The objective of this study was to determine the feasibility, reliability and validity of the Pediatric Quality of Life Inventory™ generic core scales (PedsQL™ questionnaire) in children with sickle cell disease. This was a cross-sectional study of children from an urban hospital-based sickle cell disease clinic and an urban primary care clinic. The study participants were children ages 2 to 18 years who presented to clinic for a routine visit. Health-related quality of life (HRQL) was the main outcome. HRQL of children with sickle cell disease were compared to children without disease to test validity. Missing items were used to determine feasibility and Cronbach’s alpha was used to determine reliability. Parents of 178 children (104 with sickle cell disease and 74 without disease) and 118 children (78 with sickle cell disease and 40 without disease) completed HRQL questionnaires. The PedsQL™ questionnaire was feasible and reliable. The parent-proxy and child self-report questionnaire differentiated between children with and without sickle cell disease. The parent proxy-report differentiated well between children with mild and severe sickle cell disease. The questionnaire performed well in children with sickle cell disease and is a feasible, reliable, and valid tool to measure HRQL in children with sickle cell disease.
PurposeThe objective of this study was to determine the impact of family income and sickle cell disease on the health-related quality of life (HRQL) of children.MethodsThis was a cross-sectional study of children with and without sickle cell disease. Participants completed the PedsQL™ generic core scales parent-proxy or child self-report questionnaire during a routine clinic visit. HRQL was the primary outcome measured. Family income and sickle cell disease were the primary independent variables of interest.ResultsA total of 104 children with sickle cell disease and 74 without disease participated in the study. After adjusting for family income, patient age, and the presence of co-morbidities, children with severe sickle cell disease had increased odds of worse overall HRQL (parent-proxy HRQL report odds ratio [OR] 4.0) and physical HRQL (parent-proxy report OR 5.67, child self-report OR 3.33) compared to children without sickle cell disease.ConclusionsChildren with sickle cell disease have significantly impaired HRQL, even after considering the potential detrimental effect of family income on HRQL. Targeted interventions to improve these children’s HRQL are warranted.
Previous studies have shown that poverty is associated with worse health-related quality of life (HRQL). Children with sickle cell disease have been shown to have poor HRQL. The impact that poverty and sickle cell disease have on the HRQL of children has not been well delineated. The objective of this study was to determine the impact of poverty and sickle cell disease on the HRQL of children with and without sickle cell disease. A cross-sectional study was conducted. Parents of children with sickle cell disease and parents of children without sickle cell disease (controls) who presented to the clinic (ages 2 to 18 years) were asked to complete the PedsQL questionnaire. Detailed demographic information on total family income and family size was collected to classify family income based on the federal poverty threshold. The primary outcome measured was HRQL. An impaired HRQL, a score similar to that of children with chronic disease, is defined as any score that is less than the population mean -1 standard deviation (Varni, et al. 2003). Three HRQL summary scores were assessed: total, physical and psychosocial summary scores. Poverty level and sickle cell disease were the independent variables of interest. Descriptive statistics and non-parametric tests were used to look for significant differences. An ordinal multinomial regression model was used to look at the independent effects of income and disease on the HRQL of children. A total of 104 children with sickle cell disease and 74 control patients participated in the study. Approximately half of the study population was female, 98% of the cases and 78% of the controls were African-American. Approximately half (58%) of the controls and 42% of the cases with sickle cell disease had a family income at poor or near poor (100% or 100–124% of the federal poverty threshold). There were 15% of the controls and 25% of the cases that had a low family income (125–200% of the federal poverty threshold). The remainder in each group were considered as middle/high income (greater than 200% of the federal poverty threshold). The median HRQL was significantly worse in the children with sickle cell disease compared to the control group in all summary scores, p < 0.05 adjusted for multiple comparisons (total score 67.4 versus 80.7, physical summary score 68.8 versus 87.5, psychosocial summary score 68.1 versus 75.0). When both disease and family income were examined in the regression model, children who were poor and who had sickle cell disease were more likely to have lower physical and psychosocial HRQL. Children with sickle cell disease were more than twice as likely to have an impaired physical HRQL compared to controls at each family income level. (Table 1). In conclusion, children with sickle cell disease have an increased probability of having impaired physical HRQL compared to children without sickle cell disease across each family income level. Predicted Probabilities of Poor Physical Health Score (PHS) (based on multinomial regression model) Group Income Level Pr (PHS ≥ 63.28)* 95% Confidence Interval *Denotes probability that PHS is≤ 1 standard deviation below the population mean reported by Varni et al. 2003 Control Poor/Near Poor 0.23 0.14–0.36 Low 0.24 0.12–0.41 Middle/High 0.10 0.04–0.22 Sickle Cell Disease Poor/Near Poor 0.49 0.37–0.61 Low 0.49 0.34–0.64 Middle/High 0.26 0.14–0.42
Recently it has been suggested that investigators should present health-related quality of life (HRQL) data in a more clinically interpretable way. The objective of this study was to determine the validity and reliability of the PedsQL questionnaire in children with sickle cell disease and to utilize proportions and a dichotomous outcome to demonstrate the impaired HRQL of children with sickle cell disease. A cross sectional study was conducted. Parents of children (ages 2 to 18 years) with sickle cell disease who presented to the clinic for a routine visit completed the PedsQL questionnaire. The PedsQL 4.0 generic questionnaire consists of 23 items that measure the child’s well being. The primary outcome measured was HRQL. Children were classified as severe sickle cell disease if they had a history of 1 or more major complications of sickle cell disease (overt stroke, acute chest syndrome, or priapism) or 3 or more hospitalizations for pain in the prior 3 years. All other children were considered mild. To determine validity, a two-sample t-test was used to compare HRQL scores between those with mild and severe disease with adjustment made for multiple comparisons. To determine internal reliability, a Cronbach’s alpha coefficient was calculated. To make the HRQL differences more clinically meaningful, the proportion of children who have problems walking a block or participating in sports or exercise (based on dichotomized Likert scale responses of “often/almost always” versus “sometimes/almost never/never”) was plotted against the mean physical summary score from the PedsQL. A total of 96 parents of children with sickle cell disease completed the PedsQL. Children with mild sickle cell disease had significantly better HRQL, as evidenced by a higher total (74.9), physical (75.2), and psychosocial (74.9) mean HRQL summary score, than those with severe disease (total score 60.3, physical summary score 58.9, psychosocial summary score 61.1; all p<0.01). All scales of the PedsQL had an acceptable alpha coefficient of greater than 0.7. Figure 1 shows the proportion of children with sickle cell disease, given their mean physical summary score on the PedsQL, who have problems walking a block or participating in sports or exercise. One out of 10 (10%) children with a mean score of 58.9 (mean score for severe disease) have problems walking around the block while 1 out of 6 (17%) have problems exercising or playing sports. In contrast, no children with a mean score of 75.2 (mean score for mild disease) report having problems with walking a block while just 1 out of 41 (2%) children report problems exercising or playing sports. In conclusion, the PedsQL 4.0 generic core scales demonstrated both validity, by differentiating between children with severe and mild sickle cell disease, and reliability in children with sickle cell disease. While the meaning of a 16 point difference in quality of life may not be readily apparent, a 10–15% decrease in the ability to walk a block or play sports certainly is easily understandable. Further work to broaden the HRQL field in sickle cell disease by developing a companion PedsQL sickle cell disease specific module and to promote presentation of HRQL results in an interpretable fashion is needed. Figure Figure
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