Background

Rheumatic fever usually follows an untreated beta-hemolytic streptococcal throat

infection in children. It can affect many parts of the body, and may result in
rheumatic heart disease, in which the heart valves are permanently damaged, and
which may progress to heart failure, atrial fibrillation, and embolic stroke.

Nowadays, rheumatic fever mostly affects children in developing countries,
especially where poverty is widespread. Up to 1% of all schoolchildren in Africa,
Asia, the Eastern Mediterranean region and Latin America show signs of the disease.

Of 12 million people currently affected by rheumatic fever and rheumatic heart
disease, two-thirds are children between 5 and 15 years of age. There are around
300,000 deaths each year, with two million people requiring repeated
hospitalization and one million likely to require surgery in the next 5 to 20 years.

Early treatment of streptococcal sore throat can preclude the development of
rheumatic fever. Regular long-term penicillin treatment can prevent rheumatic
fever becoming rheumatic heart disease, and can halt disease progression in people
whose heart valves are already damaged by the disease. In many developing
countries, lack of awareness of these measures, coupled with shortages of money
and resources, are important barriers to the control of the disease.


Figure 1
Rheumatic Heart Disease in Children
Estimated number of cases in 5 to 14 year-old (2003)




Although RF and RHD are rare in developed countries, they are still major public
health problems among children and young adults in developing countries.

Acute rheumatic fever (ARF) and rheumatic heart disease (RHD) represent the first
cause of cardiac mortality among children and young people in developing countries
(Marijon et al, 2012).

People who have had ARF previously are much more likely to have subsequent
episodes, and these recurrences may cause further damage to the cardiac valves.
Thus RHD steadily worsens in people who have multiple episodes of ARF (Carapetis
JR, Brown A, Wilson NJ, Edwards KN. An Australian guideline for rheumatic fever
and rheumatic heart disease: an abridged outline. Med J Aust. 2007 Jun 4;
186(11):581-6.)

ARF is predominantly a disease of children aged 5-14 years and generally does not
affect children less than 3 years old or adults (National Heart Foundation of
Australia (RF/RHD) guideline development working group) and the Cardiac Society
of Australia and New Zealand. Diagnosis and management of acute rheumatic fever
and rheumatic heart disease in Australia-an evidence based review. 2006).

The World Health Organization (WHO) outline the important part socioeconomic
and environmental factors play in contributing to the magnitude and severity of RF
and RHD, and although it is a disease rarely seen now in most developed
populations, it is still a cause of major concern in many of the worlds developing
nations and selected populations of some developed countries.

In 2005, Carapetis et al (Carapetis JR, Steer AC, Mulholland EK, Weber M. The global
burden of group A streptococcal diseases. Lancet Infect Dis. 2005; 5:685-94)
published a summary of the major findings of an in depth review performed for
WHO of the global burden of group A streptococcal diseases. From this review, they
estimated that over 2.4 million children aged 5-14 years are affected with RHD. In
addition, 79% of all RHD cases came from less developed countries. Further, they
estimated the annual number of ARF cases in children aged 5-14 years was more
than 336,000. This was extrapolated out to an estimate of 471,000 ARF cases in all
age groups. Similar to RHD, they found that 95% of cases came from less developed
countries.

From there, they estimated that of all cases of ARf, 60% would go on to develop RHD
each year. They also estimated that, in addition, there are 1.88 million people (ten
times the remaining 40% of new ARF cases each year) with a history of ARF but no
carditis presently requiring secondary prophylaxis. Finally, they estimated that
there were over 492,000 deaths per year due to RHD, with approximately 468,000
of these occurring in less developed countries.

The prevalence of RHD appears to be increasing worldwide. Based on WHO study, of
the available control strategies, secondary prophylaxis is the only one that has been
shown to be both effective and cost-effective at the community/population level and
therefore, in populations with high prevalence of RHD, delivery of secondary
prophylaxis should be the major priority for control GAS diseases. In 2004, WHO
Technical Report on RF and RHD, IM injection of benzathine benzylpenicillin every
three weeks (every four weeks in low risk areas or low risk-patients) is outlined as
the most effective strategy for prevention of recurrent attacks of RF.

Objective of the study

Generally, the ARF attack on children (which develops into RHD) can have recurrent
episodes in their third and fourth decades. To reduce the impact and burden of
ARF/RHD, the World Health Organization (WHO) introduced several approaches.
Rheumatic fever remains a serious public health problem throughout the world.
Preventing or providing early treatment for streptococcal infections will be the most
important step to worldwide ARF eradication. This study would like to evaluate
rates of adherence in people receiving BPG injections prophylaxis in NCH and to
determine the factors associated with the relapse of ARF/RHD. It is well known that
socioeconomic and environmental factors play an indirect, but important, role in the
magnitude and severity of RF and RHD. Factors such as a shortage of resources for
providing quality health care, inadequate expertise of health-care providers, and a
low level of awareness of the disease in the community can all impact the
expression of the disease in populations.

Significance of the Study

Rheumatic Fever and Rheumatic Heart Disease remain to be the number one cause
of OPD consultation at National Children every month. Lots of patient who had
relapse were admitted every now and then. Most of them lost to follow-up in the
treatment then suddenly returning and already in irreversible Heart Failure. This is
currently the real situation happening in our patients at National Childrens
Hospital. This is always how fate will be described in each patient if new ideas and
changes are not given a chance. Hence, this research was pursued.

These study hopes to generate data that will enable clinicians to implement
evidence based strategies in the care of these children. Since it aims to determine
and understand the factors affecting relapse of patients diagnosed with Rheumatic
Fever, the primary significance of this research is to help us find ways on how we
can decrease the relapse rate. The medical staff of National Childrens Hospital plays
a big role in presenting this issue to the management. Therefore, new
recommendations and necessary adjustments in the hospital programs will be
facilitated, to ensure strict compliance on the treatment among each patient.

Once this modification happened, patients will learned better conduct to adopt for
its own benefits. More cooperation is then expected thereby good compliance and
less relapse is foreseen.

Scope and Limitation of the study

This study only covers the diagnose case of patients between 2001-2010 in National
Childrens Hospital. The following socio-demographic and clinical factors are
examined in this study:
1. Socio-demographic Factors
a. Age
b. Sex
c. Sibling Rank
d. Family Income
2. Clinical Factors
a. Age of Diagnosis (5-15 years old)
b. Signs and Symptoms (minor: fever, arthralgia; major: carditis,
polyarthritis, chorea)
c. Compliance
d. Duration of Treatment
e. Supporting evidence of Group A Streptococcal Infection
f. Baseline 2-D Echo
g. Other Laboratory Findings (Elevated ESR/CRP)
h. Baseline Medication (Amoxicillin, Cephalexin, Cefuroxime, Co-
amoxiclav)

Methodology

A cross-sectional study was conducted at National Childrens Hospital and randomly
included patients diagnosed with Rheumatic Fever from year 2001 to 2010. Medical
records of each patient were reviewed individually. Data were gathered from the
clinical history and discharge summary of each patients chart. Relevant data were
recorded using a Data Abstraction Form. Compliance to treatment was determined
by counting the number of Penicillin injection in each year. Patient is considered as
with poor compliance if more than 3 Penicillin injections were missed during each
year.


What is rheumatic heart disease?
Rheumatic heart disease is caused by damage to the heart valves and heart muscle from the inflammation
and scarring caused by rheumatic fever. Rheumatic fever is caused by streptococcal bacteria, which usually
begins as a sore throat or tonsillitis in children.
Rheumatic fever mostly affects children in developing countries, especially where poverty is widespread.
Globally, almost 2% of deaths from cardiovascular diseases is related to rheumatic heart disease, while
42% of deaths from cardiovascular diseases is related to ischaemic heart disease, and 34% to
cerebrovascular disease (2).
Symptoms of rheumatic heart disease
Symptoms of rheumatic heart disease include: shortness of breath, fatigue, irregular heart beats, chest pain
and fainting.
Symptoms of rheumatic fever include: fever, pain and swelling of the joints, nausea, stomach cramps and
vomiting.
Treatment
Early treatment of streptococcal sore throat can stop the development of rheumatic fever. Regular long-
term penicillin treatment can prevent repeat attacks of rheumatic fever which give rise to rheumatic heart
disease and can stop disease progression in people whose heart valves are already damaged by the disease.

http://www.who.int/mediacentre/factsheets/fs317/en/
(2) Global atlas on cardiovascular disease prevention and control. Geneva, World Health
Organization, 2011.