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INTRODUCTION

Lymphatic flariasis (LF) caused by flarial nematodes and transmitted


by mosquitos, is one of the neglected tropical diseases (NTD) which
is still a public health problem in India and is endemic in 17 states
and 6 union territories. Wuchereria bancrofti and Brugia malayi are
the two important parasite species that give rise to this disease. Te
former accounts for almost 95% of the disease burden in the tropical
countries, while the latter is responsible for most of the remaining
5% of cases. Brugia malayi is prevalent in the states of Kerala, Tamil
Nadu, Andhra Pradesh, Odisha, Madhya Pradesh, Assam and West
Bengal. In India, the incidence of LF is of such magnitude that this
country alone accounts for 40% of the world disease burden.
1
About
31 million people are estimated to be the carriers of microflariae and
over 23 million sufer from flarial disease manifestations in India. Te
state of Bihar has highest endemicity (over 17%) and Goa showed the
lowest endemicity. Te long standing disability resulting from this
disease in the form of elephantiasis of the limbs and genital region,
hydrocele of the scrotal sac and the repeated acute attacks of fever
and infammation cause such sufering that this disease is thought
to be only next to malaria among the tropical debilitating diseases.
2

Te total disability adjusted life years (DALYs) lost in India due to
this disease is around 2.06 million, resulting in an annual wage loss
of US $811 million.
3
Recent studies have shown beyond doubt that
LF infection starts mostly in childhood, even though the disease
manifestations are seen more often, several years later in adulthood.
Te daytime antigen detection tests, ultrasonography (USG) and
lymphoscintigraphy (LSG) have documented the presence of
signifcant subclinical pathology in children, which suggest that LF
often has its origin in childhood.
4-7
DISABILITY CAUSED BY LYMPHATIC FILARIASIS
Te disease spectrum of LF ranges from the initial phase of
asymptomatic microflaremia to the later stages of acute and chronic
clinical manifestations. Te disease manifestations caused by Brugia
malayi difer from those of Wuchereria bancrofti in that, in the former
the lymphedema involves only the legs below the knee and upper
limbs below the elbow and there is no genital involvement. Tropical
pulmonary eosinophilia (TPE) syndrome is a form of occult flariasis
caused by both parasites.
Asymptomatic Microflaremia
In any endemic area for LF, among the infected individuals, the
largest group consists of otherwise healthy young adults and
children who have microflaria (MF) in their peripheral blood
without any overt clinical manifestation. Even at this stage, there
are abnormalities of the lymph vessels like dilation and tortuosity
which are demonstrable by ultrasound examination and LSG.
8,9

Tis pathology appears to be irreversible even after treatment.
10

Tis is important because ~30% of children in certain endemic
communities have been shown to acquire LF infection by the age
of 4 years, as shown either by the presence of MF or Wuchereria
bancrofti antigen in blood.
11
In a Brugia malayi endemic region
in Kerala, LSG has demonstrated subclinical lymphatic pathology
even in three year old children with asymptomatic microflaremia.
12
Acute Manifestations
Acute Dermato-Lymphangio-Adenitis
Attacks of acute dermato-lymphangio-adenitis (ADLA) associated
with fever and chills account for 97% of acute clinical manifestations
in LF.
13
Even though ADLA occurs both in the early and late stages of
the disease, it is more frequent in higher grades of lymphedema. Te
afected area, usually in the extremities or sometimes in the scrotum
is extremely painful, warm, red, swollen and tender. Te draining
lymph nodes in the groin or axilla become swollen and tender.
Depending on the presence of precipitating factors, such acute
attacks to recur several times a year in patients with flarial swelling.
It is now well-known that secondary infections due to bacteria
like streptococci are responsible for these acute episodes and
in the afected limbs, lesions favoring entry of bacteria can be
demonstrated, either in the form of interdigital candidiasis, minor
injuries, infections, eczema or cracks in the feet. In higher grades of
lymphedema, fungal infection tends to occur in the webs of the toes
and becomes aggravated during rainy season or due to household
work where the feet are soaked in water. In such situations, the
ADLA are more frequent and are responsible for the persistence and
progression of lymphedema leading on to elephantiasis.
14
Acute Epididymo-orchitis and Funiculitis
Infammation of structures in the scrotal sac may result in acute
epididymo-orchitis or funiculitis in bancroftian flariasis. Tis is
characterized by severe pain, tenderness and swelling of scrotum,
usually with fever and rigor. Te testes, epididymis or the spermatic
cord may become swollen and extremely tender. Like acute ADLA,
these attacks are also precipitated by bacterial infections.
15
Acute Filarial Lymphangitis
Acute manifestations directly caused by adult worms are usually
rare and are seen when the adult worms are killed in the lymphatics
either spontaneously or by drugs like diethylcarbamazine (DEC).
Small tender nodules form at the location where adult worms die,
Indian Scenario of Elimination
of Lymphatic Filariasis
Chapter 3
Suma TK
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Chapter 3 Indian Scenario of Elimination of Lymphatic Filariasis Section 1
either in the scrotum or along the lymphatics. Lymph nodes may
become tender. Infamed large lymphatics may stand out as long
tender cords underneath the skin, usually along the sides of chest or
medial aspect of arm, with restriction of movement of afected limb.
But, these episodes are not associated with fever, toxemia or evidence
of secondary bacterial infection.
13
Rarely abscess formation may be
seen at the site of dead adult worms.
Chronic Manifestations
Lymphedema and Elephantiasis
Lymphedema of the extremities is the most common chronic
manifestation of LF which on progression leads on to elephantiasis.
Even though lower limbs are the ones frequently afected, upper
limbs and male genitalia may also be involved. In females, rarely the
breasts may also be afected.
In the advanced stages of lymphedema, which continues to occur
the afected limb may undergo enormous enlargement and the skin
is thickened, thrown into folds, often with hypertrichosis, black
pigmentation, nodules, warty growth, intertrigo in the webs of toes
or chronic nonhealing ulcers.
16
Acute dermato-lymphangio-adenitis
is responsible for the progression of lymphedema continues to occur
with greater frequency in higher grades of edema.
Genitourinary Lesions
Hydrocele is a common chronic manifestation of bancroftian flariasis
in the males. Tis is characterized by accumulation of fuid in the
tunica vaginalis, the sac covering the testes. Te swelling gradually
increases over a period of time and in long standing cases the size of
the scrotum may be enormous. Microflaria may be detected in the
peripheral blood or in the hydrocele fuid in some of these subjects.
Occasionally, the hydrocele may have an acute onset when the
surrounding lymphatics are infamed due to the death of adult worms.
Tis is usually self-limiting and may disappear over a period of time.
17
Lymphedema of the scrotum and penis may occur in bancroftian
flariasis. In some subjects, the skin of the scrotum may be covered
with vesicles distended with lymph known as lymph scrotum.
Tese patients are prone for acute ADLA attacks involving the
skin of genitalia. Hematuria, chyluria and chylocele are the other
genitourinary manifestations associated with LF.
WHY LYMPHATIC FILARIASIS SHOULD
BE ELIMINATED?
Tis disease is prevalent mostly among the socioeconomically
challenged population in 83 tropical countries of the world. Several
studies have highlighted the social, psychological, sexual and
economic implications of this disease. Te massive swellings of
the limbs in elephantiasis interfere with the day-to-day activities of
these subjects. Tey tend to work for fewer hours or change to lighter
and less remunerative jobs, impairing their earning capacity.
18

Genitourinary disease especially hydrocele, results in strong feelings
of shame, fear and embarrassment associated with sexual disability
and dysfunction.
19
Tis disease hampers the marriage prospects
of the young mainly females. Among children the social problems
include feeling of shame, embarrassment and frequent absence from
school or even discontinuation of studies.
20
International Task Force
for Disease Eradication has identifed LF as one of only six potentially
eradicable diseases.
21
GLOBAL PROGRAM FOR ELIMINATION
OF LYMPHATIC FILARIASIS
In the year 1977, the World Health Assembly resolved to eliminate LF
globally as a public health problem.
22
Tis resolution is based on the
new knowledge of the pathogenesis of LF; the biology of the parasite;
development of better diagnostic tools and treatment strategies.
Global program for elimination of lymphatic flariasis (GPELF)
has two components:
1. Interruption of transmission of infection in communities by mass
drug administration (MDA).
2. Alleviation of the disability in those who already have the
disease.
23
Te frst component of GPELF is to interrupt transmission of
flarial infection in the entire at risk population of an endemic
region by administration of single, annual doses of a two-drug
regimen which in India consists of administration of DEC 6 mg/kg
and albendazole 400 mg for a period of 46 years. Children under 2
years of age, pregnant women and severely ill patients are excluded
from MDA. Te principle behind this strategy is as follows. Te
transmission of LF infection in a community depends on the MF
load in the human carriers and density of vector mosquitoes. Even
though vector control is in theory the ideal, it is difcult to achieve
this in practice due to difculties encountered and the cost involved
in efectively implementing this method for LF transmission control.
So, the next best is to reduce the load of MF in a given community
to such low levels that the mosquitoes will be unable to efectively
transmit the infection. Te two drug combination, in the doses
mentioned above is shown to bring down the blood microflaria
levels drastically even after annual single dose administration
which when repeated for up to 6 years would achieve the target of
preventing transmission of LF infection. Te estimated fecundity life
of the adult parasite is 46 years which is the basis for continuing the
program for the duration mentioned.
24
It has been shown further that at least 80% of the at risk
population should consume the drug once annually to achieve
transmission control in the time frame mentioned above. Otherwise
the drug administration will have to be continued for many more
years. Among the LF endemic countries, MDA program is now in
place in 51 countries. Ten countries did not require any more MDA,
since they have eliminated transmission of the disease.
25
Te benefts obtained so far from the MDA conducted globally
during the past 8 years (20002007) have been remarkable. In the
treated communities, in an estimated 6.6 million newborns who
would otherwise have acquired LF, nearly 1.4 million cases of
hydrocele, 800,000 cases of lymphedema and 4.4 million cases of
subclinical disease have been averted in their lifetimes. Similarly,
MDA has protected 9.5 million individuals who were previously
infected but without overt manifestations of disease from developing
hydrocele (6.0 million) or lymphedema (3.5 million). Tese LF
related benefts, by themselves translate into 32 million DALYs
averted.
26
In a recent study, it was clearly shown that drugs used in
MDA reversed the subclinical lymphatic pathology in children with
flarial infection.
27
PRESENT SCENARIO OF LYMPHATIC
FILARIASIS ELIMINATION IN INDIA
In India, MDA with DEC alone was launched as a pilot project
in 13 districts of 7 states in the year 1996 covering a population of
41 million. Te program was scaled up to cover a population of 77
million in 2002, when 19 districts were under DEC alone and 11 were
covered with DEC + albendazole. During the year 2004, a population
of about 468 million from 202 districts was targeted for MDA. It was
also proposed to observe National Filaria Day every year from
2004 in all endemic districts.
28
From 2006 onward, November 11
is observed as National Filaria Day. Te National Task Force on
Elimination of LF recommended the coadministration of DEC 6 mg/
kg and albendazole 400 mg to all endemic districts from 2006. From
2007 the entire endemic population of 590 million in 250 districts
8
Infectious Diseases Section 1
in India was targeted for MDA. Even though the drug distribution
ranged from 83% to 90%, actual consumption was much lower
than the desired 80% in most of the districts covered under MDA.
Even then, in most endemic districts the MF prevalence rates have
declined after initiating the MDA program. Tus, MF rates have
declined below 1% MF rate in 176 districts. In 2008, as the frst step
toward elimination of LF transmission, immunochromatographic
test (ICT) card survey for detection of Wuchereria bancrofti is initiated
in six districts with less than 1% MF rates in Tamil Nadu. Tis is being
conducted by National Institute of Communicable Disease (NICD)
and Vector Control Research Center (VCRC) for which, World Health
Organization (WHO) have supplied 19,500 ICT cards.
29
Alleviation of Disability Due to Lymphatic Filariasis
Disability management is an important arm of GPELF. In order to
achieve a visible impact of the program in the community, it is
necessary that components that address disability control are built-
in.
23
Treatment of Acute Dermato-Lymphangio-Adenitis
Te most distressing aspect of disability in LF is the acute attacks of
ADLA which prevent the patient from attending his daily activities for
several days. In most instances, they can easily be treated and further
episodes can be prevented. Bed rest and symptomatic treatment with
simple drugs like paracetamol are enough in mild cases. Any local
precipitating factor like injury and bacterial or fungal infection should
be treated with local antibiotic or antifungal ointments. Moderate or
severe attacks of ADLA require oral or parenteral administration of
antibiotics depending on the general condition of the patient, together
with analgesic/antipyretic drugs. Commonly used antibiotics like
penicillin, tetracycline, ampicillin, amoxicillin or cotrimoxazole may
be given in adequate doses till the infection subsides. Bacteriological
examination of swabs from the entry lesions may help in selecting the
proper antibiotic in severe cases. Systemic antifungal agents are rarely
required since the fungal infections of the skin act only as entry points
for the bacteria and fungi themselves do not cause the ADLA.
30
Prevention of Acute Dermato-Lymphangio-Adenitis
Presently there is a simple, efective, cheap and sustainable method
available for prevention of these attacks. Many recent studies have
shown that this can be achieved by proper local-hygiene of the
afected limbs, carried out regularly.
14
Foot-care aimed at prevention
of fungal and secondary bacterial infections have become the
mainstay for disability alleviation in GPELF.
31
Tis method requires
only the common facilities available for washing in any household
and hence can be carried out by the patients themselves in their
home. Patients, community health workers and also providers of
home-care should be trained in this foot-hygiene program.
Tis foot-care program to prevent ADLA attacks consists of the
following:
Washing the afected area, especially the webs of the toes and
deep skin folds, with soap and water twice a day or at least once
before going to bed and wiping dry with a clean cloth
Clipping the nails at intervals and keeping them clean
Preventing or promptly treating any local injuries or infections
using antibiotic ointments
Applying antifungal ointment in the webs of the toes, skin folds
and sides of the feet to prevent fungal infections
Regular use of proper footwear
Keeping the afected limb raised at night, using bricks to elevate
the foot end of the cot.
In patients with late stages of edema proper local care of the limb
is not always possible due to deep skin folds or warty excrescences.
If such patients continue to have ADLA attacks, long-term antibiotic
therapy using oral penicillin or long acting parenteral benzathine
penicillin is indicated. Recent studies have shown that antiflarial
drugs like DEC have no role either in the treatment or prevention of
the acute ADLA attacks occurring in cases of lymphedema which are
caused by bacterial infections.
32
In endemic areas, regular foot-care should be encouraged
from early age, in view of the fact that LF is frst acquired mostly in
childhood and that the early asymptomatic stage of the infection
itself is associated with irreversible lymphatic damage. Tis would
help in preventing the acute attacks and probably in arresting the
development of future lymphedema and elephantiasis in children
and young adults.
Treatment and Prevention of Lymphedema
In early stages of the disease when the adult worms are sensitive to
DEC, treatment with this drug might destroy them and thus logically
prevent the later development of lymphedema. Equally important is
the prevention of ADLA attacks in these patients since the occurrence
of lymphedema and its progression are related to these repeated
infections. Once lymphedema is established there is no permanent
cure and as mentioned already, treatment with DEC does not seem
to reverse the existing lymphatic damage. Te following treatment
modalities ofer relief and help to prevent further progression of the
swelling:
Using elastocrepe bandage or tailor made stockings while
ambulant
Keeping the limb elevated at night after removing the bandage
Regular exercising of the afected limb
Regular light massage of the limb to stimulate the lymph vessels
and to promote fow of lymph toward larger patent vessels
Intermittent pneumatic compression of the afected limb using
single or multicell jackets
Heat therapy using either wet heat or hot ovens
Surgical procedures: Tere are various surgical options available
to ofer relief of lymphedema, like lymph nodo-venous shunts,
omentoplasty and excision with skin grafting. Even after surgery
the local care of the limb should be continued for life, so that
ADLA attacks and recurrence of the swelling are prevented.
Hydrocele of the scrotum and lymphedema of the external
genitalia are amenable to surgery.
Oral and topical benzopyrones and favonoids are advocated for
the treatment of lymphedema. Tese drugs are supposed to reduce
high protein edema by stimulating macrophages to remove the
proteins from the tissues when administered for long periods. Further
controlled trials are needed to substantiate this claim.
CONCLUSION
Lymphatic flariasis is still a public health problem in India harboring
40% of world disease burden. Te social, psychological, sexual and
economic implications of this disease are enormous. Elimination is
all the more important since it is now known that LF infection frst
starts in childhood even though the clinical manifestations appear
much later. Te early pathology is dilatation of lymph vessels caused
by the adult worms which was thought to be irreversible even after
treatment. Tis has prompted the launching of GPELF to eliminate
this parasitic infection through MDA. It may be noted that drugs used
in MDA reversed the subclinical lymphatic pathology in children
with flarial infection. India aims to eliminate LF by 2015 through
annual MDA using DEC and albendazole. Tis is one of the most
economical and benefcial disease control strategies undertaken so
far in public health programs.
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