In its decades-long history, the Division of Blood Disorders (DBD) at CDC has evolved from a patient-focused, services-supporting entity at inception, to one of the world leaders in the practice of public health to improve the lives of people at risk for or affected by nonmalignant blood disorders. The DBD's earliest public health activities consisted of working with care providers in a network of hemophilia treatment centers to provide AIDS risk reduction services to people with hemophilia. Because this infectious disease threat has been reduced over time as a result of the development of safer treatment products, the DBD-under the auspices of congressional appropriations guidance-has expanded its core activities to encompass blood disorders other than hemophilia, including hemoglobinopathies such as thalassemia and sickle cell disease, and Diamond Blackfan anemia. Simultaneously, in transitioning to a greater public health role, the DBD has expanded its network of partners to new consumer and professional organizations, as well as state and other federal health agencies. The DBD has also developed and maintains many surveillance and registry activities beyond the Universal Data Collection system aimed at providing a better understanding of the health status, health needs, and health-related quality of life of people with nonmalignant blood disorders. The DBD has integrated applicable components of the Essential Services of Public Health successfully to promote and advance the agenda of blood disorders in public health.

OBJECTIVE: We sought to determine prevalence and likelihood of venous thromboembolism (VTE) among women with and without polycystic ovary syndrome (PCOS). STUDY DESIGN: We performed a cross-sectional analysis using Thomson Reuters MarketScan Commercial databases for the years 2003 through 2008. The association between VTE and PCOS among women aged 18-45 years was assessed using age-stratified multivariable logistic regression models. RESULTS: Prevalence of VTE per 100,000 was 374.2 for PCOS women and 193.8 for women without PCOS. Compared with women without PCOS, those with PCOS were more likely to have VTE (adjusted odds ratio [aOR] 18-24 years, 3.26; 95% confidence interval [CI], 2.61-4.08; aOR 25-34 years, 2.39; 95% CI, 2.12-2.70; aOR 35-45 years, 2.05; 95% CI, 1.84-2.38). A protective association (odds ratio, 0.8; 95% CI, 0.73-0.98) with oral contraceptive use was noted for PCOS women. CONCLUSION: PCOS might be a predisposing condition for VTE, particularly among women aged 18-24 years. Oral contraceptive use might be protective.

OBJECTIVE: The purpose of this study was to estimate the prevalence of polycystic ovary syndrome (PCOS) and its phenotypes as defined by the National Institutes of Health, Rotterdam criteria, and Androgen Society. STUDY DESIGN: Thomson Reuters MarketScan Commercial databases (Thomson Reuters Healthcare Inc, New York, NY) for 2003-2008 were used to calculate the prevalence of PCOS and to assess differences in demographic characteristics and comorbid conditions among women who were 18-45 years old with and without PCOS. RESULTS: The prevalence of PCOS was 1585.1 per 100,000; women with phenotype A or classic PCOS were most prevalent at 1031.5 per 100,000. Women with PCOS were more likely than those without PCOS to be 25-34 years old, be from the South, be infertile, have metabolic syndrome, have been seen by an endocrinologist, and have taken oral contraceptives. CONCLUSION: This is the first study to use all available criteria to estimate the prevalence of PCOS. Providers should evaluate women with menstrual dysfunction for the presence of PCOS.

BACKGROUND: Deep vein thrombosis and pulmonary embolism (PE) are responsible for substantial mortality, morbidity, and impaired health-related quality of life. The aim of this study was to evaluate the correlates of in-hospital deaths among hospitalizations with a diagnosis of PE in the United States. METHODS: By using data from the 2001-2008 National Hospital Discharge Survey, we assessed the correlates of in-hospital deaths among 14,721 hospitalizations with a diagnosis of PE and among subgroups stratified by age, sex, race, days of hospital stay, type of admission, cancer, pneumonia, and fractures. We produced adjusted rate ratios (aRR) and 95% confidence intervals using log-linear multivariate regression models. RESULTS: Regardless of the listing position of diagnostic codes, we observed an increased likelihood of in-hospital death in subgroups of hospitalizations with ages 50 years and older (aRR = 1.82-8.48), less than 7 days of hospital stay (aRR = 1.43-1.57), cancer (aRR = 2.10-2.28), pneumonia (aRR = 1.79-2.20), or fractures (aRR = 2.18) (except for first-listed PE), when compared to the reference groups with ages 1-49 years, 7 days or more of hospital stay, without cancer, pneumonia, or fractures while adjusting for covariates. In addition, we observed an increased likelihood of in-hospital death for first-listed PE in hospitalizations of women, when compared to those of men (aRR = 1.45). CONCLUSIONS: The results of this study provide support for identifying, developing, and implementing effective, evidence-based clinical assessment and management strategies to reduce PE-related morbidity and mortality among hospitalized PE patients who may have concurrent health conditions including cancer, pneumonia, and fractures.

We welcome the opportunity to provide additional support for the assertion in our paper on sickle cell disease (SCD) in Africa that newborn screening can result in substantially reduced mortality even in the absence of specifıc interventions such as penicillin prophylaxis or pneumococcal conjugate vaccines (PCVs).1 Three references were cited describing reductions in SCD mortality that occurred prior to the availability of those interventions.2–4 In one U.S. study, the rate of mortality among infants with sickle cell anemia diagnosed as newborns in a pilot screening study during 1975–1985 was 1.8% compared with 8% among infants diagnosed after age 3 months, a difference attributed to better follow-up and education.2 The subsequent introduction of oral penicillin prophylaxis in the U.S. was associated with a 36% reduction in SCD mortality in children aged <4 years between 1983–1986 and 1987–1990.5 The administration of PCV-7 to U.S. infants beginning in 2000, which eliminated many deaths from invasive pneumococcal disease (IPD) due to incomplete adherence to penicillin prophylaxis, was accompanied by a further 42% drop in SCD mortality in children aged <4 years between 1995–1998 and 1999–2002.5 | In Jamaica, King et al. and others documented sharp reductions in SCD mortality among infants born between 1973 and 1981 who were enrolled in the Jamaican Sickle Cell Cohort Study (JSCCS).4,6 The rate of death in the fırst 2 years of life fell by 71% from 14% in the 1973–1975 birth cohort to 4% in the 1979–1981 birth cohort. One specific intervention, parental education on the detection of enlarged spleens and need for medical attention, was shown to result in a 74% decrease in the death rate from acute splenic sequestration (ASS) from 1.3 per 100 patient-years during 1974–1978 to 0.3 per 100 patient-years during 1979–1983, calculated from data on incidence and case-fatality of ASS.4,7

INTRODUCTION: Pregnant women are four to five times more likely than nonpregnant women to develop venous thromboembolism (VTE). The aim of this review is to provide an overview of guidelines in the literature on VTE risk assessment, screening for thrombophilias, and thromboprophylaxis dissemination among pregnant women. METHODS: We performed a review of the published literature to identify evidence-based guidelines published between the years 2000 and 2011. We searched for guidelines from U.S. and international organizations that identified clinically based practice recommendations to healthcare providers on how VTE risk should be assessed, thrombophilias screened, and thromboprophylaxis disseminated among pregnant women. RESULTS: We found nine guidelines that met our requirements for assessing VTE risk and found seven guidelines addressing thrombophilia screening. Seven of the nine agreed that all women should undergo a risk factor assessment for VTE either in early pregnancy or in the preconception period. Seven of the nine agreed that pregnant women with more than one additional VTE risk factor be considered for thromboprophylaxis, and five of the seven groups addressing thrombophilia screening agreed that selected at-risk populations should be considered for thrombophilia screening. CONCLUSIONS: There is some agreement between U.S. and international guidelines that women should be assessed for VTE risk during preconception and again in pregnancy. Although there is agreement that the general population of women should not be screened for thrombophilias, no agreement exists as to the clinical subgroups for which screening should be done.

Pulmonary embolism (PE) is a potentially life-threatening condition that typically occurs when a thrombus from deep veins in the leg, pelvis, arms, or heart embolizes to the lungs.1,2 Recent data linked PE to approximately 247 000 hospitalizations in the United States in 2006.3 To our knowledge, the case-fatality rate and estimated number of in-hospital deaths among a national representative example of hospitalizations that encompass first-listed and any-listed PE diagnoses in the United States are limited. Therefore, we report nationally representative estimates of in-hospital deaths (ie, annual number and case-fatality rate) among hospitalizations with a PE diagnosis (ie, first-listed and any-listed) in the United States by analyzing data from the 2001-2008 National Hospital Discharge Survey (NHDS).4

The public health system encompasses governments, healthcare providers, and others working to improve population health. The IOM in 1988 identified three core functions of public health: assessment, policy development, and assurance.1 In 1994, the Core Public Health Functions Steering Committee, which included representatives from U.S. Public Health Service agencies and other major public health organizations, developed the framework for the ten essential services for public health.2 This framework of generic goals and strategies is used by public health programs and systems to address health challenges facing the population or community as a whole, notably environmental hazards, injuries, infections, and common chronic diseases, but has also been applied to blood disorders.3, 4 | The papers in this supplement to the American Journal of Preventive Medicine4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20 address various facets of blood disorders and public health. Specifically, the majority of the papers focus on blood disorders that are considered rare in North America or Western Europe. Because of their rarity, rare disorders often are not considered to fall under the purview of the public health system even though, collectively, rare disorders do affect large numbers of people. However, a public health framework is needed to address public health services and functions for all rare disorders, including blood disorders, regardless of the incidence or prevalence of a given disorder.

Sickle cell disease (SCD) is common throughout much of sub-Saharan Africa, affecting up to 3% of births in some parts of the continent. Nevertheless, it remains a low priority for many health ministries. The most common form of SCD is caused by homozygosity for the beta-globin S gene mutation (SS disease). It is widely believed that this condition is associated with very high child mortality, but reliable contemporary data are lacking. We have reviewed available African data on mortality associated with SS disease from published and unpublished sources, with an emphasis on two types of studies: cross-sectional population surveys and cohort studies. We have concluded that, although current data are inadequate to support definitive statements, they are consistent with an early-life mortality of 50%-90% among children born in Africa with SS disease. Inclusion of SCD interventions in child survival policies and programs in Africa could benefit from more precise estimates of numbers of deaths among children with SCD. A simple, representative, and affordable approach to estimate SCD child mortality is to test blood specimens already collected through large population surveys targeting conditions such as HIV, malaria, and malnutrition, and covering children of varying ages. Thus, although there is enough evidence to justify investments in screening, prophylaxis, and treatment for African children with SCD, better data are needed to estimate the numbers of child deaths preventable by such interventions and their cost effectiveness.

Sickle cell disease (SCD) is a collection of inherited blood disorders that affect a substantial number of people in the U.S., particularly African Americans. People with SCD have an abnormal type of hemoglobin, Hb S, which polymerizes when deoxygenated, causing the red blood cells to become misshapen and rigid. Individuals with SCD are at higher risk of morbidity and mortality from infections, vaso-occlusive pain crises, acute chest syndrome, and other complications. Addressing the public health needs related to SCD is an important step toward improving outcomes and maintaining health for those affected by the disorder. The objective of this study was to review public health activities focusing on SCD and define the need to address it more comprehensively from a public health perspective. We found that there has been some progress in the development of SCD-related public health activities. Such activities include establishing newborn screening (NBS) for SCD with all states currently having universal NBS programs. However, additional areas needing focus include strengthening surveillance and monitoring of disease occurrence and health outcomes, enhancing adherence to health maintenance guidelines, increasing knowledge and awareness among those affected, and improving healthcare access and utilization. These and other activities discussed in this paper can help strengthen public health efforts to address SCD.

Although the issue of whether sickle cell trait (SCT) is clinically benign or a significant health concern has not yet been resolved, the potential health risk to affected individuals is of vital importance and represents a tremendous challenge in protecting, promoting, and improving the health of the approximately 300 million people worldwide and 3 million people in the U.S. who possess the trait. In response to a request by the Sickle Cell Disease Association of America, in December 2009, the CDC convened a meeting of partners, stakeholders, and experts to identify the gaps in public health, clinical health services, epidemiologic research, and community-based outreach strategies and to develop an agenda for future initiatives. Through facilitated discussion and presentations in four topic areas, participants discussed pertinent issues, synthesized clinical research findings, and developed a coherent framework for establishing an agenda for future initiatives. A primary outcome of the meeting was to provide the first step of an iterative process to move toward agreement regarding appropriate counseling, care, and, potentially, treatment of people with SCT.

Deep vein thrombosis (DVT) and pulmonary embolism (PE), collectively known as venous thromboembolism (VTE), are an important and growing public health issue, associated with considerable morbidity and mortality. Presently, there is no national surveillance for DVT and PE. This article provides a summary of an expert workgroup meeting convened January 12, 2010, by the CDC. The purpose of the meeting was to inform CDC on the development of U.S. population-based public health surveillance activities for DVT/PE. Topics discussed included: (1) stakeholders, needs, gaps, and target populations; (2) requirements of surveillance systems; (3) challenges, limitations, and potential barriers to implementation of surveillance activities; and (4) integration of research and education with surveillance activities.

Venous thromboembolism (VTE) includes deep | vein thrombosis (DVT) and pulmonary embolism (PE); DVT refers to the formation of 1 or | more blood clots in a large vein, and PE results when a | portion of the blood clot breaks loose, travels through | the bloodstream, and partially or completely blocks a pulmonary artery.1 Venous thromboembolism is an important and growing public health concern; however, a national surveillance system for this condition has not been | established.2 Therefore, many of the current estimates of | VTE incidence were derived from geographically defined populations,3,4 single institutions,5 or hospital discharge databases.6 Hospital discharge data are particularly problematic because many patients are treated on | an outpatient basis.5 | Administrative data represent a potential source for | monitoring VTE trends in the absence of established public health surveillance systems. We used health insurance claims data from a large, privately insured US adult | population to estimate the prevalence of VTE during 2005 | through 2006 within that population. To account for the | potential for misclassification of DVT or PE diagnoses | in claims data, we also explored using different algorithms to calculate our estimates.

For many years, maternal mortality has been considered to be one of the leading public health problems facing health-care systems in developing countries. Global efforts to address this problem started with the Safe Motherhood Initiative in 1987; the problem was further highlighted during the 1994 International Conference on Population and Development, and most recently when reduction of maternal mortality was identified as one of the eight Millennium Development Goals (MDG 5).1, 2, 3, 4 In response to these initiatives, many countries started programmes to reduce maternal mortality, and it seems that some of these efforts are starting to show successes. A recent study estimated that about 342 000 maternal deaths occurred worldwide compared with the previous estimate of 526 000 maternal deaths in 1980.5 Despite this important progress, the challenge continues: the disparity in maternal mortality rates between developed and developing countries is higher than that of any other health outcome. The range is from fewer than ten deaths per 100 000 livebirths in most European countries to more than 500 deaths per 100 000 livebirths in many African countries. It is estimated that more than 50% of maternal deaths occur in six countries: India, Nigeria, Pakistan, Afghanistan, Ethiopia, and the Democratic Republic of the Congo. Moreover, most maternal deaths are preventable, with 73% of deaths attributed to haemorrhage, sepsis, eclampsia, unsafe abortion, and obstructed labour.6

Nonmalignant blood disorders meet all criteria for qualifying, as a group, as a very important public health problem with serious morbidities affecting over 1 million Americans every year, not including an additional 8 million individuals suffering from anemia. Many of these conditions and the morbidities and mortalities associated with them are, to a large extent, preventable. Further, the changing demographic composition of the American population is sure to increase the number of individuals affected by these conditions. Yet, nonmalignant blood disorders have not been recognized as important public health priorities. Immediate action is needed to meet the increasing challenge of blood disorders in public health. We propose a national, comprehensive, organized, coordinated, institutionalized, sustainable public health response to blood disorders based on the three core functions and the ten essential services of public health. Immediate action needs to be taken to improve surveillance and monitoring, increase public and provider awareness, increase the use of evidence-based practices, and enhance epidemiologic research on the causes, prevention, and treatment of conditions resulting in adverse outcomes.

BACKGROUND: Patients with sickle cell disease (SCD) often use emergency department services to obtain medical care. Limited information is available about emergency department use among patients with SCD. PURPOSE: This study assessed characteristics of emergency department visits made nationally by patients with SCD. METHODS: Data from the National Hospital Ambulatory Medical Care Survey (NHAMCS) for the years 1999-2007 were analyzed. The NHAMCS is a survey of hospital emergency department and outpatient visits. Emergency department visits by patients with SCD were identified using ICD-9-CM codes, and nationally weighted estimates were calculated. RESULTS: On average, approximately 197,333 emergency department visits were estimated to have occurred each year between 1999 and 2007 with SCD as one of the diagnoses listed. The expected source of payment was private insurance for 14%, Medicaid/State Children's Health Insurance Program for 58%, Medicare for 14%, and other/unknown for 15%. Approximately 29% of visits resulted in hospital admission; this was 37% among patients aged 0-19 years, and 26% among patients aged ≥20 years. The episode of care was indicated as a follow-up visit for 23% of the visits. Patient-cited reasons for the emergency department visit included chest pain (11%); other pain or unspecified pain (67%); fever/infection (6%); and shortness of breath/breathing problem/cough (5%), among other reasons. CONCLUSIONS: Substantial numbers of emergency department visits occur among people with SCD. The most common reason for the emergency department visits is pain symptoms. The findings of this study can help to improve health services delivery and utilization among patients with SCD.

The objectives of preconception care for women with blood disorders are to provide women and their partners with information on the implications of blood disorders for pregnancy; reproductive choices; and the management of potential or future pregnancies. Advances in hematology have led to improved diagnosis and treatment of blood disorders, thereby contributing to longevity and quality of life for women who are either affected by or are carriers of blood disorders. Women with blood disorders pose unique challenges: physiologic events such as pregnancy and menstruation influence the manifestations of blood disorders; blood disorders are a risk factor for adverse pregnancy outcomes; pregnancy imposes the risk of potential genetic transmission of the blood disorder to the offspring;and medications used for treatment of blood disorders pose additional challenges to conception and pregnancy. Hence, it is crucial that women of childbearing age with blood disorders be provided proper care for their conditions and be counseled before they become pregnant, in time to prevent complications to mothers and infants related to blood disorders. The purpose of this paper is to provide a brief overview of the current knowledge related to blood disorders in women of reproductive age, the interventions needed to manage these conditions, and the implications of these conditions and their management for the health of women and their infants.

BACKGROUND: Hemophilia is a hereditary bleeding disorder. Its complications can result in substantial morbidity, but few efforts have been made to quantify the disease burden. PURPOSE: The objective of this analysis was to estimate the burden of disease due to hemophilia (A and B) in the U.S., using disability-adjusted life years (DALY). METHODS: The approach taken by the WHO in its Global Burden of Disease study was followed. Assumptions were drawn from published literature, and population estimates from the U.S. Census Bureau for the Year 2007 were used. Estimations of years of life lost resulting from mortality (YLL) and years of life lost resulting from morbidity (YLD) were done separately by gender, 5-year age intervals, and severity of disease (morbidity only) with their sum representing DALYs. Disability weights were derived from the quality-of-life tool EuroQol (EQ-5D). The stability of burden estimates was tested by performing sensitivity analyses, changing one assumption at a time. RESULTS: In the U.S. in 2007, hemophilia resulted in 110,095 DALYs, composed of 13,418 YLLs and 96,677 YLDs. Large differences between men/boys (107,346) and women/girls (2749) were observed, given that females are genetic carriers of the disorder and rarely present with disease. Sensitivity analyses revealed a relatively robust estimate with a maximum variation of 4.49%. CONCLUSIONS: This first estimate of hemophilia-related DALYs in the U.S. indicates that control of hemophilia can potentially result in a gain of 1 healthy year of life for every 2700 people in the population.

Sickle cell disease (SCD) is a hemoglobinopathy and a life-long blood disorder characterized by red blood cells that assume an abnormal, rigid, sickle shape, which decreases the cells' flexibility and causes risk of various complications. About 7% of the world's population are carriers of the condition, and 300,000–400,000 affected children are born each year, making SCD the most common life-threatening monogenic disorder in the world1 (Figure 1). The highest frequency of SCD remains in tropical regions, particularly sub-Saharan Africa, India, and the Middle East, and in countries to which people from these regions have migrated. In the past 5 decades, because of migration and demographic changes, SCD has been observed with increasing frequency in previously low-incidence areas.2 There is marked variation in carrier frequency, not only between countries but also between regions within countries. For example, among the Bamba tribe in western Uganda, the carrier frequency is as high as 45%, whereas it is as low as 1%–2% in South Africa and on the North African coast.3