Conclusion
We evaluated emotional distress and other variables in the context of a biobehavioral model of HRQL outcomes for adults with SCD. Integrating the patient's perspective of the impact of the disease and its treatment with assessment of clinical indications is critical to implementing and evaluating effective therapies [25]. However, there are conceptual challenges in determining what actually contributes to HRQL from the patient’s perspective in the context of genetic disorders such as SCD [50]. Our findings highlight the importance of incorporating comprehensive psychosocial screening in order to support optimal HRQL in SCD. Providers may be reluctant to include such screening if, as is often the case, mental health services are difficult to access. Models such as the collaborative care model, which include mental health interventions within the sickle cell center or primary care provider’s office, should be implented. Barriers to care and HRQL should also be routinely evaluated for patients with SCD. Use of disease-specific tools, such as the Adult Sickle Cell Quality of Life measurement system [66], may increase the specificity needed to detect differences within adults with SCD and improvements related to interventions, whether medical or psychosocial. Contributors to HRQL in SCD go beyond clinical manifestations to include psychological and social factors, as well as provider and health system variables. Research conducted within the framework of a comprehensive conceptual model of broad clinical and life effects associated with SCD can inform clinical applications that ultimately enhance HRQL for patients with SCD.
Acknowledgment: The authors wish to thank San Keller, PhD, for her helpful comments on a previous version of this manuscript.
Corresponding author: Marsha J. Treadwell, PhD, Hematology/Oncology Dept., UCSF Benioff Children’s Hospital Oakland, 747 52nd St., Oakland, CA 94609, mtreadwell@mail.cho.org.
Funding/support: This research was conducted as part of the National Initiative for Children’s Healthcare Quality (NICHQ) Working to Improve Sickle Cell Healthcare (WISCH) project. Further support came from a grant from the Health Resources and Services Administration (HRSA) Sickle Cell Disease Treatment Demonstration Project Grant No. U1EMC16492 and from the National Institutes of Health (NIH) Clinical and Translational Science Award UL1 RR024131. The views expressed in this publication do not necessarily reflect the views of WISCH, NICHQ, HRSA or NIH.
Financial disclosures: None.
Author contributions: conception and design, MJT; analysis and interpretation of data, MJT, GG; drafting of article, MJT, GG; critical revision of the article, MJT, KK, FB; statistical expertise, GG; obtaining of funding, MJT; administrative or technical support, KK, FB; collection and assembly of data, KK, FB.