Adverse childhood experiences in children and adolescents with sickle cell disease: A retrospective cohort study.

BACKGROUND: Adverse childhood experiences (ACEs) are linked to poor health outcomes; however, the relationship between ACEs and health outcomes among children and adolescents with sickle cell disease (SCD) has limited documentation in the published literature. PROCEDURE: This retrospective cohort study involved 45 children and 30 adolescents. Participants were screened using the Center for Youth Wellness ACE Questionnaire. Parents completed the questionnaire for children. Adolescents provided self-report. ACEs were treated as continuous and categorical scales: 0-1 verus ≥2 original ACEs (individual and/or familial level); 0-1 versus ≥2 additional ACEs (community level); and 0-3 versus ≥4 expanded ACEs (original + additional). Pain and acute chest syndrome events were compared using Wilcoxon rank-sum tests, and correlated with cumulative ACE scores using Spearman's correlation. Multivariable models were fitted to examine the association between ACEs and pain/acute chest syndrome. RESULTS: The cumulative number of original ACEs positively correlated with acute chest syndrome events (rho = .53, p = .003) and pain (rho = .40, p = .028) among adolescents. Adolescents with ≥2 versus 0-1 original ACEs had a higher number of acute chest syndrome events (4.9 ± 2.6 vs. 1.6 ± 2.2, p = .002); however, this association was confounded by asthma. Acute chest syndrome events and hospitalizations for pain did not differ among child ACE groups. Emergency department (ED) pain visits were higher among children with ≥4 versus 0-3 expanded ACEs (1.6 ± 2.8 vs. 3.3 ± 3.2, p = .042), even after controlling for SCD genotype, asthma, disease-modifying treatment, and follow-up years (p = .027). CONCLUSION: ACEs are linked to increased morbidity among children and adolescents with SCD. Prospective studies are needed to further understand this relationship and test ACE-protective remedies.

Impact of telehealth visits on hydroxyurea response in sickle cell anemia.

BACKGROUND: It is important to ensure access to hydroxyurea (HU) for patients with sickle cell anemia (SCA) living in rural areas. The University of Alabama at Birmingham (UAB) Pediatric Sickle Cell program's satellite clinics reduce the barrier of transportation to the university-based clinic. However, as compared with the university clinic, these satellite clinics do not offer immediate access to HU dosing laboratory results and a nurse clinician calls families with HU dose adjustments after the clinic visit. This study evaluated the impact of telehealth dosing adjustments on HU laboratory and clinical response as compared with university-based patients. METHODS: A one-year retrospective chart review was performed to evaluate HU laboratory and clinical response based on clinic location and socioeconomic status for patients with SCA. We identified the number of clinic and acute care visits for one year and calculated the mean complete blood count and fetal hemoglobin (HbF) values for each patient. RESULTS: We identified 107 academic center participants with SCA-prescribed HU and 65 satellite clinic participants. The mean age of participants was 11 ± 5 years. We identified no difference in HbF (13.3 ± 0.7 vs 11.7 ± 0.8, P = 0.13), Hb (8.46 ± 1.1 vs 8.55 ± 1.1, P = 0.59), mean corpuscular volume (91.0 ± 10.6 vs 91.7 ± 9.5, P = 0.67), or absolute neutrophil count (4.85 ± 2.3 vs 4.87 ± 2.3, P = 0.95) when comparing Birmingham versus satellite clinics. We also identified no difference in hospital admissions (0.99 ± 0.1 versus 0.85 ± 0.2, P = 0.49), based on clinic location. CONCLUSIONS: The use of telehealth did not negatively impact laboratory response to HU. Future studies should identify novel approaches to improve access to HU among patients with SCA living in rural areas.

Nocturnal hypoxemia measured by polysomnogram is associated with acute chest syndrome in pediatric sickle cell disease.

STUDY OBJECTIVES: Nocturnal hypoxemia is associated with increased risk of sickle cell disease (SCD) complications. The association of nighttime hypoxemia and acute chest syndrome (ACS) in children with SCD has yet to be determined. METHODS: This is a retrospective study of children with SCD who underwent polysomnography at a SCD center. Univariate logistic regression was used to assess the association between nocturnal hypoxemia and ACS admissions. Multivariate logistic regression was performed to verify the effects of different clinical covariates on ACS. Secondary analysis comparing patients with one vs multiple ACS admissions was performed. RESULTS: One hundred ten individuals with SCD who completed their polysomnogram (mean age of 9.4 years) were identified. Fifty-nine (54%) had a history of at least one episode of ACS admission (mean age of 4.1 years), including 40 with multiple episodes. The percentage of total sleep time with O2 saturation < 90% was greater in the ACS group (P < .05). Similarly, mean nocturnal O2 saturation was lower in the ACS group (P < .0005). Mean nocturnal O2 saturation of < 97.3% and the percentage of total sleep time with O2 saturation < 90% higher than 2.7% were associated with ACS. There was no difference in nocturnal hypoxemia between patients with single and multiple ACS admissions. CONCLUSIONS: Nocturnal hypoxemia later in life is associated with previous ACS admissions in children with SCD. This can increase the yield of interpreting polysomnograms in this vulnerable population. Prospective studies are needed to determine the temporal relations of nocturnal hypoxemia and ACS, which may identify a modifiable risk for ACS.

Outpatient pain clinic and intranasal fentanyl to improve sickle cell disease outcomes.

BACKGROUND: Acute pain events are a leading complication for sickle cell patients. In an attempt to improve pain outcomes, we developed an outpatient pain clinic, and included intranasal fentanyl in the opioid emergency department (ED) pain order set. We evaluated admission rates and opioid administration for patients that attended both the outpatient pain clinic and ED within a 3-month period. METHODS: We recorded the admission rate, IV morphine equivalents, and time from triage for each opioid order and administration from both an outpatient pain clinic and ED visit within a 3-month period for an individual pediatric patient with sickle cell disease. RESULTS: Thirty patients received acute pain management in both settings. We identified a significant reduction in hospital admission when patients received care in the pain clinic as compared to the ED (17% vs 43%, P = .02). Additionally, outpatient pain clinic patients received significantly less IV morphine equivalents than patients received in the ED (5.6 vs 10.6 IV morphine equivalents, P < .0001). In the ED, intranasal fentanyl was administered in a significantly shorter time than patients ordered intravenous opioid (43 vs 75 min, P = .02). The mean time to receiving an opioid in the outpatient pain clinic was 57 min. CONCLUSION: The use of an outpatient pain clinic can reduce admission rates as compared to the ED. The use of intranasal fentanyl reduced the time to first opioid administration in the ED. Patient-centered research or quality improvement projects should continue to focus on novel approaches to acute pain event management.

Association between hospital admissions and healthcare provider communication for individuals with sickle cell disease.

Objective: To test the hypothesis that caregivers' or adult participants' low ratings of provider communication are associated with more hospital admissions among adults and children with sickle cell disease (SCD), respectively. Secondarily, we determined whether there was an association between the caregivers' or participants' health literacy and rating of providers' communication. Methods: Primary data were collected from participants through surveys between 2014 and 2016, across six sickle cell centers throughout the U.S. In this cross-sectional cohort study, 211 adults with SCD and 331 caregivers of children with SCD completed surveys evaluating provider communication using the Consumer Assessment of Healthcare Providers and Systems (CAHPS), healthcare utilization, health literacy, and other sociodemographic and behavioral variables. Analyses included descriptive statistics, bivariate analyses, and logistic regression. Results: Participants with better ratings of provider communication were less likely to be hospitalized (odds ratio (OR) = 0.54, 95% confidence interval (CI) = [0.35, 0.83]). Positive ratings of provider communication were associated with fewer readmissions for children (OR = 0.23, 95% CI = [0.09, 0.57]). Participants with better ratings of provider communication were less likely to rate their health literacy as lower (regression coefficient (B) = -0.28, 95% CI = [-0.46, -0.10]). Conclusions: Low ratings of provider communication were associated with more hospitalizations and readmissions in SCD, suggesting the need for interventions targeted at improving patient-provider communication which could decrease hospitalizations for this population.

Risk factors for hospitalizations and readmissions among individuals with sickle cell disease: results of a U.S. survey study.

OBJECTIVE: Hospital admissions are significant events in the care of individuals with sickle cell disease (SCD) due to associated costs and potential for quality of life compromise. METHODS: This cross-sectional cohort study evaluated risk factors for admissions and readmissions between October 2014 and March 2016 in adults with SCD (n = 201) and caregivers of children with SCD (n = 330) at six centres across the U.S. Survey items assessed social determinants of health (e.g. educational attainment, difficulty paying bills), depressive symptoms, social support, health literacy, spirituality, missed clinic appointments, and outcomes hospital admissions and 30-day readmissions in the previous year. RESULTS: A majority of adults (64%) and almost half of children (reported by caregivers: 43%) were admitted, and fewer readmitted (adults: 28%; children: 9%). The most common reason for hospitalization was uncontrolled pain (admission: adults: 84%, children: 69%; readmissions: adults: 83%, children: 69%). Children were less likely to have admissions/readmissions than adults (Admissions: OR: 0.35, 95% CI: [0.23,0.52]); Readmissions: 0.23 [0.13,0.41]). For all participants, missing appointments were associated with admissions (1.66 [1.07, 2.58]) and readmissions (2.68 [1.28, 6.29]), as were depressive symptoms (admissions: 1.36 [1.16,1.59]; readmissions: 1.24 [1.04, 1.49]). In adults, difficulty paying bills was associated with more admissions, (3.11 [1.47,6.62]) readmissions (3.7 [1.76,7.79]), and higher spirituality was associated with fewer readmissions (0.39 [0.18,0.81]). DISCUSSION: Missing appointments was significantly associated with admissions and readmissions. Findings confirm that age, mental health, financial insecurity, spirituality, and clinic attendance are all modifiable factors that are associated with admissions and readmissions; addressing them could reduce hospitalizations.

Hyperfiltration during early childhood precedes albuminuria in pediatric sickle cell nephropathy.

BACKGROUND: In patients with diabetes mellitus, hyperfiltration precedes the development of albuminuria. Pediatric sickle cell anemia (SCA) patients have a high prevalence of hyperfiltration and albuminuria during early childhood and adolescence. We tested the hypothesis that hyperfiltration precedes the development of albuminuria in a longitudinal pediatric SCA cohort. METHODS: We identified 91 participants with HbSS or SB0 thalassemia 5-21 years of age enrolled in a longitudinal sickle cell nephropathy cohort study who had a cystatin C measured during early childhood (4-10 years of age). Early hyperfiltration was defined as a mean eGFR >180 mL/min/1.73m2 using cystatin C obtained from 4 to 10 years of age. Persistent albuminuria was defined as an albumin to creatinine ratio > 30 mg/g on two of three untimed urine specimens. Time to event analysis estimated survival curves for participants with and without hyperfiltration using Kaplan-Meier curves and used logrank test for categorical variables to assess the association with time to development of the first episode persistent albuminuria. RESULTS: Persistent albuminuria occurred more often and at an earlier age in participants with early hyperfiltration compared to those without early hyperfiltration (log-rank, P = .004). Participants who developed albuminuria have a significant increase in their eGFR during childhood (P = .003) as compared to participants who have not yet progressed to albuminuria (P = .26). For every 1 g/dL increase in hemoglobin, the hazard ratio for developing persistent proteinuria decreased by 0.56 (95% CI: 0.3, 1.06, P = .07). CONCLUSION: Hyperfiltration precedes the development of persistent proteinuria in pediatric SCA patients. Intervention strategies should target lowering eGFR during early childhood.

Outcomes of febrile events in pediatric patients with sickle cell anemia.

BACKGROUND: Limited evidence exists to create institutional admission criteria guidelines for febrile sickle cell patients. In addition, evidence is lacking to understand readmission rates for febrile sickle cell patients discharged from the emergency department (ED) or hospital. PROCEDURES: We conducted a 16-year retrospective study of bacteremia outcomes for febrile sickle cell patients. Risk variables analyzed included fever (either ≥ 39.5°C or ≥40°C), abnormal white blood cell (WBC) (>30,000 or <5,000/mcL), tachycardia and hypotension, or "ill appearing." Fourteen-day readmission rates were analyzed to determine outcomes for febrile sickle cell patients discharged from the ED or discharged within 72 h. RESULTS: Bacteremia was identified in 17 (2.6%) of 653 febrile events that are presented to the ED. "Ill-appearing" patients had an 8.5-fold increased odds of being diagnosed with bacteremia. Models using WBC count, "ill appearing," and hypotension have the highest sensitivity and specificity (AUC > 0.75). Among 427 patients discharged from the ED or within 72 h of hospitalization, only 10 (2.3%) were readmitted for a new sickle cell complication. CONCLUSIONS: Institutions can develop admission criteria based on WBC count, hypotension, and "ill appearance." Persistently febrile, well-appearing patient can be discharged at 48 h with minimal risk for new complications.

Modifying factors of the health belief model associated with missed clinic appointments among individuals with sickle cell disease.

OBJECTIVES: Outpatient care is critical in the management of chronic diseases, including sickle cell disease (SCD). Risk factors for poor adherence with clinic appointments in SCD are poorly defined. This exploratory study evaluated associations between modifying variables from the Health Belief Model and missed appointments. METHODS: We surveyed adults with SCD (n = 211) and caregivers of children with SCD (n = 331) between October 2014 and March 2016 in six centres across the U.S. The survey tool utilized the framework of the Health Belief Model, and included: social determinants, psychosocial variables, social support, health literacy and spirituality. RESULTS: A majority of adults (87%) and caregivers of children (65%) reported they missed a clinic appointment. Children (as reported by caregivers) were less likely to miss appointments than adults (OR:0.22; 95% CI:(0.13,0.39)). In adults, financial insecurity (OR:4.49; 95% CI:(1.20, 20.7)), health literacy (OR:4.64; 95% CI:(1.33, 16.15)), and age (OR:0.95; 95% CI:(0.91,0.99)) were significantly associated with missed appointments. In all participants, lower spirituality was associated with missed appointments (OR:1.83; 95%CI:(1.13, 2.94)). The most common reason for missing an appointment was forgetfulness (adults: 31%, children: 26%). A majority thought reminders would help (adults: 83%, children: 71%) using phone calls (adults: 62%, children: 61%) or text messages (adults: 56%, children: 51%). CONCLUSIONS: Our findings demonstrate that modifying components of the Health Belief Model, including age, financial security, health literacy, spirituality, and lacking cues to action like reminders, are important in missed appointments and addressing these factors could improve appointment-keeping for adults and children with SCD.