Sickle cell anaemia a red blood cell hereditary disorder is common amongst Scheduled Caste, Scheduled Tribes, Nomadic Tribes and other backward class from state of Maharashtra. The disorder is very common amongst these population groups from Chandrapur, Gadchiroli and Nandurbar districts of Maharashtra state. In this geographical area on an average one out of five person is Heterozygotes (trait or carriers) and more than 1% Homozygote suffering from disease (sufferer). For last twelve years, we are working on this disorder. Up till now we identified 1316 Sickle cell anaemia patients and most of them are under our medical supervision. We have selected one Taluka (Block) from Nandurbar district known as Dhadgaon Taluka and with co-operation of tribal youths established Sickle Cell Centre which provides following facilities.
a) Diagnostic facilities
b) Possible treatment and follow up.
c) Population genetic screening programme to identify carrier and sufferer.
d) Health education.
e) Marriage counselling
f) Guidance for Pre natal diagnosis
g) Guidance for Family Planning
h) Training programme
Initially we were using Allopathic drugs like Folic Acid & NSAIDS as a routine treatment and during crisis period Paracetamol and Soda mint tablets. After two years of initial studies we switch on to Ayurvedic drug for following. It is an assumed that Sickle Cell disease is as old as Ayurvedic Science (about 3000 years old). We thought that there is likely to be treatment in Ayurvedic Science. Since Maharashtra Arogya Mandal basically has been established on Ayurvedic system. We tried to search on the basis of signs and symptoms of the disease, the nearest possible drugs and found that few ayurvedic drugs like Bilwa (Aegle marmelos) and others are useful. We found good response from patients. The general condition of the patient is improved; there is improvement in anemic condition and less severity during crisis. The drug contains about 20% Urea and probably might be acting as antisickling agent.
Sickle cell anemia is a hereditary defect confined to red blood cells. The basic defect is in the structure of hemoglobin molecule of the red blood cells. These cells acquired sickle like shape in oxygen deficient environment. Due to this effect there is early destruction of the cells leading to condition known as sickle cell anemia.
The individual with sickle cell anaemia has common symptoms
This symptom begins at early age (3 to 4 years) and severity of symptoms increases with age. Patient has painful and limited life span. In addition to these symptoms one of the typical symptoms experienced by majority of the patients is known as Sickle Cell Crisis. Whenever there is increase demand for oxygen in the body i.e. during infections, heavy physical exercise, exposed to extreme cold or hot summer or excess fluid loss, the sickling process gets accelerated. The sickled red cells entangle with each other and may cause obstruction. When blood flows through micro capillaries, the obstruction may stop further blood supply developing ischemic condition and causes tremendous pain at that site. The crisis episode varies from individual to individual. When, where, how crisis develop is difficult to predict. The frequency and period is also variable. Sometime it is once in a month or more or less. Pain remains for few minutes or few hours or few days. During crisis, pain is unbearable and pain killer drugs have limited effect. Patient cannot bear the pain and are helpless. Parents cannot bear patient's painful condition. Ultimately whole family get disturbed, worried and scarred about episode. However it is noticed that few patients do not develop crisis throughout the life. It is observed that patients have less immunity and hence more susceptible for common infections. With the chronic illness and repeated episodes of crisis every organ of the body is likely to be affected, including brain with stroke, the lungs with respiratory failure, the kidney with renal failure and Avascular necrosis of bones. The long-term studies emerge out following observations.
Hemoglobin is a protein molecule found in the red blood cells and is responsible for transport of oxygen to different parts of the body. The short form for this molecule is designated as Hb. It is compound protein and consists of heme and globin components. Heme is iron protoporphyrins and globin part is made up of two pairs of identical subunits (tetramer). The subunits are polypeptide chains and also known as globin chains. Hb is labeled as alpha (α), beta (β), gama (γ) and delta (δ).α chain consist of 141 amino acids, each attached to other amino acid with specific sequence, which is characteristic to the chain, while β, γ and δ are all three consists of 146 amino acids each. The difference between β, γ and δ chains is well-organized amino acid sequence, different for different chains and very much characteristic to each chain. The red blood cell of adult healthy individual consists of mixture of three types of hemoglobin's known as adult (Hb A), Fetal hemoglobin (Hb F) and hemoglobin A2 (Hb A2). HbA is major hemoglobin (about 96%) Hb F and Hb A2 are minor hemoglobin's with concentrations of about 2% each. Each hemoglobin protein is made up of four globin chains and to each globin chain one heme group is attached. Globin part of Hb A protein is made up of 2 α chains and 2 β chains (2 α 2 β) similarly globin part Hb F is made up of 2 α chains and 2 γ chains (α 2 γ 2) While in Hb A2 it 2 α chains and 2 δ chains (α2 δ 2).
Sickle cell hemoglobin (Hb s) is mutant form of adult hemoglobin (Hb A). It differs in organization of sequence of amino acids in β chains the difference is the sequence of single amino acid i.e.6th amino acid in normal β chain in glutamic acid while is replaced by another amino acid valine in sickle β chain. This is known as single gene defect. The gene for β globin chain is located on short arm of chromosome No. 11. Family studies of the sickle cell patients suggest that is follows Mendeline Rules of inheritance and defect is transmitted from one generation to other in autosomal recessive manner. In a population the defect occurs in two forms, one heterozygous state (suggesting defect on only one chromosome of the pair) and another homozygous state (suggesting defect of both chromosome of the pair). Heterozygote individual usually do not suffer from any symptoms of the disease and hence known as carrier and homozygote individual always remain ill and known as sufferer. In depth studies of sickle cell defect suggest following guidelines.
As per recent information available Maharashtra State ranks second in population and area-wise third in the country. As per 2001 census the total population of the state likely to be 10 crores and this is about 10% of Indian population. The total tribal population of the state is 10% of the total and will be about (1 crore). There are three mountain ranges in the state known as Sahyadri, Satpuda and Gondwan ranges. Majority of tribal population groups from the state of Maharashtra are inhabitants in these geographically difficult hilly ranges. There are in all 47 different tribal population groups, 17 are major tribal groups. Madia from Gadchiroli District, Kolam from Yeotmal District and Katkari from Raigad District are primitive tribal groups. Bhilla, Pawara from Satpuda. These tribal groups differ from each other in various aspects. They differ in language they speak, in their cultural pattern and socio-economic categories. Inter tribal groups marriages not permitted. Majority of groups remain isolated, untouched by civilization. Hence these groups remain backward in all aspects of life including health and education.
Newly created Nandurbar district is separated from original Dhule district. It consists of six Talukas. 66% of the population of district belongs to two major tribal groups known as Bhill and Pawara. These two groups differ from each other in language they speak, cultural pattern and socio economically. Generally inter group marriages are not permitted. These tribal groups are found either at the foot of hills of Satpuda ranges (Akkalkuva Taloda and Shahada taluka) or in between third, fourth, fifth and sixth ranges. (Dhadgaon and part of Akkalkuva Taluka) There is no proper communication with rest of the state because of geographically difficult hilly terrain and remain backward with poor quality of education and heath facilities.
A) Malnutrition: Unbalance dietary intake, coupled with poor availability and mal-absorption of micronutrients from routinely consumed cereal based diet. Milk and milk products are not consumed since this is taboo in their society. Net result is malnutrition. Proteins, iron, iodine, Vitamin-A and folic acid deficiencies are common.
B) Water borne and communicable diseases:Lack of hygiene and hence gastrointestinal disorders particularly dysentery and parasitic infections are very common, leading to marked morbidity and malnutrition. Several studies have indicated decrease in the level of rational, hemoglobin and circulating zinc in presence of acute or chronic infections. Anemia or infection appears to result from decrease in iron utilization for hemoglobin synthesis and also reduction in erythropoiesis. Malaria and tuberculosis is still remain a major public health problem in this tribal area.
C) Genetic disorders:High prevalence of genetic disorders mostly confined to red blood cells. Genetically transmitted single gene disorders like sickle cell anemia, red cell enzyme defects and different forms of thalassemia are common amongst tribal population groups from Satpuda Ranges. All these defects found in the red blood cells that ultimately lead to early destruction of the cells. These factors complicate the health problem further and likely to develop refractory anemia.
D) Excess consumption of alcohol:The brewing of alcohol from Mahu (Madhuka indica) flower has been practiced traditionally. However due to scanty rain falls with less availability of flowers people are switching over the commercially available local liquor and is likely to be major threat.
E) Superstitions:Majority of the superstitions is related to food and health problem.
F) Extreme poverty:Very limited source of income. Agriculture (poor) is the only source of income..
G) Poor and inadequate health facilities.:
Amongst the genetic disorder, sickle cell is very common in Nandurbar Dist. Dr. Kate and his team are working on Sickle Cell anaemia for last several years and carried out population genetic surveys in different parts of Maharashtra. The team has screened blood samples of more than 1 lakh population covering different population groups. The team has also screened extensively different Scheduled Caste and Scheduled Tribes groups from Nandurbar District. And data available can be summarized as follows: -
Total Population of Nandurbar District: 16,77,179
Total S.C. and S.T. Population (about): 10, 72, 933
Estimated Sickle cell carrier individuals: 3, 70, 389
(Prevalence 22.5 %)
Estimated sickle cell sufferer approx: > 16,500
(Prevalence 1.2 %)
The prevalence found to be very high amongst Scheduled Cast and Scheduled tribes groups form Nandurbar Dist. The groups are high risk groups since prevalence for sufferer is more than1% these high risk groups are found in geographically difficult hilly areas. Health facilities available at primary health centers, rural hospital and even civil hospital of the district is unable to provide even diagnostic facilities and hence proper treatment is difficult on this background Maharashtra Arogya Mandal have decided to work on this problem and selected difficult taluka of the district and established Sickle Cell Centre at Dhadgaon taluka.
This Taluka is located at the extreme North West side of the state, on the border line of Maharashtra with neighboring states Gujarath and Madhya pradesh. Total geographical area is 32,106 hector. There are in all 163 villages (Revenue 99 + Forest 64). As per 1991 census total population is 96,912 and total tribal population is about 86.96% recent figures available in 2001 census the total population is about 1.4 lakhs and the tribal population is about 96%. Dhadgaon Taluka headquarter is located between the third and forth range of Satpuda ranges.
Majority of the villages are scattered and located in between 2nd, 3rd, 4th, 5th and 6th ranges of Satpuda ranges. There is one road for transport from Dhadgaon to Molagi. Villages situated towards the west side of the road have no proper approach and hence remain isolated. Narmada River is now shifted due to Sardar Sarowar Dam. Bhill and Pawara are two major tribal groups in this area. In all there are 11 Ashram schools run by Govt. and 4 by private institutions. One high school and college (art faculty) located at Dhadgaon, run by private institution. Toranmal is one of the tourist place in the Dhadgaon Taluka. There are about 7 Primay Health Centres and one rural hospital at Dhadgaon.
Our institution i.e. Maharashtra Arogya Mandal, Pune is voluntary heath organization has vast experience of working in tribal areas, expertise knowledge about genetic disorder (prevalent in tribal areas), a team of devoted medical and non-medical staff.
We are working in this Taluka since 1997 and we screened 25 villages for sickle cell disorder and other health problems. We found high prevalence for nutritional anemia and sickle cell anemia. We have mobile laboratory and facilities for blood investigations. We have conducted population screenings programme in 20 villages from this Taluka for sickle cell disorder. On the basis of data collected by our team the rough estimation for sickle cell disorder is as follows.
(As per 2011 figures) - 1, 95,343.
(Prevalence 22.5%) >43,000
(Prevalence 1.2%) - More than 1900
Diagnosis of sickle cell sufferer and carrier is possible by past history, careful clinical examination along with laboratory tests on blood, which are as follows.
a) Solubility test
b) Electrophoresis at alkaline pH, using cellulose acetate membrane as a supporting medium.
Techniques for laboratory investigations, which are simple, reproducible, reliable, and cost effective and requires minimum sample of blood (5 drops) are now available.
Up till now we have identified 1316 patients of sickle cell anemia. We visit every two months, carry out medical checkup of old patients and on the basis of examination provide them Ayurvedic medicines. We also carry out their family studies. Since the treatment given to them gives relief and hence good response from patients. Patients and people are doing mouth publicity and every time our old patient brings with them new patients who have similar symptoms to that of patient. For every visit we have at least 25 new patients.
No curable treatment is available at present. Techniques like Bone Marrow Transplantation, (BMT) Gene therapy, and Hydroxyl Urea treatment are very costly and not affordable to people in whom this is public health problem.
Symptomatic treatment which include following may give some relief
1. Tab. Folic Acid (5 mg) - one daily.
2. Tab Paracetamol (500 mg) 1 t.d.s. For 5 days whenever pain is unbearable
3. Plenty of water to drink
4. Alkalisers like soda mint tablet.
Patient is advised to avoid heavy exercise, exposure to extreme cold and hot. For any illness, it is better to contact local Medical Officer. Blood transfusion usually not required but is better to take advice of Medical Officer. From available literature it was found that sickle cell disease is as old as Ayurvedic Science (about 3,000 Years old) and there must be some drugs prescribed for Sickle Cell anaemia and like wise diseases. We search for literature and find out nearest drug for this blood disorder it is Bilva.
After 2000 we switch on the Ayurvedic treatment, which include followings since we found better response from patient. We give Ayurvedic Medicines tab (SC3) for sickle cell patients and the results are favorable. The formulation is as follows:
1. Kumari Ghanasar - Aloe vera
2. Guduchi Ghanasar - Tinospora cordifolia
3. Bilwa Magaj - Aegle marmelos
4. Bhringraj - Eclipta alba
5. Sharapunkha - Tephrosia purpurea
6. Bhumyamalaki - Phyllanthus niruri
We observed -
1. Hb % is increasing and maintained.
. Crisis has disappear or become milder.
Interval between two crises is increased.
Improvement in general condition weight gain and feeling of well-being
- Bringaraj (Eclipta alba)
- Bhumyamalaki (Phyllanthus niruri)
- Kumari (Aloe vera)
- Sharapunkha (Tephrosia purpurea)
- Guduchi (Tinospora cordifolia)