A multi-institutional comparison of younger and older adults with sickle cell disease.
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ABSTRACT:
SUBMITTER: Oyedeji C
PROVIDER: S-EPMC6449149 | biostudies-literature | 2019 Apr
REPOSITORIES: biostudies-literature
Publications
A multi-institutional comparison of younger and older adults with sickle cell disease.
Oyedeji Charity C Strouse John Joseph JJ Crawford Regina D RD Garrett Melanie E ME Ashley-Koch Allison E AE Telen Marilyn J MJ
American journal of hematology 20190131 4
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Project description: Not available
| S-EPMC9429735 | biostudies-literature
Project description:Background. Patients with SCD now usually live well into adulthood. Whereas transitions into adulthood are now often studied, little is published about aging beyond the transition period. We therefore studied age-associated SCD differences in utilization, pain, and psychosocial variables. Methods. Subjects were 232 adults in the Pain in Sickle Cell Epidemiology Study (PiSCES). Data included demographics, comorbidity, and psychosocial measures. SCD-related pain and health care utilization were recorded in diaries. We compared 3 age groups: 16-25 (transition), 26-36 (younger adults), and 37-64 (older adults) years. Results. Compared to the 2 adult groups, the transition group reported fewer physical challenges via comorbidities, somatic complaints, and pain frequency, though pain intensity did not differ on crisis or noncrisis pain days. The transition group utilized opioids less often, made fewer ambulatory visits, and had better quality of life, but these differences disappeared after adjusting for pain and comorbidities. However, the transition group reported more use of behavioral coping strategies. Conclusion. We found fewer biological challenges, visits, and better quality of life, in transition-aged versus older adults with SCD, but more behavioral coping. Further study is required to determine whether age-appropriate health care, behavioral, or other interventions could improve age-specific life challenges of patients with SCD.
| S-EPMC5387810 | biostudies-literature
Project description:People with sickle cell disease (SCD) are living longer than ever before, with the median survival increasing from age 14 years in 1973, beyond age 40 years in the 1990s, and as high as 61 years in recent cohorts from academic centers. Improvements in survival have been attributed to initiatives, such as newborn screening, penicillin prophylaxis, vaccination against encapsulated organisms, better detection and treatment of splenic sequestration, and improved transfusion support. There are an estimated 100,000 people living with SCD in the United States and millions of people with SCD globally. Given that the number of older adults with SCD will likely continue to increase as survival improves, better evidence on how to manage this population is needed. When managing older adults with SCD (defined herein as age ≥ 40 years), healthcare providers should consider the potential pitfalls of extrapolating evidence from existing studies on current and emerging therapies that have typically been conducted with participants at mean ages far below 40 years. Older adults with SCD have historically had little to no representation in clinical trials; therefore, more guidance is needed on how to use current and emerging therapies in this population. This article summarizes the available evidence for managing older adults with SCD and discusses potential challenges to using approved and emerging drugs in this population.
| S-EPMC10979738 | biostudies-literature
Project description:To study degrees of chronic kidney disease (CKD) using creatinine clearance in adult Nigerian patients with sickle-cell disease (SCD).One hundred SCD patients, made up of 79 HbSS (homozygous haemoglobin S) patients and 21 HbSC (heterozygous haemoglobins S and C) patients, were investigated prospectively, along with 50 normal controls. Their sociodemographic data, weight and drug history were documented. Each participant underwent dipstick urinalysis, and creatinine clearance was calculated following a 24-hour urine collection and serum creatinine measurement. They were categorized into stages of CKD based on the creatinine clearance.Of the 79 HbSS patients, 14 (18%), 28 (35%), 33 (42%) and 4 (5%) had stage 1, 2, 3 and 4 CKD, respectively. In the HbSC group, 3 (14%), 9 (43%) and 9 (43%) patients had stage 1, 2 and 3 CKD, respectively. Proteinuria was noted in 16 (20%) HbSS patients but not in any of the HbSC patients. Of the subjects aged ?24 years (n = 49), 9 (18%), 18 (37%), 21 (43%) and 1 (2%) had stage 1, 2, 3 and 4 CKD, respectively. Of those aged >24 years (n = 51), 8 (16%), 19 (37%), 21 (41%) and 3 (6%) had stage 1, 2, 3 and 4 CKD, respectively. None of the subjects had stage 5 CKD.In this study, the adult subjects with SCD had various degrees of CKD. Adequate follow-up and active intervention are advocated to delay the onset of end-stage nephropathy.
| S-EPMC5586881 | biostudies-other
Project description:Children and adults with sickle cell disease (SCD) are at risk for neuropsychological deficits; however, the neurocognitive functioning of adults with SCD and related comorbidities has not been widely reported in the literature. We examined specific cognitive domains in symptomatic adults with SCD and compared them with their unaffected siblings. We also examined relationships between cognitive scores, patient-reported outcomes (PROs), and medical/laboratory values. Thirty patient-sibling pairs (M patient age = 32.5 years, M sibling age = 32.1 years) completed evaluations as part of a medical clinical trial (NCT00061568). All patient and sibling neurocognitive test scores were within normal limits. Patients scored significantly lower (M = 91.0 ± 11.3) than their siblings (M = 100.6 ± 12.3; t = -3.5, p < .01) on the Wechsler Processing Speed Index. They also indicated more problems than siblings on an executive functioning questionnaire, although these differences were nonsignificant after accounting for depressive symptoms. Higher fetal hemoglobin and lower creatinine correlated with better scores on particular cognitive and PRO measures. In summary, our sample of adults with symptomatic SCD demonstrated worse processing speed and experience more executive challenges than their siblings, despite treatment with hydroxyurea. These relative weakness likely relate to disease processes but the specific physiological mechanism is unclear.
| S-EPMC8493482 | biostudies-literature
Project description:AimsTo characterize the prevalence and impact of nocturnal enuresis and overactive bladder (OAB) symptomatology in the adult sickle-cell disease (SCD) population.MethodsWe performed a single-center, cross-sectional study of adult SCD patients from October 2012 to February 2014, using the validated Pfizer OAB short form questionnaire and brief voiding history surveys. Patient responses and scores were compared to that of controls having normal or sickle cell trait hemoglobin genotypes.ResultsA group of 239 SCD patients (116 males, 123 females) were compared with 104 normal and 57 sickle cell trait patients. Seven of 239 (2.9%) SCD patients compared to none of the 161 patients without SCD (P = 0.04) reported current nocturnal enuresis. The median age of nocturnal enuresis cessation was higher in SCD patients (12.0, IQR 9.0-15.0 years) compared to that of both normal (7.5, IQR 6.0-9.8 years) and sickle cell trait (7.5, IQR 6.0-8.8 years) groups (P < 0.0001). Ninety-three of 239 (38.9%) SCD patients compared to 17 of 104 (16.3%) normal and 11 of 57 (19.3%) sickle cell trait had scores indicating OAB symptomatology (P < 0.0001). Patients with SCD had higher OAB symptom severity and lower health-related quality of life (HRQL) scores compared to the normal and sickle cell trait groups (P < 0.0001 and P < 0.0001, respectively).ConclusionsWe demonstrate an elevated rate of nocturnal enuresis and OAB symptoms in the adult SCD population. An OAB phenotype may be an under-recognized complication of SCD irrespective of age. Neurourol. Urodynam. 35:642-646, 2016. © 2015 Wiley Periodicals, Inc.
| S-EPMC4605838 | biostudies-literature
Project description:Sickle cell disease (SCD) is primarily characterized by pain. This chronic pain with acute exacerbations is the most common reason for hospital visits, admissions, and readmissions, particularly in young adults (aged 18–39 years). People who present to the hospital for pain crises often report that nurses lack knowledge of SCD and, consequently, they do not provide appropriate, timely care.Because pain episodes often result in hospital admissions, this article highlights prominent issues that staff nurses need to know.Using a review of the literature and case studies, the authors provide recommendations to improve care of adults with SCD.No objective signs of a sickle cell pain crisis exist. Patients react to pain in different ways and use various coping mechanisms in response. Suspected opioid addiction should not affect the provision of nursing care. Pain must be treated appropriately to decrease the potential for prolonged admissions and/or readmissions. Patients are to be acknowledged as experts and collaborated with in developing an appropriate plan of care. Advocacy on behalf of the patient is important for better communication with providers. With this knowledge, nurses will be better equipped to provide the appropriate and timely care required to manage pain crises experienced by individuals living with SCD.
| S-EPMC5565201 | biostudies-other
Project description:Word retrieval during language production slows down with age. However, bilinguals also require language control to manage language competition, in particular when switching languages to cues. The current study examined how this bilingual language control differs between younger and older adults. It also compared bilingual control, and age-group differences, to control that might be applied when alternating between responses within one language. In Experiment 1, 40 younger and 40 older monolingual adults completed a task alternating between noun and verb responses to pictures. The task showed costs associated with language control but these did not differ between age groups. Experiment 2 was completed by 50 older and 50 younger bilingual adults. Older adults showed larger switching costs than younger adults when switching between and within languages, suggesting they experienced more difficulty with reactive control. However, while older adults showed larger mixing costs than younger adults when using two languages in the dual-language environment relative to the single-language environment, they surprisingly showed smaller mixing costs than younger adults in the noun-verb within-language naming task. These findings show that language control, and the way it differs between older and younger adults, is not the same across within- and bilingual-language competition.
| S-EPMC10555994 | biostudies-literature
Project description:Voice assistants are widely used in smart home environments. This study aimed to investigate user acceptance of a smart home voice assistant. A questionnaire was designed, and 471 Chinese adults were recruited to complete the questionnaire. The data were analyzed using exploratory factor analysis and regression analysis. The results revealed that user requirements of adults were composed of six factors: hedonic motivation and trust (β = .41, p < .001), social influence (β = .22, p < .001), performance expectancy (β = .15, p < .001), effort expectancy (β = .08, p = .018), product features (β = .15, p = .009), and facilitating conditions (β = .06, p = .049). Among these six factors, hedonic motivation and trust are considered the most important. Younger, middle-aged, and older adults differed significantly in their requirements and acceptance of a smart home voice assistant. These findings have implications for the design of smart home voice assistants so that they are more acceptable to younger and older adults.Supplementary informationThe online version contains supplementary material available at 10.1007/s10209-022-00936-1.
| S-EPMC9617746 | biostudies-literature
Project description:The photophysical properties of human sickle cell disease (SCD) Hemoglobin (Hb) is characterized by multi-photon microscopy (MPM). The intrinsic two-photon excited fluorescence (TPEF) signal associated with extracted hemoglobin was investigated and the solidified SCD variant (HbS) was found to demonstrate broad emission peaking around 510 nm when excited at 800 nm. MPM is used to dynamically induce and image HbS gelling by photolysis of deoxygenated HbS. For comparison, photolysis conditions were applied to a healthy variant of human hemoglobin (HbA) and found to remain in solution not forming fibers. The use of this signal to study the mechanism of HbS polymerization associated with the sickling of SCD erythrocytes is discussed.
| S-EPMC4605066 | biostudies-other