Background: Acute chest syndrome (ACS) is the leading cause of mortality in patients with sickle cell disease (SCD). Treatment is multimodal and includes anti-inflammatories like corticosteroids. While systemic corticosteroids may decrease length of hospital stay for ACS, the risk of readmission for vaso-occlusive crises (VOC) has limited their use. Inhaled corticosteroids (ICS) may be a safe alternative, but efficacy is currently unknown. Objectives: To investigate the effects of initiation of ICS at the time of hospital admission in reducing ACS morbidity. Design/Method: A case control study compared SCD patients with a discharge diagnosis of ACS who received ICS (cases) at admission to those who did not (controls). History of asthma and severity of ACS (unilateral infiltrate vs. bilateral) were collected. Outcome measures included transfusion and oxygen requirement, rate of PICU transfer, intubation, or BiPAP initiation, and need for readmission. Statistical analyses included Pearson chi-square and student t-tests. Results: One hundred twenty SCD patients (55 controls, 65 cases) were included in this study. A significantly higher proportion of controls had bilateral infiltrates (40% vs. 20% of cases, p=0.03), but fewer had asthma (12.7% vs. 52.3% of cases, p<0.001). Twelve cases (18.3%) and 9 controls (16.3%) were transferred to the PICU (p=0.80). While a lower proportion of cases required intubation (8.3% vs. 33.3% of controls, p=0.15), more cases required BiPAP (15.3% vs. 5.4%, p=0.02). Transfusion rates (p=0.10), oxygen requirement (p=0.09), and readmission rates (p=0.41) did not differ between groups. Conclusion: More controls had bilateral infiltrates in this study, suggesting more severe ACS, yet initiation of ICS in cases did not significantly decrease morbidity despite having less severe disease. More cases were diagnosed with asthma which may be why they were treated with ICS on admission despite less severe ACS. Overall, initiation of ICS did not decrease ACS morbidity as measured by the need for transfusion, oxygen supplementation, or PICU intervention. While systemic steroids increase the risk of readmission for VOC, cases were not more likely to be readmitted after the use of ICS. Despite a plausible pathogenic mechanism to reduce airway hyperreactivity, our study did not demonstrate a significant benefit of ICS in the treatment of ACS. Abstract source

Got Transition/Center for Health Care Transition Improvement is a cooperative agreement between the Maternal and Child Health Bureau and The National Alliance to Advance Adolescent Health. Our aim is to improve transition from pediatric to adult health care through the use of new and innovative strategies for health professionals and youth and families. With a broad range of partners, we are working to: - Expand the use of the Six Core Elements of Health Care Transition in pediatric, family medicine, and internal medicine practices; - Partner with health professional training programs to improve knowledge and competencies in providing effective health care transition supports to youth, young adults, and families; - Develop youth and parent leadership in advocating for needed transition supports and participating in transition quality improvement efforts; - Promote health system measurement, performance, and payment policies aligned with the Six Core Elements of Health Care Transition; and - Serve as a clearinghouse for current transition information, tools, and resources. Official website

Nadine Matthie is an assistant professor in Nell Hodgson Woodruff School of Nursing, Emory University. Dr. Matthie earned a PhD in Nursing Science from the University of South Florida in 2013, a MS in Nursing from the University of South Florida in 2008, and a BSN from the Florida State University in 2005. She completed a certificate in health management and leadership at the University of South Florida and is a board certified clinical nurse leader. Dr. Matthie is a postdoctoral fellow in the T32 program, Interventions for Preventing and Managing Chronic Illness, where she is developing a program of research in the areas of self-care and pain management. Specifically, her work is aimed at improving self-care behaviors and decreasing the frequency of pain crises and resulting hospitalizations in young adults with sickle cell disease. Additional interests include health promotion, health disparities, and health policy. Online profile

Dr. Cheedy Jaja is an associate professor with appointment in the College of Nursing at the University of South Carolina, Columbia. He has over ten years’ experience providing clinical care to patients with sickle cell disease (SCD), and over two decades of teaching experience in institutions of higher education in the USA and Sierra Leone. He is a board certified Psychiatric and Mental Health Advanced Registered Nurse Practitioner. His itinerant, albeit eclectic education, includes graduate degrees in psychiatric and mental health nursing, community health nursing, public administration and policy, public health genetics, philosophy, political science, and clinical and translational science. Dr. Jaja was the inaugural Pharmacogenetics, Ethics and Public Policy Fellow at Indiana University School of Medicine. His research interests are sickle cell disease analgesic pharmacogenetics. He currently holds leadership positions in the International Society of Nurses in Genetics, the International Association of Sickle Cell Nurses and Physician Assistants and is a member of the American Society of Hematology Sickle Cell Disease Coalition Group. Online profile

In addition to pain, sickle cell anaemia (HbSS) complications include neurocognitive difficulties in attention and processing speed associated with low daytime and night-time oxygen saturation. These effects can be compounded by obstructive sleep apnoea (OSA). Continuous Positive Airways Pressure (CPAP) is an accepted treatment for OSA in the general population. However, supplementary oxygen therapy in SCA may lead to bone marrow suppression. The aim of this single-blind, randomised, controlled phase II trial is to compare Auto-adjusting CPAP (APAP) with standard care to standard care alone in subjects with HbSS to determine whether the intervention is safe and improves attention and processing speed and brain structure. Methods: Eligibility criteria included ability to provide informed consent, age >8 and <16 years, diagnosis of HbSS and mean overnight saturation of <90% for <30% of the night. Key exclusion criteria were overnight respiratory support, respiratory or decompensated cardiac failure, chronic transfusion or contra-indications to APAP therapy or MRI. Minimisation factors were age group (8-11, 12-15), silent infarction on MRI, minimum overnight oxygen saturation > or < 90%, and hydroxyurea (HU) use. APAP adherence was defined as using APAP for an average of 4 hours a night for >50% of the time and was recorded using software documenting hours of use each night. Participant support in terms of appropriate facemask and facilitating adherence were provided by an unblinded sleep physiologist. Full blood counts were obtained at baseline, 2 weeks, 3 months and 6 months. Data were analysed by intention-to-treat. The primary outcome is change in the cancellation subtest from the Wechsler scales, and secondary outcomes include general cognitive functioning, assessed at baseline and after 6 months of treatment by assessors blind to treatment assignment. Analysis of Covariance (ANCOVA) models, adjusted for minimisation factors, were used to calculate least-square mean changes from baseline to 6 months. Results: 30 children with HbSS were randomised to standard care + APAP (n=15) or standard care alone (n=15) for 6 months. One child in the standard care arm withdrew after 6 weeks, and 8 children in the APAP arm were not adherent to treatment. There was no difference in hemoglobin change between the arms, but hydroxyurea use was associated with an increase in haemoglobin (p=0.01). Increase in cancellation score was numerically greater in the APAP arm (mean 1.46, SE 0.59 vs 1.01, SE 0.61) but this was not significant (mean difference 0.44, 95% CI: -1.42; 2.31; p=0.626). Increase in cancellation score was greater (mean 2.63; 95%CI: 0.95, 4.30) in those whose adherence was in the highest quartile (> 2.4 hours/night) compared with the other 3 quartiles (mean 0.71, 95%CI: -0.32, 1.75; mean difference 1.91, 95%CI: -0.06, 3.88; p=0.057). In subjects assigned to APAP, cancellation scores were significantly higher with hydroxyurea use (p=0.01). There were 7 subjects with serious adverse events in the placebo group, compared to 3 in the APAP group, all related to SCD pain. There was no evidence of decline in haemoglobin in either group. Abstract source

Complications of sickle cell disease (SCD) include pain episodes, chronic anemia, cardiopulmonary disease, and long-term end-organ damage. Hydroxyurea is efficacious in preventing these complications by increasing fetal hemoglobin levels (HbF); however, adherence is limited. Metformin, a Food and Drug Administration-approved drug used for type-2 diabetes mellitus (DM), was recently found to induce HbF through upregulation of FOXO3 and gamma-globin expression. However, the relation of metformin utilization to clinical outcomes and healthcare utilization in adults with SCD and DM has not been studied. Objectives: Our specific aims were to 1) estimate the prevalence of DM in a cohort of adult patients with SCD and 2) assess the association of metformin with clinical outcomes and healthcare utilization in this population. We hypothesized that adults with SCD and DM on metformin have less frequent SCD-related complication and lower utilization of hospital care for those complications. Methods: We analyzed subjects with SCD and DM >=18 years of age from the Truven Health MarketScan® Multi-State Medicaid Database from 2009-2015 with continuous enrollment of >=365 days. Subjects with SCD and DM were identified through the presence of >=1 inpatient or >=2 outpatient disease-specific billing codes or >=1 outpatient DM code and >=1 DM-related drug claim. Subjects with drug claims for insulin, hydroxyurea, or iron chelation were excluded. Metformin use was defined as having >=1 metformin-related drug claim in the study period. Outcomes included annual rates of all-cause inpatient hospitalizations, SCD-related hospitalizations, all-cause emergency department (ED) visits, SCD-related ED visits, vaso-occlusive episodes (VOE), strokes, acute chest syndrome (ACS) episodes, avascular necrosis (AVN) events, and gallstone events. Outcomes were identified based on claims records after the subject’s first DM diagnosis record in the dataset. Clinical events among metformin users and non-users were compared using chi square tests. Rate ratios (RR) comparing annual rates of clinical events among metformin users to non-users were estimated using negative binomial regression, controlling for age, sex, and claims-data-defined Charlson Comorbidity Index (CCI) score. Abstract source

Patients with sickle cell disease (SCD) who live at long distances from their specialty care center can have difficulty with clinic visit compliance. The Children’s Hospital of Pittsburgh (CHP) and the New York Mid-Atlantic Consortium for Genetics and Newborn Screening Services (NYMAC), a federally funded regional collaborative, recognized the need for families and providers to improve access to clinical services for these patients. Objectives: To continue to provide service utilizing telemedicine for pediatric patients with SCD. To host onsite telemedicine training to illustrate how Telehealth can improve accessibility to care, increase workforce capacity, and identify barriers to its implementation at CHP for pediatric hematologists in the NYMAC region (DC, DE, MD, NJ, NY, PA, VA, and WV). Design/Method: Telemedicine infrastructure was established in a satellite clinic at both Erie and Johnstown in Western Pennsylvania for monthly telemedicine sessions. CHP Staff and CHP SCD providers completed the telemedicine training. Patient satisfaction surveys were administered following the telemedicine visit. Pediatric hematologists in the NYMAC region were invited to participate in an onsite training program at CHP. Results: Nine pediatric patients were seen at the Erie clinic which yielded 73 completed telemedicine visits. Two pediatric patients were seen at the Johnstown clinic, yielding 7 completed telemedicine visits. Improvements with clinic access, hydroxyurea adherence, and patient satisfaction were noted as positive results of Telemedicine. The pilot at CHP in 2013 resulted in an increased proportion of patients receiving four annual clinic visits to 90%, as well as access to genetic counseling for SCD families. The proportion of patients receiving hydroxyurea increased to 50%. SCD telemedicine services saved patients/families crucial time and resources. Abstract source

Health literacy is defined as “the degree to which individuals have the capacity to obtain, process, and understand basic health information and services needed to make appropriate health decisions”. There is a paucity of literature regarding health literacy in adolescents and specifically no published data describing the state of health literacy in adolescents with sickle cell disease (SCD). Design/Method: This cross-sectional, descriptive correlational study included assessment of demographic measures and evaluation of data resulting from completion of the REALM-Teen and Newest Vital Sign (NVS) instruments by 75 Black, non-Hispanic adolescents with SCD followed at Children’s Medical Center (CMC) in Dallas, Texas. Convenience sampling was utilized. Inclusion criteria were a diagnosis of one of the four primary genotypes of SCD and age 10-19 years. UT Austin and UT Southwestern Institutional Review Boards approved the study. Results: Thirty-seven males and 38 females were recruited for the study. Their mean age was 14.7 years (SD=2.2; range 8.1). Their grade level ranged from 4 to 12 (mean 8.7; SD=2.2). The following SCD genotypes were represented: SS (81.3%); SC (14.7%); Sβ+ (2.7%) and Sβ0 (1.3%). Scores on the REALM-Teen ranged from 12 to 66 (mean 53.7; SD=12.8). Scores on the NVS ranged from 0 to 6 (mean 2.37; SD=1.33). These health literacy scores were lower using both the REALM-Teen and the NVS instruments when compared to scores in healthy adolescent and adult controls. However, the mean REALM-Teen score of this sample was slightly higher than Black controls from the validation study. Current grade level and health literacy scores showed a moderately high positive correlation (r =.52, p < .01). Health literacy scores were also significantly positively correlated with age (r =.49, p < .01) and income (r =.37, p < .01). Abstract source

Individuals living with sickle cell disease (SCD) are at risk for significant health complications including chronic pain, neuropsychological dysfunction, depression and poor mobility (Vichinsky et al, 2010; Zempsky et al, 2013; Jonassaint et al, 2016). Monitoring the day-to-day health of patients often requires burdensome daily symptoms diaries or expensive, time-consuming clinical tests. There are limited objective methods for capturing health outcomes of patients outside the clinic. Location data obtained by global positioning systems (GPSs) are increasingly used to assess physical mobility and activity spaces (Loveday et al, 2015). Activity space was found to increase with improvements in functioning post-surgery (Barzilay et al, 2011) and is smaller in older adults with depressive symptoms (Kerr et al, 2012) and cognitive deficits (Wettstein et al, 2015). However, no reported studies have assessed the utility of GPS activity space measures in younger populations with a chronic condition, such as SCD. GPS could provide an objective measure of health and wellbeing for SCD patients, as well as a method for early identification of patients at risk for poor outcomes. Abstract source Full text

Pain of sickle cell disease (SCD) has both physiological and psychological components. Current treatment of SCD pain focuses on alleviating pain using opioids, but guided imagery is rarely used. This 2-phase, attention control pre-post randomized clinical trial was conducted to test the feasibility of a protocol focused on guided imagery (GI) for stress and pain reduction intervention in adults with SCD. Patients (N=21, mean age 30±8 years [ranged from 21-47 years], 95% African-American, 67% female) completed stress and pain measures daily for 2 weeks via an Android tablet and the experimental group watched any of six video clips (2-min, 5-min, 8-min, 10-min, 15-min, and 20-min lengths) regardless of whether they are stressed or not to obtain a minimal intervention dose, at stress onset and as often as they desired via the tablet. We analyzed data using multi-level regression analysis. 100% of consented patients participated and 90% completed the study. Overall, patients used the tablet on 79% of study days; 100% reported that they liked the study; and 99% of questionnaire items were completed. At two weeks, there was a trend for effects of GI on stress and pain, wherein average stress intensity and composite pain index were lower for the GI group than the control group, but the difference was not statistically significant. The study protocol was feasible and we were able to determine the effect size for the GI intervention and calculate the sample size needed to conduct an efficacy trial of GI intervention using this protocol in adults with SCD. Patients kept the scheduled study appointments and completed a simple and cost-effective trial of GI intervention on the mobile tablet device; the GI intervention shows promise for reducing the impact of stress on SCD pain and warrants a larger randomized clinical trial to determine its efficacy. Abstract source