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This review examines the evidence for some of the common psychological complications found across the life span of patients with sickle cell disease (SCD), which are likely to be encountered by haematologists responsible for their medical management. Electronic searches of medical and psychological databases were conducted with a focus on three main areas: psychological coping, quality of life and neuropsychology. Psychological complications were identified in both children and adults with SCD, and included inappropriate pain coping strategies; reduced quality of life owing to restrictions in daily functioning, anxiety and depression; and neurocognitive impairment. There were wide variations in design and consistency of the studies, therefore, some caution needs to be observed in the findings. Moreover, interventional studies were lacking in some areas such as neuropsychology. Utilization of psychological interventions including patient education, cognitive behavioural therapy, and special educational support to help improve the quality of life of patients are recommended. [ABSTRACT FROM AUTHOR]

Objective. To examine the relationship between pain, coping, and quality of life in adult patients with sickle cell disease (SCD) in the UK, and to assess the influence of these factors on the utilization of health services. Design, This cross-sectional study involved 96 adult patients with SCD attending hospitals in London. Method. Interview and questionnaire study involving standard measures of pain, health service utilization, coping responses (measured with the Coping Strategies Questionnaire -- revised for SCD), and quality of life (measured by the SF36). Data concerning clinical history, complications and haemoglobin levels were also collected. Results. The number and duration of painful sickle cell episodes in the past 12 months varied widely between patients. We found that 49.5% of accident and emergency visits and 45% of hospitalizations were of 10.4% (10) patients. Pain experience accounted for 12.3% of hospital and general practice service use, independently of age, sex, number of SCD complications and haemoglobin levels. Three higher order factors emerged from analysis of the coping strategies questionnaire, reflecting active behavioural and cognitive coping, affecting coping, and passive adherence (e.g. resting, taking fluids). Active coping was positively associated with the number of pain episodes, while passive adherence coping was related to pain intensity. Psychological coping was unrelated to health service utilization. Marked impairment on many dimensions of quality of life was apparent in the analyses of the SF36. Affective coping was associated with impaired quality of life independently of demographic and clinical variables. Conclusions, It is evident that siclde cell disease involves not only severe recurrent pain, but also other impairments in health-related quality of life. Psychological coping patterns... [ABSTRACT FROM AUTHOR]

Purpose: Sickle cell disease (SCD) is an inherited blood disorder characterized by unpredictable episodes of acute pain and numerous health complications. Individuals with SCD often face stigma from the public, including perceptions that they are lazy or weak tending to exaggerate their pain crisis, which can profoundly impact their quality of life (QoL). Methods: In a qualitative phenomenological study conducted in Cameroon, Ghana, and Tanzania, we explored stakeholders' perceptions of SCD-related stigma using three analytical frameworks: Bronfenbrenner's Ecological Systems Theory; The Health Stigma and Discriminatory Framework; and A Public Health Framework for Reducing Stigma. Results: The study reveals that SCD-related stigma is marked by prejudice, negative labelling and social discrimination, with derogatory terms such as sickler, ogbanje (one who comes and goes), sika besa (money will finish), ene mewu (I can die today, I can die tomorrow), vampire (one who consumes human blood), and Efiewura (landlord-of the hospital), commonly used to refer to individuals living with SCD. Drivers of stigma include frequent crises and hospitalizations, distinct physical features of individuals living with SCD, cultural misconceptions about SCD and its association with early mortality. Proposed strategies for mitigating stigma include public health education campaigns about SCD, integrating SCD into school curricula, healthcare worker training and community engagement. Conclusion: The results highlight the importance of challenging stigmatizing narratives on SCD and recognizing that stigmatization represents a social injustice that significantly diminishes the QoL of individuals living with SCD. [ABSTRACT FROM AUTHOR]