SCD in Saudi Children, Teens, and Adults: A Study in Health-Related Quality of Life

Special Article - Blood & Its Components

J Blood Disord. 2016; 3(1): 1036.

SCD in Saudi Children, Teens, and Adults: A Study in Health-Related Quality of Life

Ahmed AE1,2*, Alaskar AS1,2,3, McClish DK4, Ali YZ3, Malhan HM5, Aldughither MH6, Al-Suliman AM7 and Jazieh AR3

¹King Saud Bin Abdul-Aziz University for Health Sciences, Saudi Arabia

²King Abdullah International Medical Research Center, Saudi Arabia

³King Abdul-Aziz Medical City, Saudi Arabia

4Department of Biostatistics, Virginia Commonwealth University, USA

5King Fahad Hospital Central, Saudi Arabia

6National Anti-Corruption Commission, Saudi Arabia

7King Fahad Hospital, Saudi Arabia

*Corresponding author: Anwar E. Ahmed, Department of Epidemiology and Biostatistics, College of Public Health and Health Informatics, King Saud bin Abdulaziz University for Health Sciences, MC 2350, P.O.Box 22490 Riyadh, 11426, Saudi Arabia

Received: February 24, 2016; Accepted: March 22, 2016; Published: March 29, 2016


Background: While there have been earlier studies assessing Saudi adolescents with Sickle Cell Disease (SCD), this study demonstrates that Health Related Quality of Life (HRQoL) scores were negatively associated with increasing age in the adolescent population with SCD. To date, no study has assessed HRQoL in Saudi children and adults with SCD. The aim of this study was to assess HRQoL in patients with SCD and to identify what age group was most commonly associated with poor HRQoL.

Methods: A multicenter, cross-sectional study was conducted on patients with SCD who visited the hematology outpatient clinics at King Fahad Hospital in Hofuf and King Fahad Central Hospital in Jazan, Saudi Arabia. Data were gathered using a self-administered questionnaire: patient demographics, symptoms/complications of SCD, and the RAND SF-36. Data were collected from 753 SCD patients (68 children age = 14 years, 56 teenagers age 15-17 years, 290 young adults age 18-25 years, 233 intermediate-aged adults age 26-36 years, and 106 older adults age = 37 years).

Results: The ages ranged from 9 to 75 years with a mean of age of 26.4±10 years. As compared to children, young, intermediate-aged and older adults with SCD reported quite worse physical role functions and bodily pain. Better social function was noted in children than the intermediate-aged adults and older adults.

Conclusion: The current investigation noted age differences in HRQol, particularly between children and adults. Future studies are warranted to focus on the reasons of age differences in HRQoL and on the very poor HRQol of older adults.

Keywords: Sickle cell disease; Quality of life; HRQoL; Children; Teenagers; Adults


ANOVA: Analysis of Variance (Fisher); HRQOL: Health Related Quality of Life; SCD: Sickle Cell Disease; SF-36: Short Form (36) Health Survey; WHOQOL-BREF: World Health Organization Quality-of-Life Scale


Sickle Cell Disease (SCD) is considered the most common genetic disease in the eastern, western, and southern regions of Saudi Arabia [1-6]. Specifically, it is mainly spread throughout several cities: Hufuf, Qatif, and Jazan. The frequency of the SCD gene in the Hofuf region has reached 25% [4]. SCD patients tend to report fever, swelling of the hands and feet, need for blood transfusions, pulmonary hypertension, acute joint necrosis, or severe abdominal pain [7-9]. SCD complications and symptoms usually appear in childhood and last a lifetime. However, these SCD complications and symptoms could increase considerably as age increases. The average number of complications tends to increase with age in adults SCD patients [10].

SCD patients have been shown to report poorer Health Related Quality of Life (HRQoL) compared with general populations, or patients with other chronic non-communicable diseases [10-13]. Several studies have addressed HRQoL in children or adolescents within SCD populations [12,14]. HRQoL among Saudi adolescents with SCD, aged 14-17, was assessed using the RAND Short Form (36) Health Survey (SF-36). The authors noted that poorer HRQoL has been shown to correlate with age in adolescents with SCD [14]. However, a study that utilized Pediatric Quality of Life InventoryTM (PedsQL™) Generic Scales to assess physical, social, emotional, and school functioning in children and adolescents (ages between 8-18 years), found no significant differences in PedsQL between age groups [12]. The discrepancy between the two studies could be due to the sampling populations, whereas the latest study involved children.

In adult populations, increases in age tend to reduce all SF-36 scores, which indicate poor quality of life [10]. Another study found that elderly thalassemia patients tend to report poor HRQoL [15]. Sanders et al. examined age differences in pain, coping and healthcare utilization in adults with SCD (18-62 years). They concluded that age plays an important role in healthcare utilization and coping of the SCD patients [16]. According to their study younger SCD patients (= 36 years old) tend to cope by ignoring pain. However, there were no differences in psychological distress or social support across the age groups [16]. Edwards et al. examined the role of self-efficacy of 147 African American adults with SCD (18 years or greater). It was noted that age tends to relate negatively with physical symptoms [17]. A recent study by our group of data collected from Saudi Arabia on SF- 36 in adult patients with SCD has suggested no age differences were found in SF-36 subscales [18].

All the above-mentioned literature focused on children or adults; however they lack the ability to assess and compare HRQoL across various age groups that include both children and adults. There have been a few such studies involving samples from children and adults for the reasons of assessment and comparison of the HRQoL of SCD patients in various age groups [19-21]. A study conducted at King Abdulaziz University Hospital, Saudi Arabia assessed HRQoL in patients with SCD aged between 2-48 years using the WHOQOLBREF: World Health Organization Quality-of-Life Scale (WHOQOLBREF) [18]. This study observed differences in WHOQOL-BREF among various age groups with children patients (2-12 years) tending to be happier than the adults patients (18 years or older) (74% versus 42%) [19]. A study conducted in Alagoas, Brazil on 40 SCD patients aged 12 to 43 years showed that the adults rather than adolescents exhibited significantly lower SF-36 scores in several domains [20].

The hypothesis tested in this study is whether any component of SF-36 subscales is significantly poorer/worse in certain age groups. To the best of our knowledge, no finding has been reported on the comparison of HRQoL across children, teenagers, and adults. The aim of the current study was to assess these differences in a large sample of people with SCD in Saudi Arabia.


A sample of 753 patients with sickle cell disease was recruited between October 1, 2014 to February 29, 2015 from the hematology outpatient clinics of two hospitals: King Fahad Hospital in Hofuf and King Fahad Central Hospital in Jazan, Saudi Arabia. The study participants were patients with SCD of age 9 years old or greater where the patient had the ability to read and write. The current study was approved by the Ministry of Health, Kingdom of Saudi Arabia and King Abdullah International Medical Research Center, Research Protocol - RC12/127/R. The ethical approval for this study was gained by verbal consent with the IRB Log No-15-247E. Permission and assent was obtained from parents of all children and teenagers involved in the study (9 to 17 years old). Adult SCD patients who verbally consented to participate in the study were asked to complete the survey. No identifiable data gathered from participants. Sample characteristics were collected, such as age/year, gender (male/female), and engaging in regular exercise (yes/no). Clinical characteristics were also collected such as family history of anemia (yes/no), whether the spleen had been removed (yes/no), any history of blood transfusions (yes/no), the number of visits to the Emergency Department (ED) within the past six months (<3 or =3 visits), and the presence of chronic diseases other than SCD (yes/no). Symptoms and complications of SCD during the past six months were collected, such as swelling (yes/no), fever (yes/no), and skin redness (yes/no).

Study instrument

In the present study we utilized the Medical Outcome Study 36- Item Short Form (SF-36) [21]. The SF-36 was found to be reliable across various age groups [21]. The Arabic-translated version of RAND SF-36Health Related Quality of Life Questionnaire [22] was used to evaluate Health Related Quality of Life in our population. The SF-36 questionnaire consists of 8 subscales that assess perceptions and feelings with regard to physical function (ten items); emotional role functions (three items); vitality (four items); physical role health (four items); emotional wellbeing (five items); bodily pain (two items); social function (two items); and general health perceptions (five items). The SF-36 subscales were scored according to RAND Health scoring. Each subscale has a single summary variable ranged from 0 = least favorable health state to 100 = most favorable health state. The reliability of the SF-36 has been assessed in a pilot study of eighty SCD patients. The reliability analysis showed acceptable internal consistency with Cronbach alpha (a) ranged from 0.6 to 0.86. The reliability analysis showed high internal consistency for vitality (a = 0.79); physical function (a = 0.81); bodily pain (a = 0.84); physical role health (a = 0.84); and emotional role functions (a = 0.86). The other three domains had a lower than the ideal range: general health (a = 0.60); social function (a = 0.67); and emotional wellbeing (a = 0.67).

Statistical analyses

Data were analyzed using IBM Statistical Package for Social Sciences (SPSS®) version 23 (Chicago, Illinois, USA). On the basis of age, SCD patients were classified into five groups: children age = 14 years, teenagers age 15-17 years and adults (young adults age 18-25 years, intermediate-aged adults age 26-36 years, and 106 older adults age = 37 years). Some of these cut-off points of age-groups were used in a previous study [16]. Frequency and count were used to summarize the sample characteristics (Table 1). Chi-square linear-by-linear tests were performed for associations between symptoms/complications of SCD and age spectrum (Table 1). ANOVAs (Analysis of Variance) were performed to compare quality of life subscales across various age groups (Table 2 and Figure 1). When appropriate, Tukey’s multiple comparison procedure was applied for age group comparison with significant level at 0.05.