1. 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
This program deals with a major Tribal Health issue, so far unattended (neglected) in Government Health Services, which effects the life span of our Tribal People, both physically as well as socio-economically.
This program is a public – private partnership program, originally launched in 2006 in 5 districts of South Gujarat and now it is integrated with other programs of Health services in the state of Gujarat.

2. 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.

3. Introduction
Sickle Cell Anemia is a major health problem in tribal Gujarat.
A hereditary disease caused by mutant recessive gene
Gujarat being number 5th in tribal population has also major Tribal Health issue of Sickle Cell Anemia.
All 12 Tribal districts are involved in Sickle Cell Anaemia Control Program in the Gujarat State.
All 12 Tribal districts are involved in Sickle Cell Anemia Control Program 3 3

4. Estimated Prevalence of Sickle Cell Anemia in Gujarat
India
Total Population
6,03,83,628
1,21,01,93,422
Tribal Districts 12
593
Tribal Population*
89,12,623
17,86,24,549
Suspected Sickle 10.0 %
8,91,262
1,78,62,455
Suspected Sickle Cell Disease 0.75 %
66,845
13,39.684
Situation of Sickle Cell Anemia in Gujarat
Total Population : 5,50,00,000
Tribal Districts :12
Tribal Population : 64,70,256
Estimated Suspected Sickle 10.0 % : 6,47,025 Estimated Suspected Sickle Cell Disease 0.75 %: 48,527
* Provisional

5. 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.
Public Health concern:
20% of Sickle disease children die by the age of two.
As per one of the ICMR survey 30% of Sickle disease children among the tribal community die before they reach adulthood.
Even if they survive, life time risk of disease remain same.
Early diagnosis can save the life and allow better management of the disease but screening is not a common practice and the diagnosis is usually made when a patient presents with a severe complications.
Being genetic in nature, the numbers are bound to rise, if suitable intervention is not made. 5

6. 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 , 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.
Initiative: In the year , Department of Health & Family Welfare, Government of Gujarat initiated a Public Private Partnership - Sickle Cell Anemia Control Program in the 5 districts of south Gujarat.
Now in , it has been extended to all 12 tribal districts of Gujarat since 2008 and steps are being taken to form a Gujarat Sickle Cell Anemia Control Society under society registration act 21 of 1860. 6 6

7. 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.
GOAL:
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. 7

8. Patho-physiology of Sickle Cell Anemia
Pallor
Frequent jaundice
Bone & Body ache
Enlarged Spleen
Retarded Growth
Frequent Infections
Dactylitis
(Hand-Foot Syndrome)
Treatment trough
Folic Acid, Analgesic
Antibiotic, Anti helminthes
Anti Pyretic, Iron for short duration
Anti Malarial if necessary
Hydroxyurea &
Blood Transfusion in crisis.
As I said, according to one of the hypothesis, this mutation has occurred to prevent the entry of Malaria parasites into the cell wall of Red blood Cells.
In doing so, the cell wall of Red Blood Cell has become rigid and brittle, and turns round biconcave disc like red blood cells into the shape of Sickle, a Greek word used for Grass cutter, when the oxygen is delivered to tissues, i.e. on deoxygenation.
This is an ongoing process throughout the life span of Sickle Cell disease patient.
Many sickle shaped RBCs trapped in narrow capillaries, break down in endothelial system, decreasing the life span of RBC in Sickle Cell Disease, very often to less than 30 days instead of 90 to 120 days.
Anemia result from the bone marrow’s inability to produce enough blood cells to keep pace with the rate of destruction.
Very often the destruction / breakdown of Red blood cells is so fast that these patients have hemolytic jaundice, due to conversion of free liberated hemoglobin into yellow Colour of bilirubin, causing jaundice.
The iron liberate from broken red blood cell is reabsorbed by body, hence these patients do not require iron therapy
Rigidity and abnormal shape reduce their ability to be propelled through tiny capillaries & formation of entangled masses of cells in larger blood vessels.
Sickling is more likely in small capillaries where hemoglobin releases oxygen to body tissues; causing:
Inflexible Sickled red blood cells trapped in narrow vessels
Blood Flow Slows Down
More RBCs become deoxygenated and Sickled and
Capillaries are blocked.
The obstruction of the blood flow produces temporary or permanent organ dysfunction or structural changes (damage to the organs), leading to multi organ damage all over the body.
This has created other health issues, increasing morbidity and mortality. Unfortunately the victims are all tribal people, a group where literacy is low and financially weak, mostly depends on free government health services available to them, and that is the reason why a special program is needed for Sickle Cell Anemia.

9. 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.
Sickle disease child birth can be prevented through marriage counseling, adolescent screening, antenatal screening and prenatal diagnosis.
Early diagnosis can lead to better management and reduction in morbidity and mortality.
Lives can be saved through proper treatment and care.
Productivity loss can be arrested.

10. Screening Approach Adopted in Program

11. Institutions involved in the program
No.
Institutions
Year
Year 2011 1
Medical College 2
General Hospital 3 12
PHC 44
333 4
CHC 29 70 5
NGO
Total 78
419
Institutions involved in the program
Medical College
General Hospital
PHC
CHC
NGO
Total 11

12. Facilities made available
No.
Facilities available
Year
2011 1
Primary Screening for Sickle Cell (DTT) test, Counseling and Treatment 78
419 2
Hemoglobin Electrophoresis 21 3
HPLC based Hb Variant system for quantitative estimation of different hemoglobin 4
HPLC based Variant-NBS system for New Born Screening for SCA from heel prick dry blood samples from filter paper. 5
Molecular Lab for prenatal diagnosis and Genetic Counseling Center
Primary Screening for Sickle Cell (DTT) test, Counseling and Treatment Facilities available at 78 institutes in and 419 institutes in 2010.
Hemoglobin Electrophoresis facilities available at 21 institutes in and 2010.
HPLC based Hb Variant system for quantitative estimation of different hemoglobin facilities available at 2 institutes in and 3 institutes in 2010.
HPLC based Variant-NBS system for New Born Screening for SCA from heel prick dry blood samples from filter paper is made available at 1 institutes in 2010.
Molecular Lab for prenatal diagnosis and Genetic Counseling Center is made available at 1 institutes in 2010. 12 12

13. Progress of screened person till June-2011
This graph showing year wise community screened for Sickle Cell Anemia in Gujarat, from year 2006 to We have screened about 1,42,950 population in the first year and then increasing in next years. Now we screened more than 3 lacs tribes per year and planned to increase up to 5 lacs per year.

14. Screening Output - I Total Tribal Population 64,70,256
Tribal Population Screened for Sickle Cell Anemia
14,51,936
(22.44%)
No. of Sickle Cell Trait
1,69,358
(11.66%)
No. of Sickle Cell Disease
10961 (0.75%)
From 2006 TO January, ,68,953 tribal population screened for Sickle cell anemia among the total 64,70,256 tribes. Out of them 1,62,706 (11.89 %) were found sickle cell trait and (0.77%)
**Up To June, 2011**

15. 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%)
In year ,230 tribal adolescents have been screened for Sickle cell anemia Out of them 7964 (10.87%) were found sickle cell trait and 1334 (1.82%)
**Up to June, 2011**

16. 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%)
From 2006 TO January- 2011, 94,023 tribal antenatal mothers have been screened for Sickle cell anemia Out of them 3896 (4.14 %) were found sickle cell trait and 123 (0.13%)
**Up To June, 2011**

17. Screening Output - IV PND Couple ** Up To June, 2011**
Result of Prenatal Diagnosis after Genetic Counseling
Total High Risk Couple to whom Genetic counseling for PND Provided
Eligible for Prenatal Diagnosis
Agreed for PND with informed Consent and PND carried out against eligibility
No. of Fetus Negative For Sickle Disease among screened (24)
Fetus Positive for Sickle Disease among screened (24)
Sickle Disease Child Birth Prevented
104 43 24
14+3* 7
100 %
41.34 %
55.81 %
58.33 %
29.16 %
Total 93 High Risk Couple (Both the parent have Sickle gene) found to whom Genetic counseling for Prenatal Diagnosis provided, out of them 39 (42%) were eligible for Prenatal Diagnosis.
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.
Out of this 39 eligible couple, 21(54%) couples were agreed for PND with informed Consent and their PND carried out. 15 (72%) fetus were negative for Sickle Disease and 6(28%) were positive for sickle disease. These children's birth were prevented through MTP.
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)
PND Couple
** Up To June, 2011** 17 17

18. 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.
Total 3660 Newborn Screening for Sickle Cell Disease were done from Dried Blood Spots from Heel Prick on Filter Paper out of them 461(13%) were sickle cell trait and 33 (1%) were sickle disease.
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** 18

19. Marriage Counseling Using Laminated Color Coded Cards
Normal Haemoglobin Card Sickle Trait Card Sickle Disease Card
Laminated color coded cards are given to all screened persons.
These color coded cards are further used for marriage counseling.
Laminated color coded cards are given to all screened persons.
These color coded cards are further used for marriage counseling.
White card to normal person, yellow-white card to Sickle cell trait and yellow card to sickle disease patients.

20. 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.
10,475 Sickle disease patients are given Tab. Folic acid daily by field health workers.
1469 sickle crisis patients were given treatment in year 2010.
1325 blood transfusion were given to sickle disease patients free of cost.
As per survey in 500 sickle cell disease patients, number of crisis is decreased from 2-3/year to 1/year in tribal districts.

21. Established network of counseling centers in tribal blocks.
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.
Activities.
Established network of counseling centers in tribal blocks.
Regular Folic acid tablet to all Sickle disease patients by field worker to avoid complications and compliance of regular medication.
Established mechanism for referral and management of Sickle cell crisis patients.
Free of charge blood transfusion is available all over Gujarat to Sickle Cell Crisis patients (Total 1325 BT have been given). 21

22. IPC and BCC in targeted population PPP and NGO involvement.
Activities
Training and re-training of health care personnel, village health and sanitation committee members, NGO, and PRI representatives.
IPC and BCC in targeted population
PPP and NGO involvement.
Logistic, drugs, and inventory management.
Reporting , Monitoring, Supervision and feedback.
Financial management (SOE, UTC, Input output audit and third party evaluation). 22

23. 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.
Our achievement
We have carried out 21 prenatal diagnosis and prevented birth of 6 Sickle Cell Disease in the Tribal Community.
We have counseled 9,298 sickle positive adolescents and advise them for marriage and future pregnancy.
Students having Sickle cell Disease have been exempted from exercise on Saturday and allowed to go to urinals frequently without asking permission.
The Fruits of the Program has reached to the door step of Last Common Men – The Tribal People. 23

24. 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 Under-served– The Tribal People.
Socio Economic Impact
Before
Large no. of papers were published in Journals & Conferences on Sickle Cell Anemia. But fruits of this Research had not reached to the common public.
Very few tribal were fortunate enough to be diagnosed as Sickle Cell Disease and financially capable of taking regular life time medicine and crisis management.
NOW
The Common Tribal People are most benefited. Today they get regular free medicine at their door step, Crisis are managed at CHCs, Civil Hospitals and at Medical collages. and hence marked reduction in crisis 24 24

25. 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.
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 his is a genetic disease, there is no cure & nothing can be done.
Now
Awareness among medical Practitioners have increased dramatically in respect to Diagnosis & Treatment. Today, Testing, Treatment and Marriage counseling, Antenatal screening and Prenatal diagnosis are being offered by Governmnet as well as private medical doctors. 25 25

26. Extraordinary Features of the program
Reached to the doorstep of most vulnerable tribal population-14,51,936.
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.
Extraordinary Features
It has reached to the most vulnerable tribal population-13,68,953.
Public health challenge, which was so far considered to have no solution, is being addressed.
Active involvement of NGOs.
Capacity building of both Government and NGO staff.
Involvement of international organization like SCDIO and GIOSTAR international.
Application of modern techniques and research in the field situations - stem cell research etc.

27. 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.

28. 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.
According to one of the hypothesis, this mutation has occurred to prevent the entry of Malaria parasites into the cell wall of Red blood Cells.
Most of tribal belt in India being an endemic area for Malaria, the people living in tribal area are also having health problem of Sickle Cell Anaemia.
Taking into our huge population size, more than 50 % of the world’s sickle cell anemia cases are in India.

29. Thank you very much our SICKLERS. 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. 29