Quality of Life and Sickle Cell Disease.
Authors: Adams-Graves Patricia , Johnson C. , Corley P. , Lamar K. 
Published: October 2019

Abstract
Currently, there is no validated disease-specific, global quality of life (QOL) instrument for sickle cell disease (SCD). The purpose of the current research was to develop an instrument to assess QOL in adults with SCD. The 126-item QOL Questionnaire was developed using 4 validated instruments and their original, disease-specific QOL questions. These instruments included the following: Arthritis Impact Measurement Scales (Likert scale 1 to 5) that measures physical function, psychological status, and pain; Rand Instrument that assesses general well being; Cantril Ladder Visual Analog Scaling (10-point scale) for life satisfaction and life improvement goals; and Psychological Adjustment to Illness Scale Self Report for measures of vocation and sexual relationships. Quality of and access to care were measured using scaling of patients’ perceptions of health care provider effectiveness in pain management. These questions were original to the study. Generic questions were selected based upon debriefing 10 patients who previously completed the survey. Paper questionnaires were administered to “walk-in” patients at the sickle cell clinic over a period of 3 months. QOL scores were generated for multi-item measures of: overall QOL; general health perceptions (pain, overall health, and health perceptions); physical functioning (mobility, walking and bending, arm function, and ability to perform household tasks); emotional well being (tension level, mood, life satisfaction, life improvement goals, and general feelings); social functioning (activities, support, work or vocation, and personal relationships); and quality of and access to care (social services, self care, and perception of health personnel). The overall QOL score was obtained by summing the scores for the multi-item measures. The maximum possible QOL score was 600. An independent-sample t-test was conducted to compare QOL scores for SCD and rheumatoid arthritis (RA) patients, as well as patients by sex and co-morbidity status. One-way analysis of variance (ANOVA) with post-hoc significance testing was used to determine whether the QOL varied significantly by age, income, and education. A total of 151 patients (SCD=106, RA=45) completed the questionnaire. Pilot data demonstrates there are no statistically significant differences in overall QOL scores (p=0.574) of SCD and RA patients. There were also no statistically significant differences in overall QOL scores of SCD patients than in RA patients when analyzed by gender (p=0.515), income (p=0.221), education (p=0.228), and co-morbidity status (p=0.642). Pain QOL scores were higher in SCD patients. Mobility QOL scores were significantly different between RA and SCD patients (p=0.001). Mobility, arm movement, walking and bending, and ability to perform household task QOL scores were higher in SCD patients. SCD patients scored higher on QOL measures of mood, tension, and life satisfaction. However, RA patients scored significantly higher on the measure of life improvement goal setting (p=0.0001). There were no statistically significant differences seen in social activity scores of RA and SCD patients in QOL measures of social functioning. Respondents reported good socialization with peers. We have modified our instrument to reflect SCD-specific QOL issues by deleting domains more specific for RA suggested by the patients and by adding an interruption scale. The differences that we observed were consistent with the pathophysiology of the two diseases. This instrument, Sickle Cell Impact Measurement Scales, will be administered to sickle cell patients in several centers.