Assessment of Psycho-Educational Needs in a Clinical Sample of Children With Sickle Cell Disease

Sickle cell disease (SCD) is associated with impaired cognitive and academic functioning, yet prevalence rates of educational needs and accommodations are not known. The Hematology-Oncology Psycho-Educational Needs Assessment was administered to 72 parents of youth, ages 5 to 17, with SCD. Half of the parents identified concerns about their children's learning; however, only 36% had undergone testing for learning problems, and only 26% had individualized education plans. Many parents (33%) reported concerns about attention/memory, and 36% of youth had failed a grade. Of 33 participants referred for neuropsychological evaluation, only 13 completed testing, which revealed below-average IQ and achievement scores. Considerable morbidity was reported; one third of participants missed over 20 school days per year, and 42% reported disease-related difficulties participating in school. Many youth with SCD present cognitive, academic, and functional difficulties, for which universal screening may promote earlier detection and psycho-educational intervention.     

A multidisciplinary health care team's efforts to improve educational attainment in children with sickle-cell anemia and cerebral infarcts

The primary objective of this study was to improve the educational success of children with sickle-cell disease (SCD) and cerebral infarcts. A prospective intervention trial was conducted; a multidisciplinary team was created to maximize educational resources for children with SCD and cerebral infarcts. Students were evaluated systematically before and after the intervention. A baseline evaluation was completed assessing the presence of an Individualized Education Plan (IEP), grade retention in school, and days absent from school in the year preceding the intervention. A postintervention assessment occurred 2 years later for these same measurements. At baseline, 74% (17 of 23) of the students were receiving IEPs. Two years later, 87% (20 of 23) students received IEPs (p = .34). Despite the intervention, the rate of children retained in their school grade increased from 0.6 per 100 years in school at baseline to 1.7 per 100 years, 95% CI (-3.86, 1.49). The school absenteeism rate did not significantly change after the intervention; the average days absent per student rose from 15.5 to 22.5, (p = .05). The multidisciplinary team effort alone was insufficient to decrease grade retention and absenteeism rate. Further support, from either the parents or school administration, is needed to increase education attainment of students with cerebral infarcts.     

Pediatric-Based Assessment Children With School Problems

Twenty-one children with school problems underwent pediatric assessment within a university hospital primary care clinic. Assessment included history-taking facilitated by parent and teacher questionnaires, physical examinations, vision and hearing screening and neurodevelopmental examination. For 11 of the children, psychoeducational assessment was also obtained. Parent and school personnel were interviewed 2 to 6 months following assessment to determine implementation rates for recommendations. Overall, 66% of parent recommendations were implemented, including 72% of medical, 58% of mental health and 64% of educational recommendations. Seventy-two percent of school recommendations were implemented. Interdisciplinary evaluation as compared to pediatric-based assessment alone resulted in a greater number of recommendations generated (p less than .05) and a higher percentage of school recommendations implemented (p less than .01). Implementation rates for school recommendations were greater for young children (p less than .01). This study supports the usefulness of pediatric-based assessments for school problems and documents the advantages of an interdisciplinary approach to such children.     

Knowledge of stroke risk,signs of stroke,and the need for stroke education among children with sickle cell disease and their caregivers

Objectives : This study assessed stroke knowledge and the need for stroke education among children with sickle cell disease (SCD) and their caregivers. Methods : A cross-sectional study was conducted and includes: (1) structured interviews of 44 children with SCD and their 50 caregivers, and (2) a self-administered survey completed by medical directors of 22 SCD centers. The participants were interviewed regarding stroke knowledge and stroke educational materials. Results : Among the caregivers, only 34% named stroke as a complication associated with SCD, 36% were aware that their children were at higher risk, and 46% were not able to identify any warning signs of stroke. Only 11% of the children could accurately describe a stroke. Although 82% of the medical directors provided stroke information to caregivers, only 50% provided stroke educational materials to children. Caregivers and children were interested in receiving information about stroke. The preferred format for the educational material was a pamphlet for adults and a video for the children. Conclusions : Children with SCD, caregivers, and healthcare providers need and desire educational materials about stroke. Educational materials to improve knowledge about stroke may be an important strategy to increase completion of stroke screening tests.     

Perceived stress factors and coping mechanisms among mothers of children with sickle cell disease in western Nigeria

While many studies have looked at the stressful effects of chronic illness of those who suffer such conditions, less is known about the effects on caregivers, especially in developing countries. Mothers in particular must bear the brunt of care and stress for children who have sickle cell disease (SCD). A sample of 200 mothers attending six SCD clinics in both public and private hospitals in the Ibadan-Ibarapa Health Zone of Oyo State, Nigeria, were interviewed. Stress levels were measured using an instrument comprised of stressors listed by mothers themselves in focus group discussions that preceded the survey. Higher levels of stress were associated with less educated and older women, as well as non-married women and those in polygamous households. Stress levels were also greater when there was more than one child with SCD in the family and when the index child was of school age. Coping mechanisms varied according to the category of stressor. Financial stress and disease factors were met with confrontation while family sources of stress were either complained about, accepted or avoided. Knowledge of the different types of mothers who experience more stress and of their preferred coping mechanisms can be useful in designing clinic-based counseling.     

Access to Patient-Centered Medical Homes in Children with Sickle Cell Disease

To determine the proportion of children with sickle cell disease (SCD) followed in a subspecialty clinic with access to a primary care provider (PCP) exhibiting practice-level qualities of a patient-centered medical home (PCMH). We surveyed 200 parents/guardians of children with SCD using a 44-item tool addressing PCP access, caregiver attitudes toward PCPs, barriers to healthcare utilization, perceived disease severity, and satisfaction with care received in the PCP versus SCD clinic settings. Individual PCMH criteria measured were a personal provider relationship and medical care characterized as accessible, comprehensive and coordinated. Although 94 % of respondents reported a PCP for their child, there was greater variation in the proportion of PCPs who met other individual PCMH criteria. A higher proportion of PCPs met criteria for coordinated care when compared to accessible or comprehensive care. In multivariate models, transportation availability, lower ER visit frequency and greater PCP visit frequency were associated favorably with having a PCP meeting criteria for accessible and coordinated care. Child and respondent demographics and disease severity had no impact on PCMH designation. Average respondent satisfaction scores for the SCD clinic was higher, when compared to satisfaction scores for the PCP. For children with SCD, access to a PCP is not synonymous with access to a medical home. While specific factors associated with PCMH access may be identified in children with SCD, their cause and effect relationships need further study.     

Parent perspectives on the educational barriers and unmet needs of children with cancer

Childhood cancer challenges academic success and school engagement, yet little research has described these hardships. This study documented parental perspectives of the educational barriers and unmet needs of youth treated for cancer (n = 102). Treatment-related physical sequelae were noted as common problems interfering with school attendance and engagement. One-fourth of parents reported worse academic performance since diagnosis; however, many never had their child evaluated (63.3%) or enacted formalized educational accommodations (55%). Findings reflect educational challenges across the continuum of cancer care and survivorship, and highlight the importance of education of school staff on both acute and long-term effects of cancer treatment.     

Associations between academic achievement and psychosocial variables in adolescents with cystic fibrosis

BACKGROUND: Cystic fibrosis (CF) is a chronic genetic disease that leads to the accumulation of thick mucus in multiple organ systems, leading to chronic lung infection and affecting the body's ability to absorb nutrients necessary for growth and development. This cross‐sectional, correlational study examined the potential effects of CF on students' psychosocial and academic development. METHODS: Forty adolescents with CF completed a battery of neuropsychological and psychosocial measures. Their school records were reviewed to abstract information about standardized achievement testing results and grade point average (GPA). Academic outcomes were hypothesized to be associated with (1) self‐efficacy, (2) disease and school‐specific coping strategies, (3) attitude to school, and (4) depression. RESULTS: Cognitive and academic scores were within the normal range, and self‐efficacy had the strongest association with standardized cognitive and academic measures and high school grades. School absences were associated with GPA, but not standardized test scores. CONCLUSION: Adolescents with CF require supports in school that foster their sense of self‐efficacy and accommodations that address the learning time lost from extended health‐related absences.     

Effects of STARBRIGHT World on Knowledge, Social Support, and Coping in Hospitalized Children With Sickle Cell Disease and Asthma

STARBRIGHT World is an innovative computer network for hospitalized children that provides interactive health education as well as opportunities to meet online with children in other hospitals. Fifty hospitalized children with sickle cell disease (SCD) or asthma participated in at least 1 day of a 3-day curriculum involving STARBRIGHT World activities and were compared to 60 control participants who participated in traditional hospital educational and recreational activities. Disease knowledge, perceived social support from peers, and coping skills were assessed at pre- and posttest. Participation in STARBRIGHT World resulted in a trend toward greater increases in knowledge for teens with asthma, more perceived peer support for children with SCD, and decreased negative coping among teens with SCD. The lack of more global treatment effects may be related to sample size and measurement variability issues, as well as limitations and variability in treatment intensity. Discussion focuses on ways to enhance STARBRIGHT World efficacy via outpatient and home-based program development and the need for further research using larger samples and more standardized intervention.     

Social Functioning, Peer Relations, and Internalizing and Externalizing Problems Among Youths With Sickle Cell Disease

This study examined differences in social functioning, peer relations, internalizing behaviors, and externalizing behaviors between youths (ages 4-18) with sickle cell disease (SCD) and demographically matched healthy peers using parent-, teacher-, and youth-report versions of the Child Behavior Checklist and Missouri Peer Relations Inventory. Youths with SCD were reported by themselves to have more social and externalizing problems compared to healthy peers. Moreover, a significantly greater percentage of youths with SCD had borderline and clinical levels of social, internalizing, and externalizing problems relative to the healthy comparison group, Illness severity variables were not associated with any of the dependent measures. Overall, although the social functioning of youths with SCD is perceived by parents and teachers to be adequate, adolescents with SCD view themselves as having more.     

Height and risk of sudden cardiac death: the Atherosclerosis Risk in Communities and Cardiovascular Health Studies

PurposeSudden cardiac death (SCD) is an important cause of mortality in the adult population. Height has been associated with cardiac hypertrophy and an increased risk of arrhythmias but also with decreased risk of coronary heart disease, suggesting a complex association with SCD.MethodsWe examined the association of adult height with the risk of physician-adjudicated SCD in two large population-based cohorts: the Cardiovascular Health Study and the Atherosclerosis Risk in Communities study.ResultsOver an average follow-up time of 11.7 years in Cardiovascular Health Study, there were 199 (3.6%) cases of SCD among 5556 participants. In Atherosclerosis Risk in Communities study, over 12.6 years, there were 227 (1.5%) cases of SCD among 15,633 participants. In both cohorts, there was a trend toward decreased SCD with taller height. In fixed effects meta-analysis, the pooled hazard ratio per 10 cm of height was 0.84; 95% confidence interval, 0.73–0.98; P = .03. The association of increased height with lower risk of SCD was slightly attenuated after inclusion of risk factors associated with height, such as hypertension and left ventricular hypertrophy. The association appeared stronger among men than women in both cohorts.ConclusionsIn two population-based prospective cohorts of different ages, greater height was associated with lower risk of SCD.     

PspA family typing and PCR-based DNA fingerprinting with BOX A1R primer of pneumococci from the blood of patients in the USA with and without sickle cell disease

Disease and mortality rates for Streptococcus pneumoniae infections are much higher in patients with sickle cell disease (SCD) than in age-matched patients without SCD. Pneumococcal surface protein A (PspA) has been proposed as a component in human vaccines against S. pneumoniae to provide greater breadth of coverage than can be obtained with the 7-valent conjugate vaccine. The cross-reactivity of PspA is associated with the 'PspA family' structure. In this study we examined strains of S. pneumoniae from patients with and without SCD to determine whether the strains infecting the hypersusceptible population of SCD patients were limited to the same two PspA families already known to comprise over 95% of strains infecting non-SCD patients. Each strain was also evaluated according to the presence or absence of specific PCR fragments based on repetitive BOX elements to screen for possible SCD-associated clonal structure. Strains from SCD and non-SCD patients were similarly dispersed among the most common BOX PCR groups and strains from both groups expressed a similar distribution of PspA variants. Thus, a PspA vaccine designed for the population at large should also be appropriate for patients with SCD.     

Misconceptions about sickle cell disease (SCD) among lay people in Benin

Objective

The aim of this study was to determine the accuracy of people's ideas in Benin about the common affliction of sickle cell disease (SCD).

Methods

A questionnaire asking for the degree of agreement with 111 statements about SCD was given in 2011 to 6 physicians (the gold standard) and 178 lay people living in Cotonou, Benin. A misconception was defined as a statistically significant (p < 0.001) deviation of at least 2.5 points on the 0-10 response scale of the lay people's mean response from that of the physicians.

Results

Lay people tended not to be sufficiently aware that SCD is a hereditary illness, that a genetic test can detect the gene, that having it does not automatically lead to illness, that SCD may induce severe kidney, lung, heart, or cerebrovascular disorders, and that SCD cannot be cured by traditional healers. These misconceptions were greater among those who knew they had SCD or carried the gene than among those who knew they did not.

Conclusion

Major efforts are needed to overcome the educational and cultural barriers to accurate understanding of how to prevent and treat SCD among lay people in Benin, especially among those at greatest risk.     

Transition to adult care for adolescents with sickle cell disease: results of a national survey

The objective was to present the 'voice' of adolescents with sickle cell disease (SCD) as part of the discussion of transition issues by identifying and documenting their expressed concerns and expectations, as well as what program priorities they perceive would facilitate a smooth transition to adult care. Cross-sectional data were collected by means of structured questionnaire interviews, using standard instruments. A volunteer sample of 172 adolescents with SCD aged 14 years and older still in pediatric care within community-based and medical center SCD programs across the United States was recruited. Statistically significant results indicated the top concerns of adolescents were: lack of information relating to their transition to adult care; fear of leaving the healthcare provider with whom they were already familiar, fear that adult care providers might not understand their needs; belief that an SCD transition program was needed and that it should focus on provider support; information provision about adult care programs; ways to meet adult care providers; and ways to help healthcare providers understand their needs. We conclude that many adolescents with SCD have concerns and fears about their transition to adult care. Based on findings from this study, it is recommended that transition programs address structural and interpersonal issues of adolescents and providers if they are to be successful. Strategies by which this can be achieved are recommended, including the need to encourage, support and provide assistance for peer education, outreach programs and peer-led instructions, since these hold great promise as approaches that are adolescent-centered and adolescent-delivered.     

Injuries, Absences, and Visits to the Nurse Among Children in Alternative Schools

Previous research suggests ecological and programmatic characteristics of schools may influence the health and health behavior of schoolchildren. In particular, schools with alternative educational programs, such as "magnet" curricula and extended school hours, have been found to have a higher incidence of student injuries. To examine further the health correlates of alternative educational programs, the authors studied injuries, health office visits, and school absences of 983 elementary schoolchildren during the 1983-1984 academic year. The study population comprised students attending three elementary schools-two with magnet programs and one with a regular primary curriculum. All three had an option for extended school hours. The frequency of school absences was related significantly to school hours, with fewer absences found in schools with extended hours (p less than .01). Enrollment in a magnet school was associated positively and significantly with rates of both health office visits and injuries, even after adjustment for the effects of age, gender, and extended school hours (p less than .001). Finally, an interaction effect was identified in which children enrolled in both a magnet curriculum and an extended day program had substantially higher rates of health office visits than did children enrolled in either program alone (p less than .001). These findings confirm a disproportionate use of school health services for injuries and other health concerns among students attending alternative educational programs. Possible explanations include differences in student characteristics, adult supervision, or the family lifestyles of children in alternative schools.     

Cost of workplace accommodations for individuals with disabilities: with or without personal assistance services

BackgroundThis study compared the expense associated with use of personal assistance services (PAS) for individuals with disabilities to the expense incurred by individuals with disabilities who did not use PAS. The intent of this investigation was to assess the disability accommodation costs and benefits of PAS and non-PAS cases.MethodsThe study uses 1,182 follow-up telephone surveys and 24 telephone interviews of employers who had previously contacted the Job Accommodation Network (JAN) to discuss disability-related accommodations for an employee or potential employee that were conducted from January 2004 through December 2006. The survey included 69 employers who had considered PAS. The surveys were conducted by the University of Iowa's Law, Health Policy, and Disability Center, which contacted employers who had previously contacted JAN for consultation on workplace accommodations. The interviews were conducted by the International Center for Disability Information at West Virginia University.ResultsKey findings point to the costs associated with PAS cases and with cases not involving PAS accommodations. As reported by the employers, the median “one-time cost” of accommodations (not $0) for non-PAS cases was $500. The median “one-time cost” of accommodations (not $0) for PAS cases was $1,850. When $0 cost of accommodations on PAS cases was factored in with “one-time cost” of accommodations for PAS cases, the median cost was $0. For non-PAS cases of accommodations, when $0 cost of accommodations was considered, the outcome was a median cost of $0. The annual cost for PAS accommodations was a median cost of $8,000 in comparison to $2,000 for non-PAS. The median dollar amount estimates of direct benefits were $1,600 for PAS accommodations, similar to $1,500 for non-PAS. The most frequently mentioned benefits from PAS accommodations were (a) increased productivity, (b) increased diversity, (c) retention of a valued employee, (d) improved interactions with co-workers, (e) increased overall company morale, and (f) increased overall company productivity.ConclusionsThe findings heighten awareness of the cost and benefits aspects associated with PAS for people with disabilities. Many non-PAS accommodations cost nothing to the employer (e.g., changing the work schedule, moving the individual to another location). When dollar cost was involved, the costs for PAS accommodations were more than three times greater than non-PAS accommodations.     

Is the association between socioeconomic position and coronary heart disease stronger in women than in men?

The association between socioeconomic position and health is generally believed to be weaker among women than men. However, gender differences in the relation between socioeconomic position and coronary heart disease have not been evaluated in a representative sample of the US population. The authors examined this association in the First National Health and Nutrition Examination Survey (1971-1993), a longitudinal, representative study of the US population (n = 6,913). Information on educational attainment, household income, and covariates was derived from the baseline interview, and that on incident coronary heart disease was obtained from hospital records/death certificates over 22 years of follow-up. Cox's proportional hazards models showed that education and income were inversely associated with incident coronary heart disease in age-only and multivariate models. Risk associated with education varied by gender (p = 0.01), with less than high school education associated with stronger risk of coronary heart disease in women (relative risk = 2.15, 95% confidence interval: 1.46, 3.17) than in men (relative risk = 1.58, 95% confidence interval: 1.18, 2.12) in age-adjusted models. Low education was associated with greater social and psychological risks for women than men; however, metabolic risks largely explained gender differences in the educational gradient in coronary heart disease.     

Sickle Cell Disease in Pregnancy: Maternal Complications in a Medicaid-Enrolled Population

Higher frequencies of pregnancy complications have been reported among women with sickle cell disease (SCD) compared with those without SCD; however, past studies are limited by small sample size, narrow geographic area, and use of hospital discharge data. We compared the prevalence of maternal complications among intrapartum and postpartum women with SCD to those without SCD in a large, geographically diverse sample. Data from the 2004–2010 Truven Health MarketScan^® Multi-State Medicaid databases were used to assess the prevalence of maternal complications among intrapartum and postpartum women 15–44 years of age with and without SCD whose race was reported as black. The comparison group of women without SCD was further divided into those with chronic conditions associated with multi-organ failure and those without chronic conditions. Multivariable log-binomial regression models were used to calculate adjusted prevalence ratios for outcomes for women with SCD compared with women in the two comparison groups. Of the 335,348 black women with a delivery during 2004–2010, 1,526 had a diagnosis of SCD (0.5 %). Compared with women without SCD who had chronic conditions, women with SCD had higher prevalence of deep vein thrombosis, pulmonary embolism, obstetric shock, pneumonia, sepsis, postpartum infection, and transfusions. SCD was also positively associated with acute renal failure, cerebrovascular disorder, respiratory distress syndrome, eclampsia, postpartum hemorrhage, preterm birth, and ventilation when compared with women without SCD and chronic conditions. Overall, women with SCD have increased prevalence of pregnancy complications, even when compared with a group of women with similar risk for multi-organ failure.