The number of people with sickle cell disease in the United States: National and state estimates

American Journal of Hematology (Impact Factor: 3.8). 01/2009; 85(1):77-8. DOI: 10.1002/ajh.21570
Source: PubMed


Sickle-cell disease is not a reportable condition, making it difficult to ascertain the number of affected individuals. We estimated the number of people with sickle-cell disease for the United States and each individual state, adjusting for increased mortality. US Census population data for each of the 50 states plus the District of Columbia were obtained. The published prevalence of sickle-cell disease for blacks and Hispanics of either Mexican or non-Mexican ancestry was applied. Analysis revealed 89,079 (95% confidence interval: 88,494-89,664) people with sickle-cell disease in the United States, 80,151 black and 8928 Hispanic. The state with the highest sickle-cell population was New York with 8308, followed by Florida with 7539, and Texas with 6765 people with sickle-cell disease. This study provides important information for researchers and policymakers attempting to better plan for the care of the sickle-cell population.

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Available from: Julie Panepinto, Dec 22, 2014
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    • "It is responsible for approximately 113,000 hospitalizations and USD 488 million in hospitalization costs annually (Steiner 2006). Population estimates, with mortality adjusted by age and sickle-cell type, yielded an estimate for 2005 of 89,079 people with SCD in the USA, of which 80,151 were black and 8928 Hispanic (Brousseau 2009). In the UK there are approximately 12,500 people with the disease (National Screening Committee for SC and Thal 2006). "
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    ABSTRACT: BACKGROUND: Sickle cell disease is a group of disorders characterized by deformation of erythrocytes. Renal damage is a frequent complication in sickle cell disease as a result of long-standing anemia and disturbed circulation through the renal medullary capillaries. Due to the improvement in life expectancy of people with sickle cell disease, there has been a corresponding significant increase in the incidence of renal complications. Microalbuminuria and proteinuria are noted to be a strong predictor of subsequent renal failure. There is extensive experience and evidence with angiotensin-converting enzyme (ACE) inhibitors over many years in a variety of clinical situations for patients who do not have sickle cell disease, but their effect in people with this disease is unknown. It is common practice to administer ACE inhibitors for sickle nephropathy due to their renoprotective properties; however, little is known about their effectiveness and safety in this setting. This is an update of a Cochrane Review first published in 2013. OBJECTIVES: To determine the effectiveness of ACE inhibitor administration in people with sickle cell disease for decreasing intraglomerular pressure, microalbuminuria and proteinuria and to to assess the safety of ACE inhibitors as pertains to their adverse effects. SEARCH METHODS: The authors searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's Hameoglobinopathies Trials Register comprising references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings.Date of the most recent search: 03 June 2015. SELECTION CRITERIA: Randomized or quasi-randomized controlled trials of ACE inhibitors designed to reduce microalbuminuria and proteinuria in people with sickle cell disease compared to either placebo or standard treatment regimen. DATA COLLECTION AND ANALYSIS: Three authors independently applied the inclusion criteria in order to select studies for inclusion in the review. Two authors assessed the risk of bias of studies and extracted data and the third author verified these assessments. MAIN RESULTS: Five studies were identified through the searches, only one met our inclusion criteria. The included study randomized 22 participants (seven males and 15 females) having proteinuria or microalbuminuria with sickle cell disease and treated the participants for six months (median length of follow up of three months) with captopril or placebo. The overall quality of the outcomes reported was high, since most aspects that may contribute to bias were regarded to be of low risk, although allocation concealment was not reported. At six months, the study reported no significant difference in urinary albumin excretion between the captopril group and the placebo group, although the mean urinary albumin excretion in the captopril group was lower by a mean difference of -49.00 (95% confidence interval -124.10 to 26.10) compared to that of placebo. However, our analysis on the absolute change score showed significant changes between the two groups by a mean difference of -63.00 (95% confidence interval -93.78 to -32.22). At six months albumin excretion in the captopril group was noted to decrease from baseline by a mean of 45 ± 23 mg/day and the placebo group was noted to increase by 18 ± 45 mg/day. Serum creatinine and potassium levels were reported constant throughout the study. The potential for inducing hypotension should be highlighted; the study reported a decrease of 8 mmHg in systolic pressure and 5 mmHg in diastolic and mean blood pressure. AUTHORS' CONCLUSIONS: There is not enough evidence to show that the administration of ACE inhibitors is associated with a reduction of microalbuminuria and proteinuria in people with sickle cell disease, although a potential for this was seen. More long-term studies involving multiple centers and larger cohorts using a randomized-controlled design are warranted, especially among the pediatric age group. Detailed reporting of each outcome measure is necessary to allow a clear cut interpretation in a systematic review. One of the difficulties encountered in this review was the lack of detailed data reported in the included study
    Full-text · Article · Jun 2015 · Cochrane database of systematic reviews (Online)
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    • "Worldwide it is estimated that over 200,000 children affected with SCD are born every year, primarily in sub-Saharan Africa (180,000 births per year) [3] [4]. Approximately 2000 children in the US [5] are born with SCD each year, with a disease incidence of 1 in 2474 live births (newborn screening data 1990–1999) [6]; the estimated US prevalence ranges from 70,000–140,000 [7] [8]. "
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    ABSTRACT: Sickle cell disease (SCD) is a genetic disorder characterised by anaemia and "sickling" of red blood cells, leading to chronic haemolytic anaemia, vascular injury, and organ dysfunction. Although children and adults experience many similar symptoms and problems, complications increase with age, leading to early mortality. Hydroxyurea (hydroxycarbamide), the only US Food and Drug Administration-approved treatment, continues to be under-utilised and other treatments available to children are often inaccessible for adults. Haematopoietic stem-cell transplantation is a curative option, but is limited by a lack of donors and concerns for transplant-related toxicities. Although comprehensive programs exist in paediatrics, affected adults may not have access to preventative and comprehensive healthcare because of a lack of providers or care coordination. They are often forced to rely on urgent care, leading to increased healthcare utilisation costs and inappropriate treatment. This problem highlights the importance of primary care during the transition from paediatrics to adulthood.
    Full-text · Article · Sep 2013 · Blood reviews
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    • "Recent estimates suggest that the numbers of individuals in the United States living with sickle cell disease (SCD) is approaching 100,000 of which approximately 90% are African- American (Brousseau et al., 2010). Sickle cell disease is associated with significant mortality and morbidity and the most common clinical symptom of SCD is the painful vasoocclusive crisis. "
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    ABSTRACT: To determine if dental infections increase the likelihood of hospital admission among adult patients with sickle cell disease (SCD). Cross-sectional analysis of data from the Nationwide Emergency Department Sample (NEDS) pooled for the years 2006 through 2008. Prevalence ratios (PR) for the effects of interest were estimated using Poisson regression with robust estimates of the variance. Adults, aged 18 and over, diagnosed with SCD using ICD-9-CM codes excluding participants discharged with a code for sickle cell trait. Emergency department (ED) visit disposition, dichotomised to represent whether or not the ED visit ended in admission versus being treated and released. Among patients having a sickle cell crisis, those with dental infections were 72% more likely to be admitted compared to those not having dental infections (PR = 1.72, 95% CI 1.58-1.87). No association was observed among adult SCD patients not having a sickle crisis event. Based on preliminary data from this analysis, prevention of dental infection among patients with SCD could result in an estimated cost saving of $2.5 million dollars per year. Having a dental infection complicated by a sickle cell crisis significantly increases the likelihood of hospital admission among adult SCD patients presenting to the ED.
    Full-text · Article · Sep 2013 · Community dental health
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