Health related quality of life in sickle cell patients: The PiSCES project

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Abstract

Background

Sickle cell disease (SCD) is a chronic disease associated with high degrees of morbidity and increased mortality. Health-related quality of life (HRQOL) among adults with sickle cell disease has not been widely reported.

Methods

We administered the Medical Outcomes Study 36-item Short-Form to 308 patients in the Pain in Sickle Cell Epidemiology Study (PiSCES) to assess HRQOL. Scales included physical function, physical and emotional role function, bodily pain, vitality, social function, mental health, and general health. We compared scores with national norms using t-tests, and with three chronic disease cohorts: asthma, cystic fibrosis and hemodialysis patients using analysis of variance and Dunnett's test for comparison with a control. We also assessed whether SCD specific variables (genotype, pain, crisis and utilization) were independently predictive of SF-36 subscales, controlling for socio-demographic variables using regression.

Results

Patients with SCD scored significantly worse than national norms on all subscales except mental health. Patients with SCD had lower HRQOL than cystic fibrosis patients except for mental health. Scores were similar for physical function, role function and mental health as compared to asthma patients, but worse for bodily pain, vitality, social function and general health subscales. Compared to dialysis patients, sickle cell disease patients scored similarly on physical role and emotional role function, social functioning and mental health, worse on bodily pain, general health and vitality and better on physical functioning. Surprisingly, genotype did not influence HRQOL except for vitality. However, scores significantly decreased as pain levels increased.

Conclusion

SCD patients experience health related quality of life worse than the general population, and in general, their scores were most similar to patients undergoing hemodialysis. Practitioners should regard their HRQOL as severely compromised. Interventions in SCD should consider improvements in health related quality of life as important outcomes.

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Most cited references 26

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Integrating response shift into health-related quality of life research: a theoretical model.

M A G Sprangers, C E Schwartz (1999)

Patients confronted with a life-threatening or chronic disease are faced with the necessity to accommodate to their illness. An important mediator of this adaptation process is 'response shift' which involves changing internal standards, values and the conceptualization of quality of life (QOL). Integrating response shift into QOL research would allow a better understanding of how QOL is affected by changes in health status and would direct the development of reliable and valid measures for assessing changes in QOL. A theoretical model is proposed to clarify and predict changes in QOL as a result of the interaction of: (a) a catalyst, referring to changes in the respondent's health status; (b) antecedents, pertaining to stable or dispositional characteristics of the individual (e.g. personality); (c) mechanisms, encompassing behavioral, cognitive, or affective processes to accommodate the changes in health status (e.g. initiating social comparisons, reordering goals); and (d) response shift, defined as changes in the meaning of one's self-evaluation of QOL resulting from changes in internal standards, values, or conceptualization. A dynamic feedback loop aimed at maintaining or improving the perception of QOL is also postulated. This model is illustrated and the underlying assumptions are discussed. Future research directions are outlined that may further the investigation of response shift, by testing specific hypotheses and predictions about the QOL domains and the clinical and psychosocial conditions that would potentiate or prevent response shift effects.

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Lottery winners and accident victims: is happiness relative?

Philip Brickman, Dan Coates, Ronnie Janoff-Bulman (1978)

Adaptation level theory suggests that both contrast and habituation will operate to prevent the winning of a fortune from elevating happiness as much as might be expected. Contrast with the peak experience of winning should lessen the impact of ordinary pleasures, while habituation should eventually reduce the value of new pleasures made possible by winning. Study 1 compared a sample of 22 major lottery winners with 22 controls and also with a group of 29 paralyzed accident victims who had been interviewed previously. As predicted, lottery winners were not happier than controls and took significantly less pleasure from a series of mundane events. Study 2 indicated that these effects were not due to preexisting differences between people who buy or do not buy lottery tickets or between interviews that made or did not make the lottery salient. Paraplegics also demonstrated a contrast effect, not by enhancing minor pleasures but by idealizing their past, which did not help their present happiness.

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Pain in sickle cell disease. Rates and risk factors.

O S Platt, B D Thorington, D Brambilla … (1991)

Acute episodes of pain are the principal symptom of sickle cell disease, but little is known about the epidemiologic features of these episodes or risk factors for them, nor is it known whether patients with high rates of such episodes die prematurely. We prospectively studied the natural history of sickle cell disease in 3578 patients ranging from newborns to persons up to 66 years old who were followed at clinical centers across the United States. There were 12,290 episodes of pain in 18,356 patient-years. The average rate was 0.8 episode per patient-year in sickle cell anemia, 1.0 episode per patient-year in sickle beta 0-thalassemia, and 0.4 episode per patient-year in hemoglobin SC disease and sickle beta(+)-thalassemia. The rate varied widely within each of these four groups--e.g., 39 percent of patients with sickle cell anemia had no episodes of pain, and 1 percent had more than six episodes per year. The 5.2 percent of patients with 3 to 10 episodes per year had 32.9 percent of all episodes. Among patients with sickle cell anemia who were more than 20 years old, those with high rates of pain episodes tended to die earlier than those with low rates. High rates were associated with a high hematocrit and low fetal hemoglobin levels. alpha-Thalassemia had no effect on pain apart from its association with an increased hematocrit. The "pain rate" (episodes per year) is a measure of clinical severity and correlates with early death in patients with sickle cell anemia over the age of 20. Even when the fetal hemoglobin level is low, one can predict that small increments in the level may have an ameliorating effect on the pain rate and may ultimately improve survival. This outcome is particularly encouraging to investigators studying hydroxyurea and other treatments designed to increase the fetal hemoglobin level.

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Author and article information

Journal

Journal ID (nlm-ta): Health Qual Life Outcomes

Title: Health and Quality of Life Outcomes

Publisher: BioMed Central (London )

ISSN (Electronic): 1477-7525

Publication date Collection: 2005

Publication date (Electronic): 29 August 2005

Volume: 3

Page: 50

Affiliations

[1 ]Department of Biostatistics, Virginia Commonwealth University, Richmond, VA, USA

[2 ]Division of Quality Health Care, Department of Medicine, Virginia Commonwealth University, Richmond, VA, USA

[3 ]Department of Health Evaluation Sciences, University of Virginia, Charlottesville, VA, USA

[4 ]Division of Hematology/Oncology, Department of Medicine, Virginia Commonwealth University, Richmond, VA, USA

[5 ]Department of Emergency Medicine, Virginia Commonwealth University, Richmond, VA, USA

[6 ]Department of Psychiatry, Virginia Commonwealth University, Richmond, VA, USA

[7 ]Department of Pathology, Virginia Commonwealth University, Richmond, VA, USA

Article

Publisher ID: 1477-7525-3-50

DOI: 10.1186/1477-7525-3-50

PMC ID: 1253526

PubMed ID: 16129027

SO-VID: 31cd25e7-6118-40f6-b491-f5fbc07b599e

License:

This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Health related quality of life in sickle cell patients: The PiSCES project

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Most cited references 26

Integrating response shift into health-related quality of life research: a theoretical model.

Lottery winners and accident victims: is happiness relative?

Pain in sickle cell disease. Rates and risk factors.

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