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Health-related quality of life in adults with sickle cell disease (SCD): a report from the comprehensive sickle cell centers clinical trial consortium.

ABSTRACT: Adults with Sickle Cell Disease (SCD) experience multiple disease-related complications, but few studies have examined relationships between these events and health-related quality of life (HRQOL). We determined the number and type of previous or co-occurring SCD-related complications and their reported HRQOL in a cohort of 1,046 adults from the Comprehensive Sickle Cell Centers (CSCC). Participants had a median age of 28.0 years (48% male, 73% SS or S?? thalassemia) and had experienced several SCD-related complications (mean 3.8 ± 2.0), which were influenced by age, gender, and hemoglobinopathy type (P < 0.0001). In multivariate models, increasing age reduced all SF-36 scales scores (P < 0.05) except mental health, while female gender additionally diminished physical function and vitality scale scores (P < 0.01). Of possible complications, only vaso-occlusive crisis, asthma, or avascular necrosis diminished SF-36 scale scores. Chronic antidepressants usage predominantly diminished scores on bodily pain, vitality, social functioning, emotional role, and mental health scales, whereas chronic opioid usage diminished all scale scores (P < 0.01). Our study documents substantial impairment of HRQOL in adults with SCD that was influenced by only a few of many possible medical complications. It suggests that more effective treatments of persistent pain and depression would provide the largest HRQOL benefit.

SUBMITTER: Dampier C

PROVIDER: S-EPMC3554393 | biostudies-literature | 2011 Feb

REPOSITORIES: biostudies-literature

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Health-related quality of life in adults with sickle cell disease (SCD): a report from the comprehensive sickle cell centers clinical trial consortium.

Dampier Carlton C LeBeau Petra P Rhee Seungshin S Lieff Susan S Kesler Karen K Ballas Samir S Rogers Zora Z Wang Winfred W

American journal of hematology 20110201 2

Adults with Sickle Cell Disease (SCD) experience multiple disease-related complications, but few studies have examined relationships between these events and health-related quality of life (HRQOL). We determined the number and type of previous or co-occurring SCD-related complications and their reported HRQOL in a cohort of 1,046 adults from the Comprehensive Sickle Cell Centers (CSCC). Participants had a median age of 28.0 years (48% male, 73% SS or Sβ⁰ thalassemia) and had experienced several ...[more]

PMID: 21264908

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Project description:Understanding patient experiences, quality of life, and treatment needs in individuals with sickle cell disease (SCD) is essential in promoting health and well-being. We used measures from the Adult Sickle Cell Quality of Life Measurement Information System (ASCQ-Me), Patient Reported Outcomes Measurement Information System (PROMIS), and Quality of Life in Neurological Disorders (NeuroQol) to evaluate pain impact, sleep impact, social functioning, depressive symptoms, tiredness, and cognitive function (collectively, patient reported outcomes [PROs]) and to identify associated demographic and clinical characteristics. Participants (n = 2201) between 18 and 45 years were recruited through the eight Sickle Cell Disease Implementation Consortium (SCDIC) sites. In multivariate models, PROs were significantly associated with one another. Pain impact was associated with age, education, employment, time since last pain attack, hydroxyurea use, opioid use, sleep impact, social functioning, and cognitive function (F = 88.74, P < .0001). Sleep impact was associated with household income, opioid use, pain impact, social functioning, depressive symptoms, and tiredness (F = 101.40, P < .0001). Social functioning was associated with employment, pain attacks in the past year, autoimmune/inflammatory comorbidities, pain impact, sleep impact, depressive symptoms, tiredness, and cognitive function (F = 121.73, P < .0001). Depressive symptoms were associated with sex, sleep impact, social functioning, tiredness, and cognitive function (F = 239.51, P < .0001). Tiredness was associated with sex, education, sleep impact, social functioning, depressive symptoms, and cognitive function (F = 129.13, P < .0001). These findings reflect the baseline PRO assessments among SCDIC registry participants. Further research is needed to better understand these outcomes and new targets for interventions to improve quality of life and function in people with SCD.

Project description:PurposeTo investigate the feasibility, acceptability, and preliminary efficacy of a 6-session music therapy protocol on self-efficacy, quality of life, and coping skills in adults with sickle cell disease (SCD).Patients and methodsUsing a mixed-methods intervention design, adults with SCD (ages 21-57; mean age 32.33) were randomized (1:1) to either 1) a 6-session music therapy (MT) intervention (n = 12) or 2) waitlist control (WLC) (n = 12) using stratified randomization where factors were age in years (≤30 vs >30), and sex (male, female). All participants completed two weeks of daily electronic pain diary entries and self-efficacy, quality of life, and coping skills measures before and after their assigned study condition to explore preliminary efficacy. MT participants were taught music exercises accessed via smartphone and subsequently interviewed to determine feasibility and acceptability.ResultsThe enrollment rate was 89%. All study measures were completed, with high rates of electronic pain diary completion at baseline (70%) and 2-week follow-up (66%). Interviews revealed two overall themes related to MT participants' experience: 1) participants learned new self-management skills and 2) MT improved participants' ability to cope with pain. MT participants demonstrated 100% attendance. In preliminary analyses, MT participants demonstrated significant improvements (means ± SD) in self-efficacy (5.42 ± 5.43, p = 0.008, d = 1.20), PROMIS sleep disturbance (-1.49 ± 6.68, p = 0.023, d = -0.99), PROMIS pain interference (-2.10 ± 4.68, p = 0.016, d = -1.06), and ASCQ-Me social functioning impact scores (2.97 ± 6.91, p = 0.018, d = 1.05) compared to WLC participants.ConclusionPreliminary findings support the feasibility and acceptability of music therapy for home use in adults with SCD. While music therapy may assist adults with SCD in improving self-efficacy and quality of life, subsequent, fully-powered clinical research is needed to determine its efficacy.

Project description:Background & aimsSickle cell disease (SCD), a genetic disorder resulting from the presence of a mutated hemoglobin S (HbS), has a worldwide distribution and causes significant morbidity and mortality in children and adults. Few studies addressed the determinants of SCD severity in adults; therefore, we investigated the impact of nutrition on the outcome of SCD and health-related quality of life (HRQoL) in adult patients.MethodsIn this longitudinal study, we recruited and prospectively followed 62 adults with SCD (aged ≥18 years) for a median of 93 months. At entry and follow-up, patients provided medical and dietary history, had a physical examination and anthropometric measurements, assessed protein-energy intake, measurement of micronutrient levels, estimation of SCD severity score, and determination of the HRQoL (SF-26v2). The study outcome was a composite of hospitalization due to SCD crises or death.ResultsAt baseline, 42 (67.74%) patients had macro and, or micro-undernutrition (Group A), and 20 (32.26%) were well nourished. (Group B). The BMI and most anthropometric measurements were significantly lower in SCD patients compared to control subjects. Seventy percent of SCD patients had vitamin D, vitamin B12, and zinc deficiencies. Thirty-six under-nourished patients (86%) had gastrointestinal disorders. During follow-up, 46 patients (74.19%) developed one or more vaso-occlusive pain crises or other SCD related complications that required hospitalization. Significant differences in most SF-36v2 domains existed between well-nourished and undernourished SCD patients. Protein-energy and micronutrient deficiencies were independent predictors of severe SCD and mortality. Correction of undernutrition and hydroxyurea therapy improved SCD severity scores and HRQoL.ConclusionsPatients with sickle cell disease have various degrees of macro and micro deficiencies, which increase SCD severity and hospitalizations and reduce the health-related quality of life. Early diagnosis and prompt correction of macro and micronutrient deficiencies need to be incorporated in the standard of care of SCD patients to improve the disease outcomes.

Dataset Information

Health-related quality of life in adults with sickle cell disease (SCD): a report from the comprehensive sickle cell centers clinical trial consortium.

Publications

Health-related quality of life in adults with sickle cell disease (SCD): a report from the comprehensive sickle cell centers clinical trial consortium.

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