to injectable penicillin For the last 10 years or so, long acting benzathine penicillin, the back bone of treatment for streptococcal pharyngitis has a very inconsistent availability in most parts of India. In some states injectable penicillin is not available at all even at government funded hospitals for fear of allergic reaction. Patients with RHD have to manage with oral antibiotics which have been shown to be inferior for secondary prophylaxis. Since there is no control on quality of the drug, substandard quality penicillin are available in the market. Patients are forced to buy these, since the regular brands are not available. The other problem is that injections of penicillin are not administered by most private centres, clinics and nursing homes for fear of allergic reaction. Hence, patient has to visit one of the government hospitals every 3 weeks to get the injection administered. This adds to the cost of treatment and decreases the adherence rates of secondary prophylaxis. The injection of long acting injectable form of penicillin is quite painful and deters many patients especially children, further reducing compliance with secondary prophylaxis. Absence of register based programs Register based programs have been shown to improve the rates of secondary prophylaxis and decrease the prevalence 2,30,31 of RHD. Successful register based programs can be implemented at low cost using existing infrastructure. Such programs have been established in Australia for 32,33 Aboriginal populations. Unfortunately in India, none of the centres have adapted register based control programs. This may be one of the reasons that adherence to secondary prophylaxis is abysmally low, reported as 55% only from a 24 hospital based data in a state of urban India. Declining interest of medical fraternity Most physicians and cardiologists are under the impression that RHD is declining in India and direct their research towards other cardiovascular disorders. The number of research publications related to RF or RHD has greatly reduced over the last 40 years. The published articles are mainly related to interventions for RHD patients and not to epidemiological aspects of the disease or preventive strategies. A mere 8% of global research on RHD originates from India. Suggested strategies for improvement Since, environment plays an important part in the pathogenesis of RF, improvement in living conditions, hygiene, alleviation of poverty, etc. are likely to help in controlling this disease. However, discussion on these issues is beyond the scope of this article. Define the burden of the disease Since RHD has a variable presence in various parts of India, it is very important to generate data on its prevalence from every representative population, i.e., different states, rural and urban areas, poor and rich population, different age groups and so on. Several national and international funding agencies are coming forward to help generate such data, but we need to identify local physicians / other personnel who are willing to devote time to undertake such projects. Policy change at national level RHD must be made a notifiable disease as has been done in African countries. More RHD cases will be detected this way and secondary prophylaxis will help in retarding progression to more severe valve damage. Establish registries at all levels This is the real need of the hour. Registries can be established with existing infrastructure with little extra cost, their efficacy is well reported from various countries. Simple data base which is easy to manage should be used and its uniformity maintained at all centres. RHD registers will help us track morbidity, mortality, outcomes of valve surgery, adherence to secondary prophylaxis, etc. Registries are also useful for detecting trends in disease prevalence. Improve secondary prophylaxis rates Secondary prophylaxis has been proven to be of benefit in control of RHD. The rates of secondary prophylaxis must improve to control progressive damage to cardiac valves. The primary health centres must have facilities for 259

5 Saxena A injecting long acting penicillin free of charge to the patient. This may be best done by incorporating with the existing facilities for maternal and child care at the health centre. Make penicillin available Good quality penicillin must be made available consistently all across the country. It should be either free or priced at a subsidized cost. Physicians and others involved in providing injection of penicillin must be educated about the allergic reactions and the importance of skin testing before each injection. Fortunately, allergic 34 reactions to penicillin are very rare especially in children. Industry should try to develop depot preparations of penicillin and effective penicillin alternatives. Public awareness and education These are very important aspects for success of any program. Communities and school children should be made aware of the disease, its symptoms (e.g., joint pains) and link with sore throat. Advertising on television or in print media is very effective if done in an interesting manner. Advocacy Strengthening advocacy with government, nongovernmental organizations, national and international funding agencies is difficult but very important. Celebrities and other influential persons can be used for this purpose. Conclusion The magnitude of RHD is underestimated. It may have declined in some regions, perhaps related to economic growth, literacy levels and availability of health care with ease in that region. However, RHD continues to be a major public health problem in most of states in India affecting young children and adolescents. Control of RHD requires a multipronged approach from improvement in living conditions to public awareness, free availability of penicillin drug for secondary prophylaxis and heightened interest of physicians in this disease. RHD presents several challenges and hence several opportunities. Currently, a new surge is being seen in the interest of both physicians and non-physicians in this disease. It is also heartening to see that many of these people are from countries where RHD burden is still high. We hope that RHD would meet the same fate one day as Polio did in India. References 1. Carapetis JR. Rheumatic heart disease in developing countries. N Engl J Med 2007;357: World Health Organization. Rheumatic fever and rheumatic heart disease: Report of a WHO expert consultation. Geneva, 29 Oct 1 Nov World Health Organ Tech Rep Ser 2004;923: Roy SB, Bhatia ML, Lazaro EJ, Ramalingaswami V. Juvenile mitral stenosis in India. Lancet 1963; ii: Cherian G, Vytilingam KI, Sukumar IP, Gopinath N. Mitral valvotomy in young patients. Br Heart J 1964;26: Rogers L. Gleanings from the Calcutta post mortem records. HI Diseases of the circulatory system. Indian Med Gaz 1910;45: Grover A, Dhawan A, Iyengar SD, Anand IS, Wahi PL, Ganguly NK. Epidemiology of rheumatic fever and rheumatic heart disease in a rural community in northern India. Bull World Health Organ 1993;71: Lalchandani A, Kumar HRP, Alam SM. Prevalence of rheumatic heart disease in rural and urban school children of district Kanpur (Abstr). Indian Heart J 2000;52: Vijaykumar M, Narula J, Reddy KS, Kaplan EL. Incidence of rheumatic fever and prevalence of rheumatic heart disease in India. Int J Cardiol 1994;43: Community control of rheumatic fever and rheumatic heart disease. Report of ICMR task force study. New Delhi: Indian Council of Medical Research; Shrivastava S. Rheumatic heart disease: Is it declining in India? Indian Heart J 2007;59: Misra M, Mittal M, Singh R, Verma A, Rai R, Chandra G, et al. Prevalence of rheumatic heart disease in school-going children of eastern Uttar Pradesh. Indian Heart J 2007;59: Jose VJ, Gomathi M. Declining prevalence of rheumatic heart disease in rural schoolchildren in India: Indian Heart J 2003;55: Periwal KL, Gupta BK, Panwar RB, Khatri PC, Raja S, Gupta R. Prevalence of rheumatic heart disease in school children in Bikaner: An echocardiographic study. J Assoc Physicians India 2006;54: Prevalence of rheumatic fever and rheumatic heart disease in school children: Multicenter study. Annual Report. New Delhi: Indian Council of Medical Research; 1977: Jai Vigyan Mission mode project on community control of RHD. Non-communicable diseases. Indian Council Med Res Annu Rep ; Marijon E, Ou P, Celermajer DS, Ferreira B, Mocumbi AO, Jani D, et al. Prevalence of rheumatic heart disease detected by echocardiographic screening. N Engl J Med 2007;357: Carapetis JR, Hardy M, Fakakovikaetau T, Taib R, Wilkinson L, Penny DJ, et al. Evaluation of a screening protocol using auscultation and portable echocardiography to detect asymptomatic rheumatic heart disease in Tongan schoolchildren. Nat Clin Pract Cardiovasc Med 2008;5: Reeves BM, Kado J, Brook M. High prevalence of rheumatic heart disease in Fiji detected by echocardiography screening. J Paediatr Child Health 2011;47: Paar JA, Berrios NM, Rose JD, Cáceres M, Peña R, Pérez W, et al. JA Prevalence of rheumatic heart disease in children and young adults in Nicaragua. Am J Cardiol 2010;105: Bhaya M, Panwar S, Beniwal R, Panwar RB. High prevalence of rheumatic heart disease detected by echocardiography in school children. Echocardiography 2010;27: Saxena A, Ramakrishnan S, Roy A, Seth S, Krishnan A, Misra P, et al. Prevalence and outcome of subclinical rheumatic heart disease in India: the RHEUMATIC(Rheumatic Heart Echo Utilisation and Monitoring Actuarial Trends in Indian Children) study. Heart. 2011;97:

6 Epidemiology of rheumatic heart disease in India 22.Reményi B, Wilson N, Steer A, Ferreira B, Kado J, Kumar K, et al. World Heart Federation criteria for echocardiographic diagnosis of rheumatic heart disease an evidence-based guideline. Nat Rev Cardiol. 2012;9: Jose VJ. Changes in profile and presentation of rheumatic heart disease. In: Das S (ed). Medicine Update 2003;13: Mishra TK, Routray SN, Behera M, Pattniak UK, Satpathy C. Has the prevalence of rheumatic fever/rheumatic heart disease really changed? A hospital-based study. Indian Heart J 2003;55: Seth S, Ramakrishnan S, Roy A, Bhargava B, Bahl VK, Poole- Wilson PA. Epidemiology of heart failure in India. Eur J Heart Fail Suppl 2009;8: Kumar RK, Paul M, Francis P. RHD in India Are we ready to shift from secondary prophylaxis to vaccinating high-risk children. Curr Sci 2009;97: Poverty Reduction and Livelihoods Promotion [homepage on the Internet]. [cited on Jun 2012]. Available from: org.in/whatwedo/poverty_reduction 28.Report on Conditions of Work and Promotion of Livelihoods in the Unorganised Sector", National Commission for Enterprises in the Unorganised Sector, Government of India, August, Accessed: June World Health Organization (WHO). World Health Statistics WHO Health Statistical Information System Thornley C, McNicholas A, Baker M, Lennon D. Rheumatic fever registers in New Zealand. NZ Pub Health Rep 2001;8: Nordet P, Lopez R, Duenas A, Sarmiento L. Prevention and control of rheumatic fever and rheumatic heart disease: The Cuban experience ( ) Cardiovasc J Afr. 2008;19: Noonan S, Edmond K, Krause V, et al. The top end rheumatic heart disease control program I. Report on progress. NT Dis Control Bull 2001;8: Brown A, Purton L, Schaeffer G, Wheaton G, White A. Central Australian rheumatic heart disease control program: a report to the Commonwealth, November Northern Territory Dis Control Bull 2003;10: Markowitz M, Lue HC. Allergic reactions in rheumatic fever patients on long-term benzathine penicillin G: the role of skin testing for penicillin allergy. Pediatrics, (6): Address for correspondence Dr. Anita Saxena: anitasaxena@hotmail.com 261