Improvements in survival for children with sickle cell disease (SCD) during the last 30 years have been well established. Whether similar improvements for adults with the disease have occurred is unknown. We investigated mortality rates for children and adults with SCD.
We used the National Center for Health Statistics multiple-cause-of-death files to examine age at death and calculate mortality rates from 1979 to 2005. We examined trends in mortality rates using negative binomial regression, and we examined age at death using t-tests and linear regression.
We identified 16,654 sickle cell-related deaths. Mean age at death was significantly different for males (33.4 years, 95% confidence interval [CI] 33.0, 33.7) than for females (36.9 years, 95% CI 36.5, 37.4). In a regression model controlling for gender, the mean age at death increased by 0.36 years for each year of the study. The median age at death in 2005 was 42 years for females and 38 years for males. The overall mortality rate increased 0.7% (<0.001) each year during the time period studied. The adult (>19 years of age) mortality rate increased by 1% (<0.001) each year during the time period studied. The pediatric mortality rate decreased by 3% (<0.001) each year during the time period studied.
These data confirm prior findings of a significant decrease in mortality for children with SCD. The mortality rate for adults appears to have increased during the same time period. It seems unlikely that this increase is due merely to an influx of younger patients surviving to adulthood and may reflect a lack of access to high-quality care for adults with SCD.
"Sickle cell disease (SCD) is a painful, chronic, genetic condition that affects 90,000–100,000 individuals in the U.S.1 and shortens life expectancy to around 40 years.2–5 While there is wide variation in the use patterns of healthcare by SCD patients, particularly of emergency department (ED) care, there is also substantial evidence of generally poor quality of care for SCD patients in the ED.6–8 "
[Show abstract][Hide abstract] ABSTRACT: Introduction
Patients with sickle cell disease (SCD) often seek care in emergency departments (EDs) for severe pain. However, there is evidence that they experience inaccurate assessment, suboptimal care, and inadequate follow-up referrals. The aim of this project was to 1) explore the feasibility of applying a failure modes, effects and criticality analysis (FMECA) in two EDs examining four processes of care (triage, analgesic management, high risk/high users, and referrals made) for patients with SCD, and 2) report the failures of these care processes in each ED.
A FMECA was conducted of ED SCD patient care at two hospitals. A multidisciplinary group examined each step of four processes. Providers identified failures in each step, and then characterized the frequency, impact, and safeguards, resulting in risk categorization.
Many “high risk” failures existed in both institutions, including a lack of recognition of high-risk or high-user patients and a lack of emphasis on psychosocial referrals. Specific to SCD analgesic management, one setting inconsistently used existing analgesic policies, while the other setting did not have such policies.
FMECA facilitated the identification of failures of ED SCD care and has guided quality improvement activities. Interventions can focus on improvements in these specific areas targeting improvements in the delivery and organization of ED SCD care. Improvements should correspond with the forthcoming National Heart, Lung and Blood-sponsored guidelines for treatment of patients with sickle cell disease.
The western journal of emergency medicine 07/2014; 15(4):446-58. DOI:10.5811/westjem.2014.4.20489
[Show abstract][Hide abstract] ABSTRACT: Hypoxia is a common feature in children with sickle cell disease (SCD) that is inconsistently associated with painful crises and acute chest syndrome. To assess the prevalence and risk factors of hypoxia, we recorded daytime, nocturnal, and postexercise pulse oximetry (SpO2) values in 39 SCD patients with a median age of 10.8 years. Median daytime SpO2 was 97% (range, 89%-100%), and 36% of patients had daytime hypoxia defined as SpO2<96%. Median nocturnal SpO2 was 94.7% (range, 87.7%-99.5%), 50% of patients had nocturnal hypoxia defined as SpO2≤93%, and 11(37%) patients spent more than 10% of their total sleep time with SpO2<90%. Median postexercise SpO2 was 94% (range, 72%-100%) and 44.7% of patients had postexercise hypoxia defined as an SpO2 decrease ≥3% after a 6-minute walk test. Among patients with normal daytime SpO2, 35% had nocturnal and 42% postexercise hypoxia. Compared to 9 patients without daytime, nocturnal, or postexercise hypoxia, 25 patients with hypoxia under at least one of these three conditions had greater anemia severity (P = 0.01), lower HbF levels (P = 0.04), and higher aspartate aminotransferase levels (P = 0.03). Males predominated among patients with postexercise hypoxia (P = 0.004). Hypoxia correlated neither with painful crises nor with acute chest syndrome. Of 32 evaluable patients, 6 (18.8%) had a tricuspid regurgitation velocity ≥2.6 m/s, and this feature was associated with anemia (P = 0.044). Median percentage of the predicted distance covered during a 6-minute walk test was 86% [46-120]; the distance was negatively associated with LDH (P = 0.044) and with a past history of acute chest syndrome (P = 0.009). In conclusion, severe episodes of nocturnal and postexercise hypoxia are common in children with SCD, even those with normal daytime SpO2.
PLoS ONE 05/2014; 9(5):e97462. DOI:10.1371/journal.pone.0097462 · 3.23 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Hydroxyurea is the sole approved pharmacologic therapy for sickle cell disease (SCD). Higher fetal hemoglobin (HbF) levels diminish de-oxygenated sickle globin polymerization in vitro and clinically reduce the incidence of disease morbidities. Clinical and laboratory effects of hydroxyurea largely result from induction of HbF expression, though to a highly variable extent. Baseline and hydroxyurea-induced HbF expression are both inherited complex traits. In children with SCD, baseline HbF remains the best predictor of drug-induced levels, but accounts for only portion of the induction. A limited number of validated genetic loci are strongly associated with higher baseline HbF levels in SCD. For induced HbF levels, genetic approaches using candidate single nucleotide polymorphisms (SNP) have identified some of these same loci as also associated with induction. However, SNP associations to induced HbF are only partially independent of baseline levels. Additional approaches to understanding the impact of hydroxyurea on HbF and its other therapeutic effects on SCD include pharmaco-kinetic, gene expression and epigenetic analyses in patients and through existing murine models for SCD. Understanding the genetic and other factors underlying the variability in therapeutic effects of hydroxyurea for pediatric SCD is critical for prospectively predicting good responders and for designing other effective therapies.Pediatric Research (2013); doi:10.1038/pr.2013.227.
Pediatric Research 11/2013; 75(1-2). DOI:10.1038/pr.2013.227 · 2.31 Impact Factor
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