ABSTRACT
BACKGROUND: Chronic kidney disease (CKD) is a significant complication in patients with sickle cell disease (SCD), leading to increased mortality. OBJECTIVE: 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. METHODS: This retrospective cohort study used the California Sickle Cell Data Collection program to identify and monitor individuals with SCD. Medicaid claims, vital records, emergency department, and hospitalization data from 2011 to 2020 were analyzed. CKD prevalence was assessed based on ICD (International Classification of Diseases) codes, and mortality rates were calculated. Access to specialized care was examined through outpatient encounter rates with hematologists and nephrologists. RESULTS: Among the 2345 adults with SCD, 24.4% (n=572) met the case definition for CKD. The SCD-CKD group was older at the beginning of this study (average age 44, SD 14 vs 34, SD 12.6 years) than the group without CKD. CKD prevalence increased with age, revealing significant disparities by sex. While the youngest (18-29 years) and oldest (>65 years) groups showed similar CKD prevalences between sexes (female: 12/111, 10.8% and male: 12/101, 11.9%; female: 74/147, 50.3% and male: 34/66, 51.5%, respectively), male individuals in the aged 30-59 years bracket exhibited significantly higher rates than female individuals (30-39 years: 49/294, 16.7%, P=.01; 40-49 years: 52/182, 28.6%, P=.02; and 50-59 years: 76/157,48.4%, P<.001). During this study, of the 2345 adults, 435 (18.5%) deaths occurred, predominantly within the SCD-CKD cohort (226/435, 39.5%). The median age at death was 53 (IQR 61-44) years for the SCD-CKD group compared to 43 (IQR 33-56) years for the SCD group, with male individuals in the SCD-CKD group showing significantly higher mortality rates (111/242, 45.9%; P=.009) than female individuals (115/330, 34.9%). Access to specialist care was notably limited: approximately half (281/572, 49.1%) of the SCD-CKD cohort had no hematologist visits, and 61.9% (354/572) did not see a nephrologist during this study's period. CONCLUSIONS: 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.
Subject(s)
Anemia, Sickle Cell , Health Services Accessibility , Medicaid , Renal Insufficiency, Chronic , Humans , Male , Female , California/epidemiology , Adult , Retrospective Studies , Medicaid/statistics & numerical data , Prevalence , Anemia, Sickle Cell/complications , Anemia, Sickle Cell/epidemiology , Anemia, Sickle Cell/mortality , Middle Aged , Renal Insufficiency, Chronic/epidemiology , Renal Insufficiency, Chronic/therapy , Renal Insufficiency, Chronic/mortality , Health Services Accessibility/statistics & numerical data , United States/epidemiology , Cohort Studies , Young Adult , AdolescentABSTRACT
OBJECTIVE: To estimate trends in mortality rate and average age of death, and identify sociodemographic factors associated with early death in patients with sickle cell disease (SCD). METHODS: An ecological and cross-sectional study was conducted using data from the Mortality Information System. All deaths of patients residing in the state of São Paulo from 1996 to 2015 with at least one International Disease Code for SCD in any field of the death certificate were included. Simple linear regression was used to estimate trends. The Log-rank test and multiple Cox regression were used to identify factors associated with early death. RESULTS: The age-standardized mortality rate per million inhabitants increased by 0.080 per year (R2=0.761; p<0.001). When the events were stratified by age at death, the increase was 0.108 per year for those occurring at age 20 years or older, (R2=0.789; p<0.001) and 0.023 per year for those occurring before age 20 years old (R2=0.188; p=0.056). The average age at death increased by 0.617 years (7.4 months) per year (R2=0.835; p<0.001). Sociodemographic factors associated with early death identified were male gender (hazard ratio - HR=1.30), white race (HR=1.16), death occurring in the hospital (HR=1.29), and living in the Greater São Paulo (HR=1.13). CONCLUSIONS: The mortality rate and the average age of death in patients with SCD have increased over the last two decades. Sociodemographic factors such as gender, race, place of occurrence, and residence were found to be associated with early death.
Subject(s)
Anemia, Sickle Cell , Humans , Anemia, Sickle Cell/mortality , Brazil/epidemiology , Male , Female , Cross-Sectional Studies , Adolescent , Young Adult , Child , Infant , Child, Preschool , Adult , Sociodemographic Factors , Cause of Death , Mortality/trends , Age Factors , Infant, Newborn , Middle AgedABSTRACT
OBJECTIVE: The ability to cause death is the definitive measure of an infectious disease severity, particularly one caused by a novel pathogen like severe acute respiratory syndrome-CoV-2 (COVID-19). This study describes sickle cell disease-related mortality issues during the COVID-19 pandemic in Brazil. METHODS: The provisional 2020 mortality data originated from the public databases of the Mortality Information System and were investigated using the multiple-cause-of-death methodology. RESULTS: In 2020, 688 sickle cell disease-related deaths occurred, of which 422 (61.3%) had an underlying cause of death and 266 (38.7%) had an associated cause of death. Furthermore, 98 COVID-19-related deaths occurred, of which 78 were underlying cause of death among sickle cell disease associated (non-underlying) cause of death. Sickle cell disease-related deaths occurred mostly among young adults aged 25-49 years. COVID-19 deaths occurred at ages older than among sickle cell disease-related deaths. Majority of deaths happened in the southeast (42.3%) and northeast regions (34.0%), while COVID-19 deaths prevailed in the northeast region (42.9%). Regarding overall deaths, the leading underlying cause of death was sickle cell disease itself, followed by infectious and parasitic diseases (14.8%), owing to COVID-19 deaths, and diseases of the circulatory system (8.9%). Next, in males, diseases of the digestive system (4.8%) occurred, while, in females, maternal deaths succeeded, included in the chapter on pregnancy, childbirth, and the puerperium, accounting for 5.9% of female deaths. The leading overall associated (non-underlying) cause of deaths were septicemias (29.4%), followed by respiratory failure (20.9%), pneumonias (18.3%), and renal failure (14.7%). CONCLUSION: In Brazil, COVID-19 deaths produced trend changes in sickle cell disease-related causes of death, age at death, and regional distribution of deaths in 2020.
Subject(s)
Anemia, Sickle Cell , COVID-19 , Cause of Death , Humans , COVID-19/mortality , COVID-19/epidemiology , Anemia, Sickle Cell/mortality , Anemia, Sickle Cell/complications , Anemia, Sickle Cell/epidemiology , Brazil/epidemiology , Adult , Female , Middle Aged , Male , Young Adult , SARS-CoV-2 , Adolescent , Child , Pandemics , Aged , Child, Preschool , Age DistributionSubject(s)
Anemia, Sickle Cell , Neonatal Screening , Humans , Anemia, Sickle Cell/mortality , Anemia, Sickle Cell/epidemiology , Anemia, Sickle Cell/diagnosis , Anemia, Sickle Cell/complications , Infant, Newborn , Netherlands/epidemiology , Follow-Up Studies , Female , Male , Child , Child, Preschool , Infant , AdolescentABSTRACT
Approximately 3% of pregnant women have sickle cell disease (SCD). COVID-19, caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), became a global pandemic in March 2020, resulting in more than 3,500 deaths in Jamaica by April 2023. Sickle cell disease is an immunocompromised state; therefore, contracting COVID-19 may result in adverse maternal/neonatal outcomes. Current literature focusing on individuals of Afro-Caribbean descent is limited. Our objective was to describe the obstetric and neonatal outcomes of pregnant patients with SCD who contracted COVID-19. A retrospective case series was conducted at the University Hospital of the West Indies (Jamaica) from 2020 to 2022. We describe the maternal and neonatal outcomes of three patients with COVID-19 and SCD (including two with hemoglobin SC disease and one with hemoglobin SS disease), with complications including the demise of a mother and a newborn. Vaso-occlusive crisis was the more common presentation. Two patients required ventilatory support. Although previous reports have shown similar clinical sequelae in pregnant and nonpregnant patients with SCD and COVID-19, maternal and neonatal deaths remain possible.
Subject(s)
Anemia, Sickle Cell , COVID-19 , Pregnancy Complications, Infectious , SARS-CoV-2 , Humans , COVID-19/complications , COVID-19/mortality , COVID-19/epidemiology , Female , Pregnancy , Anemia, Sickle Cell/complications , Anemia, Sickle Cell/mortality , Adult , Infant, Newborn , Pregnancy Complications, Infectious/virology , Pregnancy Complications, Infectious/mortality , Pregnancy Complications, Hematologic/epidemiology , Jamaica/epidemiology , Retrospective Studies , Pregnancy OutcomeABSTRACT
OBJECTIVES: To examine the association between chronic kidney disease (CKD) and outcomes in sickle cell disease (SCD) patients. METHODS: Patients ≥18 years with SCD in the National Inpatient Sample database between 2016 and 2018 were identified using ICD-10-CM diagnosis codes. A 1:1 propensity-score matched analysis was performed to compare in-hospital outcomes of patients with and without CKD. RESULTS: Of the 366 240 SCD admissions, 19 365 (5.3%) had CKD. The CKD group had higher odds of in-hospital mortality (odds ratio [OR] 2.59, 95% confidence interval [CI]: 1.63-4.12, p = <.01), blood transfusion (OR 1.67, 95% CI: 1.47-1.90, p < .01), mechanical ventilation (OR 2.20, 95% CI: 1.56-3.12, p = <.01), sepsis (OR 1.75, 95% CI: 1.46-2.10, p < .01), incurred higher costs ($53 255 vs. $47 294, p < .001), but had lower odds of acute chest syndrome (OR 0.71, 95% CI: 0.54-0.95, p = 0.02) and pulmonary embolism (OR 0.45, 95% CI: 0.31-0.67, p < .01). CONCLUSIONS: CKD was associated with higher mortality, higher costs, blood transfusion, sepsis, and mechanical ventilation in SCD patients. Further studies are needed to explore the reasons for the reduced odds of pulmonary embolism and acute chest syndrome.
Subject(s)
Anemia, Sickle Cell , Hospital Mortality , Renal Insufficiency, Chronic , Humans , Anemia, Sickle Cell/complications , Anemia, Sickle Cell/epidemiology , Anemia, Sickle Cell/mortality , Anemia, Sickle Cell/therapy , Male , Female , Renal Insufficiency, Chronic/epidemiology , Renal Insufficiency, Chronic/therapy , Renal Insufficiency, Chronic/complications , Renal Insufficiency, Chronic/mortality , Adult , Middle Aged , Hospitalization , Inpatients , United States/epidemiology , Odds Ratio , Databases, Factual , Adolescent , Young AdultABSTRACT
No disponible
Subject(s)
Humans , Adolescent , Young Adult , Adult , Middle Aged , Anemia, Sickle Cell/mortality , Pulmonary Diffusing Capacity , Lung/abnormalities , Dyspnea , Acute Chest SyndromeABSTRACT
Abstract Introduction There is a demand to update national mortality trends data related to sickle cell disease (SCD) in Brazil. This study describes causes of death and mortality issues related to SCD using the multiple-cause-of-death methodology. Methods The annual SCD mortality data was extracted from the public databases of the Mortality Information System by researching deaths in rubric D57 "sickle-cell disorders" of the International Classification of Diseases, Tenth Revision and processed by the Multiple Cause Tabulator. Results From 2000 to 2018 in Brazil, a total of 9817 deaths related to SCD occurred during the 19-year period, as the underlying cause in 6924 (70.5%) and as the associated cause of death in 2893 (29.5%). The mean and median ages at death during the entire period were significantly lower for males, 29.4 (±19.6) and 27.5 (15.5-41.5), respectively, than for females, 33.3 (±20.3) and 31.0 (19.5-46.5), respectively. The leading SCD overall associated causes of death were septicemias (32.1%), followed by pneumonias (19.4%) and respiratory failure (18.2%). On certificates with SCD as an associated cause, the underlying causes of death were circulatory system diseases (8.7%), followed, in males, by digestive system and infectious diseases and respiratory system failures, while in females, maternal deaths, included in the chapter on pregnancy, childbirth and the puerperium, accounting for 4.6% of female deaths, were succeeded by digestive system and infectious diseases. Conclusion This study revised mortality data on death rate trends, underlying and associated causes of death, age at death and regional distribution of death in Brazil.
Subject(s)
Humans , Male , Female , Pregnancy , Infant, Newborn , Infant , Child, Preschool , Child , Adolescent , Adult , Middle Aged , Aged , Young Adult , Mortality/trends , Anemia, Sickle Cell/mortality , Maternal Mortality , Communicable Diseases , Cause of DeathABSTRACT
Sickle cell disease (SCD) is associated with increased risk of poor health outcomes from respiratory infections, including COVID-19 illness. We used US death data to investigate changes in SCD-related mortality before and during the COVID-19 pandemic. We estimated annual age- and quarter-adjusted SCD-related mortality rates for 2014-2020. We estimated the number of excess deaths in 2020 compared with 2019 using the standardized mortality ratio (SMR). We found 1023 SCD-related deaths reported in the United States during 2020, of which 86 (8.4%) were associated with COVID-19. SCD-related deaths, both associated and not associated with COVID-19, occurred most frequently among adults aged 25-59 years. The SCD-related mortality rate changed <5% year to year from 2014 to 2019 but increased 12% in 2020; the sharpest increase was among adults aged ≥60 years. The SMR comparing 2020 with 2019 was 1.12 (95% CI, 1.06-1.19). Overall, 113 (95% CI, 54-166) excess SCD-related deaths occurred in 2020.
Subject(s)
Anemia, Sickle Cell/mortality , COVID-19/epidemiology , Adolescent , Adult , Age Distribution , Anemia, Sickle Cell/complications , COVID-19/complications , Child , Child, Preschool , Ethnicity , Humans , Infant , Middle Aged , Mortality/trends , Race Factors , SARS-CoV-2 , Time Factors , United States/epidemiologySubject(s)
Anemia, Sickle Cell , Multiple Organ Failure , Thrombomodulin/blood , Anemia, Sickle Cell/blood , Anemia, Sickle Cell/complications , Anemia, Sickle Cell/mortality , Cell Line , Female , Humans , Male , Multiple Organ Failure/blood , Multiple Organ Failure/etiology , Multiple Organ Failure/mortalityABSTRACT
BACKGROUND: Children with sickle cell disease (SCD) are at increased risk for bloodstream infections (BSIs), mainly because of functional asplenia. Immunizations and antibiotic prophylaxis have reduced the prevalence of invasive bacterial infections, but contemporary analysis of BSI in children with SCD is limited. METHODS: We conducted a retrospective cohort study of children aged <18 years with SCD who had blood cultures collected at our institution from 2010 to 2019 to identify BSI. Probable contaminant organisms were identified and not included as BSI. We calculated the annual incidence of BSI at our institution with 95% confidence intervals (CIs) and used multivariate logistic regression to evaluate associations. RESULTS: There were 2694 eligible patients with 19 902 blood cultures. Excluding repeated cultures and contaminant cultures, there were 156 BSI episodes in 144 patients. The median age at BSI was 7.5 years. The average incidence rate of BSI was 0.89 per 100 person-years (95% CI 0.45-1.32). The most common pathogens were Streptococcus pneumoniae (16.0%), Streptococcus viridans group (9.0%), Escherichia coli (9.0%), Staphylococcus aureus (7.7%), Bordetella holmesii (7.7%), Haemophilus influenzae (7.1%), and Salmonella species (6.4%). Odds of BSI were higher with sickle cell anemia genotypes (odds ratio [OR] 1.88; 95% CI 1.20-2.94) and chronic transfusions (OR 2.66; 95% CI 1.51-4.69) and lower with hydroxyurea (OR 0.57; 95% CI 0.39-0.84). CONCLUSIONS: BSI remains a risk for children with SCD. Overall incidence, risk factors, and spectrum of pathogens are important considerations to guide prevention and empirical treatment of suspected infection in SCD.
Subject(s)
Anemia, Sickle Cell/complications , Sepsis/epidemiology , Sepsis/microbiology , Adolescent , Anemia, Sickle Cell/genetics , Anemia, Sickle Cell/mortality , Child , Female , Genotype , Georgia/epidemiology , Humans , Incidence , Male , Retrospective Studies , Risk Factors , Sepsis/mortalitySubject(s)
Anemia, Sickle Cell/drug therapy , Anemia, Sickle Cell/mortality , Benzaldehydes/administration & dosage , Pyrazines/administration & dosage , Pyrazoles/administration & dosage , Adult , Benzaldehydes/adverse effects , Disease-Free Survival , Female , Humans , Male , Middle Aged , Pyrazines/adverse effects , Pyrazoles/adverse effects , Retrospective Studies , Survival RateABSTRACT
ABSTRACT OBJECTIVE Estimate rates and describe mortality trends attributed to sickle cell disease in children and adolescents in Brazil from 2000 to 2019. METHODS This is an ecological study of the time-trend of mortality rates that used the autoregressive method, proposed by Prais-Winsten, to evaluate trends in the estimated rates of sickle cell disease deaths in children and adolescents in Brazil. Deaths with code D57 were obtained from the Mortality Information System, considering age groups (0-4, 5-9, 10-14, 15-19 years) and were used to estimate age-specific and standardized rates by gender and age. RESULTS From 2000 to 2019, Brazil had 2,422 deaths from sickle cell disease in people under 20 years of age, with higher frequency in the Northeast (40.46%), followed by the Southeast (39.02%), Midwest (9.58%), North (7.84%), and South (3.10%). The main victims were people of Black skin/race (78.73%). In Brazil, the global standardized average rate was 0.20/100,000 people-year, with an elevation trend (annual percentage change - APC = 5.44%; confidence interval - 95%CI: 2.57-8.39). The pattern was repeated in males (APC = 4.38%; 95%CI: 2.17-6.64) and females (APC = 6.96%; 95%CI: 3.05-11.01). Elaborating age-specific rates showed that the range up to four years experienced the highest rates, without distinction by region. The age group of 15 and 19 years was the second most affected in Brazil and in the Northeast, Southeast, and Midwest regions. CONCLUSION Deaths due to sickle cell disorders showed an elevation trend in children and adolescents. Considering that the magnitude of deaths was more evident in the first years (0-4) and late adolescence (15-19), the study suggests that age-specific approaches may impact the control of fatal outcomes caused by sickle cell disease in Brazil.
RESUMO OBJETIVO Estimar taxas e descrever tendências de mortalidade atribuídas à doença falciforme em crianças e adolescentes no Brasil, de 2000 a 2019. MÉTODOS Este é um estudo ecológico do tipo séries temporais de taxas de mortalidade que usou o método autorregressivo, proposto por Prais-Winsten, para avaliar tendências das taxas estimadas de mortes por doença falciforme em crianças e adolescentes no Brasil. Os óbitos com código D57 foram obtidos no Sistema de Informações sobre Mortalidade, considerando as faixas etárias (0-4, 5-9, 10-14, 15-19 anos) e usados para estimar taxas específicas por idade e taxas padronizadas por sexo e idade. RESULTADOS De 2000 a 2019, houve 2.422 óbitos por doença falciforme em menores de 20 anos no Brasil, com maior frequência na região Nordeste (40,46%), seguida de Sudeste (39,02%), Centro-Oeste (9,58%), Norte (7,84%) e Sul (3,10%). As principais vítimas foram pessoas de raça/cor da pele negra (78,73%). No Brasil, a taxa média padronizada global foi de 0,20/100 mil pessoa-ano, com tendência de elevação (mudança percentual anual - APC = 5,44%; intervalo de confiança - IC95% 2,57-8,39). O padrão se repetiu no sexo masculino (APC = 4,38%; IC95% 2,17-6,64) e no sexo feminino (APC = 6,96%; IC95% 3,05-11,01). A elaboração de taxas específicas por idade mostrou que a faixa até quatro anos experimentou as maiores taxas, sem distinção por região. A faixa etária de 15 e 19 anos foi a segunda mais afetada no Brasil e nas regiões Nordeste, Sudeste e Centro-Oeste. CONCLUSÃO Houve tendência de aumento dos óbitos por transtornos falciformes em crianças e adolescentes. Considerando que a magnitude dos óbitos foi mais evidente nos primeiros anos (0-4) e no final da adolescência (15-19), o estudo sugere que abordagens específicas por faixa etária podem impactar no controle dos desfechos fatais causados pela doença falciforme no Brasil.
Subject(s)
Brazil , Child , Time Series Studies , Adolescent , Anemia, Sickle Cell/mortality , Anemia, Sickle Cell/epidemiologyABSTRACT
Little is known about patients with sickle cell disease (SCD) who require intensive care unit (ICU) admission. The goals of this study were to assess outcomes in patients admitted to the ICU for acute complications of SCD and to identify factors associated with adverse outcomes. This multicenter retrospective study included consecutive adults with SCD admitted to one of 17 participating ICUs. An adverse outcome was defined as death or a need for life-sustaining therapies (non-invasive or invasive ventilation, vasoactive drugs, renal replacement therapy, and/or extracorporeal membrane oxygenation). Factors associated with adverse outcomes were identified by mixed multivariable logistic regression. We included 488 patients admitted in 2015-2017. The main reasons for ICU admission were acute chest syndrome (47.5%) and severely painful vaso-occlusive event (21.3%). Sixteen (3.3%) patients died in the ICU, mainly of multi-organ failure following a painful vaso-occlusive event or sepsis. An adverse outcome occurred in 81 (16.6%; 95% confidence interval [95% CI], 13.3%-19.9%) patients. Independent factors associated with adverse outcomes were low mean arterial blood pressure (adjusted odds ratio [aOR], 0.98; 95% CI 0.95-0.99; p = 0.027), faster respiratory rate (aOR, 1.09; 95% CI 1.05-1.14; p < 0.0001), higher haemoglobin level (aOR, 1.22; 95% CI 1.01-1.48; p = 0.038), impaired creatinine clearance at ICU admission (aOR, 0.98; 95% CI 0.97-0.98; p < 0.0001), and red blood cell exchange before ICU admission (aOR, 5.16; 95% CI 1.16-22.94; p = 0.031). Patients with SCD have a substantial risk of adverse outcomes if they require ICU admission. Early ICU admission should be encouraged in patients who develop abnormal physiological parameters.
Subject(s)
Anemia, Sickle Cell/epidemiology , Anemia, Sickle Cell/mortality , Critical Illness , Acute Chest Syndrome/epidemiology , Acute Chest Syndrome/therapy , Adult , Blood Pressure , Critical Care , Female , France/epidemiology , Hospitalization , Humans , Intensive Care Units , Length of Stay , Male , Middle Aged , Multivariate Analysis , Odds Ratio , Pain , Retrospective Studies , Treatment Outcome , Young AdultSubject(s)
Anemia, Sickle Cell/immunology , COVID-19 Vaccines/administration & dosage , COVID-19/complications , Surveys and Questionnaires/statistics & numerical data , Adult , Anemia, Sickle Cell/epidemiology , Anemia, Sickle Cell/mortality , Anemia, Sickle Cell/virology , COVID-19/diagnosis , COVID-19/epidemiology , COVID-19/immunology , COVID-19 Vaccines/adverse effects , COVID-19 Vaccines/immunology , Comorbidity , Female , Genotype , Hospitalization/statistics & numerical data , Hospitalization/trends , Humans , Incidence , London/epidemiology , Male , Recovery of Function , SARS-CoV-2/geneticsABSTRACT
We surveyed published papers and an international sickle cell disease (SCD) registry to detect susceptibility and clinical course of coronavirus disease 2019 (COVID-19) in SCD patients. COVID-19 presentation was mild in children and moderate in many SCD adults. Regarding increased comorbidities with age, it seems severe COVID-19 to be more common in older SCD patients. Although the overall outcome of COVID-19 was favorable in SCD children, a high rate of pediatric intensive care unit admission should be considered in managing these patients. To explain COVID-19 outcome in SCD patients, the possible benefits of hydroxyurea therapy could be considered. The obtained results should be interpreted, considering low cases from sub-Saharan people, younger age of SCD patients compared to general population, a bias toward registry of the more severe form of disease, the effect of pre-existing comorbidities with multisystem organ damage, and the role of health socio-economic determinants.
Subject(s)
Anemia, Sickle Cell/mortality , COVID-19/mortality , SARS-CoV-2 , Adolescent , Adult , Age Factors , Anemia, Sickle Cell/pathology , Anemia, Sickle Cell/virology , COVID-19/pathology , Child , Disease Susceptibility/mortality , Disease Susceptibility/pathology , Disease Susceptibility/virology , Female , Humans , Male , Risk Factors , Severity of Illness IndexABSTRACT
In the Netherlands, between 1985 and 2007 secular changes in the health care of patients with sickle cell disease (SCD) have taken place, such as penicillin prophylaxis, vaccination programs and stroke prevention. We investigated the number and causes of death in a cohort of 298 SCD patients, established in 2007, before introduction of neonatal screening, to determine preventable deaths. All patients were diagnosed with SCD before the age of 18 (median age at diagnosis 5.1 y). Their vital status was determined up to January 2017. After a total follow-up period of 4565 patient years and a median time of follow-up of 15 years for all patients, 230 patients (77%) were still alive, 45 patients (15%) were lost to follow-up and a total of 23 patients (8%) had died. Estimated survival to 18 years was 92% with a global mortality rate of 0.48 deaths/100 patient years. Leading causes of death were infection (35%) followed by neurologic complications (22%) and death in the course of a painful episode (13%). Nine of the 20 known causes of death were preventable. These results strongly suggest the benefit of comprehensive care measures for patients with SCD in the Netherlands to further prevent morbidity and mortality.
Subject(s)
Anemia, Sickle Cell/mortality , Cause of Death/trends , Adolescent , Adult , Anemia, Sickle Cell/epidemiology , Anemia, Sickle Cell/etiology , Child , Child, Preschool , Female , Follow-Up Studies , Humans , Infant , Infant, Newborn , Male , Middle Aged , Netherlands/epidemiology , Prognosis , Prospective Studies , Retrospective Studies , Survival Rate , Time Factors , Young AdultABSTRACT
Patients with a primary diagnosis of sickle cell disease (SCD) with or without crisis during the 10-year period January 2009 to December 2018 were identified in the HES Admitted Patient Care (APC) dataset and matched with the Office for National Statistics (ONS) mortality dataset. Three sub-cohorts were defined: 'crises', 'transfusions' and 'other SCD'. APC records were examined for co-morbidities commonly associated with SCD and 10-year mortality rates compared with the general population. After data cleaning and exclusions, 9503 patients remained (entire cohort), with 1171, 201, and 8131 in crises, transfusions, and other SCD sub-cohorts, respectively. Median numbers of co-morbidities per patient were 2 (Interquartile range (IQR): 1-4), 2 (IQR: 1-3), and 1 (IQR: 0-2) in the crises, transfusions, and other SCD sub-cohorts, respectively. The majority of patients in the crises (63.2%) and transfusions (56.3%) cohorts had ≥2 co-morbidities, compared with 25.3% in the other SCD sub-cohort. Crude 10-year mortality rate was 5.3% (entire cohort), compared with 8.0% (crises) and 11.4% (transfusions) sub-cohorts; all rates were substantially higher than in age-sex matched general population. Our study adds further evidence that morbidity and mortality associated with SCD in England is high.
Subject(s)
Anemia, Sickle Cell/epidemiology , Adolescent , Adult , Anemia, Sickle Cell/mortality , Anemia, Sickle Cell/therapy , Blood Transfusion , Child , Cohort Studies , Comorbidity , England/epidemiology , Female , Hospitals , Humans , Male , Middle Aged , Young AdultABSTRACT
According to the Center of Disease Control and Prevention (CDC) database, the total number of deaths due to opioid overdose from 1999 through 2018 was 840,629. Given the alarming nature of these statistics, patients who requested prescription for opioids became targets of suspicion and possible accusation of maladaptive behavior. Patients with sickle cell disease (SCD) were often not exempt from such accusations and became guilty by association. In order to clarify the effect of opioids on the mortality of patients with SCD, the mortality rates for children and adults with SCD were investigated using the CDC Wide-ranging Online Data for Epidemiologic Research (WONDER) Multiple Cause of Death database which is based on all the death certificates issued in the USA from 1999 to 2018. The data showed that 15,765 patients with SCD died from 1999 to 2018. Only 348 patients with SCD died due to opioids. The CDC database contains 27 categories of death based on ICD-10 codes in patients with SCD, and opioids were the 19th ranking cause of death. Surprisingly the most common causes of death of patients with SCD included circulatory, infection, respiratory, genitourinary, and vaso-occlusive crises/acute chest syndrome disorders in decreasing frequency. The mean age of death of females was 41.9 years and of males 39.3 years (p < 0.0001). Death due to SCD and death due to SCD and opioids were highest in the Southern Region of the USA.
Subject(s)
Analgesics, Opioid/adverse effects , Anemia, Sickle Cell/mortality , Opiate Overdose/mortality , Adolescent , Adult , Anemia, Sickle Cell/complications , Cause of Death , Child , Child, Preschool , Female , Humans , Infant , Male , Middle Aged , Opiate Overdose/complications , United States , Young AdultABSTRACT
OBJECTIVE: To document pregnancy outcome in homozygous sickle cell (SS) disease and in age-matched controls with a normal haemoglobin genotype followed from birth for up to 45 years. METHODS: A total of 100 000 consecutive non-operative deliveries screened for sickle cell disease at the main Government maternity hospital in Kingston, Jamaica between 1973 and 1981 detected 311 (149 female) babies with SS disease who were matched by age and gender with 250 (129 female) controls with an AA haemoglobin phenotype. These individuals have been followed from birth with prospective assessment of menarche and detailed documentation of all pregnancies. RESULTS: There were 177 pregnancies in 71 SS patients and 226 pregnancies in 74 AA controls. Mothers with SS disease had more spontaneous abortions (adjusted relative risk [aRR] 3.2, 95% CI 1.6-6.1), fewer live births (aRR 0.7, 95% CI 0.6-0.9) and their offspring were more likely to have a gestational age <37 weeks (aRR 2.1, 95% CI 1.1-3.7) and low birthweight <2.5 kg (aRR 3.0, 95% CI 1.6-5.3). They were more prone to acute chest syndrome (aRR 13.7, 95% CI 4.1-45.5), urinary tract infection (aRR 12.8, 95% CI 1.3-125.9), pre-eclampsia/eclampsia (aRR 3.1, 95% CI 1.1-8.8), retained placenta (aRR 10.1, 95% CI 1.1-90.3), sepsis (Fisher's Exact test 0.04) and pregnancy-related deaths (Fisher's Exact test 0.02). Four of five deaths were attributable to acute chest syndrome. There was no genotypic difference in pregnancy-induced hypertension or postpartum haemorrhage. CONCLUSION: Pregnancy in SS disease carries risks for both mother and child. The variable characteristics of pregnancy-related deaths complicate their prevention. TWEETABLE ABSTRACT: Pregnancy in SS disease compared with controls showed increased abortions and stillbirths, fewer live births and maternal deaths in 7% patients.