Exploring health-related quality of life, exercise and alcohol use in adolescents with sickle cell disease and healthy siblings.

OBJECTIVE: This study explored the health-related quality of life (HRQL) and health behaviours of adolescents with sickle cell disease (SCD) and healthy siblings, drawing on Gap theory which suggests HRQL is the discrepancy between current and ideal selves. DESIGN: Twenty-three adolescents with SCD and 21 healthy siblings aged 13 to 17 years participated in eight focus groups. RESULTS: Thematic analysis identified three themes: learning to accept SCD, coping with SCD and influences on health behaviours. Adolescents appear to have normalised and adapted to SCD. Adolescents with SCD have learnt effective coping strategies, such as moderating engagement in exercise. Unlike heathy siblings, they were not encouraged to exercise by parents but were content with their level of participation. Both groups were influenced to exercise by role models or wanting to socialise, and influenced to drink alcohol by peers, but there was limited understanding of the potential negative impacts of alcohol on SCD. CONCLUSION: There does not appear to be a discrepancy between adolescents' current and ideal selves, providing optimism about their HRQL. Further consideration of engaging in healthy behaviours is needed, but it is important to strike a balance so that modifications to lifestyle do not impair HRQL.

A qualitative exploration of health-related quality of life and health behaviours in children with sickle cell disease and healthy siblings.

OBJECTIVES: This study explored the health-related quality of life (HRQL) and health behaviours of children with sickle cell disease (SCD) and healthy siblings, drawing on Gap theory, which suggests HRQL is the discrepancy between current and ideal selves. DESIGN: Thirty-two interviews, facilitated by children's drawings of their current and ideal selves were thematically analysed. RESULTS: Two themes were identified. First, limitations of SCD and adjusted expectations. Children with SCD report some discrepancy in HRQL as they would like to participate in more physical activity, but overall, they appear to have normalised their condition and adjusted their expectations in the context of the limits of their condition. Healthy siblings worry about their sibling and have greater expectations about engaging in adventurous activities and for their future. Second, coping with SCD. Children have limited social support, although children with SCD seek support from their mothers. They also modify health behaviours, like reducing exercise to help prevent and cope with sickle-related pain. CONCLUSION: Children have some discrepancies in their HRQL but adjusted expectations among children with SCD may reduce discrepancy. Adapting health behaviours may help to cope with SCD but it is important that reductions in physical activity do not impair HRQL.

Large Drill Holes Are Still Present in the Long Term After Arthroscopic Bankart Repair With Absorbable Tacks: An 18-Year Randomized Prospective Study.

BACKGROUND: Studies have demonstrated the development of an osseous reaction at the drill sites of anchors after arthroscopic shoulder surgery. PURPOSE: To investigate the drill-hole size at 18 years after arthroscopic Bankart repair using either fast polygluconate acid (PGA) or slow polylevolactic acid (PLLA) absorbable tacks and to compare the functional outcomes and development of osteoarthritis. STUDY DESIGN: Randomized controlled trial; Level of evidence, 2. METHODS: 40 patients with unidirectional anterior shoulder instability, treated with arthroscopic Bankart repair, were randomized into the PGA group (n = 20) or the PLLA group (n = 20). Plain radiographs of both shoulders, as well as computed tomography (CT) images of the operated shoulder, were used to evaluate the drill-hole size, volume, and degenerative changes. Functional outcomes were assessed by use of the Rowe score, Constant score, and Western Ontario Shoulder Instability (WOSI) index. RESULTS: Of the 40 patients, 32 patients returned for the follow-up (15 PGA and 17 PLLA). No significant differences were found in the population characteristics between the study groups. The mean follow-up time was 18 years for both groups. No significant differences were seen in range of motion, strength in abduction, or Constant, Rowe, and WOSI scores between the groups. Recurrence rate was 33% in the PGA group and 6% in the PLLA group during the follow-up period (P = .07). The drill-hole appearance on plain radiographs (invisible/hardly visible/visible/cystic) was 11/2/2/0 and 6/5/5/1 for the PGA and PLLA groups, respectively (P = .036). The mean ± SD drill-hole volume as estimated on CT images was 89 ± 94 and 184 ± 158 mm3 in the PGA and PLLA groups, respectively (P = .051). Degenerative changes (normal/minor/moderate/severe) on plain radiographs were 7/4/4/0 and 3/8/5/1 for the PGA and PLLA groups, respectively (P = .21), and on CT images were 5/7/3/0 and 2/6/6/3 for the PGA and PLLA groups, respectively (P = .030). CONCLUSION: This long-term follow-up study demonstrated that the PLLA group had significantly more visible drill holes than the PGA group on plain radiographs. However, this difference was not evident on CT imaging, with both groups having several visible cystic drill holes and a substantial drill-hole volume defect. No significant differences were found between the study groups in terms of clinical outcomes.

Assessing the quality of life of children with sickle cell anaemia using self-, parent-proxy, and health care professional-proxy reports.

OBJECTIVES: The quality of life (QoL) of children with sickle cell anaemia (SCA) in the United Kingdom has not been examined, and a discrepancy measure based on Gap theory has rarely been used. This study investigated whether (1) child self-reports of QoL using a discrepancy measure (the Generic Children's QoL Measure; GCQ) are lower than those from healthy children, (2) proxy reports from parents and health care professionals are lower than child self-reports, and (3) demographic and disease severity indicators are related to QoL. DESIGN AND METHODS: An interdependent groups, cross-sectional design was implemented. Seventy-four children with SCA, their parent, and members of their health care team completed the GCQ. Demographic and disease severity indicators were recorded. GCQ data from healthy children were obtained from the UK Data Archive. RESULTS: Contrary to past research, when examining generic discrepancy QoL, children with SCA did not report a lower QoL than healthy children, and parent- and health care professional-proxy reports were not lower than child self-reports. Few of the demographic and disease severity indicators were related to QoL. CONCLUSIONS: Proxy reports may be used to gain a more complete picture of QoL, but should not be a substitute for self-reports. The explanation for the relatively high levels of QoL reported is not clear, but children with SCA may have realistic expectations about their ideal-self, place greater emphasis on aspects other than health in shaping their QoL, and define achievements within the limits of their illness. Future research should focus on psychological factors in explaining QoL. Statement of contribution What is already known on this subject? Children with sickle cell disease (SCD) generally have a reduced QoL compared with healthy children, but there appears to be no research measuring QoL in paediatric SCD in the United Kingdom. Proxy QoL reports from parents are often lower than child self-reports, but there is less research examining proxy reports from health care professionals. Previous research has measured paediatric QoL using measures of current health-related QoL, but this is not in line with the WHO's definition of QoL as the discrepancy between current state and expectations. What does this study add? Children with Sickle cell anaemia do not have an impaired discrepancy QoL; they may have realistic expectations about their ideal-self and define achievements within the limits of their illness. Health care professionals are able to gauge a SCA child's discrepancy QoL better than parents. The GCQ (a generic discrepancy measure of QoL) takes into account expectations about ideal QoL and does not emphasize health; it may be of use to Psychologists working with SCA children.