Search

Sickle cell disease (SCD) is an inherited hemoglobinopathy that leads to blood vessel occlusion and multiorgan complications, including pain, that may be experienced daily. Symptom management often begins at home, and tools are needed to support self-management strategies that can be implemented by children with SCD and families. The purpose of this study was to assess the feasibility of the mHealth self-management intervention (application) Voice Crisis Alert V2 for children with SCD and families. Feasibility assessment was guided by the Reach, Efficacy, Adoption, Implementation, and Maintenance framework. Data were collected with 60 dyads (children with SCD/caregivers) at four time points. Self-management data were collected via application use, and postintervention interviews were conducted. Analyses included descriptive statistics and constant comparison with directed content analysis. Recruitment was completed in 28 weeks, with 82% retention at end-of-intervention. Mobile Application Rating Scale scores and interview data indicated high satisfaction. From baseline to mid-intervention, 94% of dyads used the application (75% of total use); 45% used the application from mid-intervention to the end-of-intervention. Dyads made 2,384 actions in the application; the most commonly used features were recording health history and recording and tracking symptoms. Few reported issues with the application; most issues occurred early in the study and were corrected. After the intervention period was completed, 37% continued to use the application. Feasibility was confirmed by meeting recruitment and retention goals, high adoption of the application, and high reported satisfaction with the application. Challenges with sustained use were encountered, and areas for improvement were identified.

Background Adults with cystic fibrosis have unique barriers that may decrease their ability to receive adequate social support and socialization, leading to social isolation. Social isolation has been correlated with negative health outcomes in other populations. In those with cystic fibrosis, social isolation may present additional physiological and psychological challenges, potentially interfering with clinical outcomes and quality of life. However, there is a lack of understanding as to how social isolation presents in this population. Methods The purpose of this integrative review is to identify and critically analyze how social support and social isolation are reported for adults with cystic fibrosis. PubMed, Scopus, and CINAHL Complete were searched for related publications, resulting in an initial yield of 1767 articles. After eligibility screening, 21 studies met the criteria for this review, which were all then critically analyzed and synthesized. Results There is a scarcity of literature focusing on social isolation and social support in this population. Reduced physical and mental health were the most commonly reported variables associated with reduced social functioning and social support. Conclusion Preliminary studies are warranted to understand how adults with cystic fibrosis experience social isolation, as well as its relationship to social support. This knowledge can guide future research focusing on physical and psychological effects of social isolation, along with interventions that facilitate socialization and support.