Materiały źródłowe

• #1 Global, regional, and national prevalence and mortality burden of sickle cell disease, 2000–2021 | Institute for Health Metrics and Evaluation

  https://www.healthdata.org/research-analysis/library/global-regional-and-national-prevalence-and-mortality-burden-sickle-cell

  We estimated 34400 (sickle cell) cause-specific all-age deaths globally in 2021, but total sickle cell disease mortality burden was nearly 11-times higher at 376000. […] Completed as part of the Global Burden of Diseases, Injuries, and Risk Factors Study (GBD) 2021, this study delivers a comprehensive global assessment of prevalence of sickle cell disease and mortality burden by age and sex for 204 countries and territories from 2000 to 2021. […] We estimated cause-specific sickle cell disease mortality using standardised GBD approaches, in which each death is assigned to a single underlying cause, to estimate mortality rates from the International Classification of Diseases (ICD)-coded vital registration, surveillance, and verbal autopsy data. […] Our findings show a strikingly high contribution of sickle cell disease to all-cause mortality that is not apparent when each death is assigned to only a single cause.

• #2 Sickle cell disease – Wikipedia

  https://en.wikipedia.org/wiki/Sickle_cell_disease

  Sickle cell disease occurs when a person inherits two abnormal copies of the -globin gene that makes haemoglobin, one from each parent. […] As of 2021, SCD is estimated to affect about 7.7 million people worldwide, directly causing an estimated 34,000 annual deaths and a contributory factor to a further 376,000 deaths. […] About 80% of sickle cell disease cases are believed to occur in Sub-Saharan Africa. […] The highest frequency of sickle cell disease is found in tropical regions, particularly sub-Saharan Africa, tribal regions of India, and the Middle East. […] Migration of substantial populations from these high-prevalence areas to low-prevalence countries in Europe has dramatically increased in recent decades and in some European countries, sickle cell disease has now overtaken more familiar genetic conditions such as haemophilia and cystic fibrosis.

• #3 Sickle Cell Disease – What Is Sickle Cell Disease? | NHLBI, NIH

  https://www.nhlbi.nih.gov/health/sickle-cell-disease

  Sickle cell disease affects more than 100,000 people in the United States and 8 million people worldwide. […] In the United States, 9 of 10 people who have sickle cell disease are of African ancestry or identify as Black: […] The NHLBI leads and supports research and clinical trials to find more transformative therapies for sickle cell disease.

• #4 Sickle Cell Disease (SCD): Practice Essentials, Background, Genetics

  https://emedicine.medscape.com/article/205926-overview

  SCD is present mostly in people of sub-Saharan African descent. It also is found, with much less frequency, in eastern Mediterranean and Middle East populations. Individuals of Central African Republic descent are at an increased risk for overt kidney failure. […] The sickle gene is present in approximately 8% of Black Americans. More than 2 million people in the United States, nearly all of them of African American ancestry, carry the sickle gene. The following statistics are available from the Centers for Disease Control and Prevention and the National Institutes of Health: In the United States, it is estimated that approximately 100,000 people have SCD. SCD occurs in about 1 out of every 365 Black or African-American births. SCD occurs in about 1 of every 16,300 Hispanic-American births. Approximately 1 in 13 Blacks or African Americans has sickle cell trait.

• #5 Sickle cell disease – Wikipedia

  https://en.wikipedia.org/wiki/Sickle_cell_disease

  Three-quarters of sickle cell cases occur in Africa. […] A WHO report dated 2006 estimated that around 2% of newborns in Nigeria were affected by sickle cell anaemia, giving a total of 150,000 affected children born every year in Nigeria alone. […] The number of people with the disease in the United States is about 100,000 (one in 3,300), mostly affecting Americans of sub-Saharan African descent. […] In the United States, about one out of 365 African-American children and one in every 16,300 Hispanic-American children have sickle cell anaemia. […] In France, sickle cell disease has become a major health problem, with an overall birth prevalence of one in 2,415 in mainland France. […] In Saudi Arabia, about 4.2% of the population carry the sickle cell trait and 0.26% have sickle cell disease.

• #6 Systematic Literature Review Shows Gaps in Data on Global Prevalence and Birth Prevalence of Sickle Cell Disease and Sickle Cell Trait: Call for Action to Scale Up and Harmonize Data Collection

  https://www.mdpi.com/2077-0383/12/17/5538

  Sickle cell disease (SCD) is an inherited monogenic disorder with high prevalence throughout sub-Saharan Africa, the Mediterranean basin, the Middle East, and India. […] Sources of SCD epidemiology remain scarce and fragmented. […] A systematic literature review (SLR) to identify peer-reviewed studies on SCD epidemiology was performed, with a search of bibliographic databases and key conference proceedings from 1 January 2010 to 25 March 2022 (congress abstracts after 2018). […] Prevalence was highest in Africa (~800/100,000), followed by the Middle East (~200/100,000) and India (~100/100,000), in contrast to ~30/100,000 in Europe. […] Birth prevalence was highest in Africa (~1000/100,000) and lowest in North America (~50/100,000) and Europe (~30/100,000). […] This SLR confirmed that sub-Saharan and North-East Africa, India, the Middle East, and the Caribbean islands are global SCD hotspots.

• #7 Sickle cell disease – Wikipedia

  https://en.wikipedia.org/wiki/Sickle_cell_disease

  Sickle cell disease occurs when a person inherits two abnormal copies of the -globin gene that makes haemoglobin, one from each parent. […] As of 2021, SCD is estimated to affect about 7.7 million people worldwide, directly causing an estimated 34,000 annual deaths and a contributory factor to a further 376,000 deaths. […] About 80% of sickle cell disease cases are believed to occur in Sub-Saharan Africa. […] The highest frequency of sickle cell disease is found in tropical regions, particularly sub-Saharan Africa, tribal regions of India, and the Middle East. […] Migration of substantial populations from these high-prevalence areas to low-prevalence countries in Europe has dramatically increased in recent decades and in some European countries, sickle cell disease has now overtaken more familiar genetic conditions such as haemophilia and cystic fibrosis.

• #8 A ten year review of the sickle cell program in Muhimbili National Hospital, Tanzania | BMC Hematology | Full Text

  https://bmchematol.biomedcentral.com/articles/10.1186/s12878-018-0125-0

  Africa has the highest burden of Sickle cell disease (SCD) but there are few large, systematic studies providing reliable descriptions of the disease spectrum. Tanzania, with 11,000 SCD births annually, established the Muhimbili Sickle Cell program aiming to improve understanding of SCD in Africa. […] The highest burden of sickle cell disease (SCD) is in Africa where up to 75% of the 300,000 global births of SCD per year occur and where childhood mortality remains high, ranging between 50 and 90%. […] Tanzania is amongst the 5 countries in the world with the highest estimated number of newborns with SCD a year. […] The World Health Organization (WHO) has formally classified SCD as a major public health problem and Tanzania, like other African countries has included SCD as a priority disease condition in their strategy for Non-Communicable Diseases (NCD).

• #9 Sickle cell disease – Wikipedia

  https://en.wikipedia.org/wiki/Sickle_cell_disease

  Sickle cell disease occurs when a person inherits two abnormal copies of the -globin gene that makes haemoglobin, one from each parent. […] As of 2021, SCD is estimated to affect about 7.7 million people worldwide, directly causing an estimated 34,000 annual deaths and a contributory factor to a further 376,000 deaths. […] About 80% of sickle cell disease cases are believed to occur in Sub-Saharan Africa. […] The highest frequency of sickle cell disease is found in tropical regions, particularly sub-Saharan Africa, tribal regions of India, and the Middle East. […] Migration of substantial populations from these high-prevalence areas to low-prevalence countries in Europe has dramatically increased in recent decades and in some European countries, sickle cell disease has now overtaken more familiar genetic conditions such as haemophilia and cystic fibrosis.

• #10 Sickle Cell Disease (SCD): Practice Essentials, Background, Genetics

  https://emedicine.medscape.com/article/205926-overview

  In several parts of Africa, the prevalence of sickle cell trait (heterozygosity) is as high as 30%. Although the disease is most frequently found in sub-Saharan Africa, it is also found in some regions of Sicily, Greece, southern Turkey, and India, all of which have areas in which malaria is endemic. The mutation that results in HbS is believed to have originated in several locations in Africa and India. Its prevalence varies but is high in these countries because of the survival advantage to heterozygotes in regions of endemic malaria. As a result of migration, both forced and voluntary, it is now found worldwide. […] The male-to-female ratio is 1:1. No sex predilection exists, since sickle cell anemia is not an X-linked disease. Although no particular gender predilection has been shown in most series, analysis of the data from the US Renal Data System demonstrated marked male predominance of sickle cell nephropathy in affected patients.

• #11 Sickle Cell Disease | WHO | Regional Office for Africa

  https://www.afro.who.int/health-topics/sickle-cell-disease

  Sickle cell disease (SCD), or sickle cell anaemia, is a major genetic disease that affects most countries in the African Region. […] In the Region, the majority of children with the most severe form of the disease die before the age of 5, usually from an infection or severe blood loss. In countries such as Cameroon, Republic of Congo, Gabon, Ghana and Nigeria the prevalence is between 20% to 30% while in some parts of Uganda it is as high as 45%. […] Sickle-cell disease predominates in Africa. […] The governing bodies of WHO have adopted two resolutions on haemoglobin disorders. The resolution on sickle-cell disease from the 59th World Health Assembly in May 2006 and the resolution on thalassaemia from the 118th meeting of the WHO Executive Board call upon affected countries and the Secretariat of WHO to strengthen their response to these conditions. […] WHO sickle package of interventions for sickle cell disease management.

• #12 Epidemiology of sickle cell disease in tribal population of Southern Rajasthan, India | Scientific Reports

  https://www.nature.com/articles/s41598-024-81224-1

  Sickle Cell Disease (SCD), a genetic condition, carries significant public health implications due to its impact on mortality, morbidity and quality of life. SCD is prevalent among tribal communities. […] The prevalence of SCT and SCD was 9.87% and 0.32%, respectively. The highest combined prevalence (including SCT and SCD) of 15.52% was observed among the Garasiya tribe, followed by the Bhil tribe with the prevalence of 9.68%. […] SCD is moderately prevalent, with less than a per cent among the tribal population of Southern Rajasthan. However, the prevalence of SCT is about 10%. […] The present study reports the prevalence of the HbS gene in the form of SCT and SCD among children and adults in some tribal communities of Rajasthan, India. The prevalence of SCD is notably low, at less than one per cent. However, the prevalence of SCT is remarkable, up to 15%.

• #13 Epidemiology of sickle cell disease in tribal population of Southern Rajasthan, India | Scientific Reports

  https://www.nature.com/articles/s41598-024-81224-1

  Sickle Cell Disease (SCD), a genetic condition, carries significant public health implications due to its impact on mortality, morbidity and quality of life. SCD is prevalent among tribal communities. […] The prevalence of SCT and SCD was 9.87% and 0.32%, respectively. The highest combined prevalence (including SCT and SCD) of 15.52% was observed among the Garasiya tribe, followed by the Bhil tribe with the prevalence of 9.68%. […] SCD is moderately prevalent, with less than a per cent among the tribal population of Southern Rajasthan. However, the prevalence of SCT is about 10%. […] The present study reports the prevalence of the HbS gene in the form of SCT and SCD among children and adults in some tribal communities of Rajasthan, India. The prevalence of SCD is notably low, at less than one per cent. However, the prevalence of SCT is remarkable, up to 15%.

• #14 Sickle cell disease – Wikipedia

  https://en.wikipedia.org/wiki/Sickle_cell_disease

  Three-quarters of sickle cell cases occur in Africa. […] A WHO report dated 2006 estimated that around 2% of newborns in Nigeria were affected by sickle cell anaemia, giving a total of 150,000 affected children born every year in Nigeria alone. […] The number of people with the disease in the United States is about 100,000 (one in 3,300), mostly affecting Americans of sub-Saharan African descent. […] In the United States, about one out of 365 African-American children and one in every 16,300 Hispanic-American children have sickle cell anaemia. […] In France, sickle cell disease has become a major health problem, with an overall birth prevalence of one in 2,415 in mainland France. […] In Saudi Arabia, about 4.2% of the population carry the sickle cell trait and 0.26% have sickle cell disease.

• #15 Sickle cell disease – Wikipedia

  https://en.wikipedia.org/wiki/Sickle_cell_disease

  Sickle cell disease is common in some ethnic groups of central India, where the prevalence has ranged from 9.4 to 22.2% in endemic areas of Madhya Pradesh, Rajasthan, and Chhattisgarh. […] In Jamaica, 10% of the population carry the sickle cell gene, making it the most prevalent genetic disorder in the country.

• #16 Data and Statistics on Sickle Cell Disease | Sickle Cell Disease (SCD) | CDC

  https://www.cdc.gov/sickle-cell/data/index.html

  Sickle cell disease (SCD) affects about 100,000 people in the United States; more than 90% are non-Hispanic Black or African American, and an estimated 3%9% are Hispanic or Latino. […] The exact number of people living with SCD in the United States is unknown. […] Two studies published in 2010 estimated that SCD affects approximately 100,000 people in the United States. […] More than 90% of people in the United States with SCD are non-Hispanic Black or African American (Black), and an estimated 3%9% are Hispanic or Latino. […] SCD occurs in about 1 out of every 365 Black or African American births and about 1 out of every 16,300 Hispanic American births. […] A CDC study of children and adolescents enrolled in Medicaid found that in 2019 less than half of children and adolescents received the recommended stroke screening. […] These findings highlight the ongoing gaps in health care for people with SCD and speak to the urgent need to address barriers to care.

• #17 Data and Statistics on Sickle Cell Disease | Sickle Cell Disease (SCD) | CDC

  https://www.cdc.gov/sickle-cell/data/index.html

  Sickle cell disease (SCD) affects about 100,000 people in the United States; more than 90% are non-Hispanic Black or African American, and an estimated 3%9% are Hispanic or Latino. […] The exact number of people living with SCD in the United States is unknown. […] Two studies published in 2010 estimated that SCD affects approximately 100,000 people in the United States. […] More than 90% of people in the United States with SCD are non-Hispanic Black or African American (Black), and an estimated 3%9% are Hispanic or Latino. […] SCD occurs in about 1 out of every 365 Black or African American births and about 1 out of every 16,300 Hispanic American births. […] A CDC study of children and adolescents enrolled in Medicaid found that in 2019 less than half of children and adolescents received the recommended stroke screening. […] These findings highlight the ongoing gaps in health care for people with SCD and speak to the urgent need to address barriers to care.

• #18 Sickle Cell Disease (SCD): Practice Essentials, Background, Genetics

  https://emedicine.medscape.com/article/205926-overview

  SCD is present mostly in people of sub-Saharan African descent. It also is found, with much less frequency, in eastern Mediterranean and Middle East populations. Individuals of Central African Republic descent are at an increased risk for overt kidney failure. […] The sickle gene is present in approximately 8% of Black Americans. More than 2 million people in the United States, nearly all of them of African American ancestry, carry the sickle gene. The following statistics are available from the Centers for Disease Control and Prevention and the National Institutes of Health: In the United States, it is estimated that approximately 100,000 people have SCD. SCD occurs in about 1 out of every 365 Black or African-American births. SCD occurs in about 1 of every 16,300 Hispanic-American births. Approximately 1 in 13 Blacks or African Americans has sickle cell trait.

• #19 Sickle Cell Disease (SCD): Practice Essentials, Background, Genetics

  https://emedicine.medscape.com/article/205926-overview

  SCD is present mostly in people of sub-Saharan African descent. It also is found, with much less frequency, in eastern Mediterranean and Middle East populations. Individuals of Central African Republic descent are at an increased risk for overt kidney failure. […] The sickle gene is present in approximately 8% of Black Americans. More than 2 million people in the United States, nearly all of them of African American ancestry, carry the sickle gene. The following statistics are available from the Centers for Disease Control and Prevention and the National Institutes of Health: In the United States, it is estimated that approximately 100,000 people have SCD. SCD occurs in about 1 out of every 365 Black or African-American births. SCD occurs in about 1 of every 16,300 Hispanic-American births. Approximately 1 in 13 Blacks or African Americans has sickle cell trait.

• #20 Sickle cell disease – Wikipedia

  https://en.wikipedia.org/wiki/Sickle_cell_disease

  Three-quarters of sickle cell cases occur in Africa. […] A WHO report dated 2006 estimated that around 2% of newborns in Nigeria were affected by sickle cell anaemia, giving a total of 150,000 affected children born every year in Nigeria alone. […] The number of people with the disease in the United States is about 100,000 (one in 3,300), mostly affecting Americans of sub-Saharan African descent. […] In the United States, about one out of 365 African-American children and one in every 16,300 Hispanic-American children have sickle cell anaemia. […] In France, sickle cell disease has become a major health problem, with an overall birth prevalence of one in 2,415 in mainland France. […] In Saudi Arabia, about 4.2% of the population carry the sickle cell trait and 0.26% have sickle cell disease.

• #21 Epidemiology and disease burden of sickle cell disease in France: A descriptive study based on a French nationwide claim database | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0253986

  Sickle cell disease (SCD) is a severe hematological disorder. The primary objective of this study was to estimate SCD prevalence in France. […] Our study estimates SCD prevalence in France at between 19,800 and 32,400 patients in 2016, higher than previously published. This study highlights the significant disease burden associated with vaso-occlusive events. […] Using the EGB data, we estimate that SCD prevalence in France is between 19,800 and 32,400 patients in 2016. […] This analysis confirms the significant burden of SCD, with patients presenting on average 1.51 to 1.90 VOC per year, including 0.40 to 0.63 hospitalized VOC. […] Despite these limitations, our results are consistent with previously reported data. VOC rates are consistent with reported rates. […] In conclusion, our study estimates that SCD prevalence in France in 2016 is between 19,800 and 32,400 patients, higher than previously published and one of the higher prevalence in Europe. It also confirms the heavy disease burden associated with SCD, with frequent and severe complications leading to a significant increase in healthcare use.

• #22 Surveillance for Sickle Cell Disease — Sickle Cell Data Collection Program, Two States, 2004–2018

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9552568/

  Sickle cell disease (SCD), an inherited blood disorder affecting an estimated 100,000 persons in the United States, is associated with multiple complications and reduced life expectancy. […] Population-based public health surveillance is critical to understanding the course and outcomes of SCD as well as the health care use, unmet health care needs, and gaps in essential services of the population affected by SCD. […] In 2015, CDC established the Sickle Cell Data Collection (SCDC) program to characterize the epidemiology of SCD in two states (California and Georgia). […] SCDC is a population-based tracking system that uses comprehensive data linkages in state health systems. […] As of 2021, the SCDC program had expanded to 11 states with the addition of Colorado and Wisconsin. […] During 20042018, the cumulative prevalence of confirmed and probable SCD cases identified in California and Georgia was 9,875 and 14,777 cases, respectively. […] A surveillance capacity and performance assessment of all 11 SCDC states during 20202021 indicated that states differed in the availability of data sources used for SCD surveillance and the time frames for accessing each state data source.

• #23 Surveillance for Sickle Cell Disease — Sickle Cell Data Collection Program, Two States, 2004–2018

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9552568/

  Sickle cell disease (SCD), an inherited blood disorder affecting an estimated 100,000 persons in the United States, is associated with multiple complications and reduced life expectancy. […] Population-based public health surveillance is critical to understanding the course and outcomes of SCD as well as the health care use, unmet health care needs, and gaps in essential services of the population affected by SCD. […] In 2015, CDC established the Sickle Cell Data Collection (SCDC) program to characterize the epidemiology of SCD in two states (California and Georgia). […] SCDC is a population-based tracking system that uses comprehensive data linkages in state health systems. […] As of 2021, the SCDC program had expanded to 11 states with the addition of Colorado and Wisconsin. […] During 20042018, the cumulative prevalence of confirmed and probable SCD cases identified in California and Georgia was 9,875 and 14,777 cases, respectively. […] A surveillance capacity and performance assessment of all 11 SCDC states during 20202021 indicated that states differed in the availability of data sources used for SCD surveillance and the time frames for accessing each state data source.

• #24 Surveillance for Sickle Cell Disease — Sickle Cell Data Collection Program, Two States, 2004–2018

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9552568/

  Sickle cell disease (SCD), an inherited blood disorder affecting an estimated 100,000 persons in the United States, is associated with multiple complications and reduced life expectancy. […] Population-based public health surveillance is critical to understanding the course and outcomes of SCD as well as the health care use, unmet health care needs, and gaps in essential services of the population affected by SCD. […] In 2015, CDC established the Sickle Cell Data Collection (SCDC) program to characterize the epidemiology of SCD in two states (California and Georgia). […] SCDC is a population-based tracking system that uses comprehensive data linkages in state health systems. […] As of 2021, the SCDC program had expanded to 11 states with the addition of Colorado and Wisconsin. […] During 20042018, the cumulative prevalence of confirmed and probable SCD cases identified in California and Georgia was 9,875 and 14,777 cases, respectively. […] A surveillance capacity and performance assessment of all 11 SCDC states during 20202021 indicated that states differed in the availability of data sources used for SCD surveillance and the time frames for accessing each state data source.

• #25 Surveillance for Sickle Cell Disease — Sickle Cell Data Collection Program, Two States, 2004–2018

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9552568/

  Sickle cell disease (SCD), an inherited blood disorder affecting an estimated 100,000 persons in the United States, is associated with multiple complications and reduced life expectancy. […] Population-based public health surveillance is critical to understanding the course and outcomes of SCD as well as the health care use, unmet health care needs, and gaps in essential services of the population affected by SCD. […] In 2015, CDC established the Sickle Cell Data Collection (SCDC) program to characterize the epidemiology of SCD in two states (California and Georgia). […] SCDC is a population-based tracking system that uses comprehensive data linkages in state health systems. […] As of 2021, the SCDC program had expanded to 11 states with the addition of Colorado and Wisconsin. […] During 20042018, the cumulative prevalence of confirmed and probable SCD cases identified in California and Georgia was 9,875 and 14,777 cases, respectively. […] A surveillance capacity and performance assessment of all 11 SCDC states during 20202021 indicated that states differed in the availability of data sources used for SCD surveillance and the time frames for accessing each state data source.

• #26 3 Screening, Registries, and Surveillance | Addressing Sickle Cell Disease: A Strategic Plan and Blueprint for Action | The National Academies Press

  https://nap.nationalacademies.org/read/25632/chapter/5

  The most successful example of the collection and use of population-wide data on SCD is NBS for SCD and SCT in the United States, which is now universal across all 50 states and the District of Columbia. […] Registries and surveillance systems have been in development for SCD for some time, and they show great promise for capturing much needed information about those living with the disease, their care, and their outcomes. […] Public health surveillance of SCD in the United States is typically conducted by state governments. […] The creation of the Registry and Surveillance System for Hemoglobinopathies (RuSH), a cooperative agreement among CDC, NHLBI, and seven states with significant populations of people living with SCD and thalassemia, was one outcome of this summit and its recommendations.

• #27 3 Screening, Registries, and Surveillance | Addressing Sickle Cell Disease: A Strategic Plan and Blueprint for Action | The National Academies Press

  https://nap.nationalacademies.org/read/25632/chapter/5

  The most successful example of the collection and use of population-wide data on SCD is NBS for SCD and SCT in the United States, which is now universal across all 50 states and the District of Columbia. […] Registries and surveillance systems have been in development for SCD for some time, and they show great promise for capturing much needed information about those living with the disease, their care, and their outcomes. […] Public health surveillance of SCD in the United States is typically conducted by state governments. […] The creation of the Registry and Surveillance System for Hemoglobinopathies (RuSH), a cooperative agreement among CDC, NHLBI, and seven states with significant populations of people living with SCD and thalassemia, was one outcome of this summit and its recommendations.

• #28 An overview of sickle cell disease from the socio-demographic triangle – a Nigerian single-institution retrospective study

  https://www.panafrican-med-journal.com/content/article/41/161/full/

  SCD is a hereditary red blood cell disorder of public health importance globally with Nigeria the epicenter zone in Africa. […] The objective of this study was to examine the prevalence patterns of SCD, including other associated epidemiologic and hematological parameters from laboratory data. […] A total of 138 (1.63%) newly diagnosed SCD patients aged 7 months to 41 years made up of 39% (0.63% SCD prevalence) adults and 61% (1% SCD prevalence) pediatric age-groups were seen out of 8457 consecutive patients screened within the study period. […] The pediatric department recorded the highest proportion of SCD (65%). […] The prevalence of SCD within the states in Nigeria ranges from 1%-3%. […] Nigeria accounts for 100,000-150,000 newborns living with SCD annually (33% of the global burden of SCD).

• #29 An overview of sickle cell disease from the socio-demographic triangle – a Nigerian single-institution retrospective study

  https://www.panafrican-med-journal.com/content/article/41/161/full/

  SCD is a hereditary red blood cell disorder of public health importance globally with Nigeria the epicenter zone in Africa. […] The objective of this study was to examine the prevalence patterns of SCD, including other associated epidemiologic and hematological parameters from laboratory data. […] A total of 138 (1.63%) newly diagnosed SCD patients aged 7 months to 41 years made up of 39% (0.63% SCD prevalence) adults and 61% (1% SCD prevalence) pediatric age-groups were seen out of 8457 consecutive patients screened within the study period. […] The pediatric department recorded the highest proportion of SCD (65%). […] The prevalence of SCD within the states in Nigeria ranges from 1%-3%. […] Nigeria accounts for 100,000-150,000 newborns living with SCD annually (33% of the global burden of SCD).

• #30 An overview of sickle cell disease from the socio-demographic triangle – a Nigerian single-institution retrospective study

  https://www.panafrican-med-journal.com/content/article/41/161/full/

  The study revealed a gender disparity in the months of SCD diagnosis. However, there was no statistical difference in the pediatric and adult SCD prevalence patterns. […] The socio-demographic profile of people living with SCD is a strong predictor of the clinical course and outcome of the disease. […] A SCD prevalence of 1.63% was in keeping with the national prevalence range of 1%-3% in Nigeria. […] The two hemoglobin variants SS and SC were the commonest SCD hemoglobin variants found in Nigeria. […] The overall mean age of 14 years clearly depicts that SCD is still predominated by pediatric age status. […] The study showed that the proportion of SCD among the pediatric age group was not statistically significant higher than the adult counterpart. […] A 1% prevalence from public health department shows that the level of awareness of SCD in the environment is still very low. […] Late diagnosis of SCD is a major public health challenge in Nigeria. […] Health illiteracy, poor legislation, late detection and predominance of homozygous sickle-hemoglobin variant are predictive markers of poor prognostic outcome of SCD.

• #31 A ten year review of the sickle cell program in Muhimbili National Hospital, Tanzania | BMC Hematology | Full Text

  https://bmchematol.biomedcentral.com/articles/10.1186/s12878-018-0125-0

  Africa has the highest burden of Sickle cell disease (SCD) but there are few large, systematic studies providing reliable descriptions of the disease spectrum. Tanzania, with 11,000 SCD births annually, established the Muhimbili Sickle Cell program aiming to improve understanding of SCD in Africa. […] The highest burden of sickle cell disease (SCD) is in Africa where up to 75% of the 300,000 global births of SCD per year occur and where childhood mortality remains high, ranging between 50 and 90%. […] Tanzania is amongst the 5 countries in the world with the highest estimated number of newborns with SCD a year. […] The World Health Organization (WHO) has formally classified SCD as a major public health problem and Tanzania, like other African countries has included SCD as a priority disease condition in their strategy for Non-Communicable Diseases (NCD).

• #32 Sickle Cell Disease Epidemiology and Statistics in Tanzania – African Institute for Mathematical Sciences

  https://nexteinstein.org/sickle-cell-disease-epidemiology-and-statistics-in-tanzania/

  Sickle cell disease (SCD) encompasses a group of genetic blood disorders which have a high prevalence in Tanzania. […] The Muhimbili sickle cohort (MSC), established in 2003 and maintained since then, consists of approximately 10,000 patients in Dar es Salaam. […] Approximately 5,300 of patients in the cohort are confirmed SCD patients. […] Managing patients’ clinical follow-up is crucially important since frequent drop-out events disturb the fight against SCD complications. […] This study aims to describe the loss to follow-up (LTF) pattern among patients attending the Muhimbili hospital SCD clinic. […] The results of this study will impact on improving the health of SCD patients.

• #33 JMIR Public Health and Surveillance – The Prevalence of Sickle Cell Disease in Colorado and Methodologies of the Colorado Sickle Cell Data Collection Program: Public Health Surveillance Study

  https://publichealth.jmir.org/2024/1/e64995

  Conclusions: The CO-SCDC program successfully established the capacity to perform SCD surveillance and, in doing so, identified baseline prevalence estimates for SCD in Colorado. […] Future efforts will expand surveillance to include claims data to better capture health care use and address potential underreporting. […] The crude prevalence of SCD in CO in 2019 was 7.6 per 100,000 residents. […] The birth prevalence of SCD in CO during a 5-year period also increased from 11.9 per 100,000 live births between 2010 and 2014 to 20.1 per 100,000 live births between 2015 and 2019, equivalent to 1 in 4974 live births. […] The increase in birth prevalence supports the population growth theory as SCD births in a 5-year period increased by 67% (n=26) between 2010 and 2019, likely due to the increase of the Black or African American population in CO and the 11.7% (n=2059) increase in the birth rate among this population in a 5-year period between 2010 and 2019. […] This suggests that the population of individuals with SCD in Colorado is growing faster than previously estimated, highlighting the need for increased resource allocation to support this population.

• #34 JMIR Public Health and Surveillance – The Prevalence of Sickle Cell Disease in Colorado and Methodologies of the Colorado Sickle Cell Data Collection Program: Public Health Surveillance Study

  https://publichealth.jmir.org/2024/1/e64995

  Conclusions: The CO-SCDC program successfully established the capacity to perform SCD surveillance and, in doing so, identified baseline prevalence estimates for SCD in Colorado. […] Future efforts will expand surveillance to include claims data to better capture health care use and address potential underreporting. […] The crude prevalence of SCD in CO in 2019 was 7.6 per 100,000 residents. […] The birth prevalence of SCD in CO during a 5-year period also increased from 11.9 per 100,000 live births between 2010 and 2014 to 20.1 per 100,000 live births between 2015 and 2019, equivalent to 1 in 4974 live births. […] The increase in birth prevalence supports the population growth theory as SCD births in a 5-year period increased by 67% (n=26) between 2010 and 2019, likely due to the increase of the Black or African American population in CO and the 11.7% (n=2059) increase in the birth rate among this population in a 5-year period between 2010 and 2019. […] This suggests that the population of individuals with SCD in Colorado is growing faster than previously estimated, highlighting the need for increased resource allocation to support this population.

• #35 Sickle Cell Anemia: History and Epidemiology | SpringerLink

  https://link.springer.com/chapter/10.1007/978-3-319-06713-1_2

  This chapter summarizes how a simple point mutation in the human genome has evolved to become a global public health problem, as well as a remarkable example of evolutionary biology, population genetics and clinical epidemiology. […] Sickle cell anemia is a severe disease that is still associated with a high mortality in low- and middle-income countries, where simple public health interventions could help significantly in reducing its long-term health burden, and with high health-care costs in high-income countries, where life expectancy and quality of life remain suboptimal. […] Alongside huge progress in the understanding of the natural history and epidemiology of sickle cell anemia during the last century, significant gaps, discussed in this chapter, still remain, highlighting the need for further research to better prevent the adverse consequences of this disease.

• #36 Using Public Health Surveillance to Improve Outcomes among People with Sickle Cell Disease | Duke University School of Nursing

  https://nursing.duke.edu/news/using-public-health-surveillance-improve-outcomes-among-people-sickle-cell-disease

  NC SCDC is a state-level sickle cell disease (SCD) surveillance program that addresses the limited knowledge about the epidemiology of SCD in NC and the U.S. and generates actionable data to improve health outcomes for historically marginalized people with SCD. […] The lack of a national surveillance registry remains a major limiting factor to addressing healthcare needs and health inequities for people with SCD in the U.S. To address this gap, the Centers for Disease Control and Prevention is funding state-level surveillance programs. […] The NC SCDC program aims to generate and disseminate surveillance findings informing clinical practice, research, and policy at state and national levels. […] The exact number of people living with SCD remains unknown. Whereas reliable and comprehensive data are essential for addressing the challenges of SCD. Policy makers and different organizations need data on the prevalence of SCD in specific communities, healthcare utilization patterns, the number of children born with SCD, where births occur, and the quality of care received by people with SCD. […] This is where the NC SCDC can make significant impact- implementing statewide surveillance and disseminate aggregate level findings regarding the epidemiology and healthcare utilization patterns of SCD in the state, Kayle said.

• #37 Data and Statistics on Sickle Cell Disease | Sickle Cell Disease (SCD) | CDC

  https://www.cdc.gov/sickle-cell/data/index.html

  Sickle cell disease (SCD) affects about 100,000 people in the United States; more than 90% are non-Hispanic Black or African American, and an estimated 3%9% are Hispanic or Latino. […] The exact number of people living with SCD in the United States is unknown. […] Two studies published in 2010 estimated that SCD affects approximately 100,000 people in the United States. […] More than 90% of people in the United States with SCD are non-Hispanic Black or African American (Black), and an estimated 3%9% are Hispanic or Latino. […] SCD occurs in about 1 out of every 365 Black or African American births and about 1 out of every 16,300 Hispanic American births. […] A CDC study of children and adolescents enrolled in Medicaid found that in 2019 less than half of children and adolescents received the recommended stroke screening. […] These findings highlight the ongoing gaps in health care for people with SCD and speak to the urgent need to address barriers to care.

• #38 Using Public Health Surveillance to Improve Outcomes among People with Sickle Cell Disease | Duke University School of Nursing

  https://nursing.duke.edu/news/using-public-health-surveillance-improve-outcomes-among-people-sickle-cell-disease

  NC SCDC is a state-level sickle cell disease (SCD) surveillance program that addresses the limited knowledge about the epidemiology of SCD in NC and the U.S. and generates actionable data to improve health outcomes for historically marginalized people with SCD. […] The lack of a national surveillance registry remains a major limiting factor to addressing healthcare needs and health inequities for people with SCD in the U.S. To address this gap, the Centers for Disease Control and Prevention is funding state-level surveillance programs. […] The NC SCDC program aims to generate and disseminate surveillance findings informing clinical practice, research, and policy at state and national levels. […] The exact number of people living with SCD remains unknown. Whereas reliable and comprehensive data are essential for addressing the challenges of SCD. Policy makers and different organizations need data on the prevalence of SCD in specific communities, healthcare utilization patterns, the number of children born with SCD, where births occur, and the quality of care received by people with SCD. […] This is where the NC SCDC can make significant impact- implementing statewide surveillance and disseminate aggregate level findings regarding the epidemiology and healthcare utilization patterns of SCD in the state, Kayle said.

• #39 Systematic Literature Review Shows Gaps in Data on Global Prevalence and Birth Prevalence of Sickle Cell Disease and Sickle Cell Trait: Call for Action to Scale Up and Harmonize Data Collection

  https://www.mdpi.com/2077-0383/12/17/5538

  The identified data were limited due to gaps in the published literature for large parts of the world population; the inconsistent reporting of SCD genotypes, diagnostic criteria, and settings; and a sparsity of peer-reviewed publications from countries with assumed high prevalence. […] This SLR demonstrated a lack of systematic knowledge and a need to provide uniform data collection on SCD prevalence and mortality. […] The objective of this study was to perform an SLR of recent publications, from 2010 to 2022, on the prevalence, birth prevalence, and mortality associated with SCD globally and regionally, and to identify any knowledge gaps. […] The birth prevalence of SCD was reported in this study was ~800/100,000, and the birth prevalence of SCT was ~13,000/100,000. […] The current SLR revealed important gaps in the published literature, with missing data on prevalence and/or birth prevalence from countries where SCD is believed to be highly prevalent, including African countries such as Cameroon and Mozambique.

• #40 Systematic Literature Review Shows Gaps in Data on Global Prevalence and Birth Prevalence of Sickle Cell Disease and Sickle Cell Trait: Call for Action to Scale Up and Harmonize Data Collection

  https://www.mdpi.com/2077-0383/12/17/5538

  The identified data were limited due to gaps in the published literature for large parts of the world population; the inconsistent reporting of SCD genotypes, diagnostic criteria, and settings; and a sparsity of peer-reviewed publications from countries with assumed high prevalence. […] This SLR demonstrated a lack of systematic knowledge and a need to provide uniform data collection on SCD prevalence and mortality. […] The objective of this study was to perform an SLR of recent publications, from 2010 to 2022, on the prevalence, birth prevalence, and mortality associated with SCD globally and regionally, and to identify any knowledge gaps. […] The birth prevalence of SCD was reported in this study was ~800/100,000, and the birth prevalence of SCT was ~13,000/100,000. […] The current SLR revealed important gaps in the published literature, with missing data on prevalence and/or birth prevalence from countries where SCD is believed to be highly prevalent, including African countries such as Cameroon and Mozambique.

• #41 CDC’s Sickle Cell Disease Surveillance History | CDC

  https://archive.cdc.gov/www_cdc_gov/ncbddd/hemoglobinopathies/surveillance-history.html

  The Centers for Disease Control and Prevention (CDC) and participating states began sickle cell disease (SCD) surveillance (monitoring) in 2010. SCD surveillance involves collecting information on diagnoses, treatment, and healthcare access for people with SCD in the United States. […] CDC coordinated these efforts as part of the two projects outlined below. […] To identify and collect data on people living with SCD or thalassemia in the participating states […] To evaluate and validate data collected during RuSH and to share findings from the project […] To identify and collect data on people living with SCD in the state. […] Population based surveillance in sickle cell disease: methods, findings and implications from the California registry and surveillance system in hemoglobinopathies project (RuSH). […] State-based surveillance for selected hemoglobinopathies.

• #42 CDC’s Sickle Cell Disease Surveillance History | CDC

  https://archive.cdc.gov/www_cdc_gov/ncbddd/hemoglobinopathies/surveillance-history.html

  The Centers for Disease Control and Prevention (CDC) and participating states began sickle cell disease (SCD) surveillance (monitoring) in 2010. SCD surveillance involves collecting information on diagnoses, treatment, and healthcare access for people with SCD in the United States. […] CDC coordinated these efforts as part of the two projects outlined below. […] To identify and collect data on people living with SCD or thalassemia in the participating states […] To evaluate and validate data collected during RuSH and to share findings from the project […] To identify and collect data on people living with SCD in the state. […] Population based surveillance in sickle cell disease: methods, findings and implications from the California registry and surveillance system in hemoglobinopathies project (RuSH). […] State-based surveillance for selected hemoglobinopathies.

• #43 Surveillance for Sickle Cell Disease — Sickle Cell Data Collection Program, Two States, 2004–2018

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9552568/

  Sickle cell disease (SCD), an inherited blood disorder affecting an estimated 100,000 persons in the United States, is associated with multiple complications and reduced life expectancy. […] Population-based public health surveillance is critical to understanding the course and outcomes of SCD as well as the health care use, unmet health care needs, and gaps in essential services of the population affected by SCD. […] In 2015, CDC established the Sickle Cell Data Collection (SCDC) program to characterize the epidemiology of SCD in two states (California and Georgia). […] SCDC is a population-based tracking system that uses comprehensive data linkages in state health systems. […] As of 2021, the SCDC program had expanded to 11 states with the addition of Colorado and Wisconsin. […] During 20042018, the cumulative prevalence of confirmed and probable SCD cases identified in California and Georgia was 9,875 and 14,777 cases, respectively. […] A surveillance capacity and performance assessment of all 11 SCDC states during 20202021 indicated that states differed in the availability of data sources used for SCD surveillance and the time frames for accessing each state data source.

• #44 Surveillance for Sickle Cell Disease — Sickle Cell Data Collection Program, Two States, 2004–2018

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9552568/

  Sickle cell disease (SCD), an inherited blood disorder affecting an estimated 100,000 persons in the United States, is associated with multiple complications and reduced life expectancy. […] Population-based public health surveillance is critical to understanding the course and outcomes of SCD as well as the health care use, unmet health care needs, and gaps in essential services of the population affected by SCD. […] In 2015, CDC established the Sickle Cell Data Collection (SCDC) program to characterize the epidemiology of SCD in two states (California and Georgia). […] SCDC is a population-based tracking system that uses comprehensive data linkages in state health systems. […] As of 2021, the SCDC program had expanded to 11 states with the addition of Colorado and Wisconsin. […] During 20042018, the cumulative prevalence of confirmed and probable SCD cases identified in California and Georgia was 9,875 and 14,777 cases, respectively. […] A surveillance capacity and performance assessment of all 11 SCDC states during 20202021 indicated that states differed in the availability of data sources used for SCD surveillance and the time frames for accessing each state data source.

• #45 Surveillance for Sickle Cell Disease — Sickle Cell Data Collection Program, Two States, 2004–2018

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9552568/

  Sickle cell disease (SCD), an inherited blood disorder affecting an estimated 100,000 persons in the United States, is associated with multiple complications and reduced life expectancy. […] Population-based public health surveillance is critical to understanding the course and outcomes of SCD as well as the health care use, unmet health care needs, and gaps in essential services of the population affected by SCD. […] In 2015, CDC established the Sickle Cell Data Collection (SCDC) program to characterize the epidemiology of SCD in two states (California and Georgia). […] SCDC is a population-based tracking system that uses comprehensive data linkages in state health systems. […] As of 2021, the SCDC program had expanded to 11 states with the addition of Colorado and Wisconsin. […] During 20042018, the cumulative prevalence of confirmed and probable SCD cases identified in California and Georgia was 9,875 and 14,777 cases, respectively. […] A surveillance capacity and performance assessment of all 11 SCDC states during 20202021 indicated that states differed in the availability of data sources used for SCD surveillance and the time frames for accessing each state data source.

• #46 Sickle Cell Data Collection (SCDC) Program | Texas DSHS

  https://www.dshs.texas.gov/environmental-epidemiology-disease-registries-section-eedrs/Sickle-Cell-Data-Collection

  In 2023, the Texas Department of State Health Services (DSHS) applied for and received funding from the Centers for Disease Control and Prevention (CDC) to establish a state sickle cell data collection system that informs sickle cell practices and policies in Texas. Texas SCDC is one of 16 states funded by the CDC. […] Texas SCDC serves as the foundation for measuring sickle cell burden in Texas by assessing long-term trends in sickle cell disease diagnosis, treatment, and healthcare access. In the absence of population-based surveillance, Texas SCDC provides information on all individuals with sickle cell disease in Texas, regardless of age, insurance status, or geography. […] The goal of Texas SCDC is to collect, maintain, and disseminate high quality sickle cell data that will contribute to improving diagnoses, treatments, survival, and quality of life for all individuals with sickle cell disease in Texas.

• #47 Sickle Cell Data Collection (SCDC) Program | Texas DSHS

  https://www.dshs.texas.gov/environmental-epidemiology-disease-registries-section-eedrs/Sickle-Cell-Data-Collection

  In 2023, the Texas Department of State Health Services (DSHS) applied for and received funding from the Centers for Disease Control and Prevention (CDC) to establish a state sickle cell data collection system that informs sickle cell practices and policies in Texas. Texas SCDC is one of 16 states funded by the CDC. […] Texas SCDC serves as the foundation for measuring sickle cell burden in Texas by assessing long-term trends in sickle cell disease diagnosis, treatment, and healthcare access. In the absence of population-based surveillance, Texas SCDC provides information on all individuals with sickle cell disease in Texas, regardless of age, insurance status, or geography. […] The goal of Texas SCDC is to collect, maintain, and disseminate high quality sickle cell data that will contribute to improving diagnoses, treatments, survival, and quality of life for all individuals with sickle cell disease in Texas.

• #48 Azthena logo with the word Azthena

  https://www.news-medical.net/news/20190927/CDC-to-fund-sickle-cell-disease-surveillance.aspx

  The Centers for Disease Control and Prevention (CDC) has announced a funding of $1.2 million to different states in order to collect data and information from people suffering from sickle cell disease. […] The program called the Sickle Cell Data Collection program or SCDC is at present functional in two states – California and Georgia. […] With the new funds coming in, nine states would be able to collect and send population based information on the health of persons living with sickle cell disease (SCD). […] According to the CDC website, the funds would be used to collect information regarding the disease and also frame a plan to collect unique data and conduct in depth analysis. […] The program statement says that it would help improve the health outcomes in people with sickle cell disease.

• #49 Azthena logo with the word Azthena

  https://www.news-medical.net/news/20190927/CDC-to-fund-sickle-cell-disease-surveillance.aspx

  The Centers for Disease Control and Prevention (CDC) has announced a funding of $1.2 million to different states in order to collect data and information from people suffering from sickle cell disease. […] The program called the Sickle Cell Data Collection program or SCDC is at present functional in two states – California and Georgia. […] With the new funds coming in, nine states would be able to collect and send population based information on the health of persons living with sickle cell disease (SCD). […] According to the CDC website, the funds would be used to collect information regarding the disease and also frame a plan to collect unique data and conduct in depth analysis. […] The program statement says that it would help improve the health outcomes in people with sickle cell disease.

• #50 Michigan Sickle Cell Data Collection (MiSCDC) Program | CHEAR

  https://chear.org/research/projects/MiSCDC

  The Michigan Sickle Cell Data Collection (MiSCDC) Program is a strategic collaboration led by the Susan B. Meister Child Health Evaluation and Research (CHEAR) Center at the University of Michigan and the Michigan Department of Health and Human Services (MDHHS). Through this collaboration, we ensure the implementation of sickle cell disease surveillance in Michigan and the successful collection and submission of aggregate sickle cell disease surveillance data to the CDC. […] While all SCD cases are identified at birth, there is no ongoing national surveillance of SCD. This results in significant knowledge gaps about the natural history of the disease. […] In the absence of population-based surveillance, significant improvements in the health of the SCD community will remain elusive. […] The overall purpose of the MiSCDC Program is to foster elimination of health disparities among those with SCD in Michigan through participation in the Sickle Cell Data Collection Program.

• #51 Michigan Sickle Cell Data Collection (MiSCDC) Program | CHEAR

  https://chear.org/research/projects/MiSCDC

  The Michigan Sickle Cell Data Collection (MiSCDC) Program is a strategic collaboration led by the Susan B. Meister Child Health Evaluation and Research (CHEAR) Center at the University of Michigan and the Michigan Department of Health and Human Services (MDHHS). Through this collaboration, we ensure the implementation of sickle cell disease surveillance in Michigan and the successful collection and submission of aggregate sickle cell disease surveillance data to the CDC. […] While all SCD cases are identified at birth, there is no ongoing national surveillance of SCD. This results in significant knowledge gaps about the natural history of the disease. […] In the absence of population-based surveillance, significant improvements in the health of the SCD community will remain elusive. […] The overall purpose of the MiSCDC Program is to foster elimination of health disparities among those with SCD in Michigan through participation in the Sickle Cell Data Collection Program.

• #52 Epidemiology of Sickle Cell Disease in Michigan, 2020 – Michigan Sickle Cell Data Collection

  https://www.miscdc.org/miscdc-comm/latta-mpha-2024/

  Sickle cell disease (SCD) is a lifelong hemoglobinopathy associated with serious health complications, including pain crises and stroke. The Michigan Sickle Cell Data Collection (MiSCDC) program is a CDC-funded, population-based surveillance system led by MDHHS and the CHEAR Center at the University of Michigan. MiSCDC combines data from multiple sources to understand population trends and healthcare utilization of persons living with SCD in Michigan. […] The objective is to describe the prevalence, demographics, healthcare utilization, and mortality of people living with SCD in Michigan. […] We identified 4,010 total persons (58% female) living in Michigan in 2020. […] MiSCDC can track trends in the epidemiology and healthcare utilization of people living with SCD in Michigan using linked data sources. These findings can be applied to understand the impacts of COVID-19 on this population as well as evaluate disease burden and identify policy targets aimed at improving the quality of life for persons living with SCD.

• #53 Epidemiology of Sickle Cell Disease in Michigan, 2020 – Michigan Sickle Cell Data Collection

  https://www.miscdc.org/miscdc-comm/latta-mpha-2024/

  Sickle cell disease (SCD) is a lifelong hemoglobinopathy associated with serious health complications, including pain crises and stroke. The Michigan Sickle Cell Data Collection (MiSCDC) program is a CDC-funded, population-based surveillance system led by MDHHS and the CHEAR Center at the University of Michigan. MiSCDC combines data from multiple sources to understand population trends and healthcare utilization of persons living with SCD in Michigan. […] The objective is to describe the prevalence, demographics, healthcare utilization, and mortality of people living with SCD in Michigan. […] We identified 4,010 total persons (58% female) living in Michigan in 2020. […] MiSCDC can track trends in the epidemiology and healthcare utilization of people living with SCD in Michigan using linked data sources. These findings can be applied to understand the impacts of COVID-19 on this population as well as evaluate disease burden and identify policy targets aimed at improving the quality of life for persons living with SCD.

• #54 North Carolina Sickle Cell Data Collection Program – Using surveillance to improve the lives of North Carolinians with Sickle Cell Disease.

  https://sites.duke.edu/ncscdc/

  Using surveillance to improve the lives of North Carolinians with Sickle Cell Disease. […] The North Carolina Sickle Cell Data Collection (NC SCDC) program implements state-wide sickle cell disease surveillance in NC and disseminates aggregate-level surveillance findings regarding the epidemiology and healthcare utilization patterns of sickle cell disease in the state.

• #55 Faculty Collaboration Database – Surveillance for the Rare Condition of Sickle Cell Disease in Wisconsin. WMJ 2022 Dec;121(4):297-300

  https://fcd.mcw.edu/?search/showPublication/id/2068501

  INTRODUCTION: Despite universal newborn screening, there is no comprehensive surveillance system to understand the sickle cell disease population in Wisconsin. […] METHODS: We initiated the development of a sickle cell disease surveillance system by linking newborn screening data and electronic health records from 2 large tertiary health care institutions in Wisconsin: Children’s Wisconsin and Froedtert Hospital. […] RESULTS: There were 1478 individuals within the 3 data sources. One hundred thirty-two (82%) of 159 identified by newborn screening from 2013 through 2019 received care at Children’s Wisconsin. The majority of individuals with sickle cell disease at Children’s Wisconsin and Froedtert Hospital resided in Milwaukee County. […] DISCUSSION: The new surveillance program will increase our understanding of the sickle cell disease population in Wisconsin and help improve quality of care and health outcomes.

• #56 Faculty Collaboration Database – Surveillance for the Rare Condition of Sickle Cell Disease in Wisconsin. WMJ 2022 Dec;121(4):297-300

  https://fcd.mcw.edu/?search/showPublication/id/2068501

  INTRODUCTION: Despite universal newborn screening, there is no comprehensive surveillance system to understand the sickle cell disease population in Wisconsin. […] METHODS: We initiated the development of a sickle cell disease surveillance system by linking newborn screening data and electronic health records from 2 large tertiary health care institutions in Wisconsin: Children’s Wisconsin and Froedtert Hospital. […] RESULTS: There were 1478 individuals within the 3 data sources. One hundred thirty-two (82%) of 159 identified by newborn screening from 2013 through 2019 received care at Children’s Wisconsin. The majority of individuals with sickle cell disease at Children’s Wisconsin and Froedtert Hospital resided in Milwaukee County. […] DISCUSSION: The new surveillance program will increase our understanding of the sickle cell disease population in Wisconsin and help improve quality of care and health outcomes.

• #57 CDC Releases Updated Sickle Cell Surveillance Summary

  https://www.hcplive.com/view/cdc-releases-updated-sickle-cell-surveillance-summary

  Analyses like the Sickle Cell Data Collection (SCDC) have played prominent roles in establishing new SCD clinics, educating health care providers, guiding new research initiatives, and developing state health care policies. […] The US Centers for Disease Control and Prevention (CDC) shared the first comprehensive description of its collaborative efforts to establish, maintain, and expand sickle cell disease (SCD) surveillance in order to improve health outcomes for individuals living with the rare disease. […] According to the report, a critical strategy to understanding the course and outcomes of sickle cell disease as well as the health care use, unmet care needs, and the gaps present in essential services, requires population-based public health surveillance of those affected by sickle cell disease.

• #58 CDC Releases Updated Sickle Cell Surveillance Summary

  https://www.hcplive.com/view/cdc-releases-updated-sickle-cell-surveillance-summary

  A key takeaway identified in the report was the need for more sickle cell disease specialty clinics in the participating states of California and Georgia. […] „Public health officials can use this report as a guiding framework to plan or implement surveillance programs for persons with SCD. Both data-related activities and the administrative considerations are crucial to the success of these programs,” the report stated. […] Before SCDC was established in 2015, there were 2 short-term projects conducted: Registry and Surveillance System for Hemoglobinopathies (RuSH) and Public Health Research, Epidemiology, and Surveillance for Hemoglobinopathies (PHRESH). […] By utilizing comprehensive data linkages in state health systems, the SCDC operates as a population-based tracking system that enables the synthesization and dissemination of longitudinal data.

• #59 Georgia’s SCD surveillance program gets fresh support from CDC | Sickle Cell Disease NewsEnvelope icon

  https://sicklecellanemianews.com/news/georgias-scd-surveillance-program-gets-fresh-support-from-cdc/

  To do that, the program works with teams in several states, collecting data from newborn screening and population and healthcare utilization data. […] Previous findings from the SCDC program in Georgia and California highlighted a need for more SCD specialty clinics. […] The surveillance data that was obtained has helped open new SCD clinics in underserved areas, educate healthcare providers, develop state healthcare policies, and guide new research initiatives.

• #60 Common Data Infrastructure Feasible, Useful for Sickle Cell Disease Surveillance – Georgia Health Policy Center

  https://ghpc.gsu.edu/2023/06/02/common-data-infrastructure-feasible-useful-for-sickle-cell-disease-surveillance/

  A common informatics infrastructure is feasible to standardize processes and data comparisons for sickle cell disease across states, according to a study recently published by Sickle Cell Data Collection Program researchers in JAMIA Open. […] The Sickle Cell Data Collection Program (SCDC) is funded by the Centers for Disease Control and Prevention (CDC) and is currently active in 11 states. […] The Core Surveillance Data Instrument of the Common Data Model for sickle cell disease was piloted in Tennessee, North Carolina, and Michigan. […] Not only does this common data model strengthen our networks ability to analyze data across state sickle cell disease populations, but it also provides a blueprint for expanding sickle cell data collection or establishing similar public health surveillance efforts for other rare diseases, says GHPC senior research associate and coauthor Brandon Attell.

• #61 Common Data Infrastructure Feasible, Useful for Sickle Cell Disease Surveillance – Georgia Health Policy Center

  https://ghpc.gsu.edu/2023/06/02/common-data-infrastructure-feasible-useful-for-sickle-cell-disease-surveillance/

  A common informatics infrastructure is feasible to standardize processes and data comparisons for sickle cell disease across states, according to a study recently published by Sickle Cell Data Collection Program researchers in JAMIA Open. […] The Sickle Cell Data Collection Program (SCDC) is funded by the Centers for Disease Control and Prevention (CDC) and is currently active in 11 states. […] The Core Surveillance Data Instrument of the Common Data Model for sickle cell disease was piloted in Tennessee, North Carolina, and Michigan. […] Not only does this common data model strengthen our networks ability to analyze data across state sickle cell disease populations, but it also provides a blueprint for expanding sickle cell data collection or establishing similar public health surveillance efforts for other rare diseases, says GHPC senior research associate and coauthor Brandon Attell.

• #62 Common Data Infrastructure Feasible, Useful for Sickle Cell Disease Surveillance – Georgia Health Policy Center

  https://ghpc.gsu.edu/2023/06/02/common-data-infrastructure-feasible-useful-for-sickle-cell-disease-surveillance/

  A common informatics infrastructure is feasible to standardize processes and data comparisons for sickle cell disease across states, according to a study recently published by Sickle Cell Data Collection Program researchers in JAMIA Open. […] The Sickle Cell Data Collection Program (SCDC) is funded by the Centers for Disease Control and Prevention (CDC) and is currently active in 11 states. […] The Core Surveillance Data Instrument of the Common Data Model for sickle cell disease was piloted in Tennessee, North Carolina, and Michigan. […] Not only does this common data model strengthen our networks ability to analyze data across state sickle cell disease populations, but it also provides a blueprint for expanding sickle cell data collection or establishing similar public health surveillance efforts for other rare diseases, says GHPC senior research associate and coauthor Brandon Attell.

• #63 Surveillance for Sickle Cell Disease — Sickle Cell Data Collection Program, Two States, 2004–2018

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9552568/

  Sickle cell disease (SCD), an inherited blood disorder affecting an estimated 100,000 persons in the United States, is associated with multiple complications and reduced life expectancy. […] Population-based public health surveillance is critical to understanding the course and outcomes of SCD as well as the health care use, unmet health care needs, and gaps in essential services of the population affected by SCD. […] In 2015, CDC established the Sickle Cell Data Collection (SCDC) program to characterize the epidemiology of SCD in two states (California and Georgia). […] SCDC is a population-based tracking system that uses comprehensive data linkages in state health systems. […] As of 2021, the SCDC program had expanded to 11 states with the addition of Colorado and Wisconsin. […] During 20042018, the cumulative prevalence of confirmed and probable SCD cases identified in California and Georgia was 9,875 and 14,777 cases, respectively. […] A surveillance capacity and performance assessment of all 11 SCDC states during 20202021 indicated that states differed in the availability of data sources used for SCD surveillance and the time frames for accessing each state data source.

• #64 Surveillance for Sickle Cell Disease — Sickle Cell Data Collection Program, Two States, 2004–2018

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9552568/

  This report is the first comprehensive description of CDCs efforts in collaboration with participating states to establish, maintain, and expand SCD surveillance through the SCDC program to improve health outcomes for persons living with SCD. […] Public health officials can use this report as a guiding framework to plan or implement surveillance programs for persons with SCD.

• #65 CDC Releases Updated Sickle Cell Surveillance Summary

  https://www.hcplive.com/view/cdc-releases-updated-sickle-cell-surveillance-summary

  A key takeaway identified in the report was the need for more sickle cell disease specialty clinics in the participating states of California and Georgia. […] „Public health officials can use this report as a guiding framework to plan or implement surveillance programs for persons with SCD. Both data-related activities and the administrative considerations are crucial to the success of these programs,” the report stated. […] Before SCDC was established in 2015, there were 2 short-term projects conducted: Registry and Surveillance System for Hemoglobinopathies (RuSH) and Public Health Research, Epidemiology, and Surveillance for Hemoglobinopathies (PHRESH). […] By utilizing comprehensive data linkages in state health systems, the SCDC operates as a population-based tracking system that enables the synthesization and dissemination of longitudinal data.

• #66 Michigan Sickle Cell Data Collection (MiSCDC) Program | CHEAR

  https://chear.org/research/projects/MiSCDC

  The Michigan Sickle Cell Data Collection (MiSCDC) Program is a strategic collaboration led by the Susan B. Meister Child Health Evaluation and Research (CHEAR) Center at the University of Michigan and the Michigan Department of Health and Human Services (MDHHS). Through this collaboration, we ensure the implementation of sickle cell disease surveillance in Michigan and the successful collection and submission of aggregate sickle cell disease surveillance data to the CDC. […] While all SCD cases are identified at birth, there is no ongoing national surveillance of SCD. This results in significant knowledge gaps about the natural history of the disease. […] In the absence of population-based surveillance, significant improvements in the health of the SCD community will remain elusive. […] The overall purpose of the MiSCDC Program is to foster elimination of health disparities among those with SCD in Michigan through participation in the Sickle Cell Data Collection Program.

• #67 Michigan Sickle Cell Data Collection (MiSCDC) Program | CHEAR

  https://chear.org/research/projects/MiSCDC

  The Michigan Sickle Cell Data Collection (MiSCDC) Program is a strategic collaboration led by the Susan B. Meister Child Health Evaluation and Research (CHEAR) Center at the University of Michigan and the Michigan Department of Health and Human Services (MDHHS). Through this collaboration, we ensure the implementation of sickle cell disease surveillance in Michigan and the successful collection and submission of aggregate sickle cell disease surveillance data to the CDC. […] While all SCD cases are identified at birth, there is no ongoing national surveillance of SCD. This results in significant knowledge gaps about the natural history of the disease. […] In the absence of population-based surveillance, significant improvements in the health of the SCD community will remain elusive. […] The overall purpose of the MiSCDC Program is to foster elimination of health disparities among those with SCD in Michigan through participation in the Sickle Cell Data Collection Program.

• #68 Michigan Sickle Cell Data Collection (MiSCDC) Program | CHEAR

  https://chear.org/research/projects/MiSCDC

  increase standardization of methods for SCD surveillance; […] increase understanding of the incidence and prevalence of SCD; […] increase understanding of the demographics of individuals with SCD; […] increase understanding of healthcare utilization patterns for individuals with SCD; […] improve availability of aggregate level surveillance data for utilization in health care policy making decisions; and […] increase understanding of the epidemiology of SCD among patients, family members, health care providers, and policy makers.

• #69 Sickle Cell Disease | WHO | Regional Office for Africa

  https://www.afro.who.int/health-topics/sickle-cell-disease

  Sickle cell disease (SCD), or sickle cell anaemia, is a major genetic disease that affects most countries in the African Region. […] In the Region, the majority of children with the most severe form of the disease die before the age of 5, usually from an infection or severe blood loss. In countries such as Cameroon, Republic of Congo, Gabon, Ghana and Nigeria the prevalence is between 20% to 30% while in some parts of Uganda it is as high as 45%. […] Sickle-cell disease predominates in Africa. […] The governing bodies of WHO have adopted two resolutions on haemoglobin disorders. The resolution on sickle-cell disease from the 59th World Health Assembly in May 2006 and the resolution on thalassaemia from the 118th meeting of the WHO Executive Board call upon affected countries and the Secretariat of WHO to strengthen their response to these conditions. […] WHO sickle package of interventions for sickle cell disease management.

• #70 Frontiers | Sickle cell disease: a comparative perspective on global and national initiatives

  https://www.frontiersin.org/journals/hematology/articles/10.3389/frhem.2024.1457158/full

  In Nigeria, India, and the Democratic Republic of the Congo, up to 2% of the population has SCD, with sickle cell trait prevalence of 10% to 30%. These countries host 90% of the world’s SCD population, with 150,000 infants born with SCD annually in Nigeria. […] The WHO’s guidance frameworks for SCD in Africa and globally aim to enhance care through strategic policies and advocacy. The SICKLE technical package integrates interventions, education, and community empowerment. These frameworks emphasize early diagnosis through newborn screening, public education, and improved access to healthcare services. According to McGann et al., early diagnosis and intervention have resulted in decreased mortality rates among children with SCD in several African countries. The WHO’s efforts have also led to the establishment of national programs that enhance the availability of comprehensive care, including vaccination, prophylactic antibiotics, and pain management strategies.

• #71 Frontiers | Sickle cell disease: a comparative perspective on global and national initiatives

  https://www.frontiersin.org/journals/hematology/articles/10.3389/frhem.2024.1457158/full

  In Nigeria, India, and the Democratic Republic of the Congo, up to 2% of the population has SCD, with sickle cell trait prevalence of 10% to 30%. These countries host 90% of the world’s SCD population, with 150,000 infants born with SCD annually in Nigeria. […] The WHO’s guidance frameworks for SCD in Africa and globally aim to enhance care through strategic policies and advocacy. The SICKLE technical package integrates interventions, education, and community empowerment. These frameworks emphasize early diagnosis through newborn screening, public education, and improved access to healthcare services. According to McGann et al., early diagnosis and intervention have resulted in decreased mortality rates among children with SCD in several African countries. The WHO’s efforts have also led to the establishment of national programs that enhance the availability of comprehensive care, including vaccination, prophylactic antibiotics, and pain management strategies.

• #72 Frontiers | Sickle cell disease: a comparative perspective on global and national initiatives

  https://www.frontiersin.org/journals/hematology/articles/10.3389/frhem.2024.1457158/full

  In Nigeria, India, and the Democratic Republic of the Congo, up to 2% of the population has SCD, with sickle cell trait prevalence of 10% to 30%. These countries host 90% of the world’s SCD population, with 150,000 infants born with SCD annually in Nigeria. […] The WHO’s guidance frameworks for SCD in Africa and globally aim to enhance care through strategic policies and advocacy. The SICKLE technical package integrates interventions, education, and community empowerment. These frameworks emphasize early diagnosis through newborn screening, public education, and improved access to healthcare services. According to McGann et al., early diagnosis and intervention have resulted in decreased mortality rates among children with SCD in several African countries. The WHO’s efforts have also led to the establishment of national programs that enhance the availability of comprehensive care, including vaccination, prophylactic antibiotics, and pain management strategies.

• #73 Frontiers | Sickle cell disease: a comparative perspective on global and national initiatives

  https://www.frontiersin.org/journals/hematology/articles/10.3389/frhem.2024.1457158/full

  In Nigeria, India, and the Democratic Republic of the Congo, up to 2% of the population has SCD, with sickle cell trait prevalence of 10% to 30%. These countries host 90% of the world’s SCD population, with 150,000 infants born with SCD annually in Nigeria. […] The WHO’s guidance frameworks for SCD in Africa and globally aim to enhance care through strategic policies and advocacy. The SICKLE technical package integrates interventions, education, and community empowerment. These frameworks emphasize early diagnosis through newborn screening, public education, and improved access to healthcare services. According to McGann et al., early diagnosis and intervention have resulted in decreased mortality rates among children with SCD in several African countries. The WHO’s efforts have also led to the establishment of national programs that enhance the availability of comprehensive care, including vaccination, prophylactic antibiotics, and pain management strategies.

• #74 Frontiers | Sickle cell disease: a comparative perspective on global and national initiatives

  https://www.frontiersin.org/journals/hematology/articles/10.3389/frhem.2024.1457158/full

  Nigeria’s National Sickle Cell Disease Control Program has made substantial strides in improving the management and outcomes of sickle cell disease (SCD). This program focuses on comprehensive strategies, including early screening, public education, and enhanced healthcare access. A study found that the program’s newborn screening initiatives have significantly improved early diagnosis and treatment, leading to better clinical outcomes and reduced mortality rates among children with SCD in Nigeria. […] The National Sickle Cell Anaemia Elimination Mission, launched by the Prime Minister of India on July 1, 2023, in Shahdol, Madhya Pradesh, integrates with existing National Health Mission mechanisms to optimize resources and avoid duplication of efforts. The National Sickle Cell Elimination Mission is an ambitious healthcare initiative aimed at eradicating sickle cell disease through comprehensive screening, early diagnosis, and advanced treatment protocols.

• #75 Frontiers | Sickle cell disease: a comparative perspective on global and national initiatives

  https://www.frontiersin.org/journals/hematology/articles/10.3389/frhem.2024.1457158/full

  Nigeria’s National Sickle Cell Disease Control Program has made substantial strides in improving the management and outcomes of sickle cell disease (SCD). This program focuses on comprehensive strategies, including early screening, public education, and enhanced healthcare access. A study found that the program’s newborn screening initiatives have significantly improved early diagnosis and treatment, leading to better clinical outcomes and reduced mortality rates among children with SCD in Nigeria. […] The National Sickle Cell Anaemia Elimination Mission, launched by the Prime Minister of India on July 1, 2023, in Shahdol, Madhya Pradesh, integrates with existing National Health Mission mechanisms to optimize resources and avoid duplication of efforts. The National Sickle Cell Elimination Mission is an ambitious healthcare initiative aimed at eradicating sickle cell disease through comprehensive screening, early diagnosis, and advanced treatment protocols.

• #76 Frontiers | Sickle cell disease: a comparative perspective on global and national initiatives

  https://www.frontiersin.org/journals/hematology/articles/10.3389/frhem.2024.1457158/full

  Nigeria’s National Sickle Cell Disease Control Program has made substantial strides in improving the management and outcomes of sickle cell disease (SCD). This program focuses on comprehensive strategies, including early screening, public education, and enhanced healthcare access. A study found that the program’s newborn screening initiatives have significantly improved early diagnosis and treatment, leading to better clinical outcomes and reduced mortality rates among children with SCD in Nigeria. […] The National Sickle Cell Anaemia Elimination Mission, launched by the Prime Minister of India on July 1, 2023, in Shahdol, Madhya Pradesh, integrates with existing National Health Mission mechanisms to optimize resources and avoid duplication of efforts. The National Sickle Cell Elimination Mission is an ambitious healthcare initiative aimed at eradicating sickle cell disease through comprehensive screening, early diagnosis, and advanced treatment protocols.

• #77 Frontiers | Sickle cell disease: a comparative perspective on global and national initiatives

  https://www.frontiersin.org/journals/hematology/articles/10.3389/frhem.2024.1457158/full

  Nigeria’s National Sickle Cell Disease Control Program has made substantial strides in improving the management and outcomes of sickle cell disease (SCD). This program focuses on comprehensive strategies, including early screening, public education, and enhanced healthcare access. A study found that the program’s newborn screening initiatives have significantly improved early diagnosis and treatment, leading to better clinical outcomes and reduced mortality rates among children with SCD in Nigeria. […] The National Sickle Cell Anaemia Elimination Mission, launched by the Prime Minister of India on July 1, 2023, in Shahdol, Madhya Pradesh, integrates with existing National Health Mission mechanisms to optimize resources and avoid duplication of efforts. The National Sickle Cell Elimination Mission is an ambitious healthcare initiative aimed at eradicating sickle cell disease through comprehensive screening, early diagnosis, and advanced treatment protocols.

• #78 Frontiers | Sickle cell disease: a comparative perspective on global and national initiatives

  https://www.frontiersin.org/journals/hematology/articles/10.3389/frhem.2024.1457158/full

  Nigeria’s National Sickle Cell Disease Control Program has made substantial strides in improving the management and outcomes of sickle cell disease (SCD). This program focuses on comprehensive strategies, including early screening, public education, and enhanced healthcare access. A study found that the program’s newborn screening initiatives have significantly improved early diagnosis and treatment, leading to better clinical outcomes and reduced mortality rates among children with SCD in Nigeria. […] The National Sickle Cell Anaemia Elimination Mission, launched by the Prime Minister of India on July 1, 2023, in Shahdol, Madhya Pradesh, integrates with existing National Health Mission mechanisms to optimize resources and avoid duplication of efforts. The National Sickle Cell Elimination Mission is an ambitious healthcare initiative aimed at eradicating sickle cell disease through comprehensive screening, early diagnosis, and advanced treatment protocols.

• #79 Sickle cell disease: a neglected chronic disease of increasing global health importance | Archives of Disease in Childhood

  https://adc.bmj.com/content/100/1/48

  Even today, the majority of those born with the disease in Africa, where more than 80% of affected births occur, die undiagnosed in early childhood, presumably from preventable causes that include invasive bacterial diseases, malaria and severe acute anaemia. […] Because so little is known about the clinical course and natural history of SCD in resource-poor countries, the majority of this review is focused on data from Europe and North America, where most detailed studies have been conducted. […] A programme of universal screening for SCD was implemented in England in 2001 and was subsequently rolled out to Scotland and Wales through which approximately 300 births and 17000 carriers are detected each year. […] The recent UK National Confidential Enquiry into Patient Outcome and Death for haemoglobinopathies revealed a significant inequity of specialist care in the country and the lack of adequate knowledge of haemoglobinopathies within the medical community, and recommended the establishment of a national database to capture information regarding prevalence, therapy and adverse events of SCD.

• #80 Sickle cell disease: a neglected chronic disease of increasing global health importance | Archives of Disease in Childhood

  https://adc.bmj.com/content/100/1/48

  Even today, the majority of those born with the disease in Africa, where more than 80% of affected births occur, die undiagnosed in early childhood, presumably from preventable causes that include invasive bacterial diseases, malaria and severe acute anaemia. […] Because so little is known about the clinical course and natural history of SCD in resource-poor countries, the majority of this review is focused on data from Europe and North America, where most detailed studies have been conducted. […] A programme of universal screening for SCD was implemented in England in 2001 and was subsequently rolled out to Scotland and Wales through which approximately 300 births and 17000 carriers are detected each year. […] The recent UK National Confidential Enquiry into Patient Outcome and Death for haemoglobinopathies revealed a significant inequity of specialist care in the country and the lack of adequate knowledge of haemoglobinopathies within the medical community, and recommended the establishment of a national database to capture information regarding prevalence, therapy and adverse events of SCD.

• #81 Frontiers | Sickle cell disease: a comparative perspective on global and national initiatives

  https://www.frontiersin.org/journals/hematology/articles/10.3389/frhem.2024.1457158/full

  Globally and nationally, implementing a sickle cell disease (SCD) prevention program presents a complex array of challenges, encompassing medical, social, economic, and infrastructural aspects. One of the primary obstacles is the need for widespread genetic screening and counseling, which necessitates substantial financial investment and robust healthcare infrastructure—resources often lacking in regions where SCD is most prevalent, such as sub-Saharan Africa and parts of India. […] Apart from this, inequality in research funding for sickle cell disease (SCD) starkly contrasts between the Global North and Global South. Despite the majority of SCD sufferers residing in sub-Saharan Africa and parts of Asia, most research funding and resources are concentrated in high-income countries of the Global North.

• #82 Frontiers | Sickle cell disease: a comparative perspective on global and national initiatives

  https://www.frontiersin.org/journals/hematology/articles/10.3389/frhem.2024.1457158/full

  Globally and nationally, implementing a sickle cell disease (SCD) prevention program presents a complex array of challenges, encompassing medical, social, economic, and infrastructural aspects. One of the primary obstacles is the need for widespread genetic screening and counseling, which necessitates substantial financial investment and robust healthcare infrastructure—resources often lacking in regions where SCD is most prevalent, such as sub-Saharan Africa and parts of India. […] Apart from this, inequality in research funding for sickle cell disease (SCD) starkly contrasts between the Global North and Global South. Despite the majority of SCD sufferers residing in sub-Saharan Africa and parts of Asia, most research funding and resources are concentrated in high-income countries of the Global North.

• #83 Frontiers | Sickle cell disease: a comparative perspective on global and national initiatives

  https://www.frontiersin.org/journals/hematology/articles/10.3389/frhem.2024.1457158/full

  Globally and nationally, implementing a sickle cell disease (SCD) prevention program presents a complex array of challenges, encompassing medical, social, economic, and infrastructural aspects. One of the primary obstacles is the need for widespread genetic screening and counseling, which necessitates substantial financial investment and robust healthcare infrastructure—resources often lacking in regions where SCD is most prevalent, such as sub-Saharan Africa and parts of India. […] Apart from this, inequality in research funding for sickle cell disease (SCD) starkly contrasts between the Global North and Global South. Despite the majority of SCD sufferers residing in sub-Saharan Africa and parts of Asia, most research funding and resources are concentrated in high-income countries of the Global North.

• #84 Frontiers | Sickle cell disease: a comparative perspective on global and national initiatives

  https://www.frontiersin.org/journals/hematology/articles/10.3389/frhem.2024.1457158/full

  Globally and nationally, implementing a sickle cell disease (SCD) prevention program presents a complex array of challenges, encompassing medical, social, economic, and infrastructural aspects. One of the primary obstacles is the need for widespread genetic screening and counseling, which necessitates substantial financial investment and robust healthcare infrastructure—resources often lacking in regions where SCD is most prevalent, such as sub-Saharan Africa and parts of India. […] Apart from this, inequality in research funding for sickle cell disease (SCD) starkly contrasts between the Global North and Global South. Despite the majority of SCD sufferers residing in sub-Saharan Africa and parts of Asia, most research funding and resources are concentrated in high-income countries of the Global North.

• #85 Frontiers | Sickle cell disease: a comparative perspective on global and national initiatives

  https://www.frontiersin.org/journals/hematology/articles/10.3389/frhem.2024.1457158/full

  Future research avenues for sickle cell disease (SCD) prevention are multifaceted and highly promising. A key area of focus is the advancement of gene editing technologies, such as CRISPR-Cas9, which have the potential to correct the genetic mutation responsible for SCD at its source, offering a permanent cure. Research into safer and more efficient delivery methods for these gene therapies is critical to ensure their accessibility and effectiveness.

• #86 Frontiers | Sickle cell disease: a comparative perspective on global and national initiatives

  https://www.frontiersin.org/journals/hematology/articles/10.3389/frhem.2024.1457158/full

  Future research avenues for sickle cell disease (SCD) prevention are multifaceted and highly promising. A key area of focus is the advancement of gene editing technologies, such as CRISPR-Cas9, which have the potential to correct the genetic mutation responsible for SCD at its source, offering a permanent cure. Research into safer and more efficient delivery methods for these gene therapies is critical to ensure their accessibility and effectiveness.

• #87 Frontiers | Sickle cell disease: a comparative perspective on global and national initiatives

  https://www.frontiersin.org/journals/hematology/articles/10.3389/frhem.2024.1457158/full

  Future research avenues for sickle cell disease (SCD) prevention are multifaceted and highly promising. A key area of focus is the advancement of gene editing technologies, such as CRISPR-Cas9, which have the potential to correct the genetic mutation responsible for SCD at its source, offering a permanent cure. Research into safer and more efficient delivery methods for these gene therapies is critical to ensure their accessibility and effectiveness.

• #88 Sickle Cell Disease and Other Heritable Blood Disorders Research, Surveillance, Prevention, and Treatment Act of 2018 (S.2465) Passes – AAEM

  https://www.aaem.org/news/sickle-cell-disease-and-other-heritable-blood-disorders-research-surveillance-prevention-and-treatment-act-of-2018-s-2465-passes/

  On July 18, 2018, the Sickle Cell Disease Coalition sent a letter to the Senators Lamar Alexander and Patty Murray with other members of the Senate HELP Committee copied. AAEM signed on to the letter as part of the Coalition and supported Senators Alexander and Murry in their work to pass S. 2465. The bill officially passed in the Senate on December 11, 2018 and was signed by the President on December 18, 2018. […] The Sickle Cell Disease Research, Surveillance, Prevention and Treatment Act of 2018 is bipartisan legislation introduced by Senators Tim Scott (R-SC) and Cory Booker (D-NJ). […] Authorizes a SCD surveillance program.

• #89 Sickle Cell Disease and Other Heritable Blood Disorders Research, Surveillance, Prevention, and Treatment Act of 2018 (S.2465) Passes – AAEM

  https://www.aaem.org/news/sickle-cell-disease-and-other-heritable-blood-disorders-research-surveillance-prevention-and-treatment-act-of-2018-s-2465-passes/

  On July 18, 2018, the Sickle Cell Disease Coalition sent a letter to the Senators Lamar Alexander and Patty Murray with other members of the Senate HELP Committee copied. AAEM signed on to the letter as part of the Coalition and supported Senators Alexander and Murry in their work to pass S. 2465. The bill officially passed in the Senate on December 11, 2018 and was signed by the President on December 18, 2018. […] The Sickle Cell Disease Research, Surveillance, Prevention and Treatment Act of 2018 is bipartisan legislation introduced by Senators Tim Scott (R-SC) and Cory Booker (D-NJ). […] Authorizes a SCD surveillance program.

• #90 H.R. 3884, Sickle Cell Disease and Other Heritable Blood Disorders Research, Surveillance, Prevention, and Treatment Act of 2023 | Congressional Budget Office

  https://www.cbo.gov/publication/59857

  H.R. 3884 would authorize the appropriation of $8 million each year from 2024 through 2028 for the Centers for Disease Control and Prevention and the Health Resources and Services Administration to conduct research, surveillance, prevention, and treatment of sickle cell disease and related blood disorders. […] Based on historical spending patterns for those activities and assuming the appropriation of the authorized amounts, CBO estimates that implementing the bill would cost $37 million over the 2024-2028 period and $4 million after 2028.

• #91 Surveillance for Sickle Cell Disease — Sickle Cell Data Collection Program, Two States, 2004–2018

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9552568/

  Sickle cell disease (SCD), an inherited blood disorder affecting an estimated 100,000 persons in the United States, is associated with multiple complications and reduced life expectancy. […] Population-based public health surveillance is critical to understanding the course and outcomes of SCD as well as the health care use, unmet health care needs, and gaps in essential services of the population affected by SCD. […] In 2015, CDC established the Sickle Cell Data Collection (SCDC) program to characterize the epidemiology of SCD in two states (California and Georgia). […] SCDC is a population-based tracking system that uses comprehensive data linkages in state health systems. […] As of 2021, the SCDC program had expanded to 11 states with the addition of Colorado and Wisconsin. […] During 20042018, the cumulative prevalence of confirmed and probable SCD cases identified in California and Georgia was 9,875 and 14,777 cases, respectively. […] A surveillance capacity and performance assessment of all 11 SCDC states during 20202021 indicated that states differed in the availability of data sources used for SCD surveillance and the time frames for accessing each state data source.

• #92 A ten year review of the sickle cell program in Muhimbili National Hospital, Tanzania | BMC Hematology | Full Text

  https://bmchematol.biomedcentral.com/articles/10.1186/s12878-018-0125-0

  Africa has the highest burden of Sickle cell disease (SCD) but there are few large, systematic studies providing reliable descriptions of the disease spectrum. Tanzania, with 11,000 SCD births annually, established the Muhimbili Sickle Cell program aiming to improve understanding of SCD in Africa. […] The highest burden of sickle cell disease (SCD) is in Africa where up to 75% of the 300,000 global births of SCD per year occur and where childhood mortality remains high, ranging between 50 and 90%. […] Tanzania is amongst the 5 countries in the world with the highest estimated number of newborns with SCD a year. […] The World Health Organization (WHO) has formally classified SCD as a major public health problem and Tanzania, like other African countries has included SCD as a priority disease condition in their strategy for Non-Communicable Diseases (NCD).

• #93 JMIR Public Health and Surveillance – Prevalence, Mortality, and Access to Care for Chronic Kidney Disease in Medicaid-Enrolled Adults With Sickle Cell Disease in California: Retrospective Cohort Study

  https://publichealth.jmir.org/2024/1/e57290/

  Chronic kidney disease (CKD) is a significant complication in patients with sickle cell disease (SCD), leading to increased mortality. […] This study aims to investigate the burden of CKD in Medicaid-enrolled adults with SCD in California, examine differences in disease burden between male and female individuals, and assess mortality rates and access to specialized care. […] Among the 2345 adults with SCD, 24.4% (n=572) met the case definition for CKD. […] During this study, of the 2345 adults, 435 (18.5%) deaths occurred, predominantly within the SCD-CKD cohort (226/435, 39.5%). […] This study provides robust estimates of CKD prevalence and mortality among Medicaid-enrolled adults with SCD in California. […] The findings highlight the need for improved access to specialized care for this population and increased awareness of the high mortality risk and progression associated with CKD.

• #94 JMIR Public Health and Surveillance – Prevalence, Mortality, and Access to Care for Chronic Kidney Disease in Medicaid-Enrolled Adults With Sickle Cell Disease in California: Retrospective Cohort Study

  https://publichealth.jmir.org/2024/1/e57290/

  Chronic kidney disease (CKD) is a significant complication in patients with sickle cell disease (SCD), leading to increased mortality. […] This study aims to investigate the burden of CKD in Medicaid-enrolled adults with SCD in California, examine differences in disease burden between male and female individuals, and assess mortality rates and access to specialized care. […] Among the 2345 adults with SCD, 24.4% (n=572) met the case definition for CKD. […] During this study, of the 2345 adults, 435 (18.5%) deaths occurred, predominantly within the SCD-CKD cohort (226/435, 39.5%). […] This study provides robust estimates of CKD prevalence and mortality among Medicaid-enrolled adults with SCD in California. […] The findings highlight the need for improved access to specialized care for this population and increased awareness of the high mortality risk and progression associated with CKD.

• #95 Sickle Cell Disease (SCD): Practice Essentials, Background, Genetics

  https://emedicine.medscape.com/article/205926-overview

  Risk of chronic kidney disease (CKD) is increased in SCD, with one study showing an almost 30% prevalence of baseline CKD in a cohort with mean age 31.6 years, increasing to 41.8% over 5 years. Among US sickle cell trait carriers of African or Hispanic ancestry, the risk of CKD is about 1.5-2fold higher than in noncarriers. The prevalence of sickle cell trait is twice as high in African Americans with end-stage kidney disease compared with the general African-American population (15% versus 7%, P 0.001). Certain gene variants have been linked with increased risk of kidney disease in SCD. In particular, patients who carry APOL1 G1 and G2 risk variants (which confer protection against trypanosomiasis) have a 7-fold higher risk of CKD progression and a 7-30fold greater risk of kidney failure.

• #96

  https://journals.lww.com/jaht/fulltext/2020/11010/epidemiology_and_characteristics_of_sickle_cell.3.aspx

  Sickle cell anemia (SCA) is an autosomal recessive illness caused by the formation of abnormal hemoglobin S. […] This study aimed to describe the epidemiology and characteristics of sickle cell patients admitted to hospitals in Jazan region. […] SCA was the number one cause of admissions in this study. Approximately, one out of each four patients admitted to Jazan hospitals is a sickle cell patient. […] SCA is the most common hemoglobinopathy globally. […] In Saudi Arabia, it is most prevalent in Jazan region after the eastern region. […] More than one-fifth of all patients’ admissions during 1 month were of sickle cell patients. […] The most frequent cause of admission was VOC, followed by ACS. […] The most common complications of SCA were VOC and ACS, as reported in many studies. […] Further studies to explore the burden of SCD in Jazan region, consanguinity status after the application of premarital screening and the reasons behind the proceeding of high-risk marriages are needed.

• #97

  https://journals.lww.com/jaht/fulltext/2020/11010/epidemiology_and_characteristics_of_sickle_cell.3.aspx

  Sickle cell anemia (SCA) is an autosomal recessive illness caused by the formation of abnormal hemoglobin S. […] This study aimed to describe the epidemiology and characteristics of sickle cell patients admitted to hospitals in Jazan region. […] SCA was the number one cause of admissions in this study. Approximately, one out of each four patients admitted to Jazan hospitals is a sickle cell patient. […] SCA is the most common hemoglobinopathy globally. […] In Saudi Arabia, it is most prevalent in Jazan region after the eastern region. […] More than one-fifth of all patients’ admissions during 1 month were of sickle cell patients. […] The most frequent cause of admission was VOC, followed by ACS. […] The most common complications of SCA were VOC and ACS, as reported in many studies. […] Further studies to explore the burden of SCD in Jazan region, consanguinity status after the application of premarital screening and the reasons behind the proceeding of high-risk marriages are needed.

• #98 Epidemiology of Stroke in Sickle Cell Disease

  https://www.mdpi.com/2077-0383/10/18/4232

  Sickle cell disease is the most common cause of stroke in childhood, both ischaemic and haemorrhagic, and it also affects adults with the condition. Without any screening or preventative treatment, the incidence appears to fall within the range 0.5 to 0.9 per 100 patient years of observation. […] Newborn screening with Penicillin prophylaxis and vaccination leading to reduced bacterial infection may have reduced the incidence, alongside increasing hydroxyurea prescription. Transcranial Doppler screening and prophylactic chronic transfusion for at least an initial year has reduced the incidence of stroke by up to 10-fold in children with time averaged mean of the maximum velocity >200 cm/s. […] The prevention of haemorrhagic stroke at all ages and ischaemic stroke in adults has not yet received the same degree of attention.

• #99 Epidemiology of Stroke in Sickle Cell Disease

  https://www.mdpi.com/2077-0383/10/18/4232

  Sickle cell disease is the most common cause of stroke in childhood, both ischaemic and haemorrhagic, and it also affects adults with the condition. Without any screening or preventative treatment, the incidence appears to fall within the range 0.5 to 0.9 per 100 patient years of observation. […] Newborn screening with Penicillin prophylaxis and vaccination leading to reduced bacterial infection may have reduced the incidence, alongside increasing hydroxyurea prescription. Transcranial Doppler screening and prophylactic chronic transfusion for at least an initial year has reduced the incidence of stroke by up to 10-fold in children with time averaged mean of the maximum velocity >200 cm/s. […] The prevention of haemorrhagic stroke at all ages and ischaemic stroke in adults has not yet received the same degree of attention.

• #100 Global, regional, and national prevalence and mortality burden of sickle cell disease, 2000–2021 | Institute for Health Metrics and Evaluation

  https://www.healthdata.org/research-analysis/library/global-regional-and-national-prevalence-and-mortality-burden-sickle-cell

  We estimated 34400 (sickle cell) cause-specific all-age deaths globally in 2021, but total sickle cell disease mortality burden was nearly 11-times higher at 376000. […] Completed as part of the Global Burden of Diseases, Injuries, and Risk Factors Study (GBD) 2021, this study delivers a comprehensive global assessment of prevalence of sickle cell disease and mortality burden by age and sex for 204 countries and territories from 2000 to 2021. […] We estimated cause-specific sickle cell disease mortality using standardised GBD approaches, in which each death is assigned to a single underlying cause, to estimate mortality rates from the International Classification of Diseases (ICD)-coded vital registration, surveillance, and verbal autopsy data. […] Our findings show a strikingly high contribution of sickle cell disease to all-cause mortality that is not apparent when each death is assigned to only a single cause.

• #101 Sickle cell disease – Wikipedia

  https://en.wikipedia.org/wiki/Sickle_cell_disease

  Sickle cell disease occurs when a person inherits two abnormal copies of the -globin gene that makes haemoglobin, one from each parent. […] As of 2021, SCD is estimated to affect about 7.7 million people worldwide, directly causing an estimated 34,000 annual deaths and a contributory factor to a further 376,000 deaths. […] About 80% of sickle cell disease cases are believed to occur in Sub-Saharan Africa. […] The highest frequency of sickle cell disease is found in tropical regions, particularly sub-Saharan Africa, tribal regions of India, and the Middle East. […] Migration of substantial populations from these high-prevalence areas to low-prevalence countries in Europe has dramatically increased in recent decades and in some European countries, sickle cell disease has now overtaken more familiar genetic conditions such as haemophilia and cystic fibrosis.

• #102 SciELO Brazil – Acute clinical events in patients with sickle cell disease: epidemiology and treatment Acute clinical events in patients with sickle cell disease: epidemiology and treatment

  https://www.scielo.br/j/rbhh/a/RtVg8MqtpF4RRS7MmFbSPVy/?lang=en

  The proportion of hospitalizations in which the endpoint was death (5.2%) was similar to the one of another national study with patients hospitalized with sickle cell disease and it didn’t vary between adolescents and adults. […] Mortality was significantly higher in those with bacterial infection, reinforcing the importance of the recognition and appropriate treatment of infections in these patients, even out of the age group with higher risk, the childhood. […] We considered this study relevant to allow a larger knowledge concerning morbidity and mortality among adolescent and adult patients hospitalized with sickle cell disease. There are few studies with data from hospital admissions, then the study results can be useful in public health area, especially on healthcare planning to the population with sickle cell disease.

• #103 SciELO Brazil – Acute clinical events in patients with sickle cell disease: epidemiology and treatment Acute clinical events in patients with sickle cell disease: epidemiology and treatment

  https://www.scielo.br/j/rbhh/a/RtVg8MqtpF4RRS7MmFbSPVy/?lang=en

  The proportion of hospitalizations in which the endpoint was death (5.2%) was similar to the one of another national study with patients hospitalized with sickle cell disease and it didn’t vary between adolescents and adults. […] Mortality was significantly higher in those with bacterial infection, reinforcing the importance of the recognition and appropriate treatment of infections in these patients, even out of the age group with higher risk, the childhood. […] We considered this study relevant to allow a larger knowledge concerning morbidity and mortality among adolescent and adult patients hospitalized with sickle cell disease. There are few studies with data from hospital admissions, then the study results can be useful in public health area, especially on healthcare planning to the population with sickle cell disease.

• #104 Internet Scientific Publications

  https://ispub.com/IJBA/1/2/9165

  The frequency varies from population to population (tribals as well as nontribals) depending upon the practice of community and territorial endogamy between 1-44.4%, but commuted average sickle cell disorder frequency of 4.3% has been worked out in India. […] An alarming number of the cases are added every year in India. […] The pattern of death in persons who have sickle cell anemia is bimodal, with the first peak occurring in childhood and the 2 nd occurring in people in their late 30s. […] It is interesting to note that the sickle cell patients in India do not show severe clinical manifestations unlike the African patients and can survive upto 3rd or 4 th decade of life. […] Epidemiological studies of sickle cell disorders showed age specific mortality and relatively lower fitness of sickle cell homozygotes.

• #105 Internet Scientific Publications

  https://ispub.com/IJBA/1/2/9165

  The average life span of sickle cell homozygotes is about 20 years as against the normal life span of 50 years of heterozygotes. […] High degree of marital consanguinity, caste/class and geographical endogamy, lack of medical facilities, psycho-social prejudices, certain irrational traditions and beliefs aggravating the nutritional status and health, heavy cost of treatment, poor economy, backwardness in all spheres of life, etc. are some of the salient features of these vulnerable people in India. […] An Integrated National Preventive and Management Health Policy for Hemoglobinopathies in India is repeatedly over emphasized.

• #106 JMIR Public Health and Surveillance – Prevalence, Mortality, and Access to Care for Chronic Kidney Disease in Medicaid-Enrolled Adults With Sickle Cell Disease in California: Retrospective Cohort Study

  https://publichealth.jmir.org/2024/1/e57290/

  Chronic kidney disease (CKD) is a significant complication in patients with sickle cell disease (SCD), leading to increased mortality. […] This study aims to investigate the burden of CKD in Medicaid-enrolled adults with SCD in California, examine differences in disease burden between male and female individuals, and assess mortality rates and access to specialized care. […] Among the 2345 adults with SCD, 24.4% (n=572) met the case definition for CKD. […] During this study, of the 2345 adults, 435 (18.5%) deaths occurred, predominantly within the SCD-CKD cohort (226/435, 39.5%). […] This study provides robust estimates of CKD prevalence and mortality among Medicaid-enrolled adults with SCD in California. […] The findings highlight the need for improved access to specialized care for this population and increased awareness of the high mortality risk and progression associated with CKD.

• #107 JMIR Public Health and Surveillance – Prevalence, Mortality, and Access to Care for Chronic Kidney Disease in Medicaid-Enrolled Adults With Sickle Cell Disease in California: Retrospective Cohort Study

  https://publichealth.jmir.org/2024/1/e57290/

  The prevalence of CKD by age group and sex shows a notable pattern of increasing prevalence with advancing age, alongside a distinct variation between female and male individuals. […] Male individuals have a significantly higher CKD prevalence in the 30, 40, and 50 years age groups. […] The mortality rate in the SCD-CKD cohort was high at 39.5% (226/572). […] Access to nephrology care was also limited; in the group with SCD-CKD, individuals had only approximately 1 visit to a nephrologist per person-year, and 61.9% (354/572) of people did not have any encounters with nephrologists throughout the entire study period. […] Our findings highlight the critical need for this data to guide state health care stakeholders and government policy makers in addressing these access issues.

• #108 Burden of sickle cell disease | IJGM

  https://www.dovepress.com/the-burden-of-sickle-cell-disease-in-saudi-arabia-a-single-institution-peer-reviewed-fulltext-article-IJGM

  Sickle cell disease (SCD) is a significant burden for patients and healthcare systems due to multiple factors, including high readmission rates. This study aimed to determine the general characteristics, etiology of admissions, annual admission rate, length of stay, and readmission rate of patients with SCD. […] The average annual number of emergency department (ED) visits was 4, and approximately 19% of patients had 3 annual admissions. The mean length of stay was 6 days. The readmission rates at 7, 30, 60, and 90 days were 8%, 24.5%, 13.6%, and 10.8%, respectively. […] SCD generates a significant economic burden on the Saudi society and the effects on the healthcare system and patients quality of life are evident in the high ED visits, readmission rates and prolonged hospitalization.

• #109 Burden of sickle cell disease | IJGM

  https://www.dovepress.com/the-burden-of-sickle-cell-disease-in-saudi-arabia-a-single-institution-peer-reviewed-fulltext-article-IJGM

  The prevalence is much higher in the African and middle eastern populations, as sickle cell disease affects more than 20,000 per 1,000,000 of the Nigerian population. And more than 45,100 per 1,000,000 among adults in Saudi Arabia. Moreover, among Saudi children and adolescents, 2400 per 1,000,000 were estimated to have sickle cell disease. SCD is more dominant in the eastern and southwestern regions of the country. […] Over the 6-year study period, 3826 emergency department (ED) visits and 891 admissions were attributed to SCD complications, including 33 admissions to the intensive care unit. VOC was the main reason for admission in more than 90% of cases. […] More than half of the patients (n= 93, 58%) were readmitted during the study period. Of the 891 admissions, 218 (24.5%) were within 30 days of discharge, of which 71 (32.5%) were within 7 days of discharge. In contrast, 121 (13.6%) and 95 (10.8%) readmissions occurred within 60 and 90 days, respectively.

• #110 Burden of sickle cell disease | IJGM

  https://www.dovepress.com/the-burden-of-sickle-cell-disease-in-saudi-arabia-a-single-institution-peer-reviewed-fulltext-article-IJGM

  The prevalence is much higher in the African and middle eastern populations, as sickle cell disease affects more than 20,000 per 1,000,000 of the Nigerian population. And more than 45,100 per 1,000,000 among adults in Saudi Arabia. Moreover, among Saudi children and adolescents, 2400 per 1,000,000 were estimated to have sickle cell disease. SCD is more dominant in the eastern and southwestern regions of the country. […] Over the 6-year study period, 3826 emergency department (ED) visits and 891 admissions were attributed to SCD complications, including 33 admissions to the intensive care unit. VOC was the main reason for admission in more than 90% of cases. […] More than half of the patients (n= 93, 58%) were readmitted during the study period. Of the 891 admissions, 218 (24.5%) were within 30 days of discharge, of which 71 (32.5%) were within 7 days of discharge. In contrast, 121 (13.6%) and 95 (10.8%) readmissions occurred within 60 and 90 days, respectively.

• #111 Burden of sickle cell disease | IJGM

  https://www.dovepress.com/the-burden-of-sickle-cell-disease-in-saudi-arabia-a-single-institution-peer-reviewed-fulltext-article-IJGM

  The prevalence is much higher in the African and middle eastern populations, as sickle cell disease affects more than 20,000 per 1,000,000 of the Nigerian population. And more than 45,100 per 1,000,000 among adults in Saudi Arabia. Moreover, among Saudi children and adolescents, 2400 per 1,000,000 were estimated to have sickle cell disease. SCD is more dominant in the eastern and southwestern regions of the country. […] Over the 6-year study period, 3826 emergency department (ED) visits and 891 admissions were attributed to SCD complications, including 33 admissions to the intensive care unit. VOC was the main reason for admission in more than 90% of cases. […] More than half of the patients (n= 93, 58%) were readmitted during the study period. Of the 891 admissions, 218 (24.5%) were within 30 days of discharge, of which 71 (32.5%) were within 7 days of discharge. In contrast, 121 (13.6%) and 95 (10.8%) readmissions occurred within 60 and 90 days, respectively.

• #112 Burden of sickle cell disease | IJGM

  https://www.dovepress.com/the-burden-of-sickle-cell-disease-in-saudi-arabia-a-single-institution-peer-reviewed-fulltext-article-IJGM

  The average length of stay (LOS) was 6.43 days per admission. […] The average number of admissions and ED visits per patient is one admission and four ED visits. The average Length of stay during the study period was 6.4 days. The readmission rates at 7, 30, 60, and 90 days were 8%, 24.5%, 13.6%, and 10.8%, respectively.

• #113 Burden of sickle cell disease | IJGM

  https://www.dovepress.com/the-burden-of-sickle-cell-disease-in-saudi-arabia-a-single-institution-peer-reviewed-fulltext-article-IJGM

  The average length of stay (LOS) was 6.43 days per admission. […] The average number of admissions and ED visits per patient is one admission and four ED visits. The average Length of stay during the study period was 6.4 days. The readmission rates at 7, 30, 60, and 90 days were 8%, 24.5%, 13.6%, and 10.8%, respectively.

• #114 Burden of sickle cell disease | IJGM

  https://www.dovepress.com/the-burden-of-sickle-cell-disease-in-saudi-arabia-a-single-institution-peer-reviewed-fulltext-article-IJGM

  The average length of stay (LOS) was 6.43 days per admission. […] The average number of admissions and ED visits per patient is one admission and four ED visits. The average Length of stay during the study period was 6.4 days. The readmission rates at 7, 30, 60, and 90 days were 8%, 24.5%, 13.6%, and 10.8%, respectively.

• #115 World Sickle Cell Awareness Day: Navigating the Present and Shaping the Future of Sickle Cell Disease

  https://www.ajmc.com/view/world-sickle-cell-awareness-day-navigating-the-present-and-shaping-the-future-of-sickle-cell-disease

  World Sickle Cell Awareness Day was officially established on June 19, 2009. Each year, observing this day grants opportunities to further educate the public understanding of SCD and shed light on the innovations, challenges, and barriers that clinicians, patients, families, and caregivers face in their efforts to mitigate the detrimental effects of this rare blood disorder. […] In the US, newborn screening for SCD only began within the last 50 years and wasn’t widely adopted until 1986. Even with this progress, the transition to adult care presents significant challenges. Adult programs for SCD are often under-resourced, leading many patients to rely on emergency care rather than regular, preventive treatment. This dependence on the emergency department (ED) results in a staggering $3 billion annual burden on the US health care system.

• #116 World Sickle Cell Awareness Day: Navigating the Present and Shaping the Future of Sickle Cell Disease

  https://www.ajmc.com/view/world-sickle-cell-awareness-day-navigating-the-present-and-shaping-the-future-of-sickle-cell-disease

  World Sickle Cell Awareness Day was officially established on June 19, 2009. Each year, observing this day grants opportunities to further educate the public understanding of SCD and shed light on the innovations, challenges, and barriers that clinicians, patients, families, and caregivers face in their efforts to mitigate the detrimental effects of this rare blood disorder. […] In the US, newborn screening for SCD only began within the last 50 years and wasn’t widely adopted until 1986. Even with this progress, the transition to adult care presents significant challenges. Adult programs for SCD are often under-resourced, leading many patients to rely on emergency care rather than regular, preventive treatment. This dependence on the emergency department (ED) results in a staggering $3 billion annual burden on the US health care system.

• #117 Improved Surveillance, Registry Data Could Improve Outcomes in Sickle Cell Disease

  https://www.ajmc.com/view/improved-surveillance-registry-data-could-improve-outcomes-in-sickle-cell-disease

  Population-based data is a crucial aspect to evaluating current care quality and improving outcomes for patients with sickle cell disease. […] A review published in JAMA Health Forum emphasized the importance of national surveillance programs to gather longitudinal data and evaluate care access and quality for patients with sickle cell disease (SCD). […] The review highlights a lack of population-based data and emphasizes the importance of a longitudinal clinical registry and a national surveillance program to optimize care for this patient population. […] Population-based data is a crucial aspect to evaluating current care quality and improving care for patients with SCD. This would ideally include both a longitudinal clinical registry and a national surveillance program, which could provide complementary data to paint a picture of the state of SCD care overall.

• #118 Improved Surveillance, Registry Data Could Improve Outcomes in Sickle Cell Disease

  https://www.ajmc.com/view/improved-surveillance-registry-data-could-improve-outcomes-in-sickle-cell-disease

  If well developed, a SCD surveillance system would incorporate all individuals with SCD regardless of payer, including those not seen in established SCD care centers, and would determine the true SCD prevalence and geographic distribution of individuals with SCD, the authors wrote. […] Overall, the review highlights a need to close knowledge gaps in SCD via surveillance data in tandem with clinical registry data, both on a national scale. […] A national SCD surveillance program that makes its findings available to all stakeholders (patients, health care clinicians, researchers, legislators, advocates) would make it possible for the US to finally begin to address the health disparities imperiling individuals living with SCD, the authors concluded.

• #119 Research | Center of Excellence in Sickle Cell Disease

  https://www.bu.edu/sicklecell/research/

  The Center for Excellence in Sickle Cell Disease has an active basic science, clinical and translational research program devoted to increasing understanding of disease pathogenesis, the clinical epidemiology of complications of the disease and defining new treatments for patients. […] Understanding the epidemiology of sleep-disordered breathing in sickle cell disease and its impact on endothelial function and cardiopulmonary outcomes. […] Understanding the clinical outcomes of venous thromboembolism in SCD and the role of thromboses as a pulmonary vascular modulator.

• #120 Research | Center of Excellence in Sickle Cell Disease

  https://www.bu.edu/sicklecell/research/

  The Center for Excellence in Sickle Cell Disease has an active basic science, clinical and translational research program devoted to increasing understanding of disease pathogenesis, the clinical epidemiology of complications of the disease and defining new treatments for patients. […] Understanding the epidemiology of sleep-disordered breathing in sickle cell disease and its impact on endothelial function and cardiopulmonary outcomes. […] Understanding the clinical outcomes of venous thromboembolism in SCD and the role of thromboses as a pulmonary vascular modulator.

• #121 Making the case to improve outcomes for sickle cell disease – UAB News

  https://www.uab.edu/news/research-innovation/making-the-case-to-improve-outcomes-for-sickle-cell-disease

  Without surveillance data and a longitudinal registry, a true understanding of the implementation of disease-modifying therapy is impossible, she said. Disease surveillance is important for rare diseases that result in premature mortality, or which may impart a high emotional or economic burden to those affected or to the health care system. […] Given the importance and power of systematic surveillance data, it is clear that a national SCD surveillance system should be congressionally funded and used in conjunction with longitudinal clinical registries for SCD as clearly stated by the recommendations proposed by the National Academies of Science, Engineering and Medicine, Kanter said.

• #122 Making the case to improve outcomes for sickle cell disease – UAB News

  https://www.uab.edu/news/research-innovation/making-the-case-to-improve-outcomes-for-sickle-cell-disease

  Without surveillance data and a longitudinal registry, a true understanding of the implementation of disease-modifying therapy is impossible, she said. Disease surveillance is important for rare diseases that result in premature mortality, or which may impart a high emotional or economic burden to those affected or to the health care system. […] Given the importance and power of systematic surveillance data, it is clear that a national SCD surveillance system should be congressionally funded and used in conjunction with longitudinal clinical registries for SCD as clearly stated by the recommendations proposed by the National Academies of Science, Engineering and Medicine, Kanter said.

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