Health & Family Welfare Department
Gandhinagar, Gujarat
Sickle Cell Anemia Control Program
(A Major Tribal Health Program of Gujarat)
Presentation by : Anju Sharma IAS
Mission Director (NRHM)
Government of Gujarat
Introduction
 India has the highest number
of Sickle gene carriers in the
world, >50% of the world.
Most of tribal belt in India being
an endemic area for Malaria, the
people living in tribal area are
affected by Sickle Cell Anemia.
However,
no attempts have
been made so far to address the
issue related to the disease so
far at the national level.
Introduction
 Sickle Cell Anemia is a
major health problem in
tribal Gujarat.
 A hereditary disease
caused by mutant
recessive gene
 All 12 Tribal districts are involved in Sickle Cell Anemia Control Program
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Estimated Prevalence of Sickle Cell Anemia in Gujarat
Gujarat
Total Population
Tribal Districts
India
6,03,83,628 1,21,01,93,422
12
593
Tribal Population*
89,12,623
17,86,24,549
Suspected Sickle Trait @ 10.0 %
8,91,262
1,78,62,455
66,845
13,39.684
Suspected Sickle Cell Disease
Patients @ 0.75 %
* Provisional
Public Health concern:
If left untreated –

20% of Sickle disease children die by the age of two.

As per one of the ICMR survey 30% of disease children
among the tribal community die before they reach adulthood.

Even if they survive, life time risk of disease remains same.

Being genetic in nature, the numbers are bound to rise, if
suitable intervention is not made.
Sickle Cell Anemia Control Program –
Gujarat Initiative
Initiative: In the year 2006, the Department of
Health & Family Welfare, Government of Gujarat
initiated a Public Private Partnership - Sickle Cell
Anemia Control Program in 5 districts of south
Gujarat.
Now by 2010-11, it has been extended to all 12
tribal districts of Gujarat and Gujarat Sickle Cell
Anemia Control Society has been formed under
society registration act 21 of 1860.
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GOALS:
 No Sickle Cell Disease childbirth by 2020.
 Prevention of death from Sickle Cell Crisis.
 To improve health status and quality of life of
Sickle Cell Anemia patients.
Patho-physiology of Sickle Cell Anemia
Rigid & Brittle Sickle
Shaped RBC
Sickle Cell trapped in Capillaries
Mutation
Normal Red Blood Cell
Patho -physiology of
Sickle Cell Anemia
Treatment trough
Folic Acid, Analgesic
Antibiotic, Anti helminthes
Anti Pyretic, Iron for short duration
Anti Malarial if necessary
Hydroxyurea &
Blood Transfusion in crisis.
Causes symptoms
Pallor
Frequent
jaundice
Sickle Cell
Anemia,
Bone & Body ache
Hemolytic
Jaundice,
Enlarged
Spleen
Joint Pain,
etc.
Retarded
Growth
Frequent Infections
Dactylitis
(Hand-Foot Syndrome)
Strategies:







Timely diagnosis through large scale screening with the
help of specialized laboratory tests.
Prevention of Sickle disease child birth through
adolescent screening, marriage counseling, antenatal
screening and prenatal diagnosis.
Better management of disease through supportive
treatment, follow up and counseling to reduce morbidity
and mortality.
Reduction in crisis episodes.
Saving Lives through referral treatment and specialized
hospital care.
Building Community Awareness and sensitization of the
population through interpersonal communication.
Training and capacity building of health service
providers.
Screening Approach Adopted in Program
Adolescent
Screening
Mass
Screening
Treatment and
follow up
Newborn
Screening
Antenatal Screening
&
Husband Screening
Institutions involved in the program
No.
Institutions
Year 2006-07
Year 2011
1
Medical College
1
2
2
General Hospital
3
12
3
PHC
44
333
4
CHC
29
70
5
NGO
1
2
78
419
Total
11
Facilities made available
Facilities available
No.
Year
2007-08
Year
2011
1
Primary Screening for Sickle Cell (DTT) test,
Counseling and Treatment
78
419
2
Hemoglobin Electrophoresis
21
21
3
HPLC based Hb Variant system for quantitative
estimation of different hemoglobin
2
3
4
HPLC based Variant-NBS system for New Born
Screening for SCA from heel prick dry blood
samples from filter paper.
0
1
5
Molecular Lab for prenatal diagnosis and Genetic
Counseling Center
0
1
12
Progress of screened person till June-2011
Year wise Community screened for Sickle Cell Anemia in
Gujarat, Year 2006 to 2011 (Up to June)
No. of Person screened during year
400000
350000
329224
323218
300000
342696
269171
250000
200000
150000
142950
100000
44677
50000
0
2006-07
2007-08
2008-09
2009-2010
Year
2010-11
2011-12 (Up
to June)
Screening Output - I
Total Tribal
Population
64,70,256
Tribal Population
14,51,936
(22.44%)
Screened for
Sickle Cell Anemia
No. of Sickle Cell
Trait
1,69,358
(11.66%)
No. of Sickle Cell
Disease
10961
(0.75%)
**Up To June, 2011**
Screening Output - II
No. of Adolescents
Screened for Sickle
Cell Anemia
1,81,365
No. of Adolescents
found Sickle Cell
Trait
17613
(9.71%)
No. of Adolescents
found Sickle Cell
Disease
1020
(0.56%)
**Up to June, 2011**
Screening Output - III
No. of Antenatal
Mothers
Screened for
Sickle Cell
Anemia
2,13,779
No. of Antenatal
Mothers found
Sickle Cell Trait
15367
(7.18%)
No. of Antenatal
Mothers found
Sickle Cell
Disease
848
(0.44%)
**Up To June, 2011**
Screening Output - IV
Result of Prenatal Diagnosis after Genetic Counseling
Total High
Risk Couple to
whom Genetic
counseling for
PND Provided
Eligible for
Prenatal
Diagnosis
104
43
100 %
41.34 %
No. of
Agreed for
Fetus Positive
Fetus
PND with
for Sickle
Negative
Sickle
informed
Disease
For Sickle
Disease
Consent and
among
Disease
Child Birth
PND carried
screened (24) Prevented
among
out against
screened
eligibility
(24)
24
14+3*
7
7
55.81 %
58.33 %
29.16 %
100 %
Both the parent have Sickle gene.
Rest of the 58 % High Risk Couple were
not eligible for Prenatal Diagnosis as they
were registered for ANC after 20 weeks of
Pregnancy, Hence not eligible for
Termination of Pregnancy; even if fetus is
Sickle Disease. Steps are being taken for
early registration of ANC cases.
* Result awaited (1)+ procedure fail (2)
** Up To June, 2011**
PND Couple
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Screening Output - V
Newborn Screening for Sickle Cell Disease
from Dried Blood Spots from Heel Prick on Filter Paper


Total Newborn
Screened
Normal
Sickle
Trait
Sickle
Disease
5,037
4,377
622
38
86.89 %
12.34%
0.75 %
All 33 Sickle Disease patient were
registered for comprehensive
care
under the Sickle cell program.
Their
Parents
were
provided
counseling for care of their children at
home as well as both the parents blood
were tested and counseled for future
pregnancy.
**Up to June, 2011**
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Marriage Counseling Using Laminated Color
Coded Cards
Normal Haemoglobin Card
Sickle Trait Card
Laminated color coded
cards are given to all
screened persons.
These color coded cards
are further used for
marriage counseling.
Sickle Disease Card
Treatment Outputs

10,961 Sickle disease patients are given monthly quota of
Tab. Folic acid and painkillers for daily use, by field health
workers.

57 patients are on Hydroxyurea, given to severe disease
patients.

1675 sickle crisis patients have been given treatment in year
2010.

1531 blood transfusion were given to sickle disease patients
free of cost in 2010.
Other Activities







Established network of counseling centers
in tribal blocks.
Established mechanism for referral and
management of Sickle cell crisis patients.
Capacity building and training of MPHWs,
ANMs, Village Health and Sanitation
Committees and PRI representatives.
Targeted IEC and BCC
Creation of database and regular reporting
and monitoring
Logistics and inventory management of
drugs.
Teacher counselling- Students having
Sickle Cell Disease have been exempted
from physical training and allowed to go to
urinals
frequently
without
asking
permission.
Outcomes

Many Sickle cell patients are now able to lead normal lives.
Mortality and morbidity due to the disease has reduced.

As per survey in 500 sickle cell disease patients, number of
crisis has decreased from 2-3 per year to 1 per year in
tribal districts.

Counseling of 17,613 sickle positive adolescents and for
marriage and future pregnancy. Thousands of marriages
between sicklers have been avoided in the Tribal
Community. Prevented birth of many sickle Cell Diseased
children including 7 through PNDT.

Mass Awareness about the disease.
Socio Economic Impact
Before
Sickle Cell Disease was in books and academic research papers.
NOW
The Common Tribal People are most benefited. Today they get
regular free medicine at their door step, hence there is marked
reduction in crisis.
Today they have better Life Span & Functional Status than before.
The Fruits of the Program have reached to the door step of Underserved– The Tribal People.
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Impact on Medical Services
Before
The Medical
Practitioners were not aware of sickle
phenomena existing among local public. Most of Sickle
Disease patients were misdiagnosed and mistreated. Even if
Sickle cell was diagnosed, they believed that this is a genetic
disease, there is no cure & nothing can be done.
Now
Awareness among Medical Practitioners has increased
dramatically in respect to Diagnosis & Treatment. Marriage
counseling, Antenatal Screening and Prenatal Diagnosis are
being advised by medical doctors.
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Extraordinary Features of the program








Reached to the doorstep of most vulnerable tribal population14,51,936.
Reduction in the hardship and arresting productivity loss of
tribals.
Lifecycle approach.
Active involvement of NGOs.
Community involvement.
Capacity building of both Government and NGO staff.
Utilization of National and International expertise (ICMR &
SCDIO)-Knowledge Sharing, Technical support
Application of modern techniques and research in the field
situations - Stem cell research etc.
Sustainability:





Though the program initially started with NGO
participation, it has been now been integrated with the
general health services through training and capacity
building of the government service providers.
Program approach gives wider reach and long term
focus.
Demand generation from the community due to their
sensitization and awareness building.
Training and capacity building of private service
providers.
Mass support to the program.
Replication of the Program at National level


Taking up a National Program for Sickle Cell Anemia
Control.
Components:








Population screening for tribal areas through a life cycle
approach
Color Coded Laminated Cards to all the screened
beneficiaries.
Training & Capacity building of service providers in
screening, disease management and supportive care
Free treatment including blood transfusion to all the sicklers.
Genetic counseling, patient counseling and sensitisation of
the community, family, etc.
Community awareness through Mass based campaigns.
Involvement of community & NGOs for better community
reach.
Medical education & research in Sickle Cell Anemia.
Our Indian Sicklers are
Born with Pain
Live with Pain
&
Die with Pain.
They hardly Complain.
Thank you very much
for paying attention to
our SICKLERS.
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