Sickle Cell Anemia Control Program- A New Initiative of Government of Gujarat Best Health Practices in Gujarat Health & Family Welfare Department, Gujarat Page 1 of 8
EXECUTIVE SUMMARY Sickle cell Anemia (SCA) and its variants are genetic disorders resulting from the presence of a mutated form of hemoglobin, hemoglobin S (HbS). The most common form of SCA is homozygous HbS disease (HbSS), an autosomal recessive disorder first described by Herrick in 1910. In 1952 Lehmann & Cutbush reported Hb-S in tribal population of Nilgiri in south India. Sickle Cell gene is mainly present amongst tribal group, from malaria endemic forest areas. According to one of the hypothesis, HbS is a natural mutation in Hemoglobin molecule to protect RBCs from malarial parasites by making them a little rigid, so that malarial parasites cannot enter into RBCs. Sickle shaped cells often get entangled resulting in blockage of the blood vessels leading to severe health complications. The diagnosis of SCA is done by simple blood test known as Sickling test on RBCs. Hb electrophoresis / HPLC technique finds out whether the person is Sickle Cell Trait (heterozygous) or Sickle Cell Disease (homozygous). India does not have any comprehensive national program to address the problem of sickle-cell disorder and in most states; sickle-cell disease receives scant attention from government health services. Department of Health and Family Welfare, Government of Gujarat had initiated the Sickle Cell Anemia Control programme in five districts of the state in Year 2006. Today, the programme has been extended to all 12 tribal districts of Gujarat. As Sickle-cell disease is incurable; strategy to deal with the disease focuses on prevention and early diagnosis to ensure effective management of the disease and put off a crisis situation. Till March 2011, a total of 13, 96, 904 tribal people have been screened through the programme, identifying 10, 673 sickle disease patients to whom adequate treatment, counseling and guidance is ensured. Similar control program can be replicated in other parts of our country where the disease is prevalent. This document seeks to encourage and provide assistance in any such adaptation. Background The normal human Red Blood Cells (RBCs) carries adult hemoglobin (Hb-A), which helps RBCs in transportation of oxygen in the body. Sickle Cell Anemia (SCA) occurs due to inherited abnormal hemoglobin (Hb) gene, which produces Hb-S (Hb-Sickle). Here, presence of Hb-S, converts RBCs into rigid-brittle half moon (Sickle) shaped instead of soft round biconcave shape on transferring oxygen to body tissues. The rigidity and abnormal shape reduce their ability to be propelled through tiny capillaries & formation of entangled masses of cells in larger blood vessels. This obstructs the blood flow into organs, producing temporary or permanent organ dysfunction or structural changes and retarded growth. Because of their abnormal shape, the spleen in the body destroys these RBCs, causing enlargement of spleen. Life span of RBC in SCD is less Page 2 of 8
than 30 days instead of 90 to 120 days. Anemia results from the bone marrow s inability to produce enough blood cells to keep pace with the rate of destruction. People affected by SCA are frequently misdiagnosed and mistreated. This is not a common iron deficiency (nutritional) anemia although undiagnosed SCA patients are mistreated with iron therapy. Their hemolytic jaundice due to excessive break down of red blood cell is treated as infective hepatitis. Their joint pains are treated wrongly as arthritis. Their murmurs due to anemia are wrongly treated as rheumatic fever. As screening of SCA is not a common practice, diagnosis is usually made when a patient presents with a severe complication. Epidemiology The highest frequency of Sickle Cell Disease is found in tropical regions, particularly sub-saharan Africa, India and the Middle-East. Three quarters of total Sickle-Cell Disease cases of the world occur in Africa where in few parts, the prevalence of Sickle Cell Trait (heterozygous) is as high as 30%. It is also found in some parts of Sicily, Greece, Southern Turkey, all of which have areas in which malaria is endemic. Migration of substantial populations from these high prevalence areas to low prevalence countries in Europe has dramatically increased in recent decades and in some European countries sickle cell disease has now overtaken more familiar genetic conditions such as hemophilia and cystic fibrosis. Africa: A recent WHO report estimated that around 2% of newborns in Nigeria were affected by Sickle Cell Disease, giving a total of 150,000 affected children born every year in Nigeria alone. The Sickle Cell Trait frequency ranges between 10% and 40% across equatorial Africa, decreasing to 1 2% on the North African coast and <1% in South Africa. United States: Sickle Cell Anemia is the most common inherited blood disorder in the United States; about 8% of African Americans (two million people) are Sickle Cell Trait. France: SCD has become the most common genetic disease in this country, with an overall birth prevalence of 0.04% in mainland France. United Kingdom: 0.05% babies are born with SCD. Middle East: In Saudi Arabia about 4.2% of the population carries the sickle-cell trait and 0.26% has sickle cell disease. In Eastern province approximately 17% of the population carries the gene and 1.2% has sickle cell disease. Sex distribution: No sex predilection exists, as sickle cell anemia is not an X-linked disease. The male-tofemale ratio is 1:1. Page 3 of 8
Sickle Cell Anemia in India: Sickle cell disease (SCD) with an estimated 5,200 live births each year is a major public health problem in India. Prevalence of Sickle Cell gene is as high as 5 to 34 % in various scheduled tribes, who have socio-economic disadvantage and are frequently medically underserved. As our country has tribal community about 180 million it is expected that there may be 18 million sickle cell trait and 1.4 million sickle cell disease patients. Sr. No Subject Estimated Prevalence of Sickle Cell Anemia in Gujarat and India Gujarat (Census 2011) India ( Census 2011) 1 Total Population 6,03,83,628 1,21,01,93,422 2 Tribal Districts 12 593 3 Tribal Population @ 14.76% 89,12,623 17,86,24,549 4 Suspected Sickle Trait @ 10.0 % 8,91,262 1,78,62,455 5 Suspected Sickle Cell Disease @ 0.75 % 66,845 13,39,684 Prevalence of Sickle Cell gene (%) in Some States of India Sr. No State Prevalence Sr. No State Prevalence 1 Madhya 6 0-48.5 Pradesh Gujarat 0-30 2 Maharashtra 0-45.4 7 Kerala 0-29.7 3 Tamil Nadu 0-35.3 8 Karnataka 0-25 4 Andhra Pradesh 0-34.6 9 Orissa 0-12.4 5 Uttar Pradesh 0-32.6 10 West Bengal 0-1.1 Sickle Cell Anemia Control Program of Gujarat: Gujarat has 89.12 lakh tribal population and is expected to have at least 9,00,000 Sickle Cell Trait and 70,000 Sickle Cell Disease patients. The Dhodia, Dubla, Kukna, Gavit, Chaudhary, Halpati, Varli, Kokni, Kathodi, Kolcha, Kotwadia etc. are among the major tribes having Sickle Cell problem in Gujarat. Page 4 of 8
According to ICMR survey amongst the primitive tribes of south of Gujarat, viz; Kolcha, Kotwadia & Kathodi; 30 % of Sickle Cell Disease children die before they reach adulthood (14 years) and the remaining 70 % die by the age of 50. Gujarat has incorporated Sickle cell anemia control programme in the existing health services of State Government. During 2005-06, a Comprehensive Sickle Cell Program based on public-private partnership was started in Surat, Navsari, Valsad and Dangs. It was extended to all 12 tribal districts of Gujarat in 2008. The program helps in early diagnosis New Born Screening (NBS), Antenatal Screening, treatment and counseling of Sickle Cell Disease patients including prevention by marriage counseling and prenatal diagnosis as per WHO strategy. Gujarat Sickle Cell Anemia Control Society (registered under society registration act 21 of 1860) is formed in year 2011 to fulfill following goals and objectives. Goals: 1. No Child birth with Sickle Cell Disease by 2020 2. Prevention of death from Sickle Cell Crisis 3. To improve health status and quality of life of Sickle Cell Anemia patients. Objectives: 1. To integrate all the activities done by different departments and NGOs in Gujarat for prevention and control of Sickle Cell Anemia under one umbrella 2. To achieve the goals of this program in time bound manner. 3. Focus not only on the tribal person in the tribal districts but to reach tribal person in entire Gujarat state. Hon'ble Health Minister is the Chair and Hon'ble Tribal Development Minister is Co- Chair of the Society. The Society aims to complete the screening of remaining tribal population in next three years to understand actual burden of this disease and district wise screening work has been outsourced among interested, qualified and competent agencies. Process Flow The Sickle Cell Anemia Control Program uses a three-fold approach to address sickle-cell disorder in the state. The approach involves early screening of patients, administering of treatment and counseling for managing the disease and preventing sickle cell births. It follows a PPP model wherein the Government of Gujarat works in partnership with NGO s/ Private Agencies for screening. The tribal people that the program caters to are given free of cost disease screening, treatment and counseling facilities. Page 5 of 8
Screening of Sickle Cell Anemia Experience worldwide suggests that prevention of Sickle Cell Anemia and other hemoglobinopathies requires empowering of community to take informed decision. To facilitate such empowerment, creation of all available facilities is of utmost importance. The Government of Gujarat has developed all required facilities for Premarital Screening, Antenatal Diagnosis and even Newborn Screening for Sickle Cell Anemia. The screening under this program covers the all -from new born to old age people. The main target group for screening is the adolescents, antenatal and new born. As prevention is the only way to keep away this disease, marriages between two Sicklers have to be discouraged. Diagnosing the sickle cell gene in adolescence, creating awareness regarding disease and marriage counseling is the mainstay of the program. Antenatal women in the tribal area are screened for Sickle Cell Anemia at Mamta Divas - a special Health and Nutrition Care Day in village. If mother is found positive for sickle gene, her husband is also screened. Screening of tribal Antenatal cases and their husband in case of positive ANCs will provide us an opportunity to take better care at the time of delivery and also to carry out Prenatal Diagnosis and thereby reducing birth of child with Sickle Cell Disease hence will reduce burden of disease in Gujarat. If husband and wife both found positive for sickle gene, they are advised for prenatal diagnosis. MTP is advised, if fetus is found to have sickle cell disease. If MTP is not opted/ allowed, screening is done by hill prick method to ascertain status of the new born. Premarital or Adolescent screening of tribal individuals, educating them about disease transmission and marriage counseling is high priority area under this program. This group is the focused group because we can prevent the marriages at this stage between to sicklers and prevent the spread of disease in next generation. Screening of the disease in the tribal areas where it is predominant helps in identifying disease patterns and occurrence rates. It also helps in developing treatment strategies. There is evidence to show that early screening of the disease leads to reduced infant mortality and early deaths because if the disease is screened early, comprehensive care can be arranged. Screening for SCA was carried out by Valsad Raktadan Kendra, Valsad and Indian Red Cross Society, Ahmedabad -two dedicated NGO along with Public Health Institutions from year 2006 to July 2012. But the pace of screening was slow (about 3.5 lakh screening/year) and of total 89.20 lakh tribal populations, only 18.43 lakh were screened. Page 6 of 8
Gujarat Sickle Cell Anemia Control Society aimed to complete screening of entire tribal populations by year 2015, so actual burden of disease can be understood and the proper management of the disease patient be done and thus the Goals of the Society can be achieved. In 2012, Gujarat Sickle Cell Anemia Control Society has outsourced screening work by competitive two bid process. After ensuring training to all the staff of these agencies, screening work in villages at field level has started. All Sickle Cell Disease patients diagnosed are put in to the comprehensive care system and have been regularly supplied basic medications like Folic Acid and Pain Killers through sub centers and PHCs. IV fluids, antibiotics etc. are also provided at PHCs, CHCs and at higher referral centers. Many of these patients with severe disease were put on Hydroxyurea therapy. Counselors are trained to provide them care & support with follow up. Sickle Cell Trait gene carriers were provided counseling about their status and were also provided marriage counseling. All medical, paramedical personnel are trained for Sickle Cell Anemia in tribal districts and all the SCD patients are checked by experts once in three months regularly to reduce morbidity. Arrangements were created for supply of free blood and blood products to all these patients as and when required in emergency through Regional Blood Centers and First referral Units. Screening of more than 15 lakhs tribal populations in the first year and screening of all 90 lakhs tribal population of Gujarat will be completed by year 2015. To complete targets within time frame, five more HPLC centers will be established in tribal districts for screening. Counseling: All the Sickle Cell traits and Sickle Cell Disease patients are counseled regularly by full time trained counselors under the program. Family counseling services are provided at the door step of this community. Adolescents are counseled for their marriage and future pregnancy. All the tribal persons are given color coded card according to their Sickle Cell Status, which will help them in marriage decision. After distributing above cards, adolescents are advised to avoid marriages between two yellow card holders to avoid Sickle Disease child birth. Page 7 of 8
Normal Hemoglobin Card Sickle Trait Card Sickle Disease Card Monitoring: Additional Director (Health) is overall technical in-charge for this project and Deputy Director (Epidemic) is a state nodal officer of this Program. At district level, all the activities of Sickle Cell Anemia Control Program are supervised and monitored District Epidemic Medical Officer, who is the district nodal officer for SCACP. Developing Web based software for monitoring of SCACP is under way and Sickle Cell status of all the tribal persons will be made available online through this software. Achievement Commissionerate of Health and family Welfare of Government of Gujarat is proud recipient of "Prime Minister s Award for Excellence in Public Administration" for the activities carried out in 2009-10 in tribal districts under Sickle cell Anemia Control Programme. This along with cash award Rs Five lacs was given to State in ceremony held on 21st April 2011 by Hon ble Prime Minister of India. Page 8 of 8