Facilitators and Barriers to Empowerment in Children and Young People with Cystic Fibrosis: A Meta-Synthesis of the Qualitative Literature
Cystic Fibrosis; Child; Human; Only Child; Palliative Care; Qualitative Research
PURPOSE: Patient empowerment may be particularly important in children and young people (CYP) with CF, due to high treatment burden and limited peer support opportunities. This review aimed to meta-synthesize the qualitative literature pertaining to empowerment in CYP with CF., MATERIALS AND METHODS: This work was guided by the ENTREQ framework, with a search strategy based on the SPIDER framework. A systematic search of PsycInfo, Medline, CINAHL and ASSIA databases was conducted. Identified studies were quality assessed and data analysed using thematic synthesis. PROSPERO registration: CRD42019154014., RESULTS: Seventeen studies met inclusion criteria, though none explicitly explored empowerment. Thematic synthesis identified six analytic themes<ovid:i>: relational support, information and understanding</ovid:i> and <ovid:i>feeling heard and respected</ovid:i> appeared to facilitate empowerment, while <ovid:i>prejudices and assumptions</ovid:i> were identified as potential barriers. <ovid:i>Mastery and competence</ovid:i> and <ovid:i>Navigating being different</ovid:i> appeared to be components of empowerment., CONCLUSIONS: The findings provide an initial understanding of patient empowerment in CYP with CF. Potential clinical implications include the need for more CYP-friendly information, more shared decision making and more opportunities to experience mastery. The need for further research is highlighted, particularly relating to developmental influences and factors unique to CF, which are not adequately addressed in existing patient empowerment models.Implications for rehabilitationEmpowerment in children and young people with cystic fibrosis can be facilitated by supportive and respectful relationships with family, friends and clinical teams, that enable them to feel heard and understood.It can be further supported by providing developmentally appropriate information and opportunities for children and young people to experience mastery and competency in typical childhood activities.Prejudices and assumptions about the capabilities of children and young people with CF, even when based in good intentions, can act as a barrier to empowerment.Empowerment can shape (and be shaped by) the way the children and young people navigate differences associated with living with CF.
Fairweather N; Jones FW
Disability and Rehabilitation
2022
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<a href="http://doi.org/10.1080/09638288.2021.2003876" target="_blank" rel="noreferrer noopener">10.1080/09638288.2021.2003876</a>
Building the repertoire of measures of walking in Rett syndrome
children; Rehabilitation; Rett syndrome; girls; walking; cerebral-palsy; test-retest reliability; adults; 2-minute; 6-minute; disability inventory; functional mobility scale; pediatric evaluation; two-minute walk test; tone and motor problems; tool development; scale development; Modified two-minute walk test; Rett syndrome specific functional mobility scale; RSGMS
Background: The repertoire of measures of walking in Rett syndrome is limited. This study aimed to determine measurement properties of a modified two-minute walk test (2MWT) and a modified Rett syndrome-specific functional mobility scale (FMS-RS) in Rett syndrome. Methods: Forty-two girls and women with Rett syndrome (median 18.4 years, range 2.4-60.9 years) were assessed for clinical severity, gross motor skills, and mobility. To measure walking capacity, 27 of this group completed a 2MWT twice on two different assessment days. To assess walking performance, the FMS-RS was administered to the total sample of parents (n=42) on two occasions approximately one week apart. Results: There were negative correlations between clinical severity and 2MWT (r=-0.48) and FMS-RS (r=-0.60-0.66). There were positive correlations between gross motor skills and mobility and 2MWT (r=0.51, 0.43) and FMS-RS (r=0.71-0.93, 0.74-0.94), respectively. Test-retest reliability for the 2MWT was good with high intraday and interday correlations (ICC=0.86-0.98). For the 2MWT, the standard error of measurement was 13.8 m and we would be 95% confident that changes greater than 38 m would be greater than within subject error. There was good test-retest reliability for all three distances on the FMS-RS (ICC=0.94-0.99). Conclusions: Walking capacity as measured by the 2MWT showed expected but limited relationships with measures of different constructs, providing some support for concurrent validity. Walking performance as measured with the FMS-RS was more strongly consistent with other clinical measures supporting its concurrent validity. Test-retest reliability was good for both the FMS-RS and the 2MWT. Therefore, these measures have the potential to be used in clinical practice and research.
Stahlhut M; Downs J; Leonard H; Bisgaard A M; Nordmark E
Disability and Rehabilitation
2017
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<a href="http://doi.org/10.1080/09638288.2016.1212280" target="_blank" rel="noreferrer noopener">10.1080/09638288.2016.1212280</a>
Initial assessment of the StepWatch Activity Monitor (TM) to measure walking activity in Rett syndrome
tone and motor problems; Rett syndrome; tool development; scale development; StepWatch
Purpose: In girls and women with Rett syndrome, we assessed the accuracy of the StepWatch Activity Monitor (TM) and investigated relationships between daily step counts, gross motor skills and age. Method: Twelve subjects (age 12.9 +/- 8.0 years) participating in the Australian Rett Syndrome Database wore a StepWatch during a videoed session of activities to assess agreement with the criterion method of observation. Physical activity data were also collected over the course of 6 +/- 1 whole days. Relationships between agreement, gross motor skills, average daily step count and age were analyzed. Results: The number of steps obtained using the StepWatch was similar to that viewed on video (mean difference = 0 steps per minute) and agreement did not differ with the level of general (p = 0.389) or complex gross motor skills (p = 0.221). Subjects were less active than their healthy peers (difference 6086 steps per day; p = 0.001), and physical activity was significantly greater in those who were younger and with greater levels of motor skill. Conclusions: The StepWatch provided accurate information on the physical activity of girls and women with Rett syndrome regardless of their level of gross motor function. Physical activity reduced with age despite the ability to walk. Advocacy for pro-active lifestyles is justified.
Hill K; Leonard H; Downs J
Disability and Rehabilitation
2012
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<a href="http://doi.org/10.3109/09638288.2011.630773" target="_blank" rel="noreferrer noopener">10.3109/09638288.2011.630773</a>
Initial assessment of the StepWatch Activity Monitor™ to measure walking activity in Rett syndrome
tone and motor problems; Rett syndrome; tool development; scale development; StepWatch
PURPOSE: In girls and women with Rett syndrome, we assessed the accuracy of the StepWatch Activity Monitor™ and investigated relationships between daily step counts, gross motor skills and age. METHOD: Twelve subjects (age 12.9 ± 8.0 years) participating in the Australian Rett Syndrome Database wore a StepWatch during a videoed session of activities to assess agreement with the criterion method of observation. Physical activity data were also collected over the course of 6 ± 1 whole days. Relationships between agreement, gross motor skills, average daily step count and age were analyzed. RESULTS: The number of steps obtained using the StepWatch was similar to that viewed on video (mean difference = 0 steps per minute) and agreement did not differ with the level of general (p = 0.389) or complex gross motor skills (p = 0.221). Subjects were less active than their healthy peers (difference 6086 steps per day; p = 0.001), and physical activity was significantly greater in those who were younger and with greater levels of motor skill. CONCLUSIONS: The StepWatch provided accurate information on the physical activity of girls and women with Rett syndrome regardless of their level of gross motor function. Physical activity reduced with age despite the ability to walk. Advocacy for pro-active lifestyles is justified.
Downs J; Leonard H; Hill K
Disability and Rehabilitation
2012
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<a href="http://doi.org/10.3109/09638288.2011.630773" target="_blank" rel="noreferrer noopener">10.3109/09638288.2011.630773</a>
Rett syndrome: establishing a novel outcome measure for walking activity in an era of clinical trials for rare disorders
Rehabilitation; validity; injury; Rett syndrome; walking; disease; sample; accelerometry; accuracy; activity monitors; Outcome measure; physical activity; physical-activity; step; test-retest reliability; tone and motor problems; tool development; scale development; StepWatch
Background: Rett syndrome is a pervasive neurological disorder with impaired gait as one criterion. This study investigated the capacity of three accelerometer-type devices to measure walking activity in Rett syndrome. Methods: Twenty-six participants (mean 18 years, SD 8) wore an Actigraph, ActivPAL and StepWatch Activity Monitor (SAM) during a video-taped session of activities. Agreement was determined between step-counts derived from each accelerometer and observation. Repeatability of SAM-derived step counts was determined using pairs of one-minute epochs during which the same participant was observed to walk with the same cadence. Results: The mean difference (limit of agreement) for the Actigraph, ActivPAL and SAM were -41 (SD 33), -16 (SD 21) and -1 (SD 16) steps/min, respectively. Agreement was influenced by a device/cadence interaction (p < 0.001) with greater under-recording at higher cadences. For SAM data, repeatability of step-count pairs was excellent (intraclass correlation coefficient 0.91, 95% CI 0.79-0.96). The standard error of measurement was 6 steps/min and we would be 95% confident that a change >= 17 steps/min would be greater than within-subject measurement error. Conclusions: The capacity of the SAM to measure physical activity in Rett syndrome allows focus on participation-based activities in clinical practice and clinical trials.
Downs J; Leonard H; Jacoby P; Brisco L; Baikie G; Hill K
Disability and Rehabilitation
2015
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<a href="http://doi.org/10.3109/09638288.2014.993436" target="_blank" rel="noreferrer noopener">10.3109/09638288.2014.993436</a>
The health and psychosocial functioning of caregivers of children with neurodevelopmental disorders
Purpose. Children with neurodevelopmental disorders (Neuro) pose complex parenting challenges, particularly if the condition co-occurs with behaviour problems. Such challenges are likely to impact caregiver health and well-being. This study explores the extent to which caregivers of children with both Neuro and behaviour problems differ in their physical and psychosocial outcomes from caregivers of children with either condition or neither condition. Method. The first wave of data collected in the National Longitudinal Survey of Children and Youth in Canada (1994) was used to identify four groups of caregivers of 4-to 11-year-old children: caregivers of children with a Neuro disorder and externalising behaviour problems (Both; n = 414), caregivers of children with a Neuro disorder only (Neuro Only; n = 750), caregivers of children with an externalising behaviour problem only (Ext Only; n = 1067), and caregivers of children with neither health condition (Neither; n = 7236). Results. Caregivers in the Both group were least likely to report excellent or very good health, and more frequently reported chronic conditions such as asthma, arthritis, back problems, migraine headaches, and limitations in activities as compared to the Neither group. This group also exhibited higher depression scores, experienced more problematic family functioning and reported lower social support than the Neither group. Scores for caregivers in the Ext Only and Neuro Only groups tended to lie between the Both and Neither group scores and often did not differ from one another. Conclusions. Caregivers of children with both neurovelopmental disorders and behaviour problems exhibited a greater number of health and psychosocial problems. While addressing children's behaviour problems, health care professionals should also consider caregiver physical and psychosocial health, as this may also have an impact on children's well-being.
2009
Lach LM; Kohen DE; Garner RE; Brehaut JC; Miller AR; Klassen AF; Rosenbaum PL
Disability And Rehabilitation
2009
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Journal Article
<a href="http://doi.org/10.1080/09638280802242163" target="_blank" rel="noreferrer">10.1080/09638280802242163</a>
Use of health care among adults with chronic and complex physical disabilities of childhood
Female; Humans; Male; Adult; Ontario; Emergency Service; Hospital/utilization; Adolescent Transitions; retrospective studies; Health Services/utilization; Primary Health Care/utilization; Cerebral Palsy/rehabilitation; Spinal Dysraphism/rehabilitation; Brain Injury; Chronic/rehabilitation; Disabled Persons/rehabilitation
PURPOSE. The purpose of this study was to explore the patterns of health services utilization among adults with chronic and complex physical disabilities of childhood, specifically cerebral palsy, spina bifida, and acquired brain injuries.METHODS. A cohort of 345 young adults who had graduated from the Bloorview MacMillan Children's Centre was identified. Their health care records were extracted from Ontario Health Insurance Plan (OHIP) and Canadian Institute for Health Information (CIHI) databases, for a four-year period. These data were analysed to estimate the frequency of out-patient physician visits and admissions to hospital.RESULTS. The mean age of the sample was 21.9 years (range 19.0-26.9 years). The results show that 95% of the sample visited a physician at least once per year, and 24% had a primary care physician. On average, these adults visited physicians 11.5 times per year (approximately once per month) and were admitted to hospital once every 6.8 years.CONCLUSIONS. These results suggest that adults with complex physical disabling conditions from childhood have ongoing health issues that require frequent service. Their admission rate is 9.0 times that of the general population, and few have a primary care physician. A new model of service may be necessary for this high-needs group.
2005
Young NL; Steele C; Fehlings D; Jutai J; Olmsted N; Williams JI
Disability And Rehabilitation
2005
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Journal Article
<a href="http://doi.org/10.1080/00222930500218946" target="_blank" rel="noreferrer">10.1080/00222930500218946</a>
Clinical scales for the assessment of spasticity, associated phenomena, and function: a systematic review of the literature
Non-U.S. Gov't; PedPal Lit; however; Adult Child Humans Muscle Spasticity/diagnosis/physiopathology Neurophysiology/instrumentation/methods Posture/physiology Range of Motion; Articular/physiology Reflex; as well as of functional scales with an association with spasticity. Reviewed psychometric properties included internal consistency; construct validity; ecological validity; however not in all circumstances. For many scales; interrater; intrarater as well as retest reliability; reliability data is; Stretch/physiology Research Support
2005
Platz T; Eickhof C; Nuyens G; Vuadens P
Disability And Rehabilitation
2005
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Journal Article
<a href="http://doi.org/10.1080/09638280400014634" target="_blank" rel="noreferrer">10.1080/09638280400014634</a>
Assessment of everyday functioning in young children with disabilities: an ICF-based analysis of concepts and content of the Pediatric Evaluation of Disability Inventory (PEDI)
Child; Humans; Interpersonal Relations; disabled children; caregivers; Activities of Daily Living; Self Care; Psychometrics; Disability Evaluation; PEDI Study; Mobility Limitation
BACKGROUND: Assessment of everyday functioning in children may depend to a considerable extent on the framework used to conceptualise functioning and disability. The Pediatric Evaluation of Disability Inventory (PEDI) has incorporated the mediating role of the environment on disability, using different measurement scales. The construction of the Functional Skills scales, which measure capability, and the Caregiver Assistance scales, which measure performance, was based on the Nagi disablement scheme. The International Classification of Functioning, Disability and Health (ICF) represents a new framework of functioning and disability that could be used to compare the measurement constructs and the content of different outcome measurements. PURPOSE: To examine the conceptual basis and the content of the PEDI using the ICF. METHOD: Phrases that describe the conceptual basis of the PEDI scales and of the ICF classifications were systematically collected and compared. Two researchers classified the item content of the Functional Skills scales independently before consensus was reached. RESULTS: The analyses indicate that the conceptual basis of the PEDI scales to a large extent match the ICF concepts of activity, participation and environmental factors. Both the PEDI and the ICF use the constructs of capacity and performance, but differ in how to operationalise these constructs. The classification of the Functional Skills scales shows that the PEDI primarily is a measure of activities and participation. The frequently use of environmental codes to classify the context of the requested functions demonstrates that the PEDI has incorporated the environment into the assessment. CONCLUSIONS: Our analyses indicate that the ICF could serve as a conceptual framework to clarify the measurement construct of the PEDI scales, and as taxonomy to describe and clarify the item content of the Functional Skills scales. Both as framework and taxonomy the ICF showed limitations in covering functioning in early childhood.
2006-04
Østensjø S; Bjorbaekmo W; Carlberg EB; Vøllestad NK
Disability And Rehabilitation
2006
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Journal Article
<a href="http://doi.org/10.1080/09638280500212013" target="_blank" rel="noreferrer">10.1080/09638280500212013</a>
A multi-dimensional approach to the transition of children with developmental disabilities into young adulthood: the acquisition of adult social roles
Child; Cross-Sectional Studies; Female; Humans; Male; Adult; Logistic Models; Questionnaires; Social Adjustment; adolescent; Adolescent Transitions; Social Behavior; Disabled Persons/psychology; Georgia; Developmental Disabilities/psychology
PURPOSE: To test the hypothesis that the difficulties young adults with developmental disabilities have in obtaining adult social roles are not inevitable consequences of their childhood impairment. We used the conceptual framework of the International Classification of Functioning, Disability, and Health to test this hypothesis. METHOD: We used a structured questionnaire to obtain information on the consequences of childhood impairment in young adulthood and to examine the relationship between impairment and acquisition of adult social roles. The sample (n = 635) came from the Metropolitan Atlanta Developmental Disabilities Follow-up Study of Young Adults, a population-based cohort of young adults aged 21 - 25 years identified at age 10 with childhood impairment. RESULTS: The results suggest that: (i) attaining adult social roles varies by impairment type and severity, (ii) experiencing activity limitations partially mediate the relationship between impairment and adult social roles, and (iii) attending postsecondary education increases the likelihood of attaining markers of adulthood. CONCLUSIONS: Intervention to reduce activity limitations and to develop strategies to increase attendance in postsecondary education may increase the likelihood for the acquisition of adult social roles among young adults with childhood impairment.
2006
Van Naarden Braun K; Yeargin-Allsopp M; Lollar D
Disability And Rehabilitation
2006
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Journal Article
<a href="http://doi.org/10.1080/09638280500304919" target="_blank" rel="noreferrer">10.1080/09638280500304919</a>
Psychosocial aspects of chronic widespread pain and fibromyalgia
Social Support and Chronic Pain
2005-01
Bergman S
Disability And Rehabilitation
2005
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Journal Article
<a href="http://doi.org/10.1080/09638280400009030" target="_blank" rel="noreferrer">10.1080/09638280400009030</a>
Inter-rater reliability in assigning ICF codes to children with disabilities
Disability Evaluation; PedPal Lit; Disability; Activities of Daily Living Adolescent Child Child; 17/40 codes using the PEDI; 9 months to 17.75 years old; alternative assessment strategies may be necessary to improve assigning ICF codes to children with disabilities.; and 17/40 codes using the SFA. The mean kappa statistic reached > /= 0.70 for the Self care domain; and Health (ICF) to assign to children with disabilities based on the results of standard paediatric functional assessment measures. METHOD: Children (N = 60); diagnosis/therapy; Disabled Children Female Humans Infant Interpersonal Relations Male Observer Variation Reproducibility of Results Self Care Sensitivity and Specificity Sickness Impact Profile; for 23/40 codes using the Vineland; Preschool Cohort Studies Comparative Study Developmental Disabilities/; the criterion for good agreement in this study; the Pediatric Evaluation of Disability Inventory (PEDI) (n = 20); with a range of disabilities were assessed using either the Vineland Adaptive Behavior Scales (n = 20)
2004
Ogonowski J; Kronk R; Rice C; Feldman H
Disability And Rehabilitation
2004
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Journal Article
<a href="http://doi.org/10.1080/09638280410001658658" target="_blank" rel="noreferrer">10.1080/09638280410001658658</a>
Facilitating communication in children with multiple disabilities: three case studies of girls with Rett syndrome
PedPal Lit; Abnormalities; however; Diagnosis; rehabilitation Risk Factors Sampling Studies Severity of Illness Index Sickness Impact Profile Treatment Outcome; increased communication opportunities provided by caregivers did not elicit increased responses from the girls. CONCLUSION: There is a difference in cueing by teachers in their interactions with children with multiple disabilities. Also; means findings should be viewed cautiously and that more research is indicated.; more frequent communicative interactions did not necessarily lead to increased student responses. It is suggest ed that amount and type of cueing may need to be considered to be effective in generating student responses. The small number of participants; Multiple/diagnosis/rehabilitation Child Behavior/physiology Child; Non-U.S. Gov't Rett Syndrome; Preschool Communication Disorders/diagnosis/rehabilitation Female Follow-Up Studies Humans Infant Interpersonal Relations Language Development Disorders/diagnosis/rehabilitation Research Support
2004
Ryan D; McGregor F; Akermanis M; Southwell K; Ramke M; Woodyatt G
Disability And Rehabilitation
2004
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Journal Article
<a href="http://doi.org/10.1080/09638280412331280280" target="_blank" rel="noreferrer">10.1080/09638280412331280280</a>