The evolution of the COVID-19 pandemic in paediatric patients with sickle cell disease: From Alpha to Omicron.

We compare the impact of SARS-CoV-2 variants on healthcare utilization and clinical presentation in paediatric patients with sickle cell disease (SCD). One hundred and ninety-one unique patients with SCD and positive SARS-CoV-2 polymerase chain reactions were identified between March 2020 and January 2022. Hospitalizations, which accounted for 42% (N = 81) of cases, were highest during the Delta dominant era (48%) and lowest during Omicron (36%) (p = 0.285). The most common SCD-related complication was vaso-occlusive pain (37%, N = 71), which accounted for 51% of all hospital admissions (N = 41), and acute chest was highest in the Alpha variant era (N = 15). Overall, COVID-19 remained mild in clinical severity within most paediatric SCD patients.

Telemedicine in sickle cell disease: Patient, parent, and provider perspectives.

INTRODUCTION: Patients with sickle cell disease (SCD) need frequent health maintenance visits and may face barriers accessing care. Telemedicine, during COVID pandemic, has provided a unique model of care to improve access; however, potential barriers and satisfaction with its use in SCD have not been fully evaluated. OBJECTIVE: To determine caregiver, patient, and healthcare provider (HCP) perspectives and satisfaction with telemedicine in healthcare delivery. METHODS: We surveyed patients with SCD, caregivers, and HCP, who participated in at least one telemedicine visit from March 2020 to June 2021, using the Telemedicine Usability Questionnaire (TUQ). We also accessed and compared the Press Ganey surveys completed by families who completed a telemedicine or in-person visit. Data were summarized using descriptive statistics. The internal reliability of TUQ was assessed using Cronbach's coefficient alpha. Press Ganey data comparing satisfaction with telemedicine versus in-person visits were analyzed by Mann-Whiney U test. RESULTS: Fifty-two patients/caregivers and 10 HCP completed the survey. Patients/caregivers rated satisfaction "excellent" in the five areas (Usefulness, Ease of use, Effectiveness, Reliability and Satisfaction). HCP rated Usefulness, Ease of use, Effectiveness, Satisfaction as "good," and Reliability as "excellent." Press Ganey scores for satisfaction with care for telemedicine and in-person visits were not statistically different (p > .05). DISCUSSION: We found high satisfaction for caregivers and patients as well as HCP in the delivery of clinical services via telemedicine for SCD. We suggest that telemedicine is a viable option for this population and may help overcome the barriers SCD families often face accessing care.

The genogram as a recruitment tool for identifying primary caregivers of youth living with sickle cell disease preparing for transition.

AIMS: The purpose of this secondary analysis was to describe the impact of using genograms to identify family caregivers from an original research study which used family caregiver-adolescents/young adults (AYA) dyads. BACKGROUND: Research to improve transition preparation for AYA with chronic disease is essential as 90% will survive into adulthood. Family-based transition research is specifically needed as a majority of transition preparation will occur in the home setting. Dyadic research on transition has not previously described strategies for recruiting appropriate family caregivers. DESIGN: A descriptive, secondary analysis was conducted using genograms developed during the original study conducted between October 2019 and February 2020. METHODS: For this secondary analysis conducted between July 2020 and August 2021, 50 genograms were analysed using descriptive statistics to describe family structures, relationships and responsibilities in families of AYAs living with sickle cell disease. RESULTS: In 43 genograms, there was only one primary caregiver in the family. In seven genograms, there were multiple primary caregivers who met the inclusion criteria for primary caregiver in a single family. In five genograms, there were two appropriate primary caregivers in a single family, and in two genograms, there were three appropriate individuals in a single family who met study criteria as a primary caregiver. CONCLUSIONS: Findings from the analysis of the genograms used in the original study demonstrated potential ability to improve on dyad recruitment by more specifically identifying the family member most involved in supporting the AYA's disease management. IMPACT: Genograms are an established tool for gathering information on families and application with recruitment could improve research in the realm of transition and other family-based research. NO PATIENT OR PUBLIC CONTRIBUTION: This was a secondary analysis that assessed already existing data.

Decision-Making Involvement, Self-Efficacy, and Transition Readiness in Youth With Sickle Cell Disease.

BACKGROUND: Transition to adult healthcare is a critical time for adolescents and young adults (AYAs) with sickle cell disease, and preparation for transition is important to reducing morbidity and mortality risks associated with transition. OBJECTIVE: We explored the relationships between decision-making involvement, self-efficacy, healthcare responsibility, and overall transition readiness in AYAs with sickle cell disease prior to transition. METHODS: This cross-sectional, correlational study was conducted with 50 family caregivers-AYAs dyads receiving care from a large comprehensive sickle cell clinic between October 2019 and February 2020. Participants completed the Decision-Making Involvement Scale, the Sickle Cell Self-Efficacy Scale, and the Readiness to Transition Questionnaire. Multiple linear regression was used to assess the relationships between decision-making involvement, self-efficacy, healthcare responsibility, and overall transition readiness in AYAs with sickle cell disease prior to transition to adult healthcare. RESULTS: Whereas higher levels of expressive behaviors, such as sharing opinions and ideas in decision-making, were associated with higher levels of AYA healthcare responsibility, those behaviors were inversely associated with feelings of overall transition readiness. Self-efficacy was positively associated with overall transition readiness but inversely related to AYA healthcare responsibility. Parent involvement was negatively associated with AYA healthcare responsibility and overall transition readiness. DISCUSSION: While increasing AYAs' decision-making involvement may improve AYAs' healthcare responsibility, it may not reduce barriers of feeling unprepared for the transition to adult healthcare. Facilitating active AYA involvement in decision-making regarding disease management, increasing self-efficacy, and safely reducing parent involvement may positively influence their confidence and capacity for self-management.

Reproductive health and knowledge among youth with sickle cell disease.

Knowledge related to reproductive health in adolescents with sickle cell disease (SCD) is not fully addressed. We evaluated reproductive health and knowledge among adolescent girls with SCD. Seventy-nine adolescents, 13-21 years of age completed a survey on reproductive health and knowledge with menarche age 13.2 (± 1.7) years. Fifty-four percent reported dysmenorrhea and 49% reported SCD pain a week before menstrual cycle. Sixty-two percent reported discussing contraception and pregnancy with medical providers. Adolescents reported late menarche, dysmenorrhea, and pain with menses. Knowledge of overall reproductive health was inadequate. There is an urgent need to improve reproductive education in this population.

Youths' Experiences of Transition from Pediatric to Adult Care: An Updated Qualitative Metasynthesis.

PROBLEM: Improvements in chronic disease management has led to increasing numbers of youth transitioning to adult healthcare. Poor transition can lead to high risks of morbidity and mortality. Understanding adolescents and young adults (AYA) perspectives on transition is essential to developing effective transition preparation. The aim of this metasynthesis was to synthesize qualitative studies assessing the experiences and expectations of transition to adult healthcare settings in AYAs with chronic diseases to update work completed in a prior metasynthesis by Fegran, Hall, Uhrenfeldt, Aagaard, and Ludvigsen (2014). ELIGIBILITY CRITERIA: A search of PubMed, Medline, PsycINFO, and CINAHL was conducted to gather articles published after February 2011 through June 2019. SAMPLE: Of 889 articles screened, a total of 33 articles were included in the final analysis. RESULTS: Seven main themes were found: developing transition readiness, conceiving expectations based upon pediatric healthcare, transitioning leads to an evolving parent role, transitioning leads to an evolving youth role, identifying barriers, lacking transition readiness, and recommendations for improvements. CONCLUSIONS: Findings of this metasynthesis reaffirmed previous findings. AYAs continue to report deficiencies in meeting the Got Transition® Six Core Elements. The findings highlighted the need to create AYA-centered transition preparation which incorporate support for parents. IMPLICATIONS: Improvements in transition preparation interventions need to address deficiencies in meeting the Got Transition® Six Core Elements. More research is needed to identify and address barriers implementing the transition process.

Characterization of natural killer cells expressing markers associated with maturity and cytotoxicity in children and young adults with sickle cell disease.

BACKGROUND: Sickle cell disease (SCD) is increasingly recognized as a red blood cell disorder modulated by abnormally increased inflammation. We have previously shown that in patients with SCD not on a disease-modifying therapy (hydroxyurea or chronic transfusions), natural killer (NK) cell numbers are increased. In the current study, we further investigated the NK cell function to determine if there was evidence of increased activation and cytotoxicity. PROCEDURE: We conducted a cross-sectional study of 44 patients with HbSS/HbSß0 thalassemia at steady state (hydroxyurea = 13, chronic transfusion = 11, no disease-modifying therapy = 20) and 23 healthy controls. Using a fresh blood sample, NK immunophenotyping was performed as follows: NK cells (CD3- CD56+ lymphocytes) were evaluated for makers associated with activation (NKG2D, NKp30, NKp44, and CD69) and maturity (CD57, killer immunoglobulin-like receptors (KIR), and CD56dim). Degranulation and cytotoxicity assays were performed to evaluate NK cell function. RESULTS: Patients with SCD who were not on disease-modifying therapy had a higher number of NK cells with an immunophenotype associated with increased cytotoxicity (NKG2D+ , NKp30+ , CD56dim+ , and KIR+ NK cells) compared with healthy controls and patients on hydroxyurea. NK cells from SCD patients not on disease-modifying therapy demonstrated significantly increased cytotoxicity (measured by assaying NK cell killing of the K562 cell line) compared with healthy controls (P = 0.005). Notably, NK cell cytotoxicity against K562 cells in the hydroxyurea or chronic transfusion patients was not significantly different from that in healthy controls. CONCLUSION: SCD is associated with increased NK cell function as well as increased NK cell numbers, which appears to be normalized with disease-modifying therapy.

Views of parents of children with sickle cell disease on pre-implantation genetic diagnosis.

Pre-implantation genetic diagnosis (PGD) is an option for parents who have a child with sickle cell disease (SCD) to have another child without SCD. We conducted a survey of 19 parents with at least one child with SCD to investigate views on PGD. Before education, 44% of parents were aware of PGD. All parents rated PGD education as important. All parents considering another child also reported interest in using PGD if insurance covered its costs. Parents who have a child with SCD appear to be interested in PGD and educational tools informing this group about PGD should be developed.

Measuring Transition Readiness: A Correlational Study of Perceptions of Parent and Adolescents and Young Adults with Sickle Cell Disease.

Adolescents and young adults (AYAs) often transfer from pediatric to adult care without adequate preparation, resulting in increased morbidity and mortality. The purpose of this descriptive research study of parent/AYA dyads was to measure perceptions of transition readiness. Factors that were found to be associated with perceptions of increased readiness to transition included AYA age, the amount of responsibility AYAs assume for their healthcare and the degree of parent involvement. More attention should be focused on these aspects of care to improve transition from pediatric to adult care for AYAs with sickle cell disease.