|Year : 2019 | Volume
| Issue : 2 | Page : 89-94
Awareness of ocular manifestations, complications, and treatment of sickle cell disease in the eastern province of Saudi Arabia: A cross-sectional study
Arwa Mohammed Alshehri1, Kaberi Biswas Feroze2, Moharib Khaled Amir3
1 Medical Intern, College of Medicine, King Faisal University, Al-Ahsa, Saudi Arabia
2 Department of Ophthalmology, College of Medicine, King Faisal University, Al-Ahsa, Saudi Arabia
3 Department of Statistics, Assistant Professor, King Abdulaziz University, Jeddah, Saudi Arabia
|Date of Web Publication||26-Aug-2019|
Dr. Arwa Mohammed Alshehri
College of Medicine, King Faisal University, Al-Ahsa
Source of Support: None, Conflict of Interest: None
| Abstract|| |
AIM: This study aims to assess the level of awareness about the effect of sickle cell disease (SCD) on the eye and vision and factors influencing SCD awareness.
SUBJECTS AND METHODS: The study design was cross-sectional and was carried out in 2018 among the general population in the Eastern province of Saudi Arabia. It was conducted using an online, validated questionnaire, after obtaining consent from the participants. The outcome variable was 557. The level of awareness was correlated to demographic information.
RESULTS: The study population was 557 Saudi adults. 84 were male and 473 female. Their mean age was 22 ± 23 years. Majority of the participants (57.3%) were not aware that SCD could affect the eye and vision. There was no difference in the knowledge regarding ocular complications of SCD among different ages and sexes.
CONCLUSIONS: The result of this study indicates the need for raising the knowledge regarding the disease, its ocular complications, screening methods, and management. Health educations campaigns would be an effective tool in increasing SCD awareness.
Keywords: Awareness, complications, eastern province, manifestation, Saudi Arabia, sickle cell retinopathy, treatment
|How to cite this article:|
Alshehri AM, Feroze KB, Amir MK. Awareness of ocular manifestations, complications, and treatment of sickle cell disease in the eastern province of Saudi Arabia: A cross-sectional study. Middle East Afr J Ophthalmol 2019;26:89-94
|How to cite this URL:|
Alshehri AM, Feroze KB, Amir MK. Awareness of ocular manifestations, complications, and treatment of sickle cell disease in the eastern province of Saudi Arabia: A cross-sectional study. Middle East Afr J Ophthalmol [serial online] 2019 [cited 2020 Feb 26];26:89-94. Available from: http://www.meajo.org/text.asp?2019/26/2/89/265366
| Introduction|| |
Sickle cell disease (SCD) is a recessive genetic blood disorder that targets hemoglobin. According to the WHO, SCD is particularly prevalent among people in sub-Saharan Africa, India, Saudi Arabia, and Mediterranean countries. A study conducted in Saudi Arabia shows the prevalence of 4.2% for sickle cell trait and 0.26% for SCD, but the Eastern province was found to have the highest prevalence rates of 17% for sickle cell trait and 1.2% for SCD., Furthermore, a study conducted in Saudi Arabia, showed that 56% of 11,554 recipients had consanguineous marriages, and this high rate of consanguinity could explain the increased number of patients affected with SCD., Recent developments in the management of SCD like the use of hydroxyurea have increased the life expectancy of SCD patients, and newer fundus imaging techniques have given new insight into the pathogenesis and early recognition of more complications of the disease, such as ocular complications.,, Sickle cell retinopathy, especially the proliferative form, has significant visual morbidity, especially due to its complications such as vitreous hemorrhage and retinal detachment.,, Among the different genotypes, sickle-cell hemoglobin C disease was noted to have the most severe form of retinopathy and this is probably related to blood hyperviscosity. A study conducted in Saudi Arabia about the ocular findings in Saudi Arabian patients with SCD, concludes that SCD in Saudi Arabia affects the retina and represents a potential threat to vision.
The high prevalence of SCD in Saudi Arabia, and especially in the Eastern province, requires that the population should be highly aware of the potential vision-threatening nature of this disorder. There are very minimal data regarding awareness of ocular complications of SCD in this region. The aim of the present study is to assess the level of awareness about the effect of SCD on the eye and vision and factors influencing SCD awareness.
| Subjects and Methods|| |
This cross-sectional study was conducted between August and September 2017 in the Eastern Province of Saudi Arabia. The study was conducted using an online, validated questionnaire distributed to a total of 557 random eligible participants after obtaining verbal informed consent from the subjects about the study and its purpose. It was created by the authors and validated by two senior ophthalmologists in the college. Because of the very high internet accessibility to the general public in Saudi Arabia, we chose an online questionnaire. The questionnaire was prepared as Google form and distributed through WhatsApp, Twitter, and by E-mails. The individuals were contacted by the authors in public places such as malls and colleges, informed about the purpose and significance of this research, assured confidentiality regarding their identity, and the questionnaires were sent to them, only if they volunteered to participate. An oral consent was taken to inform the individuals in person and obtain an informed consent. The first page of the Google form has a section asking if the subject wants to participate in the study. The form automatically closes if the subject replies in the negative. For the study with 95% confidence interval, 5% acceptable error margin and design effect factor of 2, we need at least 385 participants and we increased the number by 45% to have greater precision. The calculation of sample size is done by using http://www.raosoft.com/samplesize.html. The sampling frame was the Saudi adults residing in the Eastern province of Saudi Arabia.
A preliminary pilot study was conducted on 70 participants to check the clarity and reliability of the Arabic version of the questionnaire using Cronbach's alpha. Based on standardized items, the Cronbach's alpha of the translated questionnaire was 0.58, which is statistically acceptable. These participants for validation were later not included in the full study.
The questionnaire was sent to 1000 individuals. 791 individuals responded, of which 557 responses were accepted after applying the inclusion and exclusion criteria.
The inclusion criteria for the study were the Saudi adults residing in the Eastern province of Saudi Arabia. The residents of other regions, non-Saudis, patients with other hematological or chronic diseases and medical and paramedical professionals were excluded from the study.
The study had been submitted and approved by the Research and Ethical Committee of College of Medicine. Data were analyzed using the Statistical Package for the Social Sciences, Version 20 (IBM Corp., Armonk, NY, USA). Mean and standard deviations were calculated for continuous variables, independent t-test was used to determine the statistical significance between two groups, one-way anova was used to determine the statistical significance between three groups or more and percentages were calculated for categorical variables.
| Results|| |
A total of 557 Saudi adults participated in the study, 473 were female (84.9%) and 84 were male (15.1%). More than half of the participants (n = 329; 59.1%) were between 31 and 40 years of age, followed by the age group of 41–50 years (n = 131. 23.5) [Table 1].
Over two-third of the participants (n = 398, 71%) were nonsicklers. Of the remaining 159 participants, 75 (14%) of them were SCD patients and 84 (15%) were carriers [Table 2].
|Table 2: Demonstrating the sickle cell disease status of the participants|
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The majority (n = 363; 65.2%) of the participants had bachelor degree, 124 (22.3%) had high school education, 29 (5.2%) had secondary school education, 15 (2.7%) had elementary school education, 14 (2.5%) had master degree, and 11 (2%) had PhD degree.
In terms of SCD knowledge, 480 participants (86%) were aware that SCD is genetically inherited. However, 215 participants (38.6%) were aware that SCD can affect the eye and 319 (57.3%) had no knowledge. Only 94 participants (16.9%) were aware that SCD can cause permanent blindness. Half of the participants 291 (52.5%) did not know that ocular trauma in SCD patients was more dangerous than normal individuals [Table 3].
|Table 3: Awareness of ocular complications of sickle cell disease among Saudi adults in eastern province (n=557)|
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The level of awareness has neither significant difference between both gender (P = 0.7) nor age group (P = 0.5). On the other hand, there is significant difference in the awareness between different educational levels, in which, there is more awareness in the higher educational level (P = 0.01). When asked about the importance of regular screening for the detection of ocular complications of SCD, 475 of participants (85.3%) correctly answered that annual screening is important. When knowledge about the best method to treat SCD retinopathy was enquired, 330 participants (59.2%) had no knowledge about the treatment of SCD retinopathy [Table 4]. It was also noted that there was no significant difference in knowledge among the sicklers and the nonsicklers (P = 0.21).
|Table 4: Knowledge among the participants about the different methods to treat sickle cell disease retinopathy|
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When enquiry was made about the source of knowledge, the majority of the participants (n = 86, 35.5%) reported that they got the information from the internet [Table 5]. It was noted that 56.4% of the individuals could not answer about the cause of loss of vision in SCD; the others thought that it could be due to damage to the optic nerve (17.6%)/cataract (6.5%)/change in refraction (1.8%). About 17.6% of the participants correctly answered it to be due to damage to the retina.
|Table 5: Source of knowledge of ocular complications of sickle cell disease among Saudi adults (n=557)|
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263 participants (47.2%) thought that the best method to prevent blindness in patients with SCD was by regular screening, while 161 (28.9%) did not know about the prevention method in SCD retinopathy. Many participants answered that good diet prevent blindness in SCD patients (n = 112; 20.1%), few participants thought that exercise (n = 11; 2%) and using proper glasses (n = 10; 1.8%) could contribute to preventing blindness.
Education levels were significantly and positively associated with the knowledge of SCD complications, manifestations, and treatment in this study (P = 0.010) [Table 6].
|Table 6: Significant differences in the knowledge of ocular complications, manifestations and treatment of sickle cell disease retinopathy according to education level among Saudi adults in the eastern province (n=557)|
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Regarding the eye examination in sickle cell carriers and SCD patients, 124 of them (71.3%) never examined their eyes, while 50 (28.7%) examined their eyes to check if SCD had affected their eyes. When asked specifically if they have examined their eyes in the past 12 months, 106 of SCD carrier/patients (61.6%) did not have eye examination and 66 (38.4%) examined their eyes.
One-way anova test was used to measure the difference between the age groups in their knowledge. This test shows no difference between age groups in their knowledge [Table 7].
|Table 7: One-way ANOVA test for variables (age group and level of knowledge)|
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| Discussion|| |
The present study was found to be the only one to assess the knowledge of ocular complications of SCD in the Eastern province of the Kingdom of Saudi Arabia. The PubMed and Cochrane databases were searched to look for other similar researches in this area, but none was retrieved. There was an estimate of the prevalence of SCD done by The Saudi Premarital Screening Program between 2004 and 2005 and a total of 488,315 individuals were screened, 4.20% had sickle cell trait and 0.26% had SCD with the highest prevalence noted in the Eastern province. The high prevalence of ocular complications was observed more in HbSC genotype, which is a consequence of its relatively benign course.,
Our study had 557 participants, while 450 Omani adults participated in a study conducted in Oman. More than half of the candidates (59.1%) in our study were between the 31 and 40 years while 53.8% of participants in Omani study were between 18 and 29 years of age.
More number of females responded to the questionnaire, which could be due to the more responsiveness of females and readiness to participate in health surveys.
38.6% of the population in our study were aware that SCD can affect the eye, whereas 20% of the population in a study conducted in Oman and 36% of a Jamaican study were aware that SCD can cause organ damage., The difference in knowledge between the present study and the Omani study could be explained by the fact that the prevalence of sickle cell trait and SCD in the Eastern province of Saudi Arabia were 17% and 1.2%, respectively, which is higher than the prevalence of sickle cell trait and SCD in Oman was 6% and 0.2%. Increased prevalence probably leads to improve awareness. In contrast to the Jamaican study, our study showed no difference in the knowledge between the age groups, whereas important gaps were identified in the adolescents' knowledge and understanding of presentations and complications associated with SCD in the Jamaican study. Sickle cell trait was found in 1 in every 10 persons in Jamaica and the high awareness among the Jamaican population was attributed to the high prevalence and high general education in various studies.,
Educational levels affected the knowledge regarding SCD retinopathy in our study, which was similar to the Omani study which revealed that education level was the only sociodemographic factor significantly associated with mean total knowledge scores. According to the positive relationship between educational level and more awareness about the disease, awareness campaigns, both online and through brochures and leaflets would go a long way in increasing the knowledge of the population and improve coping skills. Studies in Jamaica have proved conclusively that the high awareness levels are related to the high general educational levels.
A study showed that SCD can affect both the anterior and posterior chambers which eventually lead to visual impairment. About 72% of the study population were not aware that SCD can cause permanent ocular damage, which is a cause of grave concern, as retinopathy is a serious complication, and an Egyptian study mentions sickle cell retinopathy as the most common complication of SCD.
Only 47% of the respondents were aware that regular screening is the best way to prevent the blinding complications of SCD as recommended in a study conducted in Egypt which advised for a periodic ophthalmologic examination starting at the age of 12 years to identify the retinal lesions thus minimizing the risk of threatening retinopathy.
71.26% of sickle cell carriers and 28.74% of the SCD patients who participated in this study never had their eyes checked. This is a worrying trend. Annual ophthalmological examination in adults and biennial examination in children with SCD should be strictly enforced. Yet another study confirms the early retinal changes in sickle cell anemia and SCD patients and supports the need for ophthalmological monitoring in early childhood to avoid the progression of proliferative sickle cell retinopathy to blindness. Studies recommend referral of individuals with SCD to a retinal specialist from the age of 10 years for retinal evaluation, with re-examination every 1–2 years for those without evidence of retinopathy. Additional retinal investigations like FFA are recommended in case of detection of retinal changes.,
Saudi Arabia has a policy of mandatory premarital screening, which is a strong measure to increase awareness and also is a step to reduce future prevalence of SCD in the population. Premarital screening was found to significantly reduce the number of at-risk marriages and it is hoped that it would reduce the disease load in the future.
Despite the high prevalence rates of SCD and trait among the Eastern province of Saudi Arabia, the present study shows a less than expected awareness levels. It is concluded that awareness levels among the public have to be raised to increase knowledge regarding ocular screening, early detection, and prompt management. Increasing the knowledge of the chronic disease has been reported to improve self-management. The use of campaigns, (including mass media campaigns) flyers and posters in public places would be very effective in raising awareness among the general population, as demonstrated in other studies. Due to the high internet penetrance in the Saudi population, which is almost 91%, it is proposed to utilize the internet as a source of dissemination of information regarding the eye problems in SCD. Studies have demonstrated the patient preference and effectiveness of mobile-based health applications in improving health awareness in different studies in Saudi Arabia.,,
This study is probably the first of its kind in the Eastern region of Saudi Arabia. However, the limitations of the study were thought to be a difference in the number of female and male participants and the inability to reach all strata of the general population as the study mainly focused on the population in public places. Although the high internet penetration in Saudi Arabia encouraged the authors to use an online questionnaire, it could be a limitation in reaching all the members of the population.
| Conclusion|| |
Considering the high prevalence of SCD in the Eastern province, the level of knowledge about the ocular involvement, complications and management is quite low. Awareness levels among the public has to be raised to increase knowledge regarding ocular screening, early detection and prompt management. This will help to reduce the visual morbidity from this condition. Health education would play a major role in this direction. Awareness campaigns, both online and through printed materials will go a long way in achieving this aim.
We would like to acknowledge Dalal Al-Sadoon, Fatimah Al-Ghamdi and Nourah Bonais for their participation in distributing the questionnaire. Furthermore, we would like to thank all the participants for their responses and providing information needed to come up with this study.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Creary M, Williamson D, Kulkarni R. Sickle cell disease: Current activities, public health implications, and future directions. J Womens Health (Larchmt) 2007;16:575-82.
Jastaniah W. Epidemiology of sickle cell disease in Saudi Arabia. Ann Saudi Med 2011;31:289-93.
] [Full text]
Zaini RG. Sickle-cell anemia and consanguinity among the Saudi Arabian population. Arch Med 2016;8:3.
Alotaibi MM. Sickle cell disease in Saudi Arabia: A challenge or not. J Epidemiol Glob Health 2017;7:99-101.
Do BK, Rodger DC. Sickle cell disease and the eye. Curr Opin Ophthalmol 2017;28:623-8.
Amissah-Arthur KN, Mensah E. The past, present and future management of sickle cell retinopathy within an African context. Eye (Lond) 2018;32:1304-14.
Pahl DA, Green NS, Bhatia M, Chen RW. New ways to detect pediatric sickle cell retinopathy: A comprehensive review. J Pediatr Hematol Oncol 2017;39:618-25.
Scott AW. Ophthalmic manifestations of sickle cell disease. South Med J 2016;109:542-8.
Soriano M, Aguirre G, Gordon M, Graversen V. Diagnostic Atlas of Retinal Diseases. Sharjah: Bentham Science Publishers; 2017.
de Melo MB. An eye on sickle cell retinopathy. Rev Bras Hematol Hemoter 2014;36:319-21.
Lemaire C, Lamarre Y, Lemonne N, Waltz X, Chahed S, Cabot F, et al.
Severe proliferative retinopathy is associated with blood hyperviscosity in sickle cell hemoglobin-C disease but not in sickle cell anemia. Clin Hemorheol Microcirc 2013;55:205-12.
al-Hazzaa S, Bird AC, Kulozik A, Serjeant BE, Serjeant GR, Thomas P, et al.
Ocular findings in Saudi Arabian patients with sickle cell disease. Br J Ophthalmol 1995;79:457-61.
Al-Azri MH, Al-Belushi R, Al-Mamari M, Davidson R, Mathew AC. Knowledge and health beliefs regarding sickle cell disease among Omanis in a primary healthcare setting: Cross-sectional study. Sultan Qaboos Univ Med J 2016;16:e437-44.
Bhatt K, Reid ME, Lewis NA, Asnani MR. Knowledge and health beliefs of Jamaican adolescents with sickle cell disease. Pediatr Blood Cancer 2011;57:1044-8.
Al-Riyami A, Ebrahim GJ. Genetic blood disorders survey in the sultanate of Oman. J Trop Pediatr 2003;49 Suppl 1:i1-20.
King L, Knight-Madden J, Reid M. Newborn screening for sickle cell disease in Jamaica: A review – Past, present and future. West Indian Med J 2014;63:147-50.
Olakunle OS, Kenneth E, Olakekan AW, Adenike OB. Knowledge and attitude of secondary school students in Jos, Nigeria on sickle cell disease. Pan Afr Med J 2013;15:127.
Fadugbagbe AO, Gurgel RQ, Mendonça CQ, Cipolotti R, dos Santos AM, Cuevas LE. Ocular manifestations of sickle cell disease. Ann Trop Paediatr 2010;30:19-26.
El-Ghamrawy MK, El Behairy HF, El Menshawy A, Awad SA, Ismail A, Gabal MS. Ocular manifestations in Egyptian children and young adults with sickle cell disease. Indian J Hematol Blood Transfus 2014;30:275-80.
de Almeida Oliveira DC, Carvalho MO, do Nascimento VM, Villas-Bôas FS, Galvão-Castro B, Goncalves MS. Sickle cell disease retinopathy: Characterization among pediatric and teenage patients from Northeastern Brazil. Rev Bras Hematol Hemoter 2014;36:340-4.
Alhamdan NA, Almazrou YY, Alswaidi FM, Choudhry AJ. Premarital screening for thalassemia and sickle cell disease in Saudi Arabia. Genet Med 2007;9:372-7.
Memish ZA, Saeedi MY. Six-year outcome of the national premarital screening and genetic counseling program for sickle cell disease and β-thalassemia in Saudi Arabia. Ann Saudi Med 2011;31:229-35.
] [Full text]
Sawyer SM, Aroni RA. Self-management in adolescents with chronic illness. What does it mean and how can it be achieved? Med J Aust 2005;183:405-9.
Shrestha A, Rimal J. Effectiveness of a mass media campaign on oral carcinogens and their effects on the oral cavity Asian Pac J Cancer Prev 2018;19:819-23.
AlHilali SM, AlMuammar AM, AlKahtani E, Khandekar R, AlJasser AA. Preferred method of education among patients in ophthalmic care in Saudi Arabia. Middle East Afr J Ophthalmol 2016;23:168-71.
] [Full text]
AlKlayb SA, Assery MK, AlQahtani A, AlAnazi M, Pani SC. Comparison of the effectiveness of a mobile phone-based education program in educating mothers as oral health providers in two regions of Saudi Arabia. J Int Soc Prev Community Dent 2017;7:110-5.
Alqarni AA, Alfaifi HM, Aseeri NA, Gadah T, Togoo RA. Efficacy of a self-designed mobile application to improve child dental health knowledge among parents. J Int Soc Prev Community Dent 2018;8:424-30.
[Table 1], [Table 2], [Table 3], [Table 4], [Table 5], [Table 6], [Table 7]