Development and validation of SIMS: An instrument for measuring quality of life of adults with sickle cell disease

Abstract

In recent years, there has been a growing interest in the assessment of quality of life (QOL) issues, particularly in chronic debilitating conditions. Several instruments have been developed, tested, and validated in the general population and in other chronic diseases; however, few studies have examined QOL issues in adults with sickle cell disease (SCD). We developed Sickle Cell Impact Measurement Scale (SIMS), an instrument for measuring the QOL of adults with SCD. The 142-item multi-dimensional SIMS questionnaire was developed using 4 validated instruments and additional questions based upon recommendations of patient focus groups. The SIMS was self-administered to 106 SCD and 45 rheumatoid arthritis (RA) patients over 4 sites. SIMS was evaluated on measures of both internal consistency and construct validity. Item reduction was performed based on results of factor analysis. The SIMS achieved good internal consistency, with a Cronbach's alpha coefficient reported of 0.86, and distinguished between patients with SCD and RA. Overall, QOL did not differ significantly among SCD and RA patients. However, SCD patients scored higher in both physical and social domains, which was expected and reflected the differences in the pathophysiology of each disease. The SIMS is a reliable, valid, and responsive questionnaire, which functions well as a discriminative instrument for the measure of health-related QOL (HRQOL) of adults with SCD. The SIMS is currently being administered to adults with SCD across several centers for further validation to become a disease-specific, global QOL instrument. Am. J. Hematol. 83:558-562, 2008. V

Health of adults living with a clinically significant haemoglobinopathy in New South Wales, Australia

“…The surveys were developed by adult and paediatric medical and nursing professionals involved in the management of haemoglobinopathies and drew upon previously published tools for the assessment of the health needs of people with these disorders. 10,11 Drafts of the survey were reviewed by a consumer group for validity. The surveys investigated a number of areas including symptoms, management and demographics and included both tick box answers and free text areas, with specific surveys available for adults, parents and young people for each of the two main disease groups.…”

Adult Sickle Cell Quality-of-Life Measurement Information System (ASCQ-Me)

“…87 The SIMS was developed from 4 existing instruments—3 of these instruments are general quality-of-life instruments and the fourth is an arthritis quality-of-life scale. After the SIMS was developed and psychometrically evaluated, it was modified based on a single focus group with 15 people with SCD.…”

“…75,86,87 The use of generic quality-of-life measurements with the SCD population has increased in the past decade, allowing comparison with well and other chronic populations. A recently developed SCD-specific quality-of- life measure found that overall quality of life did not differ between adults with SCD and patients with rheumatoid arthritis.…”

“…A recently developed SCD-specific quality-of- life measure found that overall quality of life did not differ between adults with SCD and patients with rheumatoid arthritis. 87 Patients with iron overload on iron chelation therapy were found to have lower quality-of-life scores on the SF-36 compared with population norms, 88 particularly if they experienced comorbidities. 89 …”

Quality of life in adults with sickle cell disease: an integrative review of the literature

“…Characterized by severe musculoskeletal pain and chronic hemolytic anemia, FD can present several complications, including: infections, heart disease, renal failure, stroke, leg ulcers and proliferative retinopathy (2)(3)(4)(5) .…”