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Knowledge, attitude and practice regarding sickle cell disease in adult sufferers and carriers in a rural area

Sickle cell disease is an autosomal recessive, unifactorial or single gene haemoglobinopathy responsible for considerable morbidity and mortality. 1,2 Sickle cell disease is expressed when sickled hemoglobin (HbS) is inherited from both parents i.e. sufferer (SS), while the heterozygous state (AS) is a carrier of disease called sickle cell trait. 3 According to WHO, about 5% of world’s population carries the gene responsible for hemoglobin disorders and about 300000 children are born worldwide with sickle cell disease every year. 4,5


INTRODUCTION
Sickle cell disease is an autosomal recessive, unifactorial or single gene haemoglobinopathy responsible for considerable morbidity and mortality. 1,2 Sickle cell disease is expressed when sickled hemoglobin (HbS) is inherited from both parents i.e. sufferer (SS), while the heterozygous state (AS) is a carrier of disease called sickle cell trait. 3 According to WHO, about 5% of world's population carries the gene responsible for hemoglobin disorders and about 300000 children are born worldwide with sickle cell disease every year. 4,5 In India, HbS was first detected in Veddoid tribe in Nilgiri hills of Tamilnadu, in 1952 by Lehman and Cutbush. 6 The incidence varies from 5% to 34% and it is mainly restricted to the tribal population. 7 In India, it is the second most common haemoglobinopathy, next to Thalassemia. 8 According to State Health Society; there are about 30 lakhs sickle cell carrier and 1.5 lakh sickle cell disease sufferers in Maharashtra. Out of these, about 10.5 lakh sickle cell carriers and 70 thousand sickle cell disease sufferers belong to the tribal population. 9,10 Sickle cell patient presents with anaemia, recurrent episodes of pain, recurrent infections, jaundice, stroke and delayed growth. Complications of sickle cell disease include serious infections, damage to vital organs, stroke, kidney damage, respiratory problems, bone marrow failure, growth failure, cognitive impairment, Progress in the scientific knowledge of SCD and community awareness programmes have made it possible for patients to have access to treatment techniques that have helped many of them to live longer than before. However, misconceptions and misbeliefs still persists that affect the health seeking behaviour of the community There is a need to assess underlying misconceptions because it will help to facilitate changes in service provision. Understanding the knowledge, attitudes and practices will help to fashion appropriate public health education programs to increase awareness and knowledge of the condition.
With this background, a study was carried out to assess knowledge, attitude and practices amongst sufferers and carriers of sickle cell disease in a rural area. Sample size of 232 was estimated from pilot study.

METHODS
Approval from the Institutional Ethics Committee was sought. Permission from District Health Officer, Chandrapur was obtained.
One house in the village was selected randomly and every consecutive house was visited for sickle cell patients aged 18 years or more, till the sample size of 232 was achieved. Informed consent was taken from each study subject after explaining the purpose of the study.
Data was collected by interview method using structured questionnaire in the local language (Marathi). Information regarding socio-demographic characteristics like age, gender, marital status, and knowledge, attitude and practices regarding sickle cell disease was recorded in a pre-designed and pre-tested proforma.

Statistical analysis
Data was analysed using statistical software EPI Info 7. Descriptive statistics (percentage, mean, standard deviation, range) were used to summarise baseline characteristics of the study subjects. Fisher Exact test was used wherever required, p value < 0.05 was considered to be statistically significant.  Out of 232 study subjects, 40 (17.24%) were sufferers and 192 (82.76%) were carriers.  Only 54.31% study subjects had correct knowledge regarding transmission of sickle cell disease. 120(51.72%) study subjects knew that sickle cell disease can be prevented. Overall knowledge regarding transmission and prevention of sickle cell disease was poor in carriers as compared to sufferers.

Mean
When the attitude of the study subjects was assessed more than half of the study subjects disagreed that sickle cell disease is a curse of god and only poor people suffer from sickle cell disease. Amongst the study subjects, 58.18% were undecided regarding sickle cell disease being a genetically transmitted disorder, and felt not much can be done to prevent it. Around 60% of the study subjects agreed that health check-up should be done regularly and there is need to take regular treatment for sickle cell disease.
Amongst the study subjects 56.46%, were undecided regarding repeated blood transfusions can cure sickle cell disease and 34.04% disagreed. Only 18.98% agreed about the need for pre-marital screening of spouse for sickle cell disease while 15.53% study subjects agreed regarding the necessity of pre-natal diagnosis for prevention of sickle cell disease. Majority of the study subjects agreed that all children should be screened for sickle cell disease and sickle cell disease as an illness is less known in society and needs more awareness.
Only 89(38.36%) study subjects were undergoing regular health check-up for sickle cell disease. All the sufferers and only 25.52% of carriers did regular health check-up for sickle cell disease.
Amongst the study subjects 85(36.63%) were taking treatment out of which 40(100%) were sufferers and only 45(23.43%) carriers. In the present study, 121(68.34%) study subjects were aware about sickle cell status of their spouses. In the study conducted by Gamit et al, only 17% patients knew the sickle cell status of their family members. 13 Treadwell et al, reported 12% and Nazir et al, found that 36% patients knew the sickle cell status of their family members. 16,17 In the present study, high proportion of study subjects was aware about sickle cell status as entire population of this area was screened for sickle cell disease.
All married sufferers were aware about sickle cell status of their spouses. However, 36.84% married carriers were not aware about sickle cell status of their spouses. Spouses of 9.21% carriers had expired and spouses of 27.63% carriers were tested but they were not aware about their SCD status.
Sufferers had fair knowledge than carriers regarding precautions to be taken by them to live healthy life like avoiding exertion, daily intake of 8-10 glasses of water, consumption of green leafy vegetables, and avoiding high altitude. This might be as more efforts were focused on sufferers since the launching of the sickle cell disease control program.  13,14,15,17 Amongst the study subjects undergoing regular health check-up, 100% were sufferers and only 25.52% were carriers. This shows that practices regarding regular health check-up for sickle cell disease were not of acceptable level in carriers.
In the present study, 36.63% study subjects were taking treatment. This is in accordance with the findings of Gamit et al, Coretta et al and Treadwell et al. 13,15,17 Practices of study subjects regarding precautions taken to live healthy life were good in sufferers but not of acceptable level in carriers. Only 28% sufferers and 3.28% carriers had done pre-marital screening of their spouses for sickle cell status. 77.96% study subjects had got their spouses screened after knowing their positive sickle cell status. Children of all study subjects were screened for sickle cell disease.
This study shows that knowledge regarding cause, types of sickle cell disease, sickle cell crisis, precautions to be taken to live healthy life, treatment and prevention of sickle cell disease is satisfactory in sufferers but not of acceptable level in carriers. Overall attitude of the sufferers as well as carriers towards treatment and prevention of sickle cell disease is more or less positive.
All sufferers are taking treatment, however almost half of the carriers are not taking treatment of any kind. Practices related to pre-marital screening of spouse and pre-natal diagnosis of sickle cell disease is infrequent in both sufferers and carriers.