REVIEW OF LITERATURE. Review of literature:

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1 Review of literature: There are number of studies till date performed on the management of the sickle cell anemia by allopathic or alternative medicine. In autosomal disorder where there is no cure than creating awareness in the youth and making positive impact into the mind of tribal people can at least be helpful in motivating people for utilizing patient counseling and genetic and premarital and neonatal screening facilities provided by the healthcare system. The current review of literature reveals various assessment studies performed on awareness and basic information about the hemoglobin disorder sickle cell anemia. Olakunle et al ( 2013) studied that comprehensive knowledge about sickle cell anemia was found to be low despite good awareness among respondents, but only few knew their hemoglobin genotype. If sickle cell disease control strategies must yield any significant results than there is a need to raise awareness about sickle cell anemia, especially among students in secondary institutions in Nigeria is recommended. Olatona FA (2012) studied the effect of health education program on the knowledge and attitudes to sickle cell disease and its screening among unmarried NYSC members in Lagos State. The study concluded that health education of youth corps members was significantly effective in improving their level of knowledge, their attitude to sickle cell disease & screening uptake. Study revealed that sustained health education through school curriculum, mass media and health institutions are relevant to influence new graduates to have better knowledge and attitudes towards sickle cell disease and hence enable them to make informed decisions about pro-creation later in life. Bazuaye G.N & Olayemi E.E. (2009) studied with 850 senior secondary school students in the urban areas of Benin City. The study has shown that majority of the senior secondary school students are in the premarital phase. The result of the study was majority of the students do not know their hemoglobin genotype and have a poor understanding of the disease and some will stigmatize patients with sickle cell disease. There is a need to encourage the education of student about the disease and encourage premarital hemoglobin genotype screening even at a lower level of secondary school. Dept of Pharmacy JJT University Page 3

2 Abubakar S et al (2010) studied that there is a deficiency in the perceptions about sickle cell disease and its prevention among undergraduates of tertiary institutions in Kano. The study concluded that there is a need to provide health education about sickle cell anemia prevention to youths in schools and through other media as well as strengthen prenatal screening and premarital counseling & testing services. The study should include the counseling of affected individuals and their education on their condition. Adeyemo et al (2007) studied the level of awareness about genetic counseling in 30 hospitals. A general informal genetic counseling took place mostly occasionally in the hospitals visited. 122 (86%) individuals have had the knowledge of genetic disease and only 43 (30.3%) individuals have been exposed to genetic counseling. 64% of individuals agreed that genetic counseling would help in the prevention of genetic disease. Kamble M & Chatruvedi P (1999) studied the epidemiology of sickle cell disease in pediatric age group in a rural hospital of central India and found sickle cell disease is prevalent in this area and most cases present before 5 year of age. Vaso-occlusive crisis is the commonest crisis seen but death often occurs due to sequestration crisis and usually below 5 years of age. Gamit C et al, (2014) assessed the Knowledge, attitude & practice about sickle cell anemia in patients with positive sickle cell status. 16% of the study participants knew correct symptoms of sickle cell anemia and 30 % patients were availing the treatment of some kind. But approximately 90% didn t know the cause of disease and only 18 % were counseled about this disease. More than 95% of the participants were unaware regarding their hemoglobin status. All the strategies for the prevention of this outrageous disease will be effective only if they are utilized to its maximal extent by creating more awareness to the affected and also the other population. Brindha B et al (2013) mentioned that there is a need to map the prevalence of this gene like its variation in different tribal groups and within tribal group that spread over a large area. Out of 55 village panchayats 33 villages are identified as infected with sickle cell anemia in which female incidence was observed high when compared to male with carrier state. Even though the disease impact in terms of speed of spread varies with demographic profile helps in also identifying the most exposed or effected tribe in the study area. Dept of Pharmacy JJT University Page 4

3 Deore A.U and Zade S. B. (2013) studied the current status of sickle cell disease in urban population of eastern part of Maharashtra State in India. Total 3479 subjects belonging to 40 ethnic groups including 29 non tribal and 8 tribal and three migratory groups were sampled. The overall prevalence of sickle cell trait was found to be 4.94% of which genotype Hb-S and Hb-SS were found to be represented by 3.88% and 1.06% respectively. Treadwell et al. (2006) surveyed 282 people from northern California about their exposure to and knowledge of sickle cell disease and sickle cell traits. Almost 68% of those interviewed in that study responded correctly to knowledge questions about sickle cell disease. There were 15% of the respondents only aware of their own sickle cell trait status. A majority of 30 respondents reported that they received sickle cell disease testing at their local community or hospital or clinic Chen, (2010) mentioned that increase in interracial marriages among the millennial generation 18 to 29 year olds had occurred. Research showed that about 85% of the millennial generation accepts interracial marriages and more people from this age group are producing a new generation of multi-racial children. As interracial marriages are a growing trend, which lead to a need for more research on interracial marriages and how they correlate with sickle cell anemia. Italia K et al (2008) studied the efficacy and safety of hydroxyurea in Indians with severe manifestations where the βs gene is linked to the Arab Indian haplotype and is associated with higher Fetal hemoglobin levels and found that Hydroxyurea was effective in reducing the clinical severity in Indian patients who initially had higher Fetal hemoglobin levels and the presence of ameliorating factors, such as α-thalassemia and the Xmn-I polymorphism. Hydroxyurea with careful monitoring can thus change the quality of life of Indians with sickle cell disease Buchanan G et al (2010) mentioned that great studies have been made in conservative management of sickle cell anemia. However, the medical and psychological cost of supporting patients with this disease is enormous and spans a lifetime. The hematopoietic stem cell transplantation can abrogate sickle cell anemia manifestation and is the best option for cure today but this treatment modality is underutilized as less than 500 transplants were reported in the centre for international blood and marrow transplant research because of the significant risk of morbidity and mortality. Dept of Pharmacy JJT University Page 5

4 Maluf S. et al (2008) studied geno-toxicity associated with Hydroxyurea dose and treatment length by evaluating muta-genicity in sickle cell anemia patients and found that increase number of micronuclei is associated with sickle cell Hydroxyurea group which confirm that patients with sickle cell anemia treated with Hydroxyurea should be carefully monitored to reduce the risk of carcinogenicity. Prabhakar et al (2009) in his study mentioned that a lack of thorough understanding of the pathophysiology of sickle cell anemia as well as observing that primary focus of sickle cell anemia clinical care revolves around treating the complications of the disease rather than focusing on prevention of complications of the disease. Serjeant et al (1997) studied that reoccurrence rate for stroke for children within 3 years of age in sickle cell anemia diagnosis is 50% to about 70 %. The study also reveals that by age of 14, a stroke has affected at least 8% and by age of 20 it has affected 11% of sickle cell patients. Fawibe et al (2008) reported that acute chest syndrome occur in 50% of sickle cell disease patients and reoccurred in approximately 80% of sickle cell disease individuals. The acute chest syndrome accounts for 25% of premature death in sickle cell anemia patients. Steinberg et al (1999) mentioned that bone marrow transplantation is limited to candidates below 16 years of age and who exhibit sickle cell disease complications like stroke, acute chest syndrome, and refractory pain. But only 1% of patients with sickle cell anemia actually meet the set parameter of transplantation. NIH (2002) mentioned that some sickle cell disease patients do not survive the chemotherapeutic process or period prior to the transplant whereas other patients may suffer from the lifethreatening infections before their bone marrow and immune system are regenerated. Yang et al (2000) suggested that many factors contribute to the lack of follow up counseling and screening and these factors include anticipatory anxiety on parent s part about learning that their infant had a sickle cell, denying and fear of being responsible for causing the disease. Dept of Pharmacy JJT University Page 6

5 Ogmaldi in (1994) concluded that individuals between ages of 19 to 30 lack of understanding of basics of sickle cell anemia. That why a need exist for more sickle cell anemia screening and education and counselling among the teen age students. Wojciechowski E A (2002) assessed treatment compliance, independence, and whether they receive uninterrupted care of sickle cell disease patients. The results indicated that patients leave pediatric care without adequate transfer preparation and readiness to transfer is not the major consideration in the decision to transfer. Follow-up often ceases once the patients leave pediatric care and patients who maintain follow-up appointments are more efficient in managing their illness. Hussain R. Yusuf et al (2011) found that there has been some progress in the development of sickle cell anemia related public health activities like newborn screening for sickle cell anemia. However still additional areas needs focus include strengthening surveillance and monitoring of disease occurrence and health outcomes, increasing knowledge and awareness among affected people, enhancing adherence to health maintenance guidelines and improving healthcare access and utilization. Mpiana PT et al (2006) studied 30 aqueous and ethanolic extracts from 13 congolese plants for their anti-drepanocytary activity. Twelve of these plants, Alchornea cordifolia leaf, Afromomum albo violaceum leaf, Annona senegalensis leaf, Cymbopogon densiflorus leaf, Bridelia ferruginea leaf, Ceiba pentandra leaf, Morinda lucida leaf, Hymenocardia acida leaf, Coleus kilimandcharis leaf, Dacryodes edulis leaf, Caloncoba welwithsi leaf, & Vinga unguiculata leaf exhibited significant activities, and thus, suggesting a possible correlation between the chemical composition of these plants and their uses in traditional medicine. Noll R B et al (1996) compared with measures of peer relationships and emotional well-being was made between youngsters with sickle cell disease and same-classroom comparison peers. Despite of chronic exposure to numerous stressful life events associated with sickle cell disease the youngsters with the illness were remarkably similar to comparison peers and showing evidence of considerable hardiness. Dept of Pharmacy JJT University Page 7

6 Al Arrayed, S. & Al Hajeri, A. (2010) studied a general population awareness service and had an excellent response rate in the study and found a good level of awareness regarding sickle cell anemia in study sample. Result suggested that some of the respondents were confused about the difference between the carrier state of a disease and the affected state. Almost all the participants support and appreciate the preventive campaigns being conducted in Bahrain such as the premarital service and the student screening program. The most remarkable and rewarding results were the ones that proved the effectiveness of the screening programs in increasing the awareness among the public about these common Hemoglobinopathies especially sickle cell anemia. Vasava B et al (2009) studied to create awareness screen samples of school adolescents and then to reach their community through them by doing surveillance for sickle cell disease. It was field based cross-sectional type of study. The study concluded that approaching community can be possible through school adolescents for conduction of surveillance of sickle cell anemia. Dept of Pharmacy JJT University Page 8