Assessment of hypoxemia among young adults with sickle cell anaemia in steady state in southwestern Nigeria: a crosssectional study.

OBJECTIVES: Hypoxia is a known feature of sickle cell anaemia (SCA) which results from chronic anaemia and recurrent vaso-occlusive crisis (VOC) which can cause tissue ischaemia that leads to an end organ damage. The hallmark of SCA is chronic anaemia and recurrent vaso-occlusive crisis. The aim of this study is to compare the oxygen saturation of sickle cell anaemic individuals with the normal haemoglobin type (Hb AA) control and also to determine the prevalence of hypoxemia among SCA. RESULTS: Two-hundred and twenty-two (136 Hb SS and 86 Hb AA) participated in the study. The mean ± SD of age (years), oxygen saturation (%) and pulse rate (bpm) of participants with sickle cell anaemia and Hb AA control were 21.85 ± 3.04 and 22.14 ± 3.18 (t = 0.701, p = 0.436), 95.21 ± 3.02 and 98.07 ± 0.81 (t=-8.598, p < 0.0001) and 77.10 ± 9.28 and 73.16 ± 8.52 (t = 3.173, p = 0.002) respectively. The prevalence of hypoxemia among SCA participants was 47.1%. Prevalence of hypoxemia in males with SCA was 60.9% while 39.1% of the females had hypoxemia.

Prevalence and predictors of iron deficiency anaemia among children with sickle cell disease in Dodoma, Tanzania: a cross-sectional study.

BACKGROUND: Patients with sickle cell disease (SCD) are prone to iron profile derangements. This study aimed to determine the prevalence of iron deficiency anaemia (IDA) and their predictors among children with SCD aged between 6 months and 14 years. Assessment of the prevalence of IDA and its predictors helps to understand ways of alleviating the magnitude of the problem so as to prevent possible complications such as shortness of breath and chest pain. METHODS: This was a cross-sectional analytical hospital-based study which included 174 patients with SCD attending SCD clinics at St. Gema hospital and Dodoma regional referral hospital in Dodoma city from October 2020 to March 2021. The cut-off points for detection of IDA was serum ferritin level < 30 µg/L and low mean corpuscular volume (MCV) for age. Data were analyzed using SPSS software version 25.0. Multivariate logistic regression analysis was used to determine the predictors of IDA. P-value less than 0.05 was considered significant. RESULTS: The prevalence of IDA in this study was (16.1%, n = 28). Family income of less than 70,000/= TZS/month (AOR = 2.2, 95% CI = 1.07-2.49, p = 0.023), being transfused with blood less than 3 times from the time of being diagnosed with SCD (AOR = 5.5, 95% CI = 1.03-8.91, p = 0.046), and eating red meat at least once per month (AOR = 3.60, 95% CI = 1.37-9.46, p = 0.010) remained the independent predictors of IDA in multivariate regression analysis. CONCLUSION: The findings of this study have shown that, support of families with children suffering from SCD in terms of financial support for improving medical services including optimal blood transfusion and affordability of diet which is rich in iron such as red meat is imperative.

Acute care utilization among individuals with sickle cell disease and related cardiopulmonary and renal complications.

Cardiopulmonary and renal end organ (CPR) complications are associated with early mortality among individuals with sickle cell disease (SCD). However, there is limited knowledge regarding acute care utilization for individuals with SCD and CPR complications. Our objective was to determine the prevalence of CPR complications in a state specific SCD population and compare acute care utilization among individuals with and without CPR complications. We leveraged 2017-2020 data for individuals with SCD identified by the Sickle Cell Data Collection program in Wisconsin. The prevalence of CPR complications is determined for distinct age groups. Generalized linear models adjusted for age compared the rate of acute care visits/person/year among individuals who had cardiopulmonary only, renal only, both cardiopulmonary and renal, or no CPR complications. There were 1378 individuals with SCD, 52% females, mean (SD) age 28.3 (18.5) years; 48% had at least one CPR complication during the study period. The prevalence of CPR complications was higher in adults (69%) compared to pediatric (15%) and transition (51%) groups. Individuals with SCD and cardiopulmonary complications had higher acute visit rates than those without CPR complications (5.4 (IQR 5.0-5.8) vs 2.4 (IQR 2.1-2.5), p <0.001)). Acute care visit rates were similar between individuals with SCD who had renal only complications and no CPR complications (2.7 (IQR 2.5-3.0) vs 2.4 (2.1-2.5), p = 0.24). The high acute care visit rates, especially for those with cardiopulmonary complications, warrant further investigation to understand risk factors for CPR complications, the underlying reasons and identify effective disease management strategies.

Social determinants of health and treatment center affiliation: analysis from the sickle cell disease implementation consortium registry.

BACKGROUND: Adults with sickle cell disease (SCD) suffer early mortality and high morbidity. Many are not affiliated with SCD centers, defined as no ambulatory visit with a SCD specialist in 2 years. Negative social determinants of health (SDOH) can impair access to care. HYPOTHESIS: Negative SDOH are more likely to be experienced by unaffiliated adults than adults who regularly receive expert SCD care. METHODS: Cross-sectional analysis of the SCD Implementation Consortium (SCDIC) Registry, a convenience sample at 8 academic SCD centers in 2017-2019. A Distressed Communities Index (DCI) score was assigned to each registry member's zip code. Insurance status and other barriers to care were self-reported. Most patients were enrolled in the clinic or hospital setting. RESULTS: The SCDIC Registry enrolled 288 Unaffiliated and 2110 Affiliated SCD patients, ages 15-45y. The highest DCI quintile accounted for 39% of both Unaffiliated and Affiliated patients. Lack of health insurance was reported by 19% of Unaffiliated versus 7% of Affiliated patients. The most frequently selected barriers to care for both groups were "previous bad experience with the healthcare system" (40%) and "Worry about Cost" (17%). SCD co-morbidities had no straightforward trend of association with Unaffiliated status. The 8 sites' results varied. CONCLUSION: The DCI economic measure of SDOH was not associated with Unaffiliated status of patients recruited in the health care delivery setting. SCDIC Registrants reside in more distressed communities than other Americans. Other SDOH themes of affordability and negative experiences might contribute to Unaffiliated status. Recruiting Unaffiliated SCD patients to care might benefit from systems adopting value-based patient-centered solutions.

National Quality Indicators in Pediatric Sickle Cell Anemia.

OBJECTIVE: To assess nationally endorsed claims-based quality measures in pediatric sickle cell anemia (SCA). METHODS: Using data from the Sickle Cell Data Collection programs in California and Georgia from 2010 to 2019, we evaluated 2 quality measures in individuals with hemoglobin S/S or S/ß-zero thalassemia: (1) the proportion of patients aged 3 months to 5 years who were dispensed antibiotic prophylaxis for at least 300 days within each measurement year and (2) the proportion of patients aged 2 to 15 years who received at least 1 transcranial Doppler ultrasound (TCD) within each measurement year. We then evaluated differences by year and tested whether performance on quality measures differed according to demographic and clinical factors. RESULTS: Only 22.2% of those in California and 15.5% in Georgia met or exceeded the quality measure for antibiotic prophylaxis, with increased odds associated with rural residence in Georgia (odds ratio 1.61; 95% confidence interval 1.21-2.14) compared with urban residence and a trend toward increased odds associated with a pediatric hematologist prescriber (odds ratio 1.28; 95% confidence interval 0.97, 1.69) compared with a general pediatrician. Approximately one-half of the sample received an annual assessment of stroke risk using TCD (47.4% in California and 52.7% in Georgia), with increased odds each additional year in both states and among younger children. CONCLUSIONS: The rates of receipt of recommended antibiotic prophylaxis and annual TCD were low in this sample of children with SCA. These evidence-based quality measures can be tracked over time to help identify policies and practices that maximize survival in SCA.

[The transition of adolescents with sickle cell disease: an interdisciplinary exchange]. / La transition des adolescents drépanocytaires, un échange interdisciplinaire.

With the rising incidence of sickle cell disease, this chronic pathology is becoming the most common genetic disease in France. Advances in care have led to a marked improvement in life expectancy. Caregivers in pediatric facilities are therefore increasingly confronted with the question of the transition to adulthood of the adolescents they have been following since birth. As nurses working in Robert-Debré's transfusion and curative medicine unit, in 2022, adolescents accounted for 57 % of sickle cell patients enrolled in our transfusion exchange program. Adolescence is a period of major change, and the repercussions of the disease are all the more pronounced. This raises the issue of transferring them to the adult sector.

Prevalence of Duffy null and its impact on hydroxyurea in young children with sickle cell disease in the United States.

Consistent with studies showing a high prevalence of the Duffy null phenotype among healthy Black Americans, this retrospective study found that Duffy null was present in >75% of a young and contemporary cohort of children with sickle cell disease (SCD) in the United States. Despite the potential for this phenotype to impact absolute neutrophil counts, hydroxyurea (HU) dosing, and outcomes, it was not associated with being prescribed a lower HU dose or having increased acute SCD visits early in the HU treatment course. Future studies are needed to confirm these findings in older children with SCD.

Description of a national, multi-center registry of patients with sickle cell disease and SARS-CoV-2 infection: Data from the Pediatric COVID-19 United States Registry.

Children with sickle cell disease (SCD) are at risk of complications from viral infections, including SARS-CoV-2. We present the clinical characteristics and outcomes of pediatric patients with SCD from the Pediatric COVID-19 United States Registry who developed acute COVID-19 due to SARS-CoV-2 infection (n = 259) or multisystem inflammatory syndrome in children (MIS-C; n = 4). Nearly half of hospitalized children with SCD and SARS-CoV-2 infection required supplemental oxygen, though children with SCD had fewer intensive care (ICU) admissions compared to the general pediatric and immunocompromised populations. All registry patients with both SCD and MIS-C required ICU admission. Children with SCD are at risk of severe disease with SARS-CoV-2 infection, highlighting the importance of vaccination in this vulnerable population.

Sickle cell trait in São Tomé e Príncipe: a population-based prevalence study in women of reproductive age.

BACKGROUND: Sickle Cell Disorder is Africa's most prevalent genetic disease. Yet, it remains a neglected condition, with high mortality under-five, and a lack of population-based studies in the region. This is the first of its kind in São Tomé e Príncipe, aiming to estimate the prevalence of sickle cell trait and other haemoglobin variants in women of reproductive age and its associated factors. METHODS: We conducted a cluster survey in 35 neighbourhoods. Haemoglobin was assessed through point-of-care capillary electrophoresis or high-performance liquid chromatography, and sociodemographic data through questionnaires. The weighted prevalence of sickle cell trait (HbAS) and HbC carriers was estimated with a 95% confidence interval (95% CI). We calculated weighted prevalence ratios (95% CI) through robust Poisson regression for its association with age and individual and collective genetic heritage. FINDINGS: The prevalence of sickle cell trait in women of reproductive age in São Tomé e Príncipe (n = 376) was 13.45% (95% CI: 9.05-19.00). The prevalence of HbC carriers was 8.00% (95% CI: 4.71-12.00). Older age and speaking Forro or Angolar were positively associated with having sickle cell trait. INTERPRETATION: The prevalence of sickle cell trait in São Tomé e Príncipe ranks high in the West African region. The country should follow international guidelines, implementing newborn screening and comprehensive healthcare management.

Birth Prevalence of Sickle Cell Disease and County-Level Social Vulnerability - Sickle Cell Data Collection Program, 11 States, 2016-2020.

Sickle cell disease (SCD) remains a public health priority in the United States because of its association with complex health needs, reduced life expectancy, lifelong disabilities, and high cost of care. A cross-sectional analysis was conducted to calculate the crude and race-specific birth prevalence for SCD using state newborn screening program records during 2016-2020 from 11 Sickle Cell Data Collection program states. The percentage distribution of birth mother residence within Social Vulnerability Index quartiles was derived. Among 3,305 newborns with confirmed SCD (including 57% with homozygous hemoglobin S or sickle ß-null thalassemia across 11 states, 90% of whom were Black or African American [Black], and 4% of whom were Hispanic or Latino), the crude SCD birth prevalence was 4.83 per 10,000 (one in every 2,070) live births and 28.54 per 10,000 (one in every 350) non-Hispanic Black newborns. Approximately two thirds (67%) of mothers of newborns with SCD lived in counties with high or very high levels of social vulnerability; most mothers lived in counties with high or very high levels of vulnerability for racial and ethnic minority status (89%) and housing type and transportation (64%) themes. These findings can guide public health, health care systems, and community program planning and implementation that address social determinants of health for infants with SCD. Implementation of tailored interventions, including increasing access to transportation, improving housing, and advancing equity in high vulnerability areas, could facilitate care and improve health outcomes for children with SCD.

Dental caries in the permanent dentition and health-related quality of life among children and adolescents with sickle cell disease.

The aim was to associate living, health and oral health conditions with the quality of life (QL) of children and adolescents (CA) with sickle cell disease (SCD). Of the 120 eligible users of a public hematological service, 106 CA with SCD from 6 to 18 years of age, and their caregivers, answered semi-structured questions about socio-demographic, health and oral health conditions. For QL, we used the validated instrument PedsQLSCD™. The oral clinical examination occurred according to the guidelines of WHO and SB Brazil 2010. The majority of CA were non-white people (88%), mean age of 10.4 (±2.9) years, family income of up to two monthly minimum wages, for 03 to 05 members, with diagnosis of sickle cell anemia by neonatal screening, hospitalizations were due allergic crises, polypharmacy and dental caries (51%) were present. "About the Impact of My Pain" was the best-fit model for the QLSCD (adjusted R²=56%; AIC=28.67; p=0.04). Dental caries in permanent dentition worsened the QLSCD (OR=0.53; IC95%=0.35-0.78; p<0.05) and was associated with the type of school, car ownership, number of family members, of complications and of the medications. To overcome this scenario, programmatic actions are required, and implementation of public policies specifically directed towards these groups.

Àgô Sankofa: an overview of the progression of sickle cell disease in Brazil in the past two decades. / Àgô Sankofa: um olhar sobre a trajetória da doença falciforme no Brasil nos últimos 20 anos.

Sickle cell disease (SCD) is an emblematic case of historical health neglect in Brazil and reflects how institutional racism produces health inequalities. This article engaged in a historical journey of this disease, showing the delayed implementation of health policies for people with sickle cell disease, often concealed in Public Power's (in)actions and omissions. The lack of commitment to implement the recommendations of the Brazilian Ministry of Health, such as neonatal screening, and the difficulty in incorporating technologies for health care result from this modus operandi. The advances and setbacks in programmatic actions and the constant pressure on several governmental entities have characterized the reported saga in the last twenty years. The present text discusses the policies for people with SCD, appropriating the Sankofa symbol, meaning that building the present is only possible by remembering past mistakes. Thus, we recognize this trajectory and this historical moment in which there is a concrete possibility of moving forward and achieving the longed-for comprehensive care for people with SCD. There is an invitation to glance at a new perspective, one in which hope is the trigger for the movements needed to guarantee the rights of people with SCD.

A doença falciforme (DF) é um caso emblemático de negligência histórica em saúde no Brasil e reflete como o racismo institucional produz iniquidades em saúde. Este artigo fez um percurso histórico até os dias atuais e mostra atraso na implementação de políticas de saúde voltadas para as pessoas com DF, tantas vezes encoberto em (in)ações e omissões do poder público. O descompromisso para a efetivação das recomendações do Ministério da Saúde, a exemplo da triagem neonatal, e a dificuldade de incorporar as tecnologias para a assistência à saúde resultam desse modus nada operandi. Os avanços e retrocessos nas ações programáticas, bem como a pressão constante sobre os diversos entes governamentais, caracterizaram a saga dos últimos 20 anos. O texto disserta sobre as políticas voltadas para as pessoas com DF, apropriando-se da simbologia Sankofa, já que só é possível construir o presente pelo aprendizado dos erros do passado. Assim, reconhecemos essa trajetória e esse momento histórico em que há possibilidade concreta de avançar e concretizar o tão almejado cuidado integral para pessoas com DF. Concluiu-se que há um convite para um novo olhar, em que esperançar seja o disparador das movimentações necessárias para a garantia do direito para as pessoas com DF.

Experiences and strategies of people with sickle cell disease in the Federal District: the biographical rupture. / Experiências e estratégias de pessoas com doença falciforme no Distrito Federal: a ruptura biográfica.

This is a study on sickle cell disease, a chronic illness that affects many Brazilians, that aims to understand and analyze how people address the adversities arising from the diagnosis and the biographical rupture. The description of people's experiences and strategies conjures a picture that expresses the respondents' habitus, built in a dialectical relationship with the vulnerability determined by the disease. We adopted a qualitative approach and focused interviews as proposed by Merton, combined with the snowball technique, applied to groups related to sickle cell disease on social networks. Seven participants were selected because they were privileged informants with the disease, were over eighteen, lived in the Federal District, and were non-exclusive users of the Unified Health System. The interview material was categorized from the focal groups employed. The results indicated the following categories: biographical rupture, experience and coping strategies, and healthcare. It is necessary to sensitize professionals and the population about the challenging living conditions of people with sickle cell disease and the consolidation of public policies and care networks to accommodate this population.

Trata-se de um estudo sobre a doença falciforme, enfermidade crônica que afeta muitas pessoas no Brasil. O objetivo foi compreender como as pessoas lidam com as adversidades oriundas do diagnóstico e a ruptura biográfica. A descrição das experiências e estratégias empregadas pelas pessoas compuseram um quadro que expressa os habitus dos entrevistados, construídos em relação dialética com a vulnerabilidade determinada pela doença. A abordagem foi qualitativa e empregou entrevistas focadas como propostas por Merton, a técnica bola de neve aplicada em grupos relacionados à doença falciforme em redes sociais. Sete participantes foram selecionados por serem informantes privilegiados, portadores da doença, maiores de 18 anos, moradores no Distrito Federal e usuários não exclusivos do Sistema Único de Saúde. O material das entrevistas foi categorizado a partir dos núcleos focais empregados. Os resultados apontaram as categorias: ruptura biográfica, experiência e estratégias de enfrentamento, atenção à saúde. Concluímos que é necessária uma sensibilização dos profissionais e da população sobre as dificuldades da condição de vida das pessoas com doença falciforme e a consolidação das políticas públicas e das redes de atendimento para acolher essa população.

Sickle Cell Disease in an Older Adult Population: A Retrospective Review of Health Care Resource Utilization.

Sickle cell disease (SCD) has a history of health inequity, as patients with SCD are primarily Black and often marginalized from the health care system. Although recent health care and treatment advancements have prolonged life expectancy, it may be insufficient to support the complex needs of the growing population of older adults with SCD. This retrospective study used a cohort (N = 812) of Medicare Advantage beneficiaries 45 years and older (ages: 45-54, 55-64, 65-74, 75-89) with SCD to identify associations of SCD-related complications and comorbidities with emergency department (ED) visits, potentially avoidable ED visits, all-cause hospitalization, and potentially avoidable hospitalizations, 2018-2020. The 75-89 age group had lower odds of an ED visit (OR 0.56; 95% CI 0.32-1.00), 65-74 age group had lower odds of an ED visit (OR 0.49; 95% CI 0.31-0.78) and hospitalization (OR 0.50; 95% CI 0.31-0.79), compared with the 45-54 age group. Acute chest syndrome was associated with increased odds of an ED visit (OR 2.02; 95% CI 1.10-3.71), avoidable ED visit (OR 1.87; 95% CI 1.14-3.06), and hospitalization (OR 3.61; 95% CI 2.06-6.31). Pain was associated with increased odds of an ED visit (OR 2.64; 95% CI 1.85-3.76), an avoidable ED visit (OR 3.08; 95% CI 1.90-4.98), hospitalization (OR 1.51; 95% CI 1.02-2.24), and avoidable hospitalization (OR 6.42; 95% CI 1.74-23.74). Older adults with SCD have been living with SCD for decades, often while managing pain crises and complications associated increased incidence of an ED visit and hospitalization. The characteristics and needs of this population must continue to be examined to increase preventative care and reduce costly emergent health care resource utilization.

Implications of climatic change on sickle cell anemia: A review.

Sickle cell anemia (SCA) is a hereditary blood disorder characterized by abnormal hemoglobin, causing red blood cells to assume a sickle shape, leading to various complications. Climate change has emerged as a significant global challenge, influencing environmental conditions worldwide. This paper explores the implications of climatic variations on the prevalence, management, and outcomes of SCA. Climate change affects weather patterns, leading to altered temperatures, increased frequency of extreme weather events, and variations in humidity levels. These changes can have a profound impact on individuals living with SCA. High temperatures exacerbate the symptoms of SCA, potentially triggering painful vaso-occlusive crises due to dehydration and increased blood viscosity. Conversely, cold temperatures may induce vaso-occlusion by causing blood vessels to constrict. Changes in rainfall patterns might also affect water accessibility, which is crucial for maintaining adequate hydration, particularly in regions prone to droughts. The management of SCA is multifaceted, involving regular medical care, hydration, and avoiding triggers that could precipitate a crisis. Adverse weather events and natural disasters can disrupt healthcare infrastructure and access to essential medications and resources for SCA patients, especially in vulnerable communities. To mitigate the implications of climatic change on SCA, interdisciplinary strategies are essential. These strategies may include enhancing healthcare systems' resilience to climate-related disruptions, implementing adaptive measures to address changing environmental conditions, and promoting public awareness and education on managing SCA amidst climate variability. In conclusion, climatic variations pose significant challenges for individuals with SCA, affecting the prevalence, management, and outcomes of the disease.

No Increase in Masked Hypertension Prevalence in Children With Sickle Cell Disease in France.

BACKGROUND: An important prevalence (32%-45%) of masked hypertension has been reported in children with sickle cell disease (SCD). Stroke screening is well established using transcranial Doppler (TCD) ultrasound. The objectives of our proof-of-concept study in childhood SCD were to evaluate the prevalence of hypertension and its relationships with cerebral vasculopathy (TCD velocity) and to further evaluate in a subgroup of children the correlations of cardiovascular autonomic nervous system indices with TCD velocity. METHODS: Ambulatory blood pressure measurement (ABPM) and TCD velocity were obtained in children with SCD and in a restricted sample, cardiac sympathovagal balance using heart rate variability analyses, baroreflex sensitivity, and pulse wave velocity were measured. RESULTS: In 41 children with SCD (median age 14.0 years, 19 girls, SS/Sß + thalassemia/SC: 33/2/6), ABPM results showed masked hypertension in 2/41 (5%, 95% confidence interval, 0-11) children, consistent with the prevalence in the general pediatric population, elevated blood pressure (BP) in 4/41 (10%) children, and a lack of a normal nocturnal dip in 19/41 children (46%). Children with increased TCD velocity had lower nocturnal dipping of systolic BP. In the 10 participants with extensive cardiovascular assessment, increased TCD velocity was associated with parasympathetic withdrawal and baroreflex failure. Exaggerated orthostatic pressor response or orthostatic hypertension was observed in 7/10 children that was linked to parasympathetic withdrawal. CONCLUSIONS: Autonomic nervous system dysfunction, namely loss of parasympathetic modulation, of SCD contributes to increase TCD velocity but is not associated with an increased prevalence of masked hypertension. CLINICAL TRIALS REGISTRATION: NCT04911049.

Molecular characterisation of sickle cell disease and classification of major haplotypes associated with the ß-globin cluster (HBB gene) by means of SNP marker sequencing in a group of samples from Bolívar, Colombia.

BACKGROUND: Colombia has a mestizo population and the prevalence of haemoglobin variants varies according to each region, but heterozygous carriers can be found in all of them. AIM: To characterise sickle cell disease (SCD) haematologically, biochemically, and molecularly, and detect classic haplotypes by DNA sequencing in a group of samples from Bolívar, Colombia. SUBJECTS AND METHODS: Blood samples were collected after informed consent from volunteers from eight communities in the Bolívar department, plus samples from the Pacific region, Providencia Island, and Bogotá were included. Data were obtained from: (1) haematological analyses; (2) biochemical tests: dHPLC was used to determine haemoglobin (Hb); and (3) DNA sequencing data through five SNPs. RESULTS: 101 samples were identified by rs334 through Sanger's Sequencing, structural haemoglobinopathies HbAS (34.65%), HbSS (2.97%) and HbAC (1.98%) were found. When contrasting the Hb identification results between SNP rs334 Vs. dHPLC/Isoelectric Focusing (IEF), a coincidence was found in 39/43 samples analysed, therefore, when comparing these techniques, a significant correlation was found (Pearson's correlation coefficient r = 0.998). 26 samples previously analysed by rs334 were classified into classical haplotypes CAR (50.0%), BEN (30.76%), CAM (7.69%), SEN (3.84%), and ATP-I (7.69%). CONCLUSIONS: SCD characterisation and SNPs-based classification through Sanger's DNA sequencing have not been performed before in Colombia. The results of this work will make it possible to expand the data or records of carriers and those affected, which will benefit patients and their families.

Comparing Hearing Loss in Children with Adults Living with Sickle Cell Disease and Sickle Cell Traits.

BACKGROUND: Hearing loss is a neurological sequelae associated with sickle cell disease (SCD) and probably sickle cell trait (SCT) in children and adults but remains understudied. AIM: This study aimed to compare the hearing impairment among children and adults living with SCD or SCT. METHODS: A comparative cross-sectional study conducted in four departments with SCD outpatient clinic in a tertiary hospital in Nigeria. Participants with Sickle cell disease (HbSS) and Sickle cell trait (HbAS) (cohort) and HbAA (control) had comprehensive ear and hearing assessments for sensorineural hearing loss. Audiometric results were categorized according to WHO classifications and data analysed with Statistical Analysis System (SAS 9.4). RESULTS: A total of 212 participants (106 cohort and control, respectively), aged 6 months to 55 years, were enrolled. Of these, 35% of children with SCD and 25% with SCT had hearing impairment, while 30% of adults with SCD, 36.1% with SCT, and 11% with HbAA had hearing impairment. There was asymmetry in the hearing impairment, with the left ear more affected in children and the right ear in adults. The odds ratio (OD) of hearing impairment was higher in HbSS (2.48 (95% confidence interval (CI):1.51-4.14); P = 0.0004) and HbAS (2.28 (95% CI: 1.1-4.58); P = 0.02) participants compared with HbAA but was not statistically significant when adjusted for frequency of hospitalization, crises, blood transfusion and routine drugs in HbAS (P = 0.49) unlike HbSS (P = 0.03). CONCLUSION: The prevalence of hearing loss among children and adults with SCD is higher than in those with HbA genotype. The frequency of hospitalization, crises, blood transfusion and taking routine drugs may influence hearing impairment in SCT but may not in SCD.