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1.
Pediatr Blood Cancer ; 71(2): e30784, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38037196

RESUMO

Depression may contribute to transition risk among young adults with sickle cell disease (SCD). It is unclear if they receive depression screening because primary care providers (PCPs) routinely perform this screening, but PCP use declines with age. This retrospective study of young adults with SCD during their final year of pediatric hematology care identified 51 (91%) had PCPs. Among those with hospital system PCPs, 20% saw their PCP and 50% of those were screened for depression by the PCP. This suggests young adults with SCD may not receive depression screening or see PCPs, leading to potential missed opportunities for intervention.


Assuntos
Anemia Falciforme , Hematologia , Criança , Humanos , Adulto Jovem , Estudos Retrospectivos , Depressão/diagnóstico , Depressão/etiologia , Anemia Falciforme/complicações , Anemia Falciforme/diagnóstico , Atenção Primária à Saúde
2.
Am J Hematol ; 98(11): 1677-1684, 2023 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-37551881

RESUMO

Pregnancy and sickle cell disease (SCD) both individually carry a risk of thromboembolism (TE). Pregnancy in people with SCD may further enhance the prothrombotic effect of the underlying disease. The objectives of this study were to determine the rate and risk factors for arterial and venous thrombosis in pregnant people with SCD. Administrative claims data from the United States Centers for Medicare and Medicaid Service Analytic eXtract from 2006 to 2018 were used. The study population included people with SCD from the start of their first identified pregnancy until 1 year postpartum and a control cohort of pregnant people without SCD of similar age and race. Outcomes of interest were identified with ICD-9 or 10 codes. Logistic regression analyses were used to analyze risk factors. We identified infant deliveries in 6388 unique people with SCD and 17 110 controls. A total of 720 venous thromboembolism (11.3%) and 335 arterial TE (5.2%) were observed in people with SCD compared to 202 (1.2%) and 95 (0.6%) in controls. People with SCD had an 8-11 times higher odds of TE compared to controls (p < .001). Within the SCD cohort, age, hemoglobin SS (HbSS) genotype, hypertension, and history of thrombosis were identified as independent risk factors for pregnancy-related TE. Pregnancy-specific factors (pre-eclampsia, eclampsia, multigestational pregnancy) were not associated with TE. In conclusion, the risk of pregnancy-related TE is considerably higher in people with SCD compared with controls without SCD. Hence, people with SCD, particularly those with multiple risk factors may be candidates for thromboprophylaxis during pregnancy and the postpartum period.


Assuntos
Anemia Falciforme , Tromboembolia Venosa , Idoso , Gravidez , Humanos , Feminino , Estados Unidos/epidemiologia , Medicaid , Anticoagulantes , Tromboembolia Venosa/epidemiologia , Tromboembolia Venosa/etiologia , Medicare , Anemia Falciforme/complicações , Anemia Falciforme/epidemiologia
3.
Pediatr Blood Cancer ; 70(7): e30384, 2023 07.
Artigo em Inglês | MEDLINE | ID: mdl-37102416

RESUMO

BACKGROUND: Despite needing to be informed about sickle cell trait (SCT) status to make informed reproductive decisions, more than 80% of adults with SCT, including parents of children with SCT who have a high prevalence of SCT, do not know their status. PROCEDURE: This was a prospective study of parents who received SCT telephone education from the state department of health and then completed SCTaware, a videoconference-administered SCT education program. The objectives were to evaluate knowledge after telephone education and explore if SCTaware closes knowledge gaps. Participants completed a demographic survey, a health literacy assessment, and reported their SCT status. They completed the Sickle Cell Trait Knowledge Assessment before receiving SCTaware, immediately after, and at follow-up visits; high knowledge was a score of 75% or higher correct. RESULTS: SCTaware and the initial surveys were completed by 61 parents; 45 completed the 6-month surveys. Only 43% of participants had high SCT knowledge after telephone education; 92% achieved high knowledge immediately after, and 84% continued with high knowledge at 6 months. Most parents reported they were aware of their SCT status after telephone education; 12 changed their response after receiving SCTaware. CONCLUSIONS: Our findings suggest that over half of parents have low SCT knowledge following telephone education, and many may be unaware of their status. SCTaware closes knowledge gaps, leads to high sustained knowledge, and is a potentially scalable tool. Future studies should refine SCTaware and determine if parents use their knowledge to inform their children and reproductive decisions.


Assuntos
Anemia Falciforme , Traço Falciforme , Adulto , Humanos , Criança , Traço Falciforme/epidemiologia , Estudos Prospectivos , Conhecimentos, Atitudes e Prática em Saúde , Pais
4.
Pediatr Blood Cancer ; 70(7): e30332, 2023 07.
Artigo em Inglês | MEDLINE | ID: mdl-37046404

RESUMO

OBJECTIVE: Hydroxyurea lowers the incidence of vaso-occlusive pain crises (VOC) and acute chest syndrome (ACS) among children with sickle cell anemia (SCA). Our objective was to assess the relationship between levels of adherence to hydroxyurea and clinical outcomes among children and adolescents with SCA. METHODS: This retrospective cohort study included Medicaid data (2005-2012) from Florida, Illinois, Louisiana, Michigan, South Carolina, and Texas. The study population consisted of children 1-17 years old with SCA enrolled in Medicaid for 3 years. Among children that initiated hydroxyurea, the medication possession ratio (MPR) was calculated as the proportion of days covered by hydroxyurea. Six months after initiation of hydroxyurea, clinical outcomes were assessed through the end of the study period: numbers of VOC-related inpatient admissions and emergency department visits, and encounters for ACS. Multivariable Poisson models were used to predict outcomes by MPR quartile adjusting for previous healthcare utilization, state, and age. RESULTS: Hydroxyurea was initiated by 515 children. The median MPR was 0.53 (interquartile range = 0.3-0.8). The annual median number of visits was 0.0 for ACS, 1.3 for VOC-related emergency department, and 1.4 for VOC-related inpatient admissions. For each outcome, the highest quartile of MPR had the lowest predicted count; this difference was significant for ACS visits when compared with the lowest quartile of MPR. CONCLUSION: This study demonstrated a high level of adherence (>75%) was essential to achieve a lower incidence of common negative clinical outcomes. Further, moderate and severe hydroxyurea nonadherence may be more common than previously appreciated among children, emphasizing the importance of developing and testing innovative strategies to increase adherence.


Assuntos
Síndrome Torácica Aguda , Anemia Falciforme , Compostos Orgânicos Voláteis , Adolescente , Humanos , Criança , Lactente , Pré-Escolar , Hidroxiureia/uso terapêutico , Estudos Retrospectivos , Compostos Orgânicos Voláteis/uso terapêutico , Anemia Falciforme/tratamento farmacológico , Síndrome Torácica Aguda/tratamento farmacológico , Antidrepanocíticos/uso terapêutico
5.
J Pediatr Hematol Oncol ; 45(6): e723-e727, 2023 08 01.
Artigo em Inglês | MEDLINE | ID: mdl-36898038

RESUMO

Comorbid pulmonary complications in people with sickle cell disease (pwSCD) are associated with high rates of morbidity and mortality, and poor access to care contributes to poor outcomes among this particularly high-risk pwSCD. Our purpose was to describe the population served and the resources required for hematology, pulmonary, nursing, respiratory therapy, social work, genetics, psychology, and school liaison providers to see these patients in an integrated clinic. We abstracted demographic, medication, clinical, and diagnostics data of the pwSCD seen at least once in this clinic from February 1, 2014 to December 10, 2020 from the electronic medical record and identified 145 unique pwSCD. Abnormal lung function and bronchodilator responsiveness were detected in 31% and 42% of participants respectively. Sleep abnormalities were found in over two-thirds of those screened and 65% had ≥1 previous acute chest syndrome episode. This clinic also allowed for direct provider communication and required relatively limited resources to serve a large number of severely affected pwSCD. Given the degree of abnormal respiratory variables detected and the limited resources required to implement this model, studies are warranted to evaluate whether it has the potential to improve outcomes in high-risk populations.


Assuntos
Síndrome Torácica Aguda , Anemia Falciforme , Humanos , Anemia Falciforme/complicações , Anemia Falciforme/terapia , Síndrome Torácica Aguda/etiologia , Fatores de Risco , Pulmão
6.
Pediatr Blood Cancer ; 69(6): e29607, 2022 06.
Artigo em Inglês | MEDLINE | ID: mdl-35373884

RESUMO

BACKGROUND: Hydroxyurea is the primary treatment for sickle cell anemia (SCA), yet real-world implementation in high-income settings is suboptimal. Variation in prescribed hydroxyurea dose and patient adherence in these settings can both affect actual exposure to hydroxyurea. Quantifying the contributions of hydroxyurea dose and medication adherence to the relationship between hydroxyurea exposure and hematologic parameters could inform strategies to optimize exposure and improve outcomes. PROCEDURE: We evaluated the relationship between hydroxyurea exposure, defined by average prescribed dose and adherence, and hematologic parameters using data from children with SCA who were enrolled in two prospective hydroxyurea adherence studies. Hydroxyurea adherence was assessed by video directly observed therapy or electronic pill bottle and medication administration record. Average prescribed dose was abstracted from prescriptions in patients' electronic medical record. Participants with a hydroxyurea exposure >20 mg/kg/day and ≤20 mg/kg/day were included in the higher and lower exposure groups, respectively. RESULTS: Forty-five participants were included in the analysis (56% male; median age 12 years [range 2-19]; 98% Black). Higher exposed participants (n = 23) were prescribed a higher dose (27.2 vs. 24.4 mg/kg/day, p = .002) and had better adherence (0.92 vs. 0.71, p ≤ .001) compared to lower exposed participants (n = 22). Higher exposure was associated with higher fetal hemoglobin (p = .04) and mean corpuscular volume (p = .02). CONCLUSIONS: Higher hydroxyurea exposure is associated with improved hematologic parameters in the high-income setting and is affected by both prescribed dose and adherence. Future studies are needed to optimize both adherence and hydroxyurea prescribing and confirm that increasing exposure improves clinical outcomes in this setting.


Assuntos
Anemia Falciforme , Antidrepanocíticos , Hidroxiureia , Adolescente , Anemia Falciforme/tratamento farmacológico , Antidrepanocíticos/uso terapêutico , Criança , Pré-Escolar , Feminino , Hemoglobina Fetal , Humanos , Hidroxiureia/uso terapêutico , Masculino , Adesão à Medicação , Estudos Prospectivos , Adulto Jovem
7.
Pediatr Blood Cancer ; 69(2): e29446, 2022 02.
Artigo em Inglês | MEDLINE | ID: mdl-34854548

RESUMO

INTRODUCTION: For decades, it has been recommended that children with sickle cell anemia (SCA) receive antibiotic prophylaxis to prevent serious infections and undergo transcranial Doppler (TCD) screening to identify those at highest risk of overt stroke. We assessed recent temporal trends in antibiotic prophylaxis prescription fills and TCD screening among children with SCA using validated quality measures. PROCEDURE: Using validated claims-based definitions, we identified children with SCA who were enrolled in Michigan or New York State (NYS) Medicaid programs (2011-2018). Among recommended age groups, two outcomes were assessed yearly: (a) filling of ≥300 days of antibiotics, and (b) receipt of greater than or equal to one TCD. The proportion of children with each outcome was calculated by state. Temporal trends in each preventive service were assessed using generalized linear models. RESULTS: A total of 1784 children were eligible for antibiotic prophylaxis (Michigan: 384; NYS: 1400), contributing 3322 person-years. Annual rates of filling ≥300 days of antibiotics ranged from 16% to 22% and were similar by state. There was no change in rates of antibiotic filling over time in Michigan (p-value: .10), but there was a decrease in NYS (p-value: .02). A total of 3439 children with SCA were eligible for TCD screening (Michigan: 710; NYS: 2729), contributing 10,012 person-years. Annual rates of TCD screening ranged from 39% to 45%, were similar by state, and did not change over time (p-values >.05). CONCLUSIONS: Most children with SCA do not receive recommended antibiotic prophylaxis and/or TCD screening. New, sustainable, and coordinated interventions across preventive services are urgently needed.


Assuntos
Anemia Falciforme , Acidente Vascular Cerebral , Anemia Falciforme/diagnóstico , Anemia Falciforme/terapia , Antibacterianos/uso terapêutico , Criança , Humanos , Programas de Rastreamento , Acidente Vascular Cerebral/prevenção & controle , Ultrassonografia Doppler Transcraniana
8.
Telemed J E Health ; 28(8): 1166-1171, 2022 08.
Artigo em Inglês | MEDLINE | ID: mdl-34986030

RESUMO

Introduction: Sickle cell anemia (SCA) is a genetic condition that predominantly affects minority populations in the United States. A lack of access to care is strongly associated with poor outcomes and quality of care among children and adolescents with SCA. The use of telehealth, which has rapidly expanded during the COVID-19 pandemic, has been shown to improve access to care for many conditions. However, the adoption of telehealth among children and adolescents with SCA is unknown. Methods: We identified children 1-17 years old with SCA continuously enrolled in Michigan Medicaid from January 2019 to December 2020. The number of in-person and telehealth outpatient visits (both urgent and routine) were summarized prepandemic (January 2019-February 2020) and during the pandemic (March 2020-December 2020); National Provider Identifier was used to identify provider specialty for telehealth visits. Results: The study population comprised 493 children with SCA with a mean age of 8.7 (±4.9) years at study entry. Prepandemic, there were 4,367 outpatient visits; 4,348 (99.6%) were in-person and 19 (0.4%) were telehealth. During the pandemic, there were 2,307 outpatient visits; 2,059 (89.3%) were in-person and 248 (10.7%) were telehealth. Telehealth visits peaked in April 2020 and declined thereafter. The majority of telehealth visits were to hematology (49%), followed by adult subspecialists (27%) and pediatrics/family medicine (14%). Discussion/Conclusions: While the overall number of outpatient visits declined during the initial months of the pandemic compared with 2019, use of telehealth rapidly increased among children and adolescents with SCA. Additional research is needed to understand patient and provider preferences for telehealth and the roles that federal and state policies can play in facilitating telehealth adoption among children and adolescents with SCA.


Assuntos
Anemia Falciforme , COVID-19 , Telemedicina , Adolescente , Adulto , Anemia Falciforme/epidemiologia , Anemia Falciforme/terapia , COVID-19/epidemiologia , Criança , Pré-Escolar , Humanos , Lactente , Medicaid , Pandemias , Estados Unidos/epidemiologia
9.
Pain Med ; 21(10): 2583-2592, 2020 10 01.
Artigo em Inglês | MEDLINE | ID: mdl-32142138

RESUMO

OBJECTIVE: To assess the impact of Ohio's 2012, 2013, and 2016 opioid prescribing guidelines on opioid and nonsteroidal anti-inflammatory drug (NSAID) prescription filling and health care utilization for pain among children with sickle cell disease (SCD). DESIGN: Quasi-experimental retrospective cohort study. SETTING: Ohio Medicaid claims data from August 2011 to August 2016. SUBJECTS: Medicaid beneficiaries under age 19 years with SCD. METHODS: Interrupted time series analyses comparing population-level rates of opioids and NSAID prescriptions filled, standardized amounts of opioids dispensed, and acute health care utilization for pain before and after release of each guideline. RESULTS: In our cohort of 1,505 children with SCD, there was a temporary but significant decrease in the opioid filling rate (-2.96 prescriptions per 100 children, P = 0.01) and in the amount of opioids dispensed (-31.39 milligram morphine equivalents per filled prescription, P < 0.001) after the 2013 guideline but a temporary but significant increase in the opioid filling rate (7.44 prescriptions per 100 children, P < 0.001) and in the amount of opioids dispensed (72.73 mg morphine equivalents per filled prescription, P < 0.001) after the 2016 guideline. The NSAID filling rate did not significantly change after any of the guidelines. Acute health care utilization rates for pain after the 2016 guideline were similar to those before the 2013 guideline (rate ratio = 1.04, P = 0.63). CONCLUSIONS: Our results suggest that Ohio's 2013 and 2016 guidelines were associated with significant but nonsustained changes in opioid prescription filling among children with SCD. Additional studies are needed to confirm that opioid guidelines have a sustained impact on excessive opioid prescribing, filling, and misuse.


Assuntos
Analgésicos Opioides , Anemia Falciforme , Adulto , Analgésicos Opioides/uso terapêutico , Anemia Falciforme/tratamento farmacológico , Criança , Prescrições de Medicamentos , Humanos , Ohio , Manejo da Dor , Padrões de Prática Médica , Prescrições , Estudos Retrospectivos , Estados Unidos , Adulto Jovem
10.
J Pediatr Psychol ; 44(10): 1196-1204, 2019 11 01.
Artigo em Inglês | MEDLINE | ID: mdl-31403687

RESUMO

OBJECTIVE: Adolescents with sickle cell disease (SCD) are at increased risk for complications. Hydroxyurea is a medication that can ameliorate risk but to benefit, adolescents must adhere to treatment. Study aims were to describe how adolescents and their caregivers decided who was responsible for treatment tasks, to describe adolescents' and caregivers' responsibility for these tasks, and to examine if hydroxyurea adherence was associated with younger adolescent age, less discrepancy between adolescents' and caregivers' reports of adolescent responsibility, and higher caregiver involvement. METHODS: Twenty-nine dyads completed treatment responsibility measures. A combination of laboratory and electronic prescription data were used to determine hydroxyurea adherence and electronic medical records were used to determine appointment adherence. RESULTS: Few dyads agreed or planned how to complete treatment tasks. Adolescents shared responsibility with caregivers for medication-taking tasks. Adolescents perceived caregivers and caregivers perceived adolescents were overall responsible for treatment, especially for appointment tasks. Half of adolescents were adherent to hydroxyurea and half were adherent to appointments but medication adherence was not associated with age, discrepancy between adolescents' and caregivers' responses, or caregiver involvement. CONCLUSIONS: Despite frequent hydroxyurea and appointment nonadherence, few adolescents and caregivers plan how to manage adolescents' SCD treatment or perceive they are overall responsible. Future studies are needed to determine the factors that influence these perceptions and if increasing adolescent and caregiver treatment planning improves adherence and clinical outcomes.


Assuntos
Anemia Falciforme/tratamento farmacológico , Cuidadores , Hidroxiureia/uso terapêutico , Adesão à Medicação/psicologia , Adolescente , Criança , Feminino , Humanos , Masculino , Adulto Jovem
11.
Pediatr Blood Cancer ; 65(2)2018 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-28944997

RESUMO

BACKGROUND/OBJECTIVE: Sickle cell disease (SCD) and hydroxyurea have implications for fertility and reproductive health. The goal of this study was to examine desire for parenthood and reproductive health knowledge among a cohort of adolescent and young adult (AYA) with SCD receiving hydroxyurea and their caregivers at a large pediatric academic center. METHODS: Patients with SCD were approached from September 2016 to July 2017 if they were: (1) 12-20 years old, (2) prescribed hydroxyurea for at least 6 months, (3) proficient in English, and (4) accompanied by a caregiver who was proficient in English and willing to participate.  Participants self-reported sociodemographic characteristics and completed surveys to assess their/their child's desire for parenthood and other life goals, and reproductive health knowledge. RESULTS: Eighteen patient-caregiver dyads completed the study (78.3% of those eligible); 61.1% indicated that they wanted to have future biological children.  Few participants reported receiving information about fertility (16.7% of AYA and 27.8% of caregivers) or birth control (11.1% of AYA and 22.2% of caregivers) from their/their child's health care provider, and the majority had received no information on these topics. Less than half of participants reported that SCD (22.2% of AYA and 50.0% of caregivers) or hydroxyurea (11.1% of AYA and 27.8% of parents) could potentially impair fertility. CONCLUSIONS: Biological parenthood was important to this cohort yet fertility and reproductive health knowledge was low, suggesting that clinicians should prioritize conversations about infertility risk and birth control options with AYA with SCD on hydroxyurea and their caregivers.  More research is needed to identify optimal approaches to these discussions.


Assuntos
Anemia Falciforme , Cuidadores , Anticoncepção , Fertilidade , Educação de Pacientes como Assunto , Saúde Reprodutiva/educação , Adolescente , Adulto , Feminino , Humanos , Hidroxiureia/administração & dosagem , Masculino , Adulto Jovem
13.
Am J Hematol ; 92(9): E520-E528, 2017 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-28589652

RESUMO

Nephropathy is a common and progressive complication of sickle cell anemia (SCA). In SCA mice, we found that hyperangiotensinemia in the absence of hypertension underlies nephropathy, and its downregulation by losartan, an angiotensin-II-receptor-1 blocker, reduced albuminuria and progression of nephropathy. Therefore, we performed a phase-2 trial of oral losartan, given for 6 months, to explore whether it reduced albuminuria in children and adults with SCA. Participants were allocated to groups defined by class of baseline urinary albumin-to-creatinine ratio (UACR): no albuminuria (NoA), microalbuminuria (MicroA), and macroalbuminuria (MacroA). The primary endpoint was a ≥25% reduction UACR from baseline. There were 32 evaluable participants (mean age 24 years; NoA = 14, MicroA = 12, MacroA = 6). The primary endpoint was met in 83% of the MacroA group (P < 0.0001) and 58% of the MicroA group (P < 0.0001). Median fold-change in UACR was -0.74 for MacroA and -0.46 for MicroA. In MacroA and MicroA, UACR classification improved in 50% but worsened in 11%. Urine osmolality and estimated glomerular filtration rate (eGFR) did not change significantly. Losartan was discontinued in three participants [leg cramps, N = 1; decline in eGFR >25% (142➝104 mL/minute/1.73 m2 ), N = 1; rise in serum creatinine >50% (0.2➝0.3 mg/dL), N = 1]. Albuminuria was associated with diastolic dysfunction and impaired functional capacity, although cardiopulmonary status was unchanged after 6 months of losartan therapy. In summary, losartan decreased urinary albumin excretion in most participants with albuminuria. Those with macroalbuminuria had the greatest benefit. This study forms the basis for a phase-3, randomized, placebo-controlled trial of losartan for the nephropathy of SCA.


Assuntos
Albuminúria , Anemia Falciforme , Losartan/administração & dosagem , Adolescente , Adulto , Fatores Etários , Albuminúria/tratamento farmacológico , Albuminúria/etiologia , Albuminúria/fisiopatologia , Albuminúria/urina , Anemia Falciforme/complicações , Anemia Falciforme/tratamento farmacológico , Anemia Falciforme/fisiopatologia , Anemia Falciforme/urina , Criança , Feminino , Taxa de Filtração Glomerular , Humanos , Masculino , Pessoa de Meia-Idade
14.
Pediatr Blood Cancer ; 63(5): 844-7, 2016 May.
Artigo em Inglês | MEDLINE | ID: mdl-26797936

RESUMO

BACKGROUND: Expert guidelines recommend that hydroxyurea (HU) be offered to all children with hemoglobin SS and Sß(0) sickle cell disease (SCD) and be considered for children with clinically severe hemoglobin SC or Sß(+) . This study aims to determine the rate of HU use in hospitalized children, if HU is differentially used in children with clinically severe SCD, and if HU users have shorter length of stay (LOS), fewer intensive care unit (ICU) admissions, and fewer inpatient transfusions compared to nonusers. PROCEDURE: Using the Pediatric Health Information System, we performed a retrospective analysis of children ages 2-18 years with SCD discharged between January 1, 2011 and September 30, 2014. We defined patients as having clinically severe SCD if they had a recent ICU admission or ≥3 admissions in the preceding year. RESULTS: Of the 2,665 unique children identified, approximately 80% had an inpatient code indicating HU use. Significantly more (p < 0.001) nonusers (30.1%) had a recent ICU admission compared to HU users (18.7%). More nonusers (33.9%) had a history of ≥3 admissions compared to HU users (21.5%) (p < 0.001). After applying propensity score weighting, the groups did not differ in their LOS, prevalence of ICU admissions, or prevalence of transfusions. CONCLUSIONS: HU use is high among hospitalized children with SCD. However, HU is not utilized by many children with clinically severe SCD. These results support that HU be considered in children with SCD to prevent hospitalization rather than as a treatment to improve hospitalization outcomes.


Assuntos
Anemia Falciforme/terapia , Transfusão de Sangue , Hidroxiureia/administração & dosagem , Admissão do Paciente , Alta do Paciente , Índice de Gravidade de Doença , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Estudos Retrospectivos
15.
J Pediatr Hematol Oncol ; 38(5): 402-5, 2016 07.
Artigo em Inglês | MEDLINE | ID: mdl-27164532

RESUMO

Hemoglobin F (HbF) concentration is used in the diagnosis of certain hemoglobinopathies and accurate quantification is central to treatment of patients with sickle cell disease. The 2 most commonly used methods to quantify HbF are high performance liquid chromatography and capillary zone electrophoresis. This study reports discrepancies in HbF quantification between these methods when hemoglobin S is present in the sample. Clinicians and investigators should be mindful of the method used for HbF quantification when evaluating and treating patients who produce hemoglobin S.


Assuntos
Cromatografia Líquida de Alta Pressão/métodos , Eletroforese Capilar/métodos , Hemoglobina Fetal/análise , Hemoglobina Falciforme/análise , Hemoglobinopatias/diagnóstico , Adolescente , Adulto , Idoso , Anemia Falciforme/diagnóstico , Criança , Pré-Escolar , Cromatografia Líquida de Alta Pressão/normas , Erros de Diagnóstico/prevenção & controle , Eletroforese Capilar/normas , Humanos , Lactente , Pessoa de Meia-Idade , Estudos Retrospectivos , Adulto Jovem
16.
Pediatr Blood Cancer ; 61(6): 1068-73, 2014 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-24436121

RESUMO

BACKGROUND: Poor hydroxyurea (HU) adherence limits effective HU use in patients with sickle cell disease (SCD). Electronic directly observed therapy (DOT) may limit costs and achieve high HU adherence in children with SCD. This study aimed to determine if electronic DOT was feasible, acceptable, and could achieve ≥ 90% HU adherence. PROCEDURE: Children with SCD were recruited for this single institution, 6-month pilot study if they had been prescribed HU for ≥ 6 months and had daily access to a smartphone or computer. Participants submitted HU administration videos daily and received electronic reminder alerts, personalized feedback, and incentives to encourage adherence as part of electronic DOT. Primary outcomes were feasibility, participant satisfaction with electronic DOT, and HU adherence. Secondary outcomes included mean corpuscular volume (MCV), hemoglobin F percentage (HbF), and overall participant satisfaction with HU therapy. RESULTS: Of 15 enrolled participants, 14 completed the study. Satisfaction surveys showed electronic DOT reminded participants to take HU and could be completed in fewer than 5 minutes daily. Participants' median medication possession ratio at study entry improved from 0.75 (0.59-0.82) to 0.91 (0.85-1.00) (P = 0.02) at the end of the study. Overall median observed HU adherence with electronic DOT was 93.3%. Median MCV and HbF increased from 96.0 to 107.2 (P = 0.009) and 10.5 to 11.4 (P = 0.03), respectively. CONCLUSIONS: This study demonstrates electronic DOT is feasible, acceptable, and can achieve high HU adherence. Further study is needed to confirm that electronic DOT can improve HU adherence and impact clinical outcomes in children with SCD.


Assuntos
Anemia Falciforme/tratamento farmacológico , Antidrepanocíticos/uso terapêutico , Terapia Diretamente Observada/métodos , Correio Eletrônico , Hidroxiureia/uso terapêutico , Adesão à Medicação , Sistemas de Alerta/instrumentação , Envio de Mensagens de Texto , Gravação em Vídeo , Adolescente , Telefone Celular , Criança , Pré-Escolar , Terapia Diretamente Observada/instrumentação , Esquema de Medicação , Retroalimentação , Feminino , Inquéritos Epidemiológicos , Humanos , Masculino , Microcomputadores , Satisfação do Paciente , Projetos Piloto , Reembolso de Incentivo , Recompensa , Adulto Jovem
17.
J Pediatr Hematol Oncol ; 36(6): 480-3, 2014 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-24633302

RESUMO

BACKGROUND: Acute chest syndrome (ACS) is associated with morbidity and mortality in children with sickle cell disease. We hypothesize that children with sickle cell disease have a distinct prodromal illness before their ACS episode. MATERIALS AND METHODS: We performed a chart review of ICD-9-CM identified ACS episodes at a pediatric hospital from 2005 to 2010. Prodromal visits were defined as acute visits that resulted in a discharge from care and occurred within 2 weeks of a hospitalization that included ACS. We reviewed the documented history, examination, laboratory studies, and radiographs for each prodromal visit. RESULTS: We identified 196 ACS episodes. Children received prodromal care in 29% of the ACS episodes. Painful vaso-occlusive crisis was a common reason for seeking this care (61%) and was commonly located in the chest or back (81%). We also observed that patients were hypoxic (53%), tachypneic (29%), had a history of asthma (39%) or ACS (80%), and presented during the winter months (38%). CONCLUSIONS: These data suggest that nearly one third of patients who develop ACS seek care for a prodromal illness. Further research is needed to confirm and better define an ACS prodromal illness that may help to identify patients at high risk for developing ACS.


Assuntos
Síndrome Torácica Aguda/diagnóstico por imagem , Síndrome Torácica Aguda/etiologia , Anemia Falciforme/complicações , Sintomas Prodrômicos , Asma/complicações , Dor nas Costas/diagnóstico por imagem , Dor nas Costas/etiologia , Dor no Peito/diagnóstico por imagem , Dor no Peito/etiologia , Criança , Criança Hospitalizada , Pré-Escolar , Diagnóstico Precoce , Feminino , Humanos , Hipóxia/etiologia , Lactente , Recém-Nascido , Masculino , Radiografia , Estudos Retrospectivos , Taquipneia/etiologia
19.
J Pediatr Adolesc Gynecol ; 36(4): 393-398, 2023 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-36934802

RESUMO

STUDY OBJECTIVE: Reproductive health counseling is important for youth with sickle cell disease (SCD) given that they experience potential infertility risks from SCD and its treatments and high rates of unplanned pregnancies. Thus, the objective of this study was to describe documented occurrences of reproductive health counseling among youth with SCD and examine differences in counseling by sociodemographic and treatment characteristics. METHODS: Data were abstracted from 167 electronic medical records of patients (age = 14-21, 54% female) with SCD (1/01/2015-12/31/19) at 2 sites (Nationwide Children's Hospital and Hassenfeld Children's Hospital at NYU Langone). Descriptive statistics, point-biserial correlations, and χ2 tests were used to examine sample characteristics and relationships between sociodemographic factors, clinical characteristics, site, and reproductive health counseling (fertility, contraception, and genetic counseling). RESULTS: Seven of 167 (4%) youth had documented discussions about the potential impacts of SCD on fertility. Fertility counseling was also low among those who received a bone marrow transplant or hydroxyurea (n = 1/2, 50%; and n = 1/104, 1%, respectively). Only 57% of youth received contraception counseling, and only 55% of sexually active youth used birth control; birth control use was associated with older age (P = .028), severe clinical disease (P = .003), and documentation of contraception counseling (P = .047). Most youth received genetic counseling (80%), although more genetic counseling occurred at Nationwide Children's Hospital (P < .001). There was no association between gender and any type of counseling. CONCLUSION: Findings suggest reproductive health counseling gaps in this population, with important implications for future infertility distress and unplanned pregnancies. Future research should examine barriers to counseling, explore fertility impacts of SCD and treatments, and inform evidence-based guidelines for reproductive health care in SCD.


Assuntos
Anemia Falciforme , Infertilidade , Gravidez , Criança , Humanos , Feminino , Adolescente , Adulto Jovem , Adulto , Masculino , Saúde Reprodutiva , Serviços de Planejamento Familiar , Anemia Falciforme/complicações , Anemia Falciforme/terapia , Anemia Falciforme/genética , Aconselhamento
20.
Blood Adv ; 7(20): 6140-6150, 2023 Oct 24.
Artigo em Inglês | MEDLINE | ID: mdl-37585480

RESUMO

Patients with sickle cell disease (SCD) are at a risk of thromboembolism (TE), and use of hormonal contraception can further increase that risk. This study aims to assess patterns of hormonal contraceptive use and compare risk of contraception-related TE between combined hormonal contraceptives (CHCs) and progestin-only contraceptives (POCs). Patients with SCD aged between 12 and 44 years with a new prescription of a hormonal contraceptive in the Centers for Medicare and Medicaid Services Medicaid Analytic eXtract database (2006-2018) were followed up to 1 year. We identified 7173 new users: 44.6% initiated CHC and 55.4% initiated POC. Combined oral contraceptive pills (OCPs; 36.5%) and progestin-only depot medroxyprogesterone acetate (33.9%) were the most frequently prescribed agents. A total of 1.8% of contraception users had a new diagnosis of TE within 1 year of the first identified contraception prescription. There were no significant differences in TE event rates between CHC and POC users (17.2 and 24.7 events per 1000 person-years, respectively). In patients prescribed OCP, there were no differences in TE event rates based on estrogen dose or progestin generation. Transdermal patch had a 2.4-fold increased risk of TE as compared with that of OCP. Although limited by the retrospective study design and use of administrative claims data, this study found no significant differences in TE rates between new users of CHC and POC in patients with SCD. Careful evaluation of underlying TE risk factors should be considered for each patient with SCD before initiation of hormonal contraception.


Assuntos
Anemia Falciforme , Tromboembolia , Estados Unidos/epidemiologia , Feminino , Humanos , Idoso , Criança , Adolescente , Adulto Jovem , Adulto , Progestinas/efeitos adversos , Contracepção Hormonal , Anticoncepcionais Orais Hormonais/efeitos adversos , Estudos Retrospectivos , Medicare , Tromboembolia/epidemiologia , Tromboembolia/etiologia , Anemia Falciforme/complicações , Anemia Falciforme/tratamento farmacológico , Anemia Falciforme/epidemiologia
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