CDC Grand Rounds: Improving the Lives of Persons with Sickle Cell Disease

Hulihan, Mary M.; Hassell, Kathryn L.; Raphael, Jean L.; Smith‐Whitley, Kim; Thorpe, Phoebe

doi:10.15585/mmwr.mm6646a2

Cited by 20 publications

(29 citation statements)

References 13 publications

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“…For children and young adults with SCD, increased access to and utilization of healthcare services are key factors in decreasing morbidity and mortality. 32 The Pain in Sickle Cell Epidemiology Study (PiSCES) demonstrated fewer physical challenges and pain frequency, HCRU, and better QoL, in transition-aged (16 to 25 years) versus older adults (37 to 64 years) with SCD. 22 Another retrospective study found patients transitioning to adulthood relied more on emergency departments for their care than their pediatric counterparts.…”

Section: Discussionmentioning

confidence: 99%

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High Healthcare Utilization in Adolescents with Sickle Cell Disease Prior to Transition to Adult Care: A Retrospective Study

Kanter

Bhor

et al. 2019

JHEOR

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Background: The transition from pediatric to adult care in the US is often difficult for individuals with sickle cell disease (SCD). Young adults (18 to 25 years of age) have higher acute care utilization and an increased risk of poor outcomes. The current study was designed to provide greater insights into patients (16 to 18 years of age) with SCD prior to their transition to adult care. Objectives: To describe current treatment patterns, pain crises prevalence, SCD-related complications, and healthcare resource utilization (HCRU) in 16-to-18-year-old patients with SCD. Methods: From 1/1/2015-6/30/2017 using MarketScan Databases, patients were included if they were 16 to 18 years old at the index date, had ≥1 inpatient or 2 outpatient SCD diagnosis claims during the identification period, and were continuously enrolled in the database for at least one year prior (baseline) and post index date. Outcomes included medications, disease management interventions, Charlson Comorbidity Index (CCI), vaso-occlusive (VOC) crises requiring healthcare visits, HCRU, and SCD complications. Results: 1,186 patients were included; most (64.3%) were female. The mean CCI was 1.3 (SD: 0.7). In the overall cohort, patients experienced an average of 3.9 (SD: 4.2) VOCs and most patients (61.1%, n=725) had chronic complications during the one-year follow-up. Pulmonary disease (31.1%, n=369) was the most frequent complication; blood transfusions (mean: 8.4 [SD:7.0]) and iron-chelating therapies (mean: 8.6 [SD:10]) were the most common interventions and medications, respectively. In the 16 to 18 year old group, patients with SCD had 2.0 (SD = 2.3) hospital admissions, 3.4 (SD = 4.0) ER visits, and 5.0 (SD = 4.2) office visits. Conclusions: Prior to transition to adult care, adolescents with SCD already have significant acute and chronic disease-related complications, possibly contributing to frequent healthcare visits. Increased attention to this age group, including improvements in disease modifying therapy, are needed prior to transition to adult care systems to improve outcomes.

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Section: Discussionmentioning

confidence: 99%

“…13,20,[23][24][25][26][27] More information is needed about healthcare resource utilization (HCRU) and treatment patterns in young patients with SCD before they transition to adult care. 15,21,[28][29][30][31][32] Improved understanding of the HCRU in adolescents may help predict individuals at higher risk post-transition.…”

Section: Intr Introduc Oduction Tionmentioning

confidence: 99%

High Healthcare Utilization in Adolescents with Sickle Cell Disease Prior to Transition to Adult Care: A Retrospective Study

Kanter

Bhor

et al. 2019

JHEOR

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“…It is estimated that approximately 300,000 children are born annually with the disease, and the global mortality rate is reported to be 0.64 per 100 years of child observation, with Africa recording the highest rate (7.3 per 100 years of observation) [1]. Improved health care systems in some regions of the world have Improved SCD management with a consequent increase in life expectancy [2]. This has led to the increase in people living beyond their fifth decade and, therefore, a resultant increase in the incidence of the chronic complications of the disease [3,4].…”

Section: Introductionmentioning

confidence: 99%

Variations and characteristics of the various clinical phenotypes in a cohort of Nigerian sickle cell patients

et al. 2021

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Background: Sickle cell anaemia affects about 4 million people across the globe, making it an inherited disorder of public health importance. Red cell lysis consequent upon haemoglobin crystallization and repeated sickling leads to anaemia and a baseline strain on haemopoiesis. Vaso-occlusion and haemolysis underlies majority of the chronic complications of sickle cell. We evaluated the clinical and laboratory features observed across the various clinical phenotypes in adult sickle cell disease patients. Methods: Steady state data collected prospectively in a cohort of adult sickle cell disease patients as out-patients between July 2010 and July 2020. The information included epidemiological, clinical and laboratory data. Results: About 270 patients were captured in this study (165 males and 105 females). Their ages ranged from 16 to 55 years, with a median age of 25 years. Sixty-eight had leg ulcers, 43 of the males had priapism (erectile dysfunction in 8), 42 had AVN, 31 had nephropathy, 23 had osteomyelitis, 15 had osteoarthritis, 12 had cholelithiasis, 10 had stroke or other neurological impairment, 5 had pulmonary hypertension, while 23 had other complications. Frequency of crisis ranged from 0 to >10/year median of 2. Of the 219 recorded, 148 of the patients had been transfused in the past, while 71 had not. Conclusion:The prevalence of SLU, AVN, priapism, nephropathy and the other complications of SCD show some variations from other studies. This variation in the clinical parameters across different clinical phenotypes indicates an interplay between age, genetic and environmental factors.

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“…A gestão de cuidados de saúde com alocação de recursos governamentais e de ações da comunidade podem auxiliar na inserção e monitoramento dessas e de outras intervenções como educação para os pais e pacientes, profilaxia para infecções com antibioticoterapia e imunobiológicos, suplementação com ácido fólico, além de alimentação adequada desde os primeiros meses de vida (Hulihan, Hassell, Raphael, Smith-Whitley, & Thorpe, 2017;Mburu & Odame, 2019).…”

Section: Discussionunclassified

Sobrevida de pacientes com doença falciforme diagnosticados durante a triagem neonatal: revisão sistemática

Pompeo

Júnior

Cardoso

et al. 2021

RSD

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Objetivo: Analisar os dados relativos à sobrevida de pacientes com doença falciforme diagnosticados durante o processo de triagem neonatal. Método: Revisão sistemática, realizada nas bases eletrônicas Current Index to Nursing and Allied Health Literature, Cochrane Library, National Library of Medicine, Scopus e Web of Science com descritores controlados indexados no Medical Subject Headings. Sem delimitação temporal ou de idioma. Resultados: Sete artigos compuseram a amostra final, todos publicados em inglês e de delineamento de coorte. A avaliação de risco de vieses pelo Newcastle Otawa Scale indicou que seis artigos foram considerados de alta qualidade. Foram estudadas as taxas de sobrevida global, estratificadas por genótipos, eventos clínicos agudos, sexo e níveis de hemoglobina, além da sobrevida livre de eventos. A sobrevida global apresentou variação entre 100% aos seis meses e 85,6% aos 18 anos, enquanto para o genótipo Sβº foi estimada em 55,2% aos 40 anos. Conclusão: Houve diminuição da sobrevida após os 30 anos de idade, entretanto, com aumento anual entre os períodos estudados. A elaboração de políticas públicas com inserção da comunidade no contexto de suas ações pode auxiliar na manutenção das estratégias de melhoria da sobrevida desta população.

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CDC Grand Rounds: Improving the Lives of Persons with Sickle Cell Disease

Cited by 20 publications

References 13 publications

High Healthcare Utilization in Adolescents with Sickle Cell Disease Prior to Transition to Adult Care: A Retrospective Study

High Healthcare Utilization in Adolescents with Sickle Cell Disease Prior to Transition to Adult Care: A Retrospective Study

Variations and characteristics of the various clinical phenotypes in a cohort of Nigerian sickle cell patients

Sobrevida de pacientes com doença falciforme diagnosticados durante a triagem neonatal: revisão sistemática

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