Health related quality of life among adolescents with sickle cell disease in Saudi Arabia
Reported, January 4, 2012
Sickle cell disease (SCD) is a group of inherited red blood cell disorders characterized by the presence of abnormal hemoglobin. The clinical manifestations are diverse and may include vaso-occlusive, hematological and infectious crises . SCD is present throughout Saudi Arabia; particularly common in the eastern and southern provinces: Qatif (eastern region) 17.0 %, Gizan (southern region), 10.3%, Ula (Northern region) 8.1 % and Mecca (western region) 2.5 % . Increased life expectancy due to recent medical advances has increased the need to understand more fully the quality of life (QoL) in patients with SCD and factors predicting disease adaptation . QoL measures are used not only to assess the psychosocial impact of the disease but also in evaluating the efficacy of medical care and it is the goal of health care providers to enhance treatment outcome and restore comfort and well-being of their patients . In particular, health related quality of life (HRQoL) assessments in adolescents with chronic disease condition facilitate doctorpatient communication, they point to areas where patients may experience serious problems, they can be used as diagnostic tools for problem-oriented followup care, and the data are strong predictors of survival .
Previous research indicated that patients with SCD experienced a lower HRQoL compared to the general adults population Dampier et al found that children with SCD as well as their parents scored significantly lower on several HRQoL domains including; general physical, motor and independent daily functioning. Trzepacz et al identified deteriorations in social and school competence for children with SCD, compared to healthy peers, but they did not find an association with disease severity as measured by sickle cell genotype. A number of disease-related factors have been found to affect HRQoL in pediatric SCD. Several studies that examined the influence of various determinants including the role of sociodemographics, disease severity and the presence of complications on HRQoL in SCD patients were carried out in the developed countries while in the developing countries similar studies do not exist. Furthermore, adolescent health is a relatively unexplored component of public health in many developing countries .
Researches on chronic diseases have indicated that HRQoL varies according to socio-demographic characteristics such as income level, educational status, ethnicity, occupational status, age, and gender, with the disadvantaged groups typically reporting lower HRQoL. This association has been reported for many chronic conditions including cancer , HIV infection , renal disease , and sickle cell disease . Also, studies involving healthy populations have indicated that there is an inverse association between childrens HRQoL and family variables such as low parental education, socio-economic status (SES) with low family income contributing to caregiver distress in families of children with chronic conditions . Also, Simon et al reported that HRQoL is poorest for children and youth in lower socioeconomic status groups, those with access barriers, adolescents compared with children, and individuals with medical conditions.
Studies on the relationship of socio-economic variables and the HRQoL in patients with SCD revealed conflicting results. Van den Tweel et al indicated that children of parents with low educational level perceived a significantly better HRQoL. This phenomenon is difficult to explain, since previous research mainly pointed out that high quality of life scores were related to high parental education, or that education had no effect at all . Also, previous research has shown that children with low socioeconomic status (SES) functioned worse than children from middle SES backgrounds .
Limitations in HRQoL have been documented consistently for youth with SCD, particularly as children move into adolescence and young adulthood . Sickle cell pain, a common manifestation that is recurrent, acute, and unpredictable, may be the most important disease complication associated with deterioration in physical and psychosocial domains of HRQoL . Palermo et al reported that sickle cell complications (including pain), in addition to child age and gender, are central to physical but not psychosocial HRQoL in their sample of adolescents with SCD. Yet, Panepinto and colleagues found that only pain, no other SCD complications, was associated with the deterioration in the physical domain of HRQoL but not the psychosocial.
Although pain and other sickle cell complications show an association with decrements in engagement in physical activities and in physical domains of HRQoL, documentation of a significant association of pain with psychosocial domains of HRQoL are not consistent . Recently, Brandow et al.
examined HRQoL in children with SCD specifically in relation to painful events at presentation to the emergency room and seven days postdischarge. They found that painful events diminished all domains of HRQoL and that these domains improve after the pain resolves. If these variables (socio-demographic and disease related complications) are crucial in determining HRQoL among adolescent patients with SCD, by controlling of these factors, we may better support them with successful transition to adulthood and with less burden on healthcare services . We specifically hypothesized that adolescents with SCD would have decreased mean scores along the different subscales of the HRQoL measure compared to adolescents without SCD. We also predicted that certain demographic factors (increasing age, gender, low socio-economic status) would be related to HRQoL with males and adolescents from lower SES backgrounds reporting lower quality of life. Additionally, we predicted that adolescents with SCD who experienced disease-related complications, frequent pain episodes, and greater health care utilization would report lower quality of life than adolescents with SCD who did not report these factors. The objective of this study were to assess the impairment of the different domains of HRQoL among Saudi adolescents with SCD compared to healthy peers and to define the relationship between socio-demographic variables, the presence of diseases related complications with the degree of impairment in HRQoL.
This is the first study to assess HRQoL in adolescent patients with SCD in an Arabic community. The data reported here showed that adolescents with SCD experiencing impairment in HRQoL when compared with their healthy peers. The smallest differences between adolescent controls and SCD group were evident in the role emotional and emotional wellbeing domains. Similar to our findings, McClish et al reported that young adult with SCD experienced a poor HRQoL except for mental health. Patel and Palham reported that Indian children with SCD experienced a poor HRQoL along physical, psychosocial and cognitive domains.
It has been suggested that people with chronic diseases may report good psychological well-being as a result of increased social support, religiousness, lack of other stressors, or due to the presence of a response shift” associated with coping and managing of their illness .
In Saudi culture, several factors may explain the process of psychological coping among adolescents with SCD. First, the extended family pattern in Saudi Arabia; family provide people suffering from anxiety the presence of safe persons around them and hide active symptoms of anxiety, maintaining them functional in a compensatory behavior . Adolescence is typically a period of rapid physical, cognitive and psychosocial change that takes place in the context of shifting relationships and roles within the family . The Family Adjustment and Adaptation Response (FAAR) model highlights how adaptation to chronic childhood illness is explained in the balance of family demands (stressors from individual, family, and community sources) as well as family resources such as positive parent-child relationships, family functioning, and active coping and positive beliefs/attitudes . Second, the health care management policy in Saudi Arabia, where the services are free of charge and all SCD patients received Hydroxyurea (HU) which can improve some domains of HRQoL including social function, general health perception and pain recall . Finally, recorded fetal hemoglobin level in this study had a mean of 13.1±6.4 % (median of 12.5%) with an interquartile range of 19.0%, higher than that reported in patients with African haplotypes . Pearson and Perrine et al. found that serious complications (i.e. jaundice, splenectomy, and hematuria) occurred in only 6% to 25% of Saudi patients compared to North American Blacks.
Despite the increased attention paid to determine factors that affect QoL in SCD fewer studies addressed the influence of gender. In our study, female participants was less and we speculated that this may be due to the disease associated stigma and fear of disclosure as many females with SCD preferred to receive disease management in private health facilities where more confidentiality is provided.
Despite increased attention being paid to determine factors that affect the quality of life in SCD, there are few studies that address the influence of gender. Some studies suggested that females have a poorer quality of life than their male counterparts while, others did not reveal gender difference . There is also suggestion that gender may influence the way adolescents perceiving of health .
Among our adolescents with SCD, gender differences in HRQoL existed between males and females. Adolescent girls with SCD achieved significantly lower scores in physical functioning, role physical, bodily pain and general health domains. Ironically, they have higher score along social function and emotional well-being compared to their healthy controls.
Studies dealt with behavior adjustment in children with chronic diseases showed conflicting results in relation to the effect of gender. Zandieh et al found that QoL in Iranian boys with bronchial asthma were more deteriorated than females.
While in Swedish study, Hanberger et al. reported lower Generic HRQoL and Diabetes-specific HRQoL mean scores in adolescent girls with type 1 diabetes mellitus than boys. HRQoL did not show a significant relationship with SCD genotype. The relationship between SCD genotype, disease severity and HRQoL may have been mediated by other variables, particularly disease-related complications. In addition, some of HRQoL subscales were significantly deteriorated with increasing hospitalization frequency. Anie et al found a relationship between pain and some HRQoL domains physical, social functions, mental and general health while others found no significant association with health care utilization measures.
In this study nearly all domains of HRQoL were negatively associated with increasing age of adolescent with SCD especially energy, emotional wellbeing, social functioning and general health domain. Baskin et al. found that there was an interactive effect between age and disease adjustment which become more problematic as SCD patient mature with additional difficulties as the disease worsens in severity (e.g., increase in vaso-oclusive episodes). Furthermore, physical manifestations of SCD notably in the form of abnormal sexual and physical development, as evidenced by delayed growth and puberty, priapism and short stature may add more difficulties to the growing patient.
Adolescents with SCD in our study reported significant educational delay due to frequent hospitalization, emergency admissions, and appointments for checkup. This is consistent with other studies reported that adolescents with SCD commonly experience role limitation due to emotional problems with subsequent educational problems .
Rural residence was a significant negative predictor for vitality and pain of HRQoL subscales. There are limited studies on rural/ urban differences in QoL among patients with SCD. Telfair et al have shown that rural patients had lower health service access and utilization with lower physical functioning. Future researches should investigate the role the differential levels of social support, coping styles, and perceptions of illness in urban-rural differences in QoL among SCD patients.
Another risk factor for lower HRQoL among our patients was the low family income. Saudi youth with lower socioeconomic standard were more likely to have lowered physical functioning scores. Children living under poorer economic conditions are more prone to anxiety, depression and negative thinking with potential exaggeration with adolescents with SCD through increasing exposure to stressful experiences and decreasing psychological and social coping resources . Panepinto et al have found a significant negative effect of poverty and disease severity on the HRQoL among pediatric patients and it was chiefly affecting the physical functions of the included children with SCD.
Health care providers have limited ability to improve the poverty status of the affected patients but improvement of disease status and prevention of disease complications may help in improvement of their well-being especially physical wellbeing .
The combined effects of SCD-related pain and low socioeconomic status on school attendance may limit opportunities for physical education with subsequent reduction of physical HRQoL.
Socio-demographic variables were found to adversely affect the HRQoL in patients with chronic conditions as reveled by several cross-sectional studies, yet prospective studies are required to better elucidate the predictive value of family variables. Barakat et al found that family resources were not significant predictors for QoL among patients with childhood cancer. Furthermore, they stated that the potential negative impact of lower family education, income, employment, and function may be indirect and dynamic over time.
Adolescents with SCD experience health related quality of life worse along several domains of HRQOL including physical functioning, role physical, bodily pain and general health compared to their comparison group of apparently healthy adolescents. Socio-demographics including older age of the adolescents with SCD, female gender, rural residence, low family income and the occurrence of disease-related complications were associated mainly with deterioration of physical rather than mental health of our sample of adolescents with SCD.
Controlling of possible modifiable determinants, e.g., disease-related complications and some socio-demographics that affect the HRQoL among adolescents with SCD might improve these patients outcome with smooth transition to adulthood. HRQoL assessments in adolescents with SCD facilitate doctorpatient communication, point to areas where patients may experience serious problems during this transitory phase, can be used as diagnostic tools for followup care, and assessing the efficiency of different treatment modalities.
Credits:Mostafa Abdel-Monhem Amr,Amin Tarek Tawfik and Ahmed Omar Al-Omair
More information:
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3201577/?tool=pubmed