Robert Gibson

Transition from pediatric to adult care in sickle cell disease: Establishing evidence-based practice and directions for research

Transition of young adults with sickle cell disease (SCD) from pediatric to adult medical care is an important priority, given medical advances that have transformed SCD into a lifelong chronic condition, rather than a disease of childhood. Successful transfer from pediatric to adult care has its foundation in collaboration among the young adult, the family, and the health care system to support building skills in positive disease management and independent living. Systemic issues in transition from pediatric to adult care for individuals with SCD include limited access to adult providers with the skills and/or interest in caring for people with SCD; poor communication and follow-up between pediatric and adult providers; and insurance coverage and reimbursement for care coordination. Family and patient issues in transition include lack of skill development for successful transition into adulthood; absence of financial independence; fear of the unknown; and increasing morbidity with age. The design and evaluation of successful transition programming in SCD requires clarity in conceptual frameworks and consistent measurement, both before and after transfer to adult care. Strategies used by three SCD transition programs and future directions for research and program development are presented.

Rural ED transfers due to lack of radiology services

PURPOSE Our objectives were to determine the frequency of patient transfers to a tertiary care emergency department (Tertiary ED) due to a lack of radiology services in rural hospital EDs (Rural EDs), and examine the community and patient attributes that are associated with these transfers. METHODS This was a retrospective chart review of patients transferred to a Tertiary ED from Rural EDs. Transfers excluded from the study included pediatric patients (age <18 years old) and patients transferred for trauma surgeon evaluation. Only those patients who were transferred for radiology services were included in the final analysis. RESULTS Over a 12-month period, 1445 patients were transferred to the Tertiary ED with 73.8% (n = 1066) of this population being transferred from a Rural ED. Excluding 381 trauma and pediatric patients, 64.3% (n = 685) of patients were transferred from a Rural ED and were included in the study. Of these 685 transfers, 24.5% (n = 168) were determined to be due primarily to a lack of a radiology service. DISCUSSION Lack of radiology services in Rural EDs leads to numerous patient transfers to the Tertiary ED each year. A disproportionate number of these transfer patients are African American. These transfers place additional financial and social burdens on patients and their families. This study discusses these findings and alternative diagnostic options (ie, telemedicine and ultrasound video transfer) to address the lack of radiology services available in Rural EDs. The use of these alternate diagnostic options will likely reduce the number of patient transfers to Tertiary EDs.

CYP2C9 Allelic Variants and Frequencies in a Pediatric Sickle Cell Disease Cohort: Implications for NSAIDs Pharmacotherapy

Nonsteroidal anti‐inflammatory drugs (NSAIDs) used to treat pain in patients with sickle cell disease (SCD) are metabolized by the CYP2C9 enzyme. Racial differences in CYP2C9 allele frequencies impact NSAIDs efficacy and safety. We determined the frequencies of CYP2C9 alleles in an African American pediatric SCD cohort. Genomic DNA was isolated from blood samples of 30 patients aged between 7 and 17 years. Genotyping of nine CYP2C9 alleles (*1,*2, *3, *4, *5, *6, *8, *11, and *13) was performed using restriction fragment length polymorphism‐PCR assays and the Tag‐It™ Mutation Detection System. The wild type *1 allele frequency was 0.850. The most common variant allele detected was CYP2C9*8 (0.067). The combined frequency of the *2, *5, *6, *8, and *11 variants was 0.151. Seventy percent of the study cohort were predicted extensive metabolizers (*1/*1) and 30% were intermediate metabolizers due mainly to the *1/*8 genotype. Analysis of CYP2C9 using an expanded assay panel facilitated improved classification of predicted drug metabolic phenotypes in our cohort. However, the pharmacokinetic effects of the CYP2C9*5,*6,*8, and *11 alleles on NSAIDs metabolism has not been evaluated and underscores the need for studies on substrate‐specific effects of variant alleles common in populations with genetic susceptibility to SCD.

Determining Adherence to Quality Indicators in Sickle Cell Anemia Using Multiple Data Sources.

INTRODUCTION Advances in primary prophylaxis have resulted in improved outcomes for patients with sickle cell anemia (SCA; i.e., hemoglobin SS- and Sβ(0)-thalassemia). Standard prophylactic measures include a first pneumococcal polysaccharide vaccine (PPV) and transcranial Doppler ultrasound (TCD) at age 2 years. Though efficacious, evidence suggests that delivery of these interventions is suboptimal. This study reports adherence to these measures and examines concordance across various data sources, using Registry and Surveillance for Hemoglobinopathies project data. METHODS Retrospective database and SCA center chart review identified children with SCA aged 24-36 months between January 1, 2004, and December 31, 2008. PPV and TCD administration were determined through Medicaid and Childrens Health Insurance Program administrative claims data, medical record review, and Georgia Registry of Immunization Transaction and Services. Analysis was conducted in 2015. RESULTS A total of 125 children met inclusion criteria. Forty-five (36.0%) children had documentation of both interventions, whereas 19 (15.2%) had no documentation of either intervention. Sixty-one (48.8%) children obtained only one intervention. Of these, more were likely to have had PPV than TCD (77.0% vs 23.0%, respectively, p<0.001). Agreement between claims data and medical record review was moderate for PPV (κ=0.55) and substantial for TCD (κ=0.74). CONCLUSIONS No single, reliable data source for tracking standard of care for children with SCA statewide was found. According to study data, prophylaxis measures were not universally implemented during the surveillance period. Further research is needed to adequately track changes over time, determine risk groups, and develop methods of evaluating important metrics.

Advancing Genomic Research and Reducing Health Disparities: What Can Nurse Scholars Do?

PURPOSE Advances in genomic research are improving our understanding of human diseases and evoking promise of an era of genomic medicine. It is unclear whether genomic medicine may exacerbate or attenuate extant racial group health disparities. We delineate how nurse scholars could engage in the configuration of an equitable genomic medicine paradigm. ORGANIZING CONSTRUCT We identify as legitimate subjects for nursing scholarship the scientific relevance, ethical, and public policy implications for employing racial categories in genomic research in the context of reducing extant health disparities. FINDINGS Since genomic research is largely population specific, current classification of genomic data will center on racial and ethnic groups. Nurse scholars should be involved in clarifying how putative racial group differences should be elucidated in light of the current orthodoxy that genomic solutions may alleviate racial health disparities. CONCLUSIONS Nurse scholars are capable of employing their expertise in concept analysis to elucidate how race is used as a variable in scientific research, and to use knowledge brokering to delineate how race variables that imply human ancestry could be utilized in genomic research pragmatically in the context of health disparities. CLINICAL RELEVANCE In an era of genomic medicine, nurse scholars should recognize and understand the challenges and complexities of genomics and race and their relevance to health care and health disparities.

Advancing genomic research and reducing health disparities

PURPOSE Advances in genomic research are improving our understanding of human diseases and evoking promise of an era of genomic medicine. It is unclear whether genomic medicine may exacerbate or attenuate extant racial group health disparities. We delineate how nurse scholars could engage in the configuration of an equitable genomic medicine paradigm. ORGANIZING CONSTRUCT We identify as legitimate subjects for nursing scholarship the scientific relevance, ethical, and public policy implications for employing racial categories in genomic research in the context of reducing extant health disparities. FINDINGS Since genomic research is largely population specific, current classification of genomic data will center on racial and ethnic groups. Nurse scholars should be involved in clarifying how putative racial group differences should be elucidated in light of the current orthodoxy that genomic solutions may alleviate racial health disparities. CONCLUSIONS Nurse scholars are capable of employing their expertise in concept analysis to elucidate how race is used as a variable in scientific research, and to use knowledge brokering to delineate how race variables that imply human ancestry could be utilized in genomic research pragmatically in the context of health disparities. CLINICAL RELEVANCE In an era of genomic medicine, nurse scholars should recognize and understand the challenges and complexities of genomics and race and their relevance to health care and health disparities.

The utility of routine reticulocyte count in uncomplicated vaso-occlusive events due to sickle cell disease

Labs are routinely ordered for patients admitted to the emergency room for uncomplicated vaso-occlusive events (VOE), however, there are no “standard” screening practices. Review articles and expert opinions regarding routine testing vary widely in their recommendations [1-3]. In addition, recent literature has called into question the need for routine screening laboratory analysis in clinically uncomplicated VOE [1,4,5]. The objective of this study was to evaluate the utility of one measure, reticulocyte count, in cases of uncomplicated VOE in Sickle Cell Disease.

Process-Oriented Guided-Inquiry Learning: A Natural Fit for Occupational Therapy Education

ABSTRACT After a brief review of the major group cooperative learning strategies, this article presents the format and use of Process-Oriented Guided-Inquiry Learning (POGIL) as a recommended teaching strategy for occupational therapy classes. This recommendation is based upon evidence of effectiveness of this strategy for enhancing critical thinking, content retention, and teamwork. Strategies for learning the process and suggestions for its use are based upon literature evidence and the authors’ experiences with this strategy over 4 years in a class on evidence-based practice.