scholarly journals Geriatric Assessment for Older Adults with Sickle Cell Disease: protocol for a prospective cohort pilot study

2020 ◽  
Author(s):  
Charity I Oyedeji ◽  
Katherine Hall ◽  
Alison Luciano ◽  
Miriam Morey ◽  
John Joseph Strouse
Keyword(s):  
Sickle Cell Disease ◽  
Geriatric Assessment ◽  
Sickle Cell ◽  
Prospective Cohort ◽  
Weight Status ◽  
Functional Decline ◽  
Psychological State ◽  
Cell Disease ◽  
Multiple Organs ◽  
Age Related

Abstract Background: The life expectancy for people with sickle cell disease (SCD) has improved tremendously over the last 50 years. This population experiences hemolysis and vaso-occlusion in multiple organs that lead to complications such as cardiopulmonary disease, strokes, and avascular necrosis. These complications can limit mobility and aerobic endurance, similar to limitations that often occur in geriatric populations. These sickle-cell and age-related events lead to frequent hospitalization, which further increases the risk of functional decline. We have few tools to measure functional decline in people with SCD. The purpose of this paper is to describe a protocol to evaluate the feasibility of sickle cell disease geriatric assessment (SCD-GA).Methods/Design: We will enroll 40 adults with SCD (20 age 18-49 years and 20 age ≥ 50 years) in a prospective cohort study to assess the feasibility of SCD-GA. The SCD-GA includes validated measures from the oncology geriatric assessment enriched with additional physical and cognitive measures. The SCD-GA will be performed at the first study visit, 10 to 20 days after hospitalization, and at after 12-months (exit visit). With input from a multidisciplinary team of sickle cell specialists, geriatricians, and experts in physical function and physical activity, we selected assessments across 7 domains: functional status (11 measures), comorbid medical conditions (1 measure), psychological state (1 measure), social support (2 measures), weight status (2 measures), cognition (3 measures), and medications (1 measure). We will measure the proportion completing the assessment with feasibility as the primary outcome. Secondary outcomes include the proportion consenting and completing all study visits, duration of the assessment, acceptability, and adverse events.Discussion: We present the protocol and rationale for selection of the measures included in SCD-GA. We also outline the methods to determine feasibility and subsequently to optimize the SCD-GA in preparation for a larger multicenter validation study of the SCD-GA.

scholarly journals Geriatric Assessment for Older Adults with Sickle Cell Disease: protocol for a prospective cohort pilot study

2020 ◽  
Author(s):  
Charity I Oyedeji ◽  
Katherine Hall ◽  
Alison Luciano ◽  
Miriam Morey ◽  
John Joseph Strouse
Keyword(s):  
Sickle Cell Disease ◽  
Geriatric Assessment ◽  
Sickle Cell ◽  
Prospective Cohort ◽  
Weight Status ◽  
Functional Decline ◽  
Psychological State ◽  
Cell Disease ◽  
Multiple Organs ◽  
Age Related

Abstract Background The life expectancy for people with sickle cell disease (SCD) has improved tremendously over the last 50 years. This population experiences hemolysis and vaso-occlusion in multiple organs that lead to complications such as cardiopulmonary disease, strokes, and avascular necrosis. These complications can limit mobility and aerobic endurance, similar to limitations that often occur in geriatric populations. These sickle-cell and age-related events lead to frequent hospitalization, which further increases the risk of functional decline. We have few tools to measure functional decline in people with SCD. The purpose of this paper is to describe a protocol to evaluate the feasibility of sickle cell disease geriatric assessment (SCD-GA). Methods/Design We will enroll 40 adults with SCD (20 age 18-49.99 years and 20 age ≥ 50 years) in a prospective cohort study to assess the feasibility of SCD-GA. The SCD-GA includes validated measures from the oncology geriatric assessment enriched with additional physical and cognitive measures. The SCD-GA will be performed at the first study visit, 10 to 20 days after hospitalization, and at after 12-months (exit visit). With input from a multidisciplinary team of sickle cell specialists, geriatricians, and experts in physical function and physical activity, we selected assessments across 7 domains: functional status (11 measures), comorbid medical conditions (1 measure), psychological state (1 measure), social support (2 measures), weight status (2 measures), cognition (3 measures), and medications (1 measure). We will measure the proportion completing the assessment with feasibility as the primary outcome. Secondary outcomes include the proportion consenting and completing all study visits, duration of the assessment, acceptability, and adverse events. Discussion We present the protocol and rationale for selection of the measures included in SCD-GA. We also outline the methods to determine feasibility and subsequently to optimize the SCD-GA in preparation for a larger multicenter validation study of the SCD-GA.

scholarly journals Geriatric Assessment for Older Adults with Sickle Cell Disease: protocol for a prospective cohort pilot study

2020 ◽  
Author(s):  
Charity I Oyedeji ◽  
Katherine Hall ◽  
Alison Luciano ◽  
Miriam Morey ◽  
John Joseph Strouse
Keyword(s):  
Sickle Cell Disease ◽  
Geriatric Assessment ◽  
Sickle Cell ◽  
Prospective Cohort ◽  
Weight Status ◽  
Functional Decline ◽  
Psychological State ◽  
Cell Disease ◽  
Multiple Organs ◽  
Age Related

Abstract Background The life expectancy for people with sickle cell disease (SCD) has improved tremendously over the last 50 years. This population experiences hemolysis and vaso-occlusion in multiple organs that lead to complications such as cardiopulmonary disease, strokes, and avascular necrosis. These complications can limit mobility and aerobic endurance, similar to limitations that often occur in geriatric populations. These sickle-cell and age-related events lead to frequent hospitalization, which further increases the risk of functional decline. We have few tools to measure functional decline in people with SCD. The purpose of this paper is to describe a protocol to evaluate the feasibility of sickle cell disease geriatric assessment (SCD-GA).Methods/Design Forty adults with SCD (20 age 18–49 years and 20 age ≥ 50 years) will be enrolled in a prospective cohort study to assess the feasibility of SCD-GA. We used a validated oncology geriatric assessment and enriched with additional physical and cognitive measures. The SCD-GA will be performed at the first study visit, 10 to 20 days after hospitalization, and at after 12-months (exit visit). With input from a multidisciplinary team of sickle cell specialists, geriatricians, and experts in physical function and physical activity, we selected assessments across 7 domains: functional status (11 measures), comorbid medical conditions (1 measure), psychological state (1 measure), social support (2 measures), weight status (2 measures), cognition (3 measures), and medications (1 measure). We will measure the proportion completing the assessment with feasibility as the primary outcome. Secondary outcomes include the proportion consenting and completing all study visits, duration of the assessment, acceptability, and adverse events.Discussion We present the protocol and rationale for selection of the measures included in SCD-GA. We also outline the methods to determine feasibility and subsequently to optimize the SCD-GA in preparation for a larger multicenter validation study of the SCD-GA.

scholarly journals Geriatric assessment for older adults with sickle cell disease: protocol for a prospective cohort pilot study

2020 ◽  
Vol 6 (1) ◽  
Author(s):  
Charity I. Oyedeji ◽  
Katherine Hall ◽  
Alison Luciano ◽  
Miriam C. Morey ◽  
John J. Strouse
Keyword(s):  
Sickle Cell Disease ◽  
Geriatric Assessment ◽  
Sickle Cell ◽  
Prospective Cohort ◽  
Weight Status ◽  
Functional Decline ◽  
Psychological State ◽  
Cell Disease ◽  
Multiple Organs ◽  
Age Related

Abstract Background The life expectancy for people with sickle cell disease (SCD) has improved tremendously over the last 50 years. This population experiences hemolysis and vaso-occlusion in multiple organs that lead to complications such as cardiopulmonary disease, strokes, and avascular necrosis. These complications can limit mobility and aerobic endurance, similar to limitations that often occur in geriatric populations. These sickle-cell and age-related events lead to frequent hospitalization, which further increases the risk of functional decline. We have few tools to measure functional decline in people with SCD. The purpose of this paper is to describe a protocol to evaluate the feasibility of sickle cell disease geriatric assessment (SCD-GA). Methods/design We will enroll 40 adults with SCD (20 age 18–49.99 years and 20 age ≥ 50 years) in a prospective cohort study to assess the feasibility of SCD-GA. The SCD-GA includes validated measures from the oncology geriatric assessment enriched with additional physical and cognitive measures. The SCD-GA will be performed at the first study visit, at 10 to 20 days after hospitalization, and at 12 months (exit visit). With input from a multidisciplinary team of sickle cell specialists, geriatricians, and experts in physical function and physical activity, we selected assessments across 7 domains: functional status (11 measures), comorbid medical conditions (1 measure), psychological state (1 measure), social support (2 measures), weight status (2 measures), cognition (3 measures), and medications (1 measure). We will measure the proportion completing the assessment with feasibility as the primary outcome. Secondary outcomes include the proportion consenting and completing all study visits, duration of the assessment, acceptability, and adverse events. Discussion We present the protocol and rationale for selection of the measures included in SCD-GA. We also outline the methods to determine feasibility and subsequently to optimize the SCD-GA in preparation for a larger multicenter validation study of the SCD-GA.

Age-related prescription medication utilization for the management of sickle cell disease among Texas Medicaid patients

2021 ◽  
Vol 17 (4) ◽  
pp. 301-310
Author(s):  
Nidhi Shukla, MS, MBA ◽  
Jamie C. Barner, PhD, FAACP, FAPhA ◽  
Kenneth A. Lawson, PhD, FAPhA ◽  
Karen L. Rascati, PhD
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Age Groups ◽  
Dual Therapy ◽  
Cell Disease ◽  
Opioid Use ◽  
Medication Utilization ◽  
Age Related ◽  
Nonopioid Analgesics ◽  
Chi Square Analysis

Introduction: Sickle cell disease (SCD) is associated with recurrent complications and healthcare burden. Although SCD management guidelines differ based on age groups, little is known regarding actual utilization of preventative (hydroxyurea) and palliative therapies (opioid and nonopioid analgesics) to manage complications. This study assessed whether there were age-related differences in SCD index therapy type and SCD-related medication utilization.Design and patients: Texas Medicaid prescription claims from September 1, 2011 to August 31, 2016 were retrospectively analyzed for SCD patients aged 2-63 years who received one or more SCD-related medications (hydroxyurea, opioid, or nonopioid analgesics).Outcome measures: The primary outcomes were SCD index drug type and medication utilization: hydroxyurea adherence, and days’ supply of opioid, and nonopioid analgesics. Chi-square, analysis of variance, and Kruskal–Wallis tests were used.Results: Index therapy percentages for included patients (N = 2,339) were the following: opioids (45.7 percent), nonopioids (36.6 percent), dual therapy-opioids and nonopioids (11.2 percent), and hydroxyurea (6.5 percent), and they differed by age-groups (χ2 = 243.0, p 0.0001). Hydroxyurea as index therapy was higher among children (2-12:9.1 percent) compared to adults (26-40:3.7 percent; 41-63:2.9 percent). Opioids as index therapy were higher among adults (18-25:48.0 percent; 26-40:54.9 percent; 41-63:65.2 percent) compared to children (2-12:36.6 percent). Mean hydroxyurea adherence was higher (p 0.0001) for younger ages, and opioid days’ supply was higher for older ages.Conclusions: Texas Medicaid SCD patients had low hydroxyurea utilization and adherence across all age groups. Interventions to increase the use of hydroxyurea and newer preventative therapies could result in better management of SCDrelated complications and reduce the frequency of pain crises, which may reduce the need for opioid use.

Overlapping Onset and Sequential Progression of Vasculopathy in Multiple Organs in Sickle Cell Disease

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 3660-3660
Author(s):  
Shruti Chaturvedi ◽  
Djamila Ghafuri ◽  
Adetola A. Kassim ◽  
Michael DeBaun
Keyword(s):  
Pulmonary Hypertension ◽  
Magnetic Resonance ◽  
Sickle Cell Disease ◽  
Mortality Rate ◽  
Sickle Cell ◽  
Conflicts Of Interest ◽  
Age Of Onset ◽  
Cell Disease ◽  
Multiple Organs ◽  
Hydroxyurea Therapy

Abstract Background: Sickle cell disease (SCD) is associated with vasculopathy in multiple vital organs, which ultimately leads to complications such as stroke, proliferative retinopathy, chronic kidney disease and pulmonary hypertension. Existing studies focus on single organ specific vasculopathy without an emphasis on shared mechanisms and simultaneous progression of vasculopathy in multiple organs. We conducted this retrospective cohort study to determine the onset and progression, as well as sequence of involvement of vasculopathy in the central nervous system (CNS), eye, kidney and lungs of adults with SCD. Methods: Our institutional practice is to perform annual magnetic resonance imaging with magnetic resonance angiography (MRI/MRA, for CNS vasculopathy and silent cerebral infarcts), echocardiography (for tricuspidregurgitant jet velocity > 2.5 m/sec, a surrogate of pulmonary hypertension), retinal examination, and measurement of urinaryalbumin:creatinine ratio, and serum creatinine in all adults with SCD. All patients were followed until death or last clinical encounter. Data were summarized as counts and proportions. Multivariable logistic regression was used to identify associations of number of organs affected with mortality. Results: We identified 280 adults with SCD followed for a median period of 66 months (interquartile range [IQR] 15.7 to 112 months). Median age was 31.1 (IQR 25.4 to 39.7) years and 49.6% were female. Over half (51.8%) were on hydroxyurea therapy. The prevalence of vasculopathy in different organs was: CNS, 37.8%; retinopathy 26.1%, proteinuria, 20.7% (nephropathy 5.71%); and pulmonary hypertension, 15.36%. There was no evidence of vasculopathy in 103 (36.8%) individuals. Of the remaining 177 (63.2%) adults, vasculopathy was present in one, two, three and all four end organs in100, 55, 18, and 4 individuals respectively. Median age of onset was earliest for CNS vasculopathy [25.42 (IQR 19.31, 38.85)] years followed by retinopathy [28.41 (IQR 23.04, 35.79)] years, proteinuria [31.25 (IQR 25.6, 46.0)] years, and pulmonary hypertension [33.08 (IQR 23.83, 47.17)] years (Figure 1). Mortality rate was 1.69 per 100 patient-years. Patients with vasculopathy affecting 3 or 4 organs had a significantly higher mortality rate than those with 0-2 organs affected by vasculopathy [odds ratio 5.50 (95%CI 4.49-20.35), p=0.007], adjusted for phenotype, age, sex, hydroxyurea therapy, and smoking status. Conclusion: Vasculopathy in SCD occurs in multiple organs simultaneously, with a predisposition to affectthe CNS first. These data strongly support that multiple vasculopathy is common, and when present in at least three organs, is associated with earlier mortality. Disclosures No relevant conflicts of interest to declare.

Factors predictive of leg-ulcer healing in sickle cell disease: a multicentre, prospective cohort study

2017 ◽  
Vol 177 (1) ◽  
pp. 206-211 ◽  
Author(s):  
P. Senet ◽  
C. Blas-Chatelain ◽  
P. Levy ◽  
E.M. Manea ◽  
M. Peschanski ◽  
...  
Keyword(s):  
Cohort Study ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Prospective Cohort Study ◽  
Ulcer Healing ◽  
Prospective Cohort ◽  
Leg Ulcer ◽  
Cell Disease

scholarly journals Low FEV1 is Associated with Fetal Death in Pregnant Women with Sickle Cell Disease: a prospective cohort study

Authorea ◽  
2020 ◽  
Author(s):  
Charles Hayfron Benjamin ◽  
Eugenia Asare ◽  
Theodore Boafor ◽  
Edeghonghon Olayemi ◽  
Yvonne Dei Adomakoh ◽  
...  
Keyword(s):  
Cohort Study ◽  
Sickle Cell Disease ◽  
Pregnant Women ◽  
Sickle Cell ◽  
Prospective Cohort Study ◽  
Prospective Cohort ◽  
Fetal Death ◽  
Cell Disease

scholarly journals Quantification and Comprehensive Analysis of Mesenchymal Stromal Cells in Bone Marrow Samples from Sickle Cell Disease Patients with Osteonecrosis

2020 ◽  
Vol 2020 ◽  
pp. 1-12
Author(s):  
Tiago O. Ribeiro ◽  
Paula B. Daltro ◽  
Gildasio Cerqueira Daltro ◽  
Songeli M. Freire ◽  
Roberto Meyer ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Mesenchymal Stromal Cells ◽  
Stromal Cells ◽  
Cell Number ◽  
Cell Phenotype ◽  
Cell Disease ◽  
Age Related

The potential use of bone marrow mesenchymal stromal cells (BM-MSCs) for the treatment of osteonecrosis in sickle cell disease (SCD) patients is increasing. However, convenient BM-MSC quantification and functional property assays are critical factors for cell-based therapies yet to be optimized. This study was designed to quantify the MSC population in bone marrow (BM) samples from SCD patients with osteonecrosis (SCD group) and patients with osteoarticular complications not related to SCD (NS group), using flow cytometry for CD271+CD45-/low cell phenotype and CFU-F assay. We also compared expanded BM-MSC osteogenic differentiation, migration, and cytokine secretion potential between these groups. The mean total cell number, CFU-F count, and CD271+CD45-/low cells in BM mononuclear concentrate were significantly higher in SCD than in NS patients. A significant correlation between CD271+CD45-/low cell number and CFU-F counts was found in SCD ( r = 0.7483 ; p = 0.0070 ) and NS ( r = 0.7167 ; p = 0.0370 ) BM concentrates. An age-related quantitative reduction of CFU-F counts and CD271+CD45-/low cell number was noted. Furthermore, no significant differences in the morphology, replicative capacity, expression of surface markers, multidifferentiation potential, and secretion of cytokines were found in expanded BM-MSCs from SCD and NS groups after in vitro culturing. Collectively, this work provides important data for the suitable measurement and expansion of BM-MSC in support to advanced cell-based therapies for SCD patients with osteonecrosis.

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