scholarly journals A multi‐institutional comparison of younger and older adults with sickle cell disease

2019 ◽  
Vol 94 (4) ◽  
Author(s):  
Charity Oyedeji ◽  
John Joseph Strouse ◽  
Regina D. Crawford ◽  
Melanie E. Garrett ◽  
Allison E. Ashley‐Koch ◽  
...  
Keyword(s):  
Older Adults ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease

scholarly journals A descriptive study of the characteristics of older adults with sickle cell disease

2017 ◽  
Vol 93 (2) ◽  
pp. E38-E40 ◽  
Author(s):  
Anjum B. Khan ◽  
Rachel Kesse-Adu ◽  
Cormac Breen ◽  
Patrick B. Murphy ◽  
John Chambers ◽  
...  
Keyword(s):  
Older Adults ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Descriptive Study ◽  
Cell Disease

scholarly journals Self-Care Recommendations of Middle-Aged and Older Adults with Sickle Cell Disease

2011 ◽  
Vol 2011 ◽  
pp. 1-5 ◽  
Author(s):  
Coretta M. Jenerette ◽  
Cheryl Brewer ◽  
Ashley N. Leak
Keyword(s):  
Older Adults ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Care Management ◽  
Self Care ◽  
The Self ◽  
Cell Disease ◽  
Middle Aged ◽  
Younger Adults ◽  
Semistructured Interviews

Self-care management is an important part of living with a chronic illness. Sickle cell disease (SCD) is a chronic disease with acute, painful exacerbations that often results in a shortened life expectancy. Some middle-aged and older adults with SCD lived with the disease prior to having a diagnosis and without modern advances. The purpose of this study is to share the self-care recommendations of middle-aged and older adults with SCD. Using descriptive qualitative methods, data were gathered through semistructured interviews from 11 individuals living with SCD, including 6 women and 5 men. Self-care recommendations themes included physiological, psychological, and provider-related. The self-care recommendations may be seen as an additional resource or “words of wisdom” for younger adults with SCD who can use the recommendations to better manage their own disease. Additionally, providers may be able to use these recommendations to inform their practice.

scholarly journals Functional Assessment in Younger and Older Adults with Sickle Cell Disease

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 26-27
Author(s):  
Charity I Oyedeji ◽  
Carl Pieper ◽  
Katherine Hall ◽  
Miriam Morey ◽  
Heather Whitson ◽  
...  
Keyword(s):  
Older Adults ◽  
Sickle Cell Disease ◽  
Avascular Necrosis ◽  
Sickle Cell ◽  
Functional Assessment ◽  
Activity Monitoring ◽  
Step Count ◽  
Cell Disease ◽  
Younger Adults ◽  
Age Related

Background Nearly 95% of individuals with sickle cell disease (SCD) live to become adults (age ≥ 18 years). As individuals with SCD age, they acquire both SCD and age-related complications leading to functional decline. There is a growing need for stragies to improve their function and quality of life.The purpose of this study was to assess the feasibility and acceptability of a sickle cell disease functional assessment (SCD-FA) in both younger and older adults with SCD. Methods We enrolled 20 younger adults (age 18-49 years) and 20 older adults (age ≥ 50 years) in a prospective cohort study. We included measures previously validated in functional geriatric assessment for oncology patients enriched with additional physical and cognitive functional measures. We monitored physical activity for 7 days using the Actigraph wT3X-BT. The primary endpoint was the proportion of subjects who complete the assessment. Secondary endpoints were duration of the SCD-FA, proportion who completed activity monitoring and biospecimen collection, and acceptability. Results Eighty percent (44/55) of adults approached for the study consented, and 91% (40) of consented participants completed the SCD-FA. The median duration of the assessment was 89 minutes (IQR 80-98 minutes), and there were no adverse events. A comparison of characteristics of younger and older participants is shown in Table 1. The mean usual gait speed (GS) was similar between younger and older adults, 1.10 m/s and 1.14m/s, respectively (p = 0.57). Both males and females in each age group had a GS similar to non-SCD adults over the age of 80 (Table 2). There was no difference detected in GS between participants with and without avascular necrosis (AVN) (1.12 m/s vs 1.13 m/s, respectively, p = 0.89) nor between severe SCD genotypes (HbSS and HbSß0) and less severe genotypes (HbSC and HbSß+) (1.08 m/s vs 1.18 m/s, respectively, p =0.13). There was no correlation between hemoglobin and GS (ρ = 0.22, p = 0.17). Young and old exhibited similar performance on the Timed Up and Go (TUG) with a mean time of 9.2 seconds for younger adults and 10.1 seconds for older adults (p = 0.14). Younger adults walked farther on the six-minute walk test (6MWT) compared to older adults (546 meters vs 482 meters p = 0.04). There was no difference in 6MWT between participants with and without AVN (499 meters vs 537 meters, p = 0.22), and no correlation between hemoglobin and distance on 6MWT (rs = 0.20, p = 0.21). Younger adults had a better performance on the 30-second chair stand test (30sCST) with a mean of 14 (range 4-22) vs 11 (range 3-16) in older adults, p = 0.02. There was no difference in 30sCST in people with or without avascular necrosis (12 vs 14, respectively, p = 0.24). Nearly all participants (95%) completed activity monitoring (18 younger adults and 18 older adults). Younger adults wore the monitors for a mean of 8 days (range 4-12) and older adults wore it for a mean of 7 days (range 4-10). The median step count for younger adults was 9253 steps/day (IQR 6449-10546) and the median step count for older adults was 6839 steps/day (IQR 6304-8144) (p = 0.44). The majority of both younger and older adult's activity was sedentary (42-44% of weartime) or light (47-48% of weartime). On the acceptability survey, 95% (38/40) reported that the length of the assessment was appropriate and particiants even had suggestions for additional questions to add. One participant found a question upsetting, about a history of drug use. 5% (2/40) reported portions as difficult to understand. When asked about feedback on removing items from the SCD-FA, 10% (4/40) recommended removing the Montreal Cognitive Assessment due to difficulty. Conclusions The SCD-FA was feasible, acceptable, and safe in both older and younger adults with SCD. There was no difference in GS in older and younger adults with SCD. Similar to older adults with SCD, younger adults had a mean GS similar to non-SCD adults over the age of 80. Younger adults did have significantly a better performance on the 6MWT and 30sCST compared to older adults, which may suggest better aerobic endurance and lower body strength. GS is one of the most powerful predictors of morbidity and mortality in non-SCD geriatric populations but its role in SCD awaits larger longitudinal samples. In future studies we will explore the role of GS and other components of the SCD-FA in predicting patient-important outcomes and mortality. Disclosures Strouse: Takeda: Research Funding.

scholarly journals Assessing Feasibility of a Focused Geriatric Assessment in Older Adults with Sickle Cell Disease to Address Functional Risk Factors for Morbidity and Mortality

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 518-518
Author(s):  
Charity I Oyedeji ◽  
Carl Pieper ◽  
Katherine Hall ◽  
Miriam Morey ◽  
Heather Whitson ◽  
...  
Keyword(s):  
Older Adults ◽  
Sickle Cell Disease ◽  
Physical Function ◽  
Geriatric Assessment ◽  
Sickle Cell ◽  
Activity Monitoring ◽  
Assessment Tools ◽  
Cell Disease ◽  
The Mean ◽  
At Home

Background During the last five decades the life expectancy for people living with sickle cell disease (SCD) has improved markedly, with median survival of 61 years in recent cohorts enrolled from academic centers. Older adults with SCD (defined herein as age ≥ 50 years) make up 13% of the adult population at four major academic medical health systems in North and South Carolina. As this population continues to grow, more data are needed to guide medical management appropriate to their needs, as a lifetime of vaso-occlusion often leads to functional decline and premature development of complications seen in geriatric populations. There are no validated assessment tools and interventions to improve physical function in older adults with SCD. In this study we evaluated the feasibility of a focused geriatric assessment (FGA) for older adults with SCD. Methods Twenty adults with SCD ≥ 50 years old were enrolled in a prospective cohort study. Measures previously validated in the oncology FGA were included and enriched with additional physical and cognitive functional measures. Activity monitoring was performed for 7 days using the Actigraph wT3X-BT, and biomarkers were collected at each study visit (baseline, 10-20 days after a hospitalization [for those who experienced a hospitalization during the study period] and 12-month after baseline). The primary endpoint was the proportion of subjects who complete the assessment. Secondary endpoints were duration of the FGA, proportion who completed the activity monitoring and lab collection, and acceptability. Results Twenty-one of 25 older adults approached for the study consented. Nearly all (20/21, 95%) completed the FGA. One was removed after missed study visits. The median duration of the assessment was 97 minutes (range 73-175 minutes), and there were no adverse events. The mean age was 57 years (range 50-71), 50% (10) were female, 30% (6) were on disability and 50% (10) were working. 45% (9) were former smokers but only 5% (1) were current smokers (Table). On the acceptability survey, 95% (19) reported the length of the assessment as appropriate. 10% (2) subjects reported a portion of the Montreal Cognitive Assessment (MoCA) as difficult. No subject found the questions upsetting, and 2 subjects reported that they would remove redundant questions. All subjects had a Karnofsky Performance Score of at least 80% and were able to complete activities of daily living. A third (35%) had been admitted in the last 6 months, and most (75%) had had severe pain crises at home that limited their activity. 40% (8) had > 4 severe crises at home in the last 6 months. The mean usual gait speed was 1.14 m/sec (range 0.90-1.50) (Figure). Mean Timed Up and Go (TUG) was 10.1 seconds (range 7.7-14.0). Two (10%) subjects had a TUG consistent with increased fall risk (≥ 12 seconds). Mean grip strength of the dominant hand was 39 kg (range 22-54 kg) for males and 25 kg (range 20-34 kg) for females, which is 38% and 43% lower than expected for age and gender. Mean six-minute walk (6MW) was 465m for males and 499m for females, which is 22% and 4% lower than expected for age/gender/height/weight. In the 17 subjects with heart rate recovery (HRR) recorded after 6MW, the mean HRR at 1 minute (HRR1) was 20 bpm (range 0-46). 31% of tested subjects had a HRR1 consistent with impaired HRR (12 bpm or fewer). HRR negatively correlated with age (HRR1 B=-1.1; 95% CI -0.2 - -2; p<0.05). Half of the subjects had mild cognitive impairment (MoCA score < 26). MoCA scores correlated with literacy testing (B= 0.39; 95% CI 0.10-0.68; p=0.02). The first 12 subjects wore the activity monitor 6 days (range 4-8 days) for an average of 15 hours a day. Average activity was sedentary for 7 hours (47%), light 7.5 hours (50%), and moderate 1 hour (7%) per day. There was a median of 7070 steps/day. Conclusions We found FGA to be feasible, acceptable, and safe. The duration of the FGA in our population was 3 times longer than the FGA for oncology. One of our most remarkable findings is that the older adults with SCD in this study have a physical function similar to non-SCD adults over the age of 80. They also demonstrate impaired HRR, an independent predictor of mortality in the elderly. After completion of the 12-month follow-up assessments, we will develop a briefer assessment to be evaluated in a larger study. Future steps will be to determine if FGA can predict outcomes such as risk of hospitalization and mortality and to develop interventions to improve these outcomes. Disclosures Strouse: Global Blood Therapeutics: Consultancy.

Pain, Coping and Health Care Utilization in Younger and Older Adults with Sickle Cell Disease

2010 ◽  
Vol 15 (1) ◽  
pp. 131-137 ◽  
Author(s):  
Kathryn A. Sanders ◽  
Susan M. Labott ◽  
Robert Molokie ◽  
Sarah R. Shelby ◽  
Joseph Desimone
Keyword(s):  
Older Adults ◽  
Health Care ◽  
Sickle Cell Disease ◽  
Health Care Utilization ◽  
Sickle Cell ◽  
Care Utilization ◽  
Pain Coping ◽  
Cell Disease

scholarly journals The Sickle Cell Disease Functional Assessment (SCD-FA) Tool: A Feasibility Pilot Study

2021 ◽  
Author(s):  
Charity I. Oyedeji ◽  
Katherine Hall ◽  
Alison Luciano ◽  
Miriam C. Morey ◽  
John J. Strouse
Keyword(s):  
Older Adults ◽  
Sickle Cell Disease ◽  
Adverse Events ◽  
Sickle Cell ◽  
Functional Assessment ◽  
Functional Decline ◽  
Adverse Outcomes ◽  
Functional Impairments ◽  
Cell Disease ◽  
Patient Reported

Abstract Background: The life expectancy for individuals with sickle cell disease (SCD) has greatly increased over the last 50 years. Adults with SCD experience multiple complications such as cardiopulmonary disease, strokes, and avascular necrosis that lead to limitations that geriatric populations often experience. There are no dedicated instruments to measure functional decline and functional age to determine risk of future adverse outcomes in older adults with SCD. The objective of this study was to assess the feasibility of performing the Sickle Cell Disease Functional Assessment (SCD-FA).Methods: We enrolled 40 adults with SCD (20 younger adults age 18-49 years as a comparison group and 20 older adults age 50 years and older) in a single-center prospective cohort study. Participants were recruited from a single comprehensive sickle cell clinic in an academic center in the southeastern United States. We included measures validated in an oncology geriatric assessment enriched with additional physical, cognitive, and patient-reported measures at the intersection of sickle cell disease and geriatrics. The primary outcome was the proportion completing the assessment. Secondary outcomes were the proportion consenting, duration of the assessment, acceptability, and adverse events.Results: Eighty percent (44/55) of individuals approached consented, 91% (40/44) completed the SCD-FA in its entirety, and the median duration was 89 minutes (IQR 80-98). There were no identified adverse events. On the acceptability survey, 95% (38/40) reported the length as appropriate, 2.5% (1/40) reported a question as upsetting, and 5% (2/40) reported portions as difficult. Exploratory analyses of physical function showed 63% (25/40) had a slow usual gait speed (< 1.2 meter/second).Conclusion: The SCD-FA is feasible, acceptable, and safe and physical performance tests were useful in identifying functional impairments in adults with SCD. These findings will inform the next phase of the study where we will assess the validity of the SCD-FA to predict patient-important outcomes in a larger sample of adults with SCD.

scholarly journals AVPR1A and Stress in Adults With Sickle Cell Disease–Related Chronic Pain

2020 ◽  
Vol 4 (Supplement_1) ◽  
pp. 785-785
Author(s):  
Keesha Roach ◽  
Brenda Dyal ◽  
Srikar Chamala ◽  
Yingwei Yao ◽  
Roger Fillingim ◽  
...  
Keyword(s):  
Older Adults ◽  
Chronic Pain ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Perceived Stress ◽  
Acute Pain ◽  
Vasopressin Receptor ◽  
Mean Stress ◽  
Cell Disease ◽  
Younger Adults

Abstract Purpose: Emotional stress is a known pain trigger in patients with sickle cell disease (SCD). The Arginine vasopressin receptor 1A gene (AVPR1A), SNP rs10877969, is associated with acute pain and stress-related pain. Our study investigated the association between AVPR1A genotype with stress and age in adults with SCD pain. Methods: 169 participants with SCD and chronic pain (100% African descent; mean age 36.4 ± 11.6 years [range =18-74 years]) completed the Perceived Stress Questionnaire. The SNP was evaluated as the imputed score was R2&gt;0.8. ANOVA compared stress by genotype and age. Findings: Mean stress scores were significantly lower (p&lt;0.05) for the older adults (0.35 ± 0.18) than the younger adults (0.41 ± 0.17). Mean stress scores were not significantly different by genotype for younger or older adults. Discussion: The rs10877969 genotype frequency was not different by age. In contrast to prior research, there was no association between genotype and stress.

scholarly journals Chronic organ failure in adult sickle cell disease

Hematology ◽  
2017 ◽  
Vol 2017 (1) ◽  
pp. 435-439 ◽  
Author(s):  
Elliott Vichinsky
Keyword(s):  
Risk Factors ◽  
Older Adults ◽  
Renal Failure ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Neurocognitive Impairment ◽  
Cortical Atrophy ◽  
Modifiable Risk Factors ◽  
Cell Disease ◽  
Timely Initiation

Abstract Sickle cell disease is now a chronic adult illness characterized by progressive multiorgan failure, particularly involving the brain and kidney. The etiology is multifactorial; it includes hemolysis and nitric oxide deficiency. As patients age, most experience neurologic insult. Twenty-five percent of older adults have had a clinical stroke and at least half of the population have had a silent infarct, cortical atrophy, and neurocognitive impairment. Periodic screening with neuroimaging and neurocognitive testing is recommended. Identification and correction of modifiable risk factors such as nocturnal hypoxemia, obstructive sleep apnea, and physical exercise programs should be implemented. Patients with neurocognitive impairment require cognitive remediation and educational accommodations. Chronic renal disease occurs in 25% of older adults and results in 50% of their deaths. Renal failure often develops insidiously. It can be prevented or minimized by early screening and treatment of modifiable risk factors including hypertension and microalbuminuria. There is an increasing number of therapeutic options, including inhibitors of the renin angiotensin system, angiotensin-II receptor blockers, endothelin-1 receptor antagonist, and haptoglobin therapy. Patients with sickle cell disease have increased mortality rates from renal failure compared with nonsickle cell patients, in part from a lack of access to early multidisciplinary care, including timely initiation of dialysis and renal transplantation.

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