scholarly journals Rigorous and practical quality indicators in sickle cell disease care

Hematology ◽  
2017 ◽  
Vol 2017 (1) ◽  
pp. 418-422 ◽  
Author(s):  
Suzette O. Oyeku ◽  
Elissa Z. Faro

Abstract In recent years, several sickle cell–specific quality indicators have been developed using rigorous approaches. A review of the history and current status of the development of sickle cell–specific indicators highlights opportunities for future refinement. Despite efforts at alignment, lack of strong evidence hinders the adoption of current quality indicators across stakeholder groups. There are many directions in which to take the current existing quality indicators, including expanding to different age groups, aims of care such as safety and equity, and better understanding of contextual and environmental factors.

2021 ◽  
Vol 27 ◽  
pp. 107602962110029
Author(s):  
Mira Merashli ◽  
Alessia Arcaro ◽  
Maria Graf ◽  
Matilde Caruso ◽  
Paul R. J. Ames ◽  
...  

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.


Hemato ◽  
2022 ◽  
Vol 3 (1) ◽  
pp. 82-97
Author(s):  
Anna Daniel Fome ◽  
Raphael Z. Sangeda ◽  
Emmanuel Balandya ◽  
Josephine Mgaya ◽  
Deogratius Soka ◽  
...  

Hematological and biochemical reference values in sickle cell disease (SCD) are crucial for patient management and the evaluation of interventions. This study was conducted at Muhimbili National Hospital (MNH) in Dar es Salaam, Tanzania, to establish laboratory reference ranges among children and adults with SCD at steady state. Patients were grouped into five age groups and according to their sex. Aggregate functions were used to handle repeated measurements within the individual level in each age group. A nonparametric approach was used to smooth the curves, and a parametric approach was used to determine SCD normal ranges. Comparison between males and females and against the general population was documented. Data from 4422 patients collected from 2004–2015 were analyzed. The majority of the patients (35.41%) were children aged between 5–11 years. There were no significant differences (p ≥ 0.05) in mean corpuscular hemoglobin concentration (MCHC), lymphocytes, basophils, and direct bilirubin observed between males and females. Significant differences (p < 0.05) were observed in all selected parameters across age groups except with neutrophils and MCHC in adults, as well as platelets and alkaline phosphatase in infants when the SCD estimates were compared to the general population. The laboratory reference ranges in SCD at steady state were different from those of the general population and varied with sex and age. The established reference ranges for SCD at steady state will be helpful in the management and monitoring of the progress of SCD.


Hematology ◽  
2002 ◽  
Vol 2002 (1) ◽  
pp. 10-34 ◽  
Author(s):  
Mark C. Walters ◽  
Arthur W. Nienhuis ◽  
Elliott Vichinsky

Abstract In this update, selected clinical features of sickle cell disease and their management are reviewed. In addition, the current status of interventions that have curative potential for sickle cell disease is discussed, with particular attention focused on indications, methodology, recent results, and challenges to wider clinical application. In Section I, Dr. Nienhuis describes recent improvements in vector technology, safety, and replacement gene expression that are creating the potential for clinical application of this technology. In Section II, Dr. Vichinsky reviews our current understanding of the pathophysiology and treatment of pulmonary injury in sickle cell disease. The acute and chronic pulmonary complications of sickle cell disease, modulators and predictors of severity, and conventional and novel treatment of these complications are discussed. In Section III, Dr. Walters reviews the current status of hematopoietic cell transplantation for sickle cell disease. Newer efforts to expand its availability by identifying alternate sources of stem cells and by reducing the toxicity of transplantation are discussed.


2021 ◽  
Vol 17 (4) ◽  
pp. 301-310
Author(s):  
Nidhi Shukla, MS, MBA ◽  
Jamie C. Barner, PhD, FAACP, FAPhA ◽  
Kenneth A. Lawson, PhD, FAPhA ◽  
Karen L. Rascati, PhD

Introduction: Sickle cell disease (SCD) is associated with recurrent complications and healthcare burden. Although SCD management guidelines differ based on age groups, little is known regarding actual utilization of preventative (hydroxyurea) and palliative therapies (opioid and nonopioid analgesics) to manage complications. This study assessed whether there were age-related differences in SCD index therapy type and SCD-related medication utilization.Design and patients: Texas Medicaid prescription claims from September 1, 2011 to August 31, 2016 were retrospectively analyzed for SCD patients aged 2-63 years who received one or more SCD-related medications (hydroxyurea, opioid, or nonopioid analgesics).Outcome measures: The primary outcomes were SCD index drug type and medication utilization: hydroxyurea adherence, and days’ supply of opioid, and nonopioid analgesics. Chi-square, analysis of variance, and Kruskal–Wallis tests were used.Results: Index therapy percentages for included patients (N = 2,339) were the following: opioids (45.7 percent), nonopioids (36.6 percent), dual therapy-opioids and nonopioids (11.2 percent), and hydroxyurea (6.5 percent), and they differed by age-groups (χ2 = 243.0, p 0.0001). Hydroxyurea as index therapy was higher among children (2-12:9.1 percent) compared to adults (26-40:3.7 percent; 41-63:2.9 percent). Opioids as index therapy were higher among adults (18-25:48.0 percent; 26-40:54.9 percent; 41-63:65.2 percent) compared to children (2-12:36.6 percent). Mean hydroxyurea adherence was higher (p 0.0001) for younger ages, and opioid days’ supply was higher for older ages.Conclusions: Texas Medicaid SCD patients had low hydroxyurea utilization and adherence across all age groups. Interventions to increase the use of hydroxyurea and newer preventative therapies could result in better management of SCDrelated complications and reduce the frequency of pain crises, which may reduce the need for opioid use.


1988 ◽  
Vol 34 (7) ◽  
pp. 1443-1446 ◽  
Author(s):  
G I Ekeke ◽  
G O Ibeh

Abstract Neuraminic (sialic) acid concentrations in serum from normal and sickle cell (HbSS) subjects were determined for discrete age groups from childhood through adolescence. Values in sickle cell disease were consistently lower over the entire age range. We further investigated the effect of exogenous sialic acid on the rate of sickling reversion of HbSS erythrocytes and demonstrated that this compound in millimole per liter concentrations could revert pre-sickled erythrocytes to their normal morphology in a concentration-dependent manner. When subjected to partial de-sialation with sialidase (EC 3.2.1.18), the HbSS erythrocytes not only sickled faster upon deoxygenation, they also reverted more slowly on treatment with phenylalanine (a more efficient anti-sickling agent than sialic acid) than did untreated cells. We conclude that, in sickle cell disease, erythrocyte sialic acid content could play a significant role, not only in the control of the sickling rate in vivo, but also, after sickling has occurred, in the rate of recovery from a sickling crisis.


Haematologica ◽  
2016 ◽  
Vol 102 (4) ◽  
pp. 666-675 ◽  
Author(s):  
Frédéric B. Piel ◽  
Sanjay Tewari ◽  
Valentine Brousse ◽  
Antonis Analitis ◽  
Anna Font ◽  
...  

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 3790-3790
Author(s):  
Deborah Yallop ◽  
Edward R. Duncan ◽  
Ellie Norris ◽  
Gary Fuller ◽  
Nikki Thomas ◽  
...  

Abstract The clinical severity of sickle cell disease (SCD) is dependent on genetic and environmental variables. The impact of environmental factors on disease is a major public health issue and air pollution has been consistently correlated with poor health outcomes. Environmental factors in SCD have been poorly studied. We have retrospectively studied the numbers of daily admissions with vaso-occlusive sickle cell pain to King’s College Hospital, London, in relation to local daily air quality measurements. We analysed 1047 patient episodes over 1400 days (1st January 1998 to 31st October 2001). Statistical time series analysis was performed using cross-correlation function (CCF), where the observations of one series are correlated with the observations of another series at various lags and leads, values >0.05 being significant. This showed a significant association between increased numbers of admissions and low levels of nitric oxide (NO) (CCF=0.063), low levels of carbon monoxide (CO) (CCF=0.064) and high levels of ozone (O3) (CCF=0.067). There was no association with sulphur dioxide (SO2), nitric dioxide (NO2) or dust. The significant results were further examined using quartile analysis. This confirmed that increased numbers of hospital admissions were associated with high levels of O3 (oneway ANOVA p=0.039) and low levels of CO (oneway ANOVA p=0.042). Low NO levels were also associated with increased admissions, not however reaching statistical significance on quartile analysis (oneway ANOVA p=0.158). O3 levels show marked seasonal variation, with high levels occurring in summer months in the UK. We have previously shown a trend towards increased admissions in the summer months with acute sickle related pain, whereas other groups, which are primarily based in rural tropical climates, found increased admissions in cold, rainy months. The adverse effect of high O3 levels may explain this difference. There is no direct evidence to explain the mechanism by which high O3 levels leads to vaso-occlusion in SCD but high levels of O3 have been linked to reduced respiratory function, which may in turn precipitate vaso-occlusion. Independent studies have shown high CO levels are linked to increased respiratory and cardiovascular admissions. Paradoxically we found that high CO levels were linked to decreased admission numbers and may be protective against acute pain in SCD. CO may confer benefit by forming carboxyhaemoglobin that cannot polymerise resulting in decreased sickling. Previous studies have shown prolonged red cell survival in vivo following administration of CO to patients with SCD. Our study also suggests higher levels of atmospheric NO are linked to fewer admissions. NO is known to be central in the pathophysiology of vaso-occlusion and sickle cell patients are thought to have functional deficiency of NO. Many groups have reported inhaled NO as beneficial in the treatment of sickle pain. Our study suggests air quality has a significant effect on acute pain in SCD and that patients should be counselled accordingly. Based on these findings it would be appropriate to warn patients that high O3 levels might precipitate complications of SCD. The potential beneficial effect of CO and NO is intriguing and requires further investigation.


Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 83-83 ◽  
Author(s):  
Bradd G. Hemker ◽  
David C Brousseau ◽  
Ke Yan ◽  
Raymond G Hoffmann ◽  
Julie A. Panepinto

Abstract Abstract 83 Background: Improved survival of patients with sickle cell disease has led to increased attention on the transition of care from pediatric to adult providers. Recent studies have shown increased emergency department (ED) utilization as well as an increased risk of mortality during this transition period for patients with sickle cell disease. Suggested reasons for such changes include loss of a medical home, loss of insurance, and loss of access to outpatient clinics as pediatric sickle cell patients transition to adult services. Emergency Department Reliance (EDR), defined as the number of ED visits divided by the number of ED and outpatient visits, views ED visits in relation to all ambulatory visits. Those with more severe disease, who utilize the ED more, should also have more outpatient visits, while those without adequate outpatient clinic access simply use the ED more without a rise in outpatient clinic visits, thus increasing EDR. EDR, therefore, is used to differentiate increased ED use due to need for care from increased ED use as an access issue. We hypothesized that as patients transition from pediatric to adult centered care, loss of a primary medical home would lead to an increase in the EDR. Methods: We conducted a retrospective cohort study using the Wisconsin State Medicaid database from 2003–2007. This database includes 757 patients of any age with sickle cell disease who are part of the Wisconsin Medicaid system. Consistent with previous literature, having sickle cell disease was defined by having at least one inpatient hospitalization, or two outpatient visits at least one month apart, with a diagnosis of sickle cell disease. All encounters during the study period were extracted from the database and linked by unique anonymous identifiers. Information extracted included age, all diagnosis codes, and classification as ED visit, outpatient visit, or inpatient hospitalization. The study population was divided into three mutually exclusive age groups: 1) children: ≤ 18 years old for the entire study period, 2) the transition group who turned 19 years old during the study period; and 3) Young adults age ≥ 19–45 years at first encounter. Adults age 3 46 at first encounter were eliminated from the analysis as the goal was to compare the transition group to the age groups immediately above and below. The median Emergency Department Reliance (ED visits/[ED + outpatient visits]) was compared for each age group for sickle cell related diagnoses, all diagnoses, and sickle cell crisis diagnoses using non-parametric, multiple-comparisons adjusted tests. Results: The 687 remaining patients had a total of 32,258 ambulatory visits over the five year study period, including 20,418 outpatient visits and 11,840 emergency department visits. There were 345 children, 65 patients in the transition group, and 277 young adults. For sickle cell diagnoses, the transition group had a significantly higher EDR than children (0.50 vs. 0.39, p=0.031) and a higher EDR than young adults, though this increase failed to achieve statistical significance (0.50 vs. 0.41, p=0.360). The difference in EDR between the children and adults for sickle cell diagnoses was not significant. A similar pattern emerged when computing EDR based on all diagnoses, with the EDR for the transition group being significantly higher than for children (0.35 vs. 0.28, p<0.001) as well as increased compared to adults, although this did not reach statistical significance (0.35 vs. 0.31, p=0.073). Lastly, the EDR was highest in all age groups for sickle cell crisis diagnoses (EDR ≥ 0.68 in all three groups). Conclusions: Patients with sickle cell disease who are transitioning from pediatric to adult centered care have an increased reliance on the emergency department as a location of care for sickle cell diagnoses. As adults, emergency department reliance returns to similar levels as childhood, suggesting a stabilization of a medical home following the transition period. The EDR is highest for all groups at times of acute pain crisis related to sickle cell disease, consistent with the need for emergent care in those situations. These findings support the hypothesis that increased ED utilization during the transition period from pediatric to adult providers is potentially due to limitations in access to care. Disclosures: No relevant conflicts of interest to declare.


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