Population-Based Analysis Of High Hospital Utilization (HHU) Among Children and Adolescents With Sickle Cell Disease (SCD)

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 1740-1740
Author(s):  
Rodney S Theodore ◽  
Maa-Ohui Quarmyne ◽  
James Bost ◽  
Carlton Dampier ◽  
Peter A. Lane
Keyword(s):  
Health Care ◽  
Sickle Cell Disease ◽  
Children And Adolescents ◽  
Sickle Cell ◽  
Insurance Coverage ◽  
Population Based ◽  
Hospital Utilization ◽  
Cell Disease ◽  
Relationship Of ◽  
The Relationship

Abstract Background Sickle cell disease (SCD) is characterized by marked heterogeneity in clinical manifestations, severity and utilization of health care services. This heterogeneity is particularly evident with regards to utilization of inpatient services, which account for a large percentage of total health care costs and significant absence from school and work. Previous studies of utilization, based on analysis of administrative datasets, have been limited by inaccuracies in ICD-coding, inability to differentiate among SCD genotypes, and/or inability to capture individuals never hospitalized. Single center studies have been limited by potential disease severity referral bias, inability to capture service utilization at outside facilities, and relatively small numbers. Objectives We sought to identify children and adolescents with SCD with high hospital utilization (HHU) and to determine the relationship of HHU with age, SCD genotype, and insurance coverage. We also sought to examine trends over a three year period (2010-2012) and explore the relationship of any trends in hospital utilization over time. Methods The clinical database of the SCD Program at Children's Healthcare of Atlanta (CHOA) was used to identify all patients with SCD, ages ≥ 5 years, who received comprehensive outpatient, acute care, and inpatient services at three CHOA facilities in metro Atlanta during 2010-2012. SCD genotype was confirmed for each patient by review of hematologic and clinical data, including results of diagnostic hemoglobin electrophoresis. All outpatient clinic, emergency department, and inpatient utilization was captured as well as insurance (payer) status at each encounter. HHU was defined as ≥3 admissions during a calendar year. Data were analyzed for significance by Chi-square. The extent to which the data were population-based was determined from the Georgia Hospital Association database with includes DRG-level data for all inpatient hospitalizations in Georgia. Results Hospitalizations at CHOA accounted for 96.1% of all SCD admissions for children within the 28-county greater metro Atlanta area. During 2010-2012, 1348 individual children and adolescents ≥ 5yr of age with SCD received comprehensive care at CHOA (1076 in 2010, 1112 in 2011, 1159 in 2012): 928 (68.8%) had SS/S β°thal, 334 (24.8%) SC, 86 (6.4%) S β+thal. Of 1159 active patients in 2012, 441 (38.1%) were 5-9 yr of age, 419 (36.2%) 10-14 yr, 299 (25.8%) ≥ 15 yr; 474 (40.9%) had traditional medicaid (MC), 205 (17.7%) managed-care medicaid (MMC), 374 (32.3%) private payer (PP), and 105 (9.1%) multiple payers (MP). HHU (≥ 3 admissions/yr) decreased from 119 of 1076 (11.1%) in 2010 to 96 of 1112 (8.6%) in 2011 and 95 of 1159 (8.2%) in 2012 (p <.01). The Table shows percentage HHU for each year by age, genotype, and payer. Conclusions For children and adolescents with SCD, HHU decreased during the three year period. HHU was significantly associated with older age and with MC and MP insurance coverage during all three years. HHU was significantly associated with the SS/S β°thal genotype in 2010, but not in 2011-12. The extent to which the decline of HHU among patients with SS/S β°thal genotype was related to increasing use of hydroxyurea is being investigated. Disclosures: Off Label Use: Hydroxyurea is not FDA approved for use in children.

scholarly journals Relationship of Hydroxyurea Adherence to Depression and Fatigue Among Children and Adolescents with Sickle Cell Disease: A Longitudinal Cohort Study

Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 3104-3104
Author(s):  
Sherif M. Badawy ◽  
Kathryn King ◽  
Olivia E. Atherton ◽  
Daniel Mroczek ◽  
Alexis A. Thompson ◽  
...  
Keyword(s):  
Cohort Study ◽  
Sickle Cell Disease ◽  
Children And Adolescents ◽  
Sickle Cell ◽  
Research Funding ◽  
Self Report ◽  
Cell Disease ◽  
Laboratory Markers ◽  
Relationship Of ◽  
Over Time

Abstract Background: Sickle cell disease (SCD) is the most common genetic disorder in the United States, seen in 100,000 Americans. SCD complications include pain episodes, chronic anemia and long-term end organ damage, leading to significant impairment in health-related quality of life (HRQOL) across the lifespan. Hydroxyurea (HU) reduces morbidity and mortality, improves HRQOL and lowers healthcare utilization, yet adherence remains suboptimal. Limited evidence from cross-sectional studies demonstrates an association between lower HU adherence and worse HRQOL scores. Objective: To assess the longitudinal relationship of HU adherence to HRQOL domains, including fatigue and depression. We hypothesized that higher HU adherence over time would be associated with improvement in HRQOL domain scores, especially depression and fatigue. Methods: In this longitudinal cohort study (NCT04675645), patients were enrolled from the comprehensive sickle cell clinic at Lurie Children's Hospital of Chicago. Patients were eligible if they were ³8 years old, had SCD (any genotype), and on HU with a stable dose for ³2 months. Study assessments included PROMIS ® measures for depression and fatigue, self-report of adherence using visual analogue scale (VAS), and patient demographics. Assessments were completed at baseline and every 3 months with a total of 5 visits (0, 3, 6, 9 and 12 months). Laboratory markers of adherence collected from chart review, including fetal hemoglobin (HbF%) and mean corpuscular volume (MCV). We conducted bivariate correlations among demographic variables, adherence markers and HRQOL scores as well as among adherence variables (VAS, HbF, MCV) at each visit. We conducted different multilevel models (MLMs), fixed and random effects, to understand the extent to which between- and within-person variation in adherence was associated with HRQOL scores over the 12-month period. We report unstandardized betas (B) and 95% Confidence Intervals (CI) from the MLMs. Results: Twenty-three patients have been enrolled (96% HbSS, 65% females, 100% Black, median age 15 [range 9-22] years old). At baseline, participants had a median Hb level of 9.5 (IQR 8.3-10.3 g/dl) with a HbF of 16.4% (IQR 13.1-28.7%) and MCV of 106.5 fl (IQR 91.6-113.9 fl). Participants' MCV levels significantly correlated with HbF% and VAS at visit 1 (r=0.58, P &lt;0.01; r=0.6, P &lt;0.01), visit 2 (r=0.66, P &lt;0.01; r=0.63, P &lt;0.01), visit 4 (r=0.76, P &lt;0.01; r=0.72, P &lt;0.01) and visit 5 (r=0.71, P &lt;0.01; r=0.59, P &lt;0.05), respectively. Participants' VAS adherence levels significantly increased from visit 1 to visit 5 (median 72 [IQR 60-92] vs. 88 [IQR 75-95], P=0.04, respectively) along with significant improvement in their fatigue scores (median 52.8 [IQR 35.1-70.5] vs. 30.8 [IQR 13.2-48.4], P=0.001, respectively). Variation in fatigue and depression scores across the study period was due to between-person differences (38% and 71%, respectively) or within-person fluctuations (62% and 29%, respectively). Using fixed and random effect MLMs, between-person differences in HU adherence over 12 months using VAS and HbF% were significantly related to participants' reported depression (B -0.43, 95% CI -0.69 to -0.17, P &lt;0.01; B -0.58, 95% CI -1 to -0.15, P &lt;0.05, respectively) (Figure 1) and fatigue scores (B -0.42, 95% CI -0.68 to -0.16, P &lt;0.01; B -0.43, 95% CI -0.78. to -0.06, P &lt;0.05, respectively) (Figure 2). In contrast, we found no statistically significant effects of within-person variation in adherence, using VAS and HbF, on participants' reported fatigue and depression scores over 12 months, which could be due our small sample size. Conclusions: Children and adolescents who were more adherent to HU across the entire study period were less likely to experience fatigue and depression, compared to those who were less adherent. Participants' self-report and laboratory markers of adherence were significantly correlated across study visits. Within-person fluctuations in adherence were not associated with changes in fatigue and depression scores across the study period. Future multi-institutional studies with a larger sample size are needed to better understand the within-person effects of variation in HU adherence on HRQOL scores over time. Behavioral interventions, such as mHealth apps, that are focused on improving HU adherence among children and adolescents with SCD has the potential to improve HRQOL and other important health outcomes. Figure 1 Figure 1. Disclosures Badawy: Bluebird Bio Inc: Consultancy; Vertex Pharmaceuticals Inc: Consultancy; Sanofi Genzyme: Consultancy. Thompson: Biomarin: Research Funding; Baxalta: Research Funding; bluebird bio, Inc.: Consultancy, Research Funding; Celgene/BMS: Consultancy, Research Funding; CRISPR Therapeutics: Research Funding; Vertex: Research Funding; Editas: Research Funding; Graphite Bio: Research Funding; Novartis: Research Funding; Agios: Consultancy; Beam: Consultancy; Global Blood Therapeutics: Current equity holder in publicly-traded company. Cella: FACIT: Membership on an entity's Board of Directors or advisory committees.

scholarly journals Inpatient Utilization Among Children and Adolescents with Sickle Cell Disease: Analysis of a Large Populated-Based Retrospective Cohort

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 3262-3262 ◽  
Author(s):  
Rodney Theodore ◽  
Vaughn Barry ◽  
Maa-Ohui Quarmyne ◽  
Carlton Dampier ◽  
Peter A. Lane
Keyword(s):  
Sickle Cell Disease ◽  
Children And Adolescents ◽  
Hospital Discharge ◽  
Sickle Cell ◽  
Hospitalization Rate ◽  
Hospital Utilization ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Readmission Rates ◽  
Hospitalization Rates

Abstract Background: Sickle cell disease (SCD) is characterized by marked heterogeneity in clinical outcomes, severity and utilization of health care services. This heterogeneity is particularly evident with regards to utilization of inpatient hospital services. Some children with SCD are frequently admitted to the hospital while others are rarely or never admitted. In addition, rates of readmission within 30 days of hospital discharge are high in SCD. The causes of this variable utilization and high rates of readmission are not well understood. Objectives: We sought to determine rates and primary causes of SCD-related hospital utilization among children and adolescents with SCD and to determine whether rates of hospitalization and of 7-, 14- and 30-day readmissions varied by age, SCD genotype, gender and cause of hospitalization. Methods: Children who lived in the 28-county greater metro Atlanta area with a confirmed SCD diagnosis and who received care at the ChildrenÕs Healthcare of Atlanta (CHOA) SCD program between January 1, 2010 through December 31, 2014 were included. The earliest and latest encounter during the five-year period was used to determine total length of observation for each patient. To ensure a substantial period of observation, individuals with two consecutive encounters >18 months apart or with < 2 yr of observation were excluded. SCD genotype (SS, SC, and Sβ0 and Sβ+ thalassemia) was confirmed for each patient by review of hematologic and clinical data, including results of diagnostic hemoglobin electrophoresis; those with rare SCD genotypes (e.g. SD, SE) were excluded. The primary cause for each admission was determined through medical chart review and classified into 4 mutually exclusive hierarchical categories in the following descending order: acute chest syndrome (ACS), pain crisis, fever/infection, and other complications of SCD (i.e. an admission for both pain and ACS was categorized as ACS). Scheduled hospitalizations for elective procedures (e.g. splenectomy, cholecystectomy, venous catheter placement) were excluded. A readmission was defined as a hospitalization occurring within 7, 14, or 30 days of a previous hospital discharge. Results: The analysis included 1,331 individuals with 5,362 person-years of observation; 68% had SS/Sβ0 thal genotypes, 49% were male. Age at the time of the earliest encounter ranged from 2 months to 19 years; average length of observation was 4.02 years (min, max= 2, 5). Total n of hospital admissions was 5,317; 19.4% were never hospitalized, and 44.8% were hospitalized less than once per year. The most common primary cause for hospital admission was pain (53.1% of admissions). Overall and cause-specific hospitalization rates varied by age and genotype (Figure) with overall hospitalization rates lowest among children 4-9 years old compared to other ages (0.68 vs. 1.00 admissions per person-year) and highest among those with SS or Sβ0 thal compared to those with SC or Sβ+ thal (0.97 vs. 0.68 admissions per person-year). Hospitalization rates associated with pain increased with age while rates for fever/infection decreased with age. Of the 1,073 patients who were admitted, 356 were readmitted within 30 days of a previous admission at least once. All-cause 7-, 14- and 30-day readmission rates were 5.6%, 10.0% and 18.2%, respectively. 30-day readmission rates were lower among younger age groups (15.7% for age 1-3 yr, 15.3% for 4-6 yr, 15.8% for 7-9 yr) but higher in older patients (18.3% for 10-12yr, 19.9% for 13-15yr, and 23.3% for 16-18yr). Readmission rates were highest following index admissions for pain (20.4%) and lowest for ACS (11.3%). Gender was not associated with readmission or cause of readmission. Conclusion: SCD-related hospitalization rates were highest in early life and in later adolescence with admissions for fever/infection most common in younger children and admissions for pain crises in older children. Rates of readmission strongly correlated with age and were highest following admissions for pain. These data highlight to need to develop and implement improved strategies prevention and treatment of pain in children and adolescents with SCD. Further studies should also explore individual and/or family factors that may contribute to the high rates of hospital utilization and 30-day readmissions that occur among a relatively small percentage of patients. Figure 1. Hospitalization rate per person-year by type of admission and age Figure 1. Hospitalization rate per person-year by type of admission and age Disclosures No relevant conflicts of interest to declare.

scholarly journals Hydroxyurea Effectiveness in Children and Adolescents with Sickle Cell Anemia: A Large Retrospective, Population-Based Cohort Study

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 2179-2179 ◽  
Author(s):  
Maa-Ohui Quarmyne ◽  
Wei Dong ◽  
Vaughn Barry ◽  
Rodney Theodore ◽  
Olufolake A. Adisa ◽  
...  
Keyword(s):  
Clinical Trials ◽  
Sickle Cell Disease ◽  
Children And Adolescents ◽  
Selection Bias ◽  
Sickle Cell ◽  
Poisson Regression ◽  
Clinical Effectiveness ◽  
Population Based ◽  
Acute Chest Syndrome ◽  
Cell Disease

Abstract Background: The clinical efficacy of hydroxyurea (HU) in patients with sickle cell anemia (SCA) is well established. In clinical trials, HU has been demonstrated to alter the clinical course of SCA; HU decreases rates of pain, dactylitis, acute chest syndrome (ACS), transfusions, hospitalizations and improves hemoglobin levels. Other studies have shown that HU improves quality of life and decreases mortality. While evidence of efficacy has been well demonstrated, published data about clinical effectiveness is limited. Additionally, attempts to compare outcomes of patients taking HU to those not taking HU have been limited by the inherent selection bias for greater disease severity in patients on HU. We sought to examine the clinical effectiveness of HU in the large population of patients with sickle cell disease (SCD) at Children's Healthcare of Atlanta (CHOA). The program provides comprehensive care to >1,700 active patients; because they include about 95% of all children and adolescents with SCD in the greater Atlanta Metropolitan Area, the program provides a population-based sample. Currently 57% of the 922 children ≥ 1 yr of age with SCA (SS and Sβ0 thalassemia) who are not on chronic transfusions are receiving HU. Methods: Using a retrospective cohort, pre-post treatment study design to control for disease severity selection bias we evaluated the clinical effectiveness of HU in patients with SCA who received care at CHOA and who first initiated HU in 2009-2011. Children on chronic transfusions, or children with inadequate follow up data and/or who ever took HU in the 3 years prior were excluded. Clinical guidelines for dosing HU have been standardized locally and recommend initial dosage of 20mg/kg/day, followed by dose escalation every 2 months to maximum tolerated dose. For each patient, healthcare utilization, laboratory values and clinical outcomes for the 2-year period prior to HU initiation were compared to those for two years after initiation. Medians were compared using the Wilcoxon Signed Rank test and means using T test. Rate ratios were computed using unadjusted Poisson regression. Interactions testing whether the effect of HU varied by age, sex, or insurance status, were assessed using multivariable Poisson regression. Results: Of 211 children with SCA who initiated HU in 2009-2011, 134 met eligibility criteria. After initiation of HU, the rate of hospitalization was 0.53 the rate before HU, a 47% reduction (Table). The number of inpatient days, emergency room (ER) visits, pain encounters, episodes of acute chest syndrome (ACS), and transfusions were also significantly reduced (Table). The hemoglobin level (mean +/- SD) pre- and post-HU initiation was 7.3+/-2.7 g /dl and 8.1+/- 2.9 respectively; an increase of 0.8+/-1.1, p<0.0001. Similarly, pre-HU mean corpuscular volume increased from 82.9+/-7.5 fl pre-HU to 95.7+/-10.7 post-HU; an increase of 12.8+/-7.2, p <0.0001. There was a statistically significant interaction between the effect of HU and age of initiation of the drug on hospitalization rates; patients < 7 years of age had a greater reduction in hospitalizations than older children (58% vs 33%, p=0.03). The effect of HU on hospitalizations did not vary by sex or insurance type. Table 1. Effect of HU initiation on clinical outcomes using Poisson Regression Rate Ratio (Confidence Limits) P Value Hospitalizations 0.53 (0.43 - 0.66) <0.0001 Inpatient Days 0.50 (0.40 - 0.63) <0.0001 ER visits 0.57 (0.49 - 0.67) <0.0001 Pain Encounters 0.64 (0.51 - 0.81) 0.0001 ACS 0.57 (0.39 - 0.83) 0.0036 Blood Exposure 0.43 (0.29 - 0.64) <0.0001 Conclusions: HU is clinically effective in children and adolescents with SCA. HU decreased hospitalizations, ER visits, pain encounters, ACS, use of transfusions and improved hemoglobin levels. HU effectiveness was similar across gender, insurance types and age, although there was a slightly greater reduction in hospitalizations in younger patients. These results are important as they parallel results obtained from HU efficacy studies, thus demonstrating that in 'real life' settings, even without the additional monitoring and adherence incentives of clinical trials, HU improves short-term outcomes in pediatric SCA. Disclosures Off Label Use: Hydroxyurea use as disease modifying therapy in pediatric patients with sickle cell disease.

scholarly journals The Relationship of Transcranial Doppler Ultrasonography with Attention, Motor, and Social-Emotional Functioning in Pediatric Sickle Cell Disease

2019 ◽  
Vol 34 (6) ◽  
pp. 842-842
Author(s):  
M Balderrama ◽  
C Merrill ◽  
A Whitaker ◽  
K Kayser
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Transcranial Doppler ◽  
Emotional Functioning ◽  
Neuropsychological Functioning ◽  
Cell Disease ◽  
Social Emotional ◽  
Social Emotional Functioning ◽  
Relationship Of ◽  
The Relationship

Abstract Objective Children with sickle cell disease (SCD) are at greater risk for certain neuropsychological deficits due to their medical condition and associated complications. Previous studies have explored the relationship between Transcranial Doppler (TCD) ultrasonography and various domains of neuropsychological functioning in pediatric SCD populations; however, these studies are dated, present variable and inconsistent findings, and are somewhat limited in scope. The goal of this study was to update and expand upon previous research by examining the predictive relationship of TCD results with measures of attention, motor, and social-emotional functioning. Methods Thirty-six patients ages 3-19 with SCD with no known history of stroke, with TCD completed within the past 12 months, underwent a brief neuropsychological exam. Attention, motor, and social-emotional functioning were assessed as appropriate for age using the Conners Continuous Performance Test (K-CPT2/CPT3), Purdue Pegboard, and PROMIS, respectively. TCD values were gathered via medical record review, using the highest value of most recent TCD. Results TCD significantly predicted certain aspects of attention and motor ability, but not social-emotional functioning. Specifically, TCD significantly predicted Detectability (p = .005), Omissions (p = .001), Commissions (p = .012), Perseverations (p = .035), and HRT SD (p = .046) on K-CPT2/CPT-3 and the non-dominant (p = .009) and bilateral (p = .024) trial scores on the Purdue Pegboard. Conclusion This study provides new evidence that TCD may be predictive of motor functioning in pediatric SCD. Results confirm that TCD is predictive of attentional function, though subdomains impacted varied from previous research. While no significant relationship between psychosocial symptoms and TCD were identified, further studies utilizing more comprehensive measurement within this domain is warranted.

scholarly journals Sleep Moderating the Relationship Between Pain and Health Care Use in Youth With Sickle Cell Disease

2020 ◽  
Vol 36 (2) ◽  
pp. 117-123
Author(s):  
Cecelia R. Valrie ◽  
Kristen Alston ◽  
Beng Fuh ◽  
Rupa Redding-Lallinger ◽  
India Sisler
Keyword(s):  
Health Care ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Health Care Use ◽  
Cell Disease ◽  
The Relationship

scholarly journals Patient Perception on Health Care Delivery for Sickle Cell Disease and Opportunities for Quality Improvement

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 5862-5862
Author(s):  
Ofelia A. Alvarez ◽  
Sandra Echenique ◽  
Hector Rodriguez-Cortes ◽  
Thomas J. Harrington ◽  
E. Leila Jerome Clay ◽  
...  
Keyword(s):  
Health Care ◽  
Quality Improvement ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Health Care Delivery ◽  
Research Funding ◽  
Care Delivery ◽  
Insurance Coverage ◽  
Cell Disease ◽  
Pain Medications

The Health Resources and Services (HRSA)-sponsored Sickle Cell Disease (SCD)Treatment Demonstration Regional Collaborative Program, EMBRACE SCD Florida has three aims: 1. To educate providers about SCD, 2. To assess patients' perception on health care and barriers encountered, and 3. To increase hydroxyurea (HU) treatment as disease-modifying therapy. We report the patients' perception on health care delivery as assessed by surveys of 65 patients at two SCD centers in South Florida. All participants signed the IRB-approved informed consent/assent and completed a 46-question survey. Surveys addressed patient demographics, HU use, pain experienced, facility utilization and confidence in SCD doctors and primary care providers (PCPs). Parents represented 62 % of the sample respondents. There were 37 female and 28 male patients, with a mean age of 14.8 years, median 12 years (range 1-45). Patients were self-identified as SS (N=42, 65%), SC (N=8), sickle-β+ thalassemia (N=5), sickle-β0 thalassemia (N=2), other (N=1), and 7 (11%) did not know their genotype. Most (55%) were African-Americans; 26% Haitians, 12% Black non-African-American or other, and 6% were White. Nine percent were Hispanic. Insurance coverage was identified as Medicaid (75%), Managed Care Organization (7%), Medicare (13%), and private insurance (5%). Of the 44 patients with SS and Sβ0, 84% (N=37) received medical advice to take HU from SCD provider. In addition, 12 patients (4 unknown type, 5 Sβ+ and 3 SC) were advised to take HU for a total of 49 or 75% of all patients surveyed. Seventy-eight percent (N=38) of those agreed to be treated, and 22% (N=11) declined HU. Eighty-four percent of those who agreed to be treated (N=32) reported taking HU as prescribed. Those patients who have received HU identified many barriers: lack of money to pay (N=5), nausea (N=5), hair loss (N=1), do not like the taste (N=8), do not like to take medications so they either do not take it frequently (N=7) or force themselves to take it (N=12), lack of effectiveness for their pain (N=10), and forgetting to take HU (N=18). Eighteen of 49 (37%) said they had no problems taking HU. We asked all patients about their pain experience. Of the 63 who responded, 30 (47.6%) said pain was managed well all the time, whereas the rest perceived difficulties in pain management: 20 (31.7%), 12 (19%), and one (1.6%) perceived their pain was managed well 75% of time, 50% of the time, and 25% or less of the time, respectively. The frequency of taking pain medications was variable among patients. The most common frequency of taking pain medications was several times a month (24 of 63 or 38%), but ranged from taking pain medication daily (N=9, 14%) to never or almost never (N=10, 16%). The emergency room (ER) was the first place they would go to when they get sick. One patient reported he prefers staying home. Patients reported a median of two ER visits (range 0-45) and two hospitalizations (range 0-30) in the previous 12 months. Almost all (92%) patients reported having a PCP. Sixty-seven percent visited the PCP 1-3 times per year, whereas 88% visited the SCD doctor 4-12 times per year. Whereas 68.3% patients trusted their PCP to manage their SCD, their level of trust with the SCD doctor was 95.2% (p <0.01). Similarly, 68% patients agreed that both the PCP and the SCD doctor should share responsibilities in their care. The table shows what participants considered most important for their PCP and their SCD doctor to know or learn about SCD. The most common single thing participants would change about the health care system was insurance/health care coverage (N=7), improved communication with providers (N=4), better care (N=2), or to have a cure for SCD (N=2). Six would not change anything. Eighty-four percent of patients did not know how to contact Sickle Cell Disease of America (SCDAA). Nine patients or parents (14%) felt discriminated against by health providers (N=3, doctors, nurses, pharmacy) and others (N=6, employers, classmates, others) as a result of their SCD. We identified five opportunities for quality improvement: 1. Improve pain control as 52.4% patients viewed their pain was sub-optimally managed, 2. Further examine and decrease barriers for HU treatment, 3. PCP education, as 31.7% of patients lacked trust in their competence, 4. Educate and encourage patient interaction with community-based organizations like SCDAA, and 5. Explore policy change in health care insurance coverage. Table Disclosures Alvarez: Forma Therapeutics: Consultancy; Novartis: Consultancy. Clay:Novartis: Speakers Bureau. Black:NHLBI: Research Funding; Micelle BioPharma: Research Funding; Pfizer: Research Funding; Sancilio and Company: Research Funding; Sanofi: Consultancy; Prolong Pharmaceuticals: Consultancy; Novartis: Research Funding; HRSA: Research Funding. Osunkwo:Novartis: Consultancy, Speakers Bureau; Pfizer: Consultancy; Terumo: Speakers Bureau; Micella Biopharma: Other: DSMB member.

Comprehensive Handbook of Childhood Cancer and Sickle Cell Disease

2006 ◽  
Keyword(s):  
Quality Of Life ◽  
Sickle Cell Disease ◽  
Childhood Cancer ◽  
Children And Adolescents ◽  
Peer Relationships ◽  
Sickle Cell ◽  
Cell Disease ◽  
Life Issues ◽  
Art Research

Over recent decades, tremendous advances in the prevention, medical treatment, and quality of life issues in children and adolescents surviving cancer have spawned a host of research on pediatric psychosocial oncology. This important volume fulfills the clear need for an up-to-date, comprehensive handbook for practitioners that delineates the most recent research in the field--the first of its kind in over a decade. Over 60 renowned authors have been assembled to provide a thorough presentation of the state-of-the art research and literature, with topics including: -Neuropsychological effects of chemotherapy and radiation therapy -Bone marrow transplantation -Important issues about quality of life during and following treatment -Collaborative research among child-focused psychologists -Standards of psychological care for children and adolescents -Stress and coping in the pediatric cancer experience -The role of family and peer relationships The Comprehensive Handbook of Childhood Cancer and Sickle Cell Disease represents both multidisciplinary and international efforts, an alliance between physicians and parents, and a combination of research and service. With a wealth of information of great interest to patients and their families, this volume will also be a welcome resource to the psychologists, psychiatrists, pediatricians, oncologists, nurses, and social workers who confront these issues as they help children and their families through the treatment, recovery, and grieving processes.

scholarly journals Antiphospholipid Antibodies in Sickle Cell Disease: A Systematic Review and Exploratory Meta-Analysis

2021 ◽  
Vol 27 ◽  
pp. 107602962110029
Author(s):  
Mira Merashli ◽  
Alessia Arcaro ◽  
Maria Graf ◽  
Matilde Caruso ◽  
Paul R. J. Ames ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Antiphospholipid Antibodies ◽  
Meta Analysis ◽  
Age Groups ◽  
Anticardiolipin Antibodies ◽  
Cell Disease ◽  
Pooled Prevalence ◽  
The Relationship

The relationship between antiphospholipid antibodies (aPL) and sickle cell disease (SCD) has never been systematically addressed. Our aim was to evaluate potential links between SCD and aPL in all age groups. EMBASE/PubMed was screened from inception to May 2020 and Peto odds ratios for rare events were calculated. The pooled prevalence (PP) of IgG anticardiolipin antibodies (aCL) was higher in individuals with SCD than in controls (27.9% vs 8.7%, P < 0.0001), that of IgM aCL was similar in the two groups (2.9% vs 2.7%); only individuals with SCD were positive for lupus anticoagulant (LA) (7.7% vs 0%, P < 0.0001). The PP of leg ulcers was similar between aPL positive and negative individuals (44% vs 53%) and between patients in acute crisis and stable patients (5.6% vs 7.3%). Reporting of aPL as a binary outcome and not as a titer precluded further interpretation. The results indicate that a prospective case-control study with serial measurements of a panel of aPL in SCD patients might be warranted, in order to understand further the possible pathogenic role of aPL in SCD.

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