Attitude and Behavior of Parents of Children With Sickle Cell Disease Toward the Disease: An Observational Study in Saudi Arabia

Background: Sickle cell disease (SCD) is a significant hematological disorder affecting populations worldwide, with a notable prevalence in certain regions of Saudi Arabia. Despite extensive screening programs, there is a critical need for improved public health education to enhance understanding and management of SCD. This study examines the relationship between the attitudes and behaviors of parents toward their children's disease and its management. Methods: We conducted a cross-sectional observational study at the King Fahd Medical Research Center in Jeddah. This research encompassed children aged 5-16 years with SCD and their parents. Comprehensive questionnaires assessed sociodemographic data, attitudes toward SCD, and behavioral responses to the illness and treatment. Results: The study included 66 parents, predominantly in the age range of 30-39 years and earning below 5000 Saudi Riyals, who exhibited varying attitudes towards SCD, with a majority questioning the availability of a cure and expressing caution towards new treatments. Despite a cautious approach to invasive treatments, parents relied on information from healthcare providers. Attitudes towards treatment showed significant differences based on gender and education level, with females and less-educated parents exhibiting more hesitancy towards new treatment and blood transfusions. Conclusion: The study indicates that while parents show a positive and proactive attitude toward SCD, there is hesitancy towards new and invasive treatments, reflecting the need for continued educational support. The results underscore the importance of tailored healthcare communication strategies to address the diverse needs of families affected by SCD.


Introduction
Sickle cell disease (SCD) is a common life-threatening hematological disease affecting millions of the world's population [1,2].In the Kingdom of Saudi Arabia (KSA), epidemiological data regarding SCD prevalence is scarce [3].However, SCD is mainly prevalent in the Eastern and Southern provinces being concentrated in regions like Qatif (Eastern region) 17%, Jazan (Southern region) 10.3%, Ula (Northern region) 8.1%, and Mecca (Western region) 2.5% [4][5][6][7][8].The reports for SCD prevalence from multiple singlecenter studies revealed that 2% to 27% are carriers, and up to 1.4% have SCD, in other areas [7,8].The only national screening program reported in 2007 was a premarital screening for thalassemia and SCD from February 2004 to January 2005 [7].Among 488,315 participants who were screened, 4.2% had sickle cell trait, and 0.26% had SCD.Notably, the screening program successfully decreased the number of at-risk marriages, and the burden of genetic disease was substantially reduced in Saudi Arabia in the later decades.
Given the harmful physical and emotional effects of SCD [9,10], the World Health Organization (WHO) and the United Nations (UN) have considered SCD as a global public health problem [11].The WHO reported that of the 330,000 affected infants who were born annually, around 83% had sickle cell disorders and hemoglobin disorders accounting for about 3.4% of deaths in children less than five years of age.Therefore, most countries should include screening for hemoglobin disorders as part of the basic health services.
Despite efforts in screening, population health education plays a crucial role in addressing the challenges associated with SCD and is mandatory for the prevention and comprehensive management of SCD.By promoting health education and awareness about SCD, individuals can gain a better understanding of the disease, its symptoms, and available treatment options.This knowledge helps the patients to adhere to their treatment, which can be important evidence of the health-related quality of life in such patients [12].It also empowers affected individuals and their caregivers to recognize early signs, seek appropriate medical care, and take preventive measures.
Though all governmental agencies and health ministries do their best in providing education, surveys, awareness, and counseling, a lot more is yet needed for their improvement.For such programs to be successful, the families of affected people should be involved.They should possess a proper attitude and behavior in creating awareness about the disease, and handling of the resulting complications.
Parental attitude is very important as they are the first teachers and healers for the affected children.Here the role of a mother is very crucial as her association with the child is from birth.The contributions and roles of mothers need to be assessed to improve the quality of life of these SCD children.The mother's attitude and behavior, towards the child's illness, nutrition, and care, needs to be assessed to bring about a positive change in the system.
Furthermore, the education of the families, especially the mother, is very contributory to the child's health.This would improve the quality of life of SCD patients, which was reported to be deteriorated among Saudi SCD patients [13,14].Assessment of awareness about SCD among patients, caregivers, students, and the public has been published elsewhere [15][16][17][18].Additionally, positive and proactive attitudes from parents can significantly impact the child's emotional well-being and overall quality of life.Developing educational models and implementing awareness programs can help deliver the best care and support for SCD patients [19].

Materials And Methods
This was a prospective observational study conducted at the King Fahd Medical Research Center, King Abdulaziz University, Jeddah, Saudi Arabia, from September 2019 to August 2020.In this study, we aimed to assess the parental attitudes and behaviors toward SCD, including beliefs about the availability of a cure and the willingness to try new treatments.It is also to explore any differences in attitudes and behaviors based on parental gender and education level.The study was approved by King Abdulaziz University Institutional Committee (approval number: 2/36/8390).

Study population
The study included children with SCD in the age group of 5-16 years and their parents.Information about the patient's genotypes was collected from the hospital.No phenotype and gender bias was considered during the study.Those not willing to participate were excluded from the study.Parents of patients with SCD fitting our inclusion criteria who visited the OPD of the pediatric hematology ward of the hospital were recruited.They were directed to the Medical Research Center, where we had arranged a setup for the questionnaire session.We made the parents comfortable while questions were asked.Our research team was adept at English as well as Arabic, so the question was well understood by the parents.Appropriate consent forms were taken from all enrolled parents before the commencement of the study.

Data collection
Appropriate questionnaires were completed by the parents concerning their personal information, background, and education.Also, certain specific questionnaires were filled in by the psychologist on attitudes and behaviors of the parents toward their affected children.Focus was given on (i) the sociodemographics of informants and (ii) the attitude of parents towards the illness, towards the children with SCD, towards the treatment, and their behavior related to it.Behavior questions were adapted based on earlier studies [18].The tool was validated and endorsed by the faculties of pediatric and hematology departments.The interview was structured around the parent's behavior and attitudes towards their affected children and the illness per se.The questionnaire as shown in the Appendices included varied questions about their personal information (15 questions), their general attitude about the disease (six questions), their attitude toward treatment (five questions), social attitude toward illness (seven questions), their behavior toward the child (six questions) and their compliance regarding treatment (one question).

Statistical analysis
All data were entered and analyzed using IBM SPSS Statistics for Windows, Version 26.0 (Released 2019; IBM Corp., Armonk, New York, United States).Descriptive statistics were done for all variables.Frequency and percentages were calculated for categorical variables.Inferential statistics was used to compare results in different categories; sex, and educational levels.Statistical associations between demographic variables and knowledge and attitude about SCD were evaluated with Fisher's exact test/chi-square test as appropriate.A p-value of ˂0.05 was considered statistically significant.Representation of certain variables was done using the bar graphs.

Results
The demographic profile (Table 1) revealed that among the parents of children with SCD, 27 (40.9%)were in the 30-39 age bracket, highlighting this as the most common age range.Gender distribution was skewed towards males, who constituted 37 (56.1%) of the respondents.In terms of education, the largest group of parents, 47 (71.2%), reported having education up to high school and below.

Characteristics Number Percentage
Age (years)

TABLE 1: Demographics of the informant
The attitude of parents towards their children's illness with SCD, as captured in Table 2, presents a mixed perspective.A slight majority of the respondents, 35 (53%), believe there is currently no cure for SCD, while 31 (47%) believe there is.A large majority, 60 (90.9%), do not consider it shameful to discuss the disease.Interestingly, 63 (95.5%) agree that one should not avoid discussing the illness and its cure, and a majority, 52 (78.8%), are proactive, preferring not to wait until extreme pain arises to seek a doctor's consultation.

Frequency Percentage
Is there currently a cure for SCD?With regard to the attitudes of parents towards the treatment of SCD, a significant majority, 51 (77.3%), reported they would not risk trying a new treatment for their child's condition.Similarly, a majority of 46 (69.7%) were hesitant about the option of blood transfusion, reflecting a cautious stance towards more invasive treatments.Despite their concerns about treatments, most parents acknowledged the helpfulness of information on SCD from healthcare providers, with 59 (89.4%) agreeing that it aided in managing the disease and facing future risks.
In analyzing the differences in attitudes towards SCD treatment by sex and education level, several significant findings emerged.Notably, the genetic information given by the hospital to help reduce distress was significantly less among males compared to females, with a p-value of 0.012.Moreover, the need for more support from health care providers was higher among males (91.9%) compared to females (75.9%), though not significant (p-value = 0.053).This underscores a gender disparity in perceived support needs (Table 3).This underscores a gender disparity in perceived support needs (Table 3).*The Fisher exact statistic is significant at the .05level.

Sex
a, b where the statistical significance exists in the pairwise comparison.

SCD: sickle cell disease
Educational differences were also evident.Regardless of their level of education, most parents (around 70%) were hesitant to consider blood transfusion as a treatment option (35 parents with lower education and 11 parents with higher education).Despite their higher education, 15.8% of those who had a Bachelor's degree or above were not sure about considering blood transfusion compared to 2.1% of parents with lesser education (p-value = 0.03) (  *The Fisher exact statistic is significant at the .05level.
a, b where the statistical significance exists in the pairwise comparison.

SCD: sickle cell disease
Both male and female parents exhibit higher levels of high compliance to treatment modalities compared to moderate and poor compliance, with males slightly outnumbering females in the high compliance category.Moderate compliance is more balanced between sexes, with males (just over 10) exhibiting a marginally higher count than females.Notably, the count of poor compliance is significantly lower for both sexes, with males showing a marginally higher count than females, both being less than five (Figure 1).The social attitude towards children with SCD and the impact of the parents' sex and educational level, as reported in Table 5, revealed a generally positive and supportive environment.Most parents, irrespective of their sex or education, reported that their children were treated normally and sympathetically by others.When stratifying the data by sex, there were no significant differences in the social attitudes experienced by the children.Similarly, the educational level of the parents did not significantly affect the social attitudes toward their children.First aid treatment, being analgesics, is given by the parents before taking the child to the hospital.Also, appropriate precautions seem to be taken by the parents, except when going to a crowded place like malls which is unavoidable in today's context in cities (Figure 2).

Data shown in percentages
There was a statistically significant difference between males (N=26) and females (N=13), as parents taking precautions in crowded places.The fathers were more cautious than the mothers in our study with a p-value of 0.037 (Table 6) but there was no difference based on the level of education (Table 7).

Discussion
This study aimed to explore the association between the attitude and behavior of the parents caring for the affected SCD children towards the disease.Most of the parents had some level of education, with more than 28.8% having a bachelor's and above education.In another study, 20% of the parents were uneducated [20].
Education level has been reported to have an impact on the attitude of the parents towards their child's disease [21].In a study from the United States, parental education increased the positive attitude toward parental genetic testing for SCD [22].In a study in Nigeria too, education level had a significant correlation with a positive attitude toward newborn screening for SCD [23].
The parents' attitudes about their child's disease and behavior towards the affected child were acceptable in the current study.In a study by Acharya et al. in 2009, parents of children with SCD in the United States had significant misunderstandings about the disease and its inheritance [24].In another study in Brazzaville, Congo, more information about SCD was required by 88% of parents of children with SCD [20].This indicates an improvement in the dissemination of information about SCD by the Saudi health authorities and denotes a more appropriate approach to educate and improve the attitude and behavior of the public regarding the disease.
In the current study, the parents perceived minimal stigma associated with their child's disease, which was the same case in the previously mentioned study in the United States [24].Most of the parents in the current study received adequate information from their child's healthcare provider in the hospital, which suggests that while there is apprehension about new or invasive treatment methods, there remains a reliance on and appreciation for medical guidance in the ongoing management of SCD.This is in contrast to the United States study, where formal professional counseling was rare outside the family [24].In the Congo study, 66% of parents received adequate information from their health workers [20].Seeking an appropriate source of information is a sign of increased public awareness, a good omen for improved overall community health.
While previous studies reported a psychological impact of the presence of an SCD child within the family [21], most of our SCD children did not face negative attitudes/behavior towards their disease from the community.This reflects wider social acceptance and understanding of the illness.The attitudes towards SCD treatments are influenced by both gender and educational background.In low-income-countries, caregivers of SCD children are faced with enormous psychological and financial burdens [25].Even in highincome countries, the frequency of children's vaso-occlusive crises has been reported to affect the parent's psychological state [26], which in turn may impact their attitudes and behavior.
The parents had reasonable information about SCD complications, pain management, and vaso-occlusive crisis management.The responses reflect a proactive and open stance towards the management and social perception of SCD.Previous studies reported that 65% of the parents sought medical advice during a crisis, while 20% exercised self-medication [20].Local beliefs, herbal medications, and traditional remedies were seldom used in the current study.A study from Jeddah found that females (mothers) had better information about SCD and its complications than their male partners (fathers) [27].However, the present study showed that male parents had higher levels of compliance with treatment modalities compared to female parents.This may be attributed to social inhibition in society which may reduce interactions between females and doctors.It is usually the males who interact with the doctor.Therefore, their awareness as well as compliance are better than females.This should address educational program creators to target the male population.
Based on the current study, data suggests that most parents recognize the illness as the cause of suffering, while a small percentage adhere to traditional beliefs or are uncertain about the cause suggesting that societal perceptions of SCD may be uniformly empathetic across the demographic variables.Therefore, measures must be considered to maintain and improve the attitudes and behavior of parents of children with SCD towards the disease.These factors are crucial to the health of the affected child.A consensus needs to be reached in Saudi Arabia to acquire a broader understanding of the attitude of the affected child's parents.This will affect healthcare costs and create a lesser burden on the family and society.
The Saudi health authorities spent an excellent effort in the premarital screening program.However, some authors suggest that screening at the university level before any commitment may be preferable to premarital screening [28].Moreover, more effort is needed for primary and secondary prevention of the disease.This can be achieved through the training of healthcare professionals, increasing accessibility to healthcare services, and the development of genetic testing protocols [29].Implementing awareness programs at a very young age level, among high school students, for example, can help the population overcome understanding barriers [30].

Recommendations
A few recommendations that would assist in improving the application of knowledge to improve attitude are: (i) Enhancing educational programs; (ii) Increasing counseling set-ups; (iii) Increasing awareness campaigns through varied sources; (iv) Mass screening to increase interactions and improve attitudes; (v) Motivating people to implement their information about the disease; (vi) Improving work attitude towards giving the child that extra bit of importance and attention so that he does not fall ill frequently.

Limitations
The current study is not without limitations, being a single-center study, directed towards patients of Saudi nationality as well as other nationalities who reside in Saudi Arabia, and questionnaires were collected from a single region in Saudi Arabia, thereby limiting its generalizability and external validity.Also, the number of respondents in our study is less in number.A larger sample size, with a focus on only the Saudi population, from different regions of the kingdom, would result in a better representative cohort.

Conclusions
We believe that awareness programs by the governmental agencies have increased which brought about positive changes in attitudes and behaviors in Saudi Arabia.Overall, attitudes and behavior towards SCD and affected children seem good but implementation of the imbibed knowledge toward their attitude is lacking in certain areas.The study serves as a prelude for the policymakers to take appropriate steps toward its implementation.

TABLE 2 : General attitude of parents about the disease
SCD: sickle cell disease

TABLE 4 : Attitude of parents towards treatment based on the education of parents.
Do siblings treat the child normally?

TABLE 6 : Behavior of parents based on gender of parent.
*The Fisher exact statistic is significant at the .05level.a, b where the statistical significance exists in the pairwise comparison.2024 Halawani et al.Cureus 16(3): e55552.DOI 10.7759/cureus.5555210 of 19

TABLE 7 : Behavior of parents based on education level of the parent
*The Fisher exact statistic is significant at the .05level.

55552 Question Reply Tick mark against the column whatever applicable
Would you risk with a new treatment?

Question Reply Tick mark against the column whatever applicable
Do siblings treat the child normally?

mark against the column whatever applicable
Do you regularly and promptly attend to the pain / wait for it to