Analyzing the Lived Experiences of Young People with Sickle Cell Disease

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A recent metasynthesis investigation identified common experiences among children with sickle cell disease (SCD). The recognition of SCD symptoms in children with the rare disease was prominent in the findings.¹

Prevalent themes in the children’s experience consisted of understanding the disease and its impact on the body and daily life; identifying ways to balance responsibilities, which involves managing competing demands and prioritizing self-care; and positioning oneself to thrive with SCD by developing a positive outlook, setting goals, and actively pursuing one's passions and interests.

Megan Coco, ND, School of Nursing, University of Connecticut, and a team of investigators aimed to investigate these experiences in a qualitative metasynthesis of children, adolescents, and young adults living with SCD.

The Analysis

The study used Noblit and Hare's phases of metaethnographic research to guide the synthesis of qualitative data. Data from 12 studies were analyzed, including 177 participants ranging in age from 6-35 years old from 6 different countries.

Studies were selected based on the inclusion and quality appraisal criteria, as well as the inclusion of diverse samples of patients with SCD.

Investigators were able to identify 3 key metaphors that capture the experience of living with SCD:

• Ubiquitous Intrusion
• Learning to Cope
• Part of the Whole.

According to the results, the first metaphor, ubiquitous Intrusion, refers to how SCD permeates every aspect of the individual's life. The disease is always present, affecting physical, emotional, and social well-being.

The second, learning to cope, reflects the adaptive process that children, adolescents, and young adults undergo as they learn to manage the challenges of living with SCD. This included developing coping skills and strategies, such as pain management techniques, self-care routines, and building support networks.

The third metaphor, part of the whole, highlighted the importance of social support and the need for individuals living with SCD to feel connected to their communities and broader society.

Balancing Sickle Cell Disease to Thrive

A few essential concepts elucidated the common metaphors:

• Recognition of SCD implications
• Identifying ways to balance responsibilities
• Positioning oneself to thrive with SCD

Investigators stated these are underlying concepts of the experiencing SCD.

According to the analysis, children and adolescents with SCD often feel the need to keep a "watching brief" over their pain and other symptoms. The hospital experience is viewed as overwhelming and disruptive to their lives, taking away their sense of autonomy.

It’s common for them to realize that SCD is an inherited, chronic, and incurable disease, at a young age. Prayer and a spiritual foundation are important to adolescents and young adults across all studies reviewed, regardless of religious affiliation.

Empowerment was another common theme, with adolescents using personal strengths, skills, and interpersonal resources to manage SCD. Non-pharmacologic approaches such as relaxation, deep breathing, and rest are also used to help with pain and fatigue.

The global theme of "Growing Beyond SCD" was noted in the study, with the intent of reflecting the shared complexity of living with SCD in young people of diverse cultures. Investigators also found that many individuals yearn for a normal life and are motivated to adapt and manage the disease with the support of their network.

According to the findings, the key metaphors and essential concepts identified in this metasynthesis can inform the development of nursing interventions designed to promote self-acceptance, coping, and adaptation skills among children, adolescents, and young adults living with SCD.

By understanding the lived experience of individuals with SCD, healthcare providers can better support patients and their families in managing the disease and achieving a fulfilling life.

References:

1. Coco, M., Henderson, W. A., Park, C. L., & Starkweather, A. R. (2023). Growing beyond sickle cell disease: A metasynthesis of children, adolescents, and young adult experiences living with sickle cell disease. Research in Nursing & Health, 1– 14. https://doi.org/10.1002/nur.22310