Descriptive Report of the Impact of Fatigue and Current Management Strategies in Cerebral Palsy.

Purpose: Describe the impact of fatigue and self-management practices for adolescents and young adults with cerebral palsy (CP). Methods: Survey of 124 people with CP with the Fatigue Impact and Severity Self-Assessment (FISSA). Results: People in GMFCS Level I experienced little impact of fatigue with high proportions of this group disagreeing to statements about fatigue impacting their general activities, mobility and social activities. People in GMFCS Levels II-V reported impact of fatigue on activities. Differences between groups were evident in questions related to fatigue interference with length of time for physical activity and with motivations to participate in social activities. All other items related to management of fatigue were not significantly different between groups. Conclusions: Fatigue impact is greater for people with more functional limitations. Lack of significant differences between groups on the Management and Activity Modification subscale, indicates more research is needed regarding strategies for fatigue management

Measuring Fatigue In Adolescents And Young Adults With Cerebral Palsy

Fatigue is a significant issue and has been estimated to affect between 30-50% of individuals with cerebral palsy (CP) in various studies; however, there is no validated measure of fatigue for this population. A systematic review revealed no one single measure with adequate psychometric properties for use with individuals with CP. As a result a new tool was created: the Fatigue Impact and Severity Self-Assessment (FISSA). Interviews were conducted with youth and young adults with CP to understand the bodily experience of living with CP and as a client-centered approach to item creation for the FISSA. Focus groups with healthcare professionals were used to reduce the number of items on the FISSA and to ensure relevance to the population. A large survey was conducted to assess the validity, reliability and factor structure of the FISSA. The bodily experience of CP revolved around, and emphasized, fatigue that occurs with walking and prolonged activity. Self-awareness of the individuals’ own bodies emerged as the most important theme and strategies employed to prevent and manage fatigue were elucidated. In the validation study, individuals who self-classified as level I on the Gross Motor Function Classification System (GMFCS) were shown to experienced less fatigue than individuals in any other GMFCS level (II-V) (p\u3c .001). Individuals with higher pain (both impact and severity) also reported higher fatigue scores (p\u3c .001). The FISSA was shown to be reliable (α = 0.95; ICC(3,1)=0.74 (95% CI 0.53-0.87)) and contains 31 items related to two factors (Impact of Fatigue and Management/Activity Modification) that together explain 48.7% of the variance in fatigue scores. The FISSA was created to examine the severity, impact and management of fatigue for youth and young adults with CP. The FISSA is reliable and was able to discriminate between groups expected to experience more fatigue including those with a more severe motor disability according to the GMFCS and individuals with a higher degree of pain. The FISSA allows for individualized identification of the activities of daily living that may be compromised by fatigue, which may enhance collaborative goal setting and intervention planning by clinicians and their clients

Validity and reliability of two abbreviated versions of the Gross Motor Function Measure.

Aim: The “gold standard” to measure gross motor functioning for children with cerebral palsy (CP) is the Gross Motor Function Measure (GMFM-66). The purpose of this study was to estimate the validity and reliability of two abbreviated versions (item set (GMFM-66 IS) and basal and ceiling (GMFM-66 B&C) approaches) of the GMFM-66. Methods: Twenty-six children with CP aged 2 to 6 years across all GMFCS levels participated. At session one, both abbreviated versions were administered by two independent raters, followed by the full GMFM-66. In the subsequent session, only the abbreviated versions were administered, by the same raters. Concurrent validity, comparability between versions and test-retest reliability were determined using intraclass correlation coefficients (ICC 2,1). Results: Both versions demonstrated high levels of validity with ICCs reflecting associations with the GMFM-66 of 0.99 (95% CIs ranging from 0.972-0.997). Both versions were also shown to be highly reliable with ICCs greater than 0.98 (95% CIs ranging from 0.965-0.994). Interpretation: Both versions can be used in clinical practice or research. However, the GMFM-66-B&C is recommended as the preferred abbreviated version

Construction and validation of the fatigue impact and severity self-assessment for youth and young adults with cerebral palsy.

Purpose: The Fatigue Impact and Severity Self-Assessment (FISSA) was created to assess the impact, severity and self-management of fatigue for individuals with cerebral palsy (CP) aged 14-31. Methods Used: Items were generated from a review of measures and interviews with individuals with CP. Focus groups with healthcare professionals were used for item reduction. A mailed survey was conducted (n=163/367) to assess the factor structure, known-groups validity and test-retest reliability. Results Obtained: The final measure contained 31-items in two factors and discriminated between individuals expected to have different levels of fatigue. Individuals with more functional abilities reported less fatigue (p\u3c 0.002) and those with higher pain reported higher fatigue (p\u3c 0.001). The FISSA was shown to have adequate test-retest reliability, ICC(3,1)=0.74 (95% CI 0.53-0.87). Conclusions Reached: The FISSA valid and reliable for individuals with CP. It allows for identification of the activities that may be compromised by fatigue to enhance collaborative goal setting and intervention planning

Profiles of Fatigue Severity and Variability Among Adolescents and Young Adults with Cerebral Palsy

Background: Individuals with cerebral palsy (CP) experience progressive changes in functional status with the development of secondary impairments such as fatigue. Detailed accounts of the fatigue experience in CP are lacking. Purpose: This study describes the severity and variability of fatigue in CP using the Fatigue Impact and Severity Self-Assessment. Methods: This was a descriptive cross-sectional study surveying 130 (61 males) individuals (mean age 18 years, 11 months; SD four years, six months) with CP. Analyses comprised comparisons between two groups (Gross Motor Function Classification System (GMFCS) I and II-V), frequency counts and proportions. Results: Significant differences exist between GMFCS groups for all fatigue severity questions. In addition, 38% of individuals classified as GMFCS level I reported their average fatigue as moderate to severe and 53% experienced fatigue on three or more days in the previous week. Sixty-eight percent of individuals classified as GMFCS II-V reported their average fatigue to be moderate to severe and 78% were fatigued on three or more days in the previous week. Conclusions: Fatigue is a highly individualized phenomenon with significant burden for individuals with CP regardless of functional ability. Individuals in GMFCS Levels II-V reported increased fatigue severity and would benefit from specific management interventions

A Narrative Review of the Disenfranchisement of Single Mothers in Highland Ecuador

Background: The Andean South American country of Ecuador presents social challenges that contribute to inequities. The social determinants of health have impacts on the physical, psychological and social health of individuals across all societies. Ecuador is an example of how the interactions of gender roles and social determinants of health impact the health of single mothers and their children. Methods: A retrospective historical literature review was conducted on gender role expectations within the rural context Ecuador to inform future public health strategies and health interventions. Results: Gender inequality contributes to higher rates of single parenting, child labour, and migration. Food insecurity and poverty are affected through the interface of economic hardships and rural agricultural livelihoods. Conclusion: The disenfranchisement of poor rural women in Ecuador is deeply rooted in historical gender discrimination, societal attitudes, and institutionalized gender bias that incur onto the society as a whole in terms of becoming less protectors and producers of human resources. The health of single mothers and children living in poverty and their ability to create a healthy family environment will not improve until women explore their productivity and creativity amid social tensions and livelihood struggles

Parents\u27 Experiences and Perceptions when Classifying their Children with Cerebral Palsy: Recommendations for Service Providers.

AIMS: This study investigated the experiences and perceptions of parents of children with cerebral palsy (CP) when classifying their children using the Gross Motor Function Classification System (GMFCS), the Manual Ability Classification System (MACS), and the Communication Function Classification System (CFCS). The second aim was to collate parents\u27 recommendations for service providers on how to interact and communicate with families. METHODS: A purposive sample of seven parents participating in the On Track study was recruited. Semi-structured interviews were conducted orally and were audiotaped, transcribed, and coded openly. A descriptive interpretive approach within a pragmatic perspective was used during analysis. RESULTS: Seven themes encompassing parents\u27 experiences and perspectives reflect a process of increased understanding when classifying their children, with perceptions of utility evident throughout this process. Six recommendations for service providers emerged, including making the child a priority and being a dependable resource. CONCLUSIONS: Knowledge of parents\u27 experiences when using the GMFCS, MACS, and CFCS can provide useful insight for service providers collaborating with parents to classify function in children with CP. Using the recommendations from these parents can facilitate family-provider collaboration for goal setting and intervention planning

The bodily experience of cerebral palsy: a journey to self-awareness.

PURPOSE: The aim of the study was to describe the lived bodily experience of cerebral palsy (CP). METHOD: This was a descriptive phenomenological inquiry. Ten participants were interviewed about their bodily experiences of living with CP. Interviews were semi-structured around pain and fatigue. Inductive thematic analysis was used to identify themes. RESULTS: The bodily experience of CP centered on issues of fatigue and pain as a feeling of muscle soreness. An overwhelming amount of the discussion on fatigue emphasized the fatigue that occurs with walking and prolonged activity. Self-awareness of the individuals\u27 own bodies and adapting activity to continue to participate in various aspects of their lives emerged as the most important theme. Some participants used strategies to manage their pain or fatigue; other participants were not yet fully aware of how to recognize signs of fatigue and/or how to adapt their activities. CONCLUSIONS: Self-awareness appears to be an important process to be fostered by service providers and parents. Specifically, encouraging youth with CP to be aware of their own bodies and the effects (positive and negative) of activity on pain and fatigue should be incorporated into transition programs as the individual becomes responsible for his or her own health care needs. Implications for Rehabilitation Fatigue is a major concern for some youth and young adults with cerebral palsy. Adolescents and young adults with cerebral palsy use a variety of techniques (including adapting or restricting activity and building in rest breaks) to manage fatigue. The process of self-awareness should be fostered by health care professionals leading up to and during transition from pediatric to adult care. Clinical conversations should explore the role of exercise, adaptive equipment, rest and other strategies for dealing with fatigue with a focus on understanding each client\u27s needs individually

More Than Just Adolescence: Differences in Fatigue Between Youth With Cerebral Palsy and Typically Developing Peers

Objective To quantify differences in fatigue and disordered sleep between adolescents with cerebral palsy (CP) and their typically developing peers. A secondary aim was to investigate the association between fatigue and disordered sleep in adolescents with CP. Methods A convenience sample of 36 youth with CP aged 10-18 years was matched for age and sex with 36 typically developing peers. The Fatigue Impact and Severity Self-Assessment (FISSA), the Patient-Reported Outcome Measurement Information System (PROMIS) fatigue profile, and the Sleep Disturbance Scale for Children (SDSC) were collected. Results Higher fatigue was reported in participants with CP than in their typically developing peers based on the FISSA total score (mean paired difference=19.06; 99% confidence interval [CI], 6.06-32.1), the FISSA impact subscale (mean paired difference=11.19; 99% CI, 3.96-18.4), and the FISSA Management and Activity Modification subscale (mean paired difference=7.86; 99% CI, 1.1-14.6). There were no differences between groups in the PROMIS fatigue profile (mean paired difference=1.63; 99% CI, -1.57-4.83) or the SDSC total score (mean paired difference=2.71; 99% CI, -2.93-8.35). Conclusion Youth with CP experienced significantly more fatigue than their peers as assessed by a comprehensive measure that considered both general and diagnosis-specific concerns. Sleep did not differ between youth with CP and their typically developing peers. These findings underscore the need to consider the clinical management of fatigue across the lifespan of individuals with CP to prevent the associated deterioration of functional abilities

Characterisation of the Stromal Microenvironment in Lobular Breast Cancer

SIMPLE SUMMARY: Invasive lobular breast cancer (ILC) accounts for approximately 5–15% of breast cancers, and although response rates to treatments are initially good, an ILC diagnosis is associated with adverse long-term outcomes; better treatments, specifically targeted to this sub-type of breast cancer, are required to improve patient survival. The tumor microenvironment (TME) plays an important role in determining how cancers respond to treatment, and in this study, we carried out an in-depth analysis of the TME in ILC following laser-capture microdissection of the tumor stroma, and analysis of primary cancer-associated fibroblasts (CAFs), which comprise the majority of non-malignant cells within tumors. This identified changes in genes involved in regulation of the extracellular matrix and also growth factor signaling pathways that were differentially regulated in ILC. Further analysis of breast cancer datasets showed that two of these genes which encode a secreted metalloproteinase (PAPPA) and a metalloproteinase inhibitor (TIMP2) were associated with survival outcomes in ILC. ABSTRACT: Invasive lobular carcinoma (ILC) is the second most common histological subtype of breast cancer, and it exhibits a number of clinico-pathological characteristics distinct from the more common invasive ductal carcinoma (IDC). We set out to identify alterations in the tumor microenvironment (TME) of ILC. We used laser-capture microdissection to separate tumor epithelium from stroma in 23 ER+ ILC primary tumors. Gene expression analysis identified 45 genes involved in regulation of the extracellular matrix (ECM) that were enriched in the non-immune stroma of ILC, but not in non-immune stroma from ER+ IDC or normal breast. Of these, 10 were expressed in cancer-associated fibroblasts (CAFs) and were increased in ILC compared to IDC in bulk gene expression datasets, with PAPPA and TIMP2 being associated with better survival in ILC but not IDC. PAPPA, a gene involved in IGF-1 signaling, was the most enriched in the stroma compared to the tumor epithelial compartment in ILC. Analysis of PAPPA- and IGF1-associated genes identified a paracrine signaling pathway, and active PAPP-A was shown to be secreted from primary CAFs. This is the first study to demonstrate molecular differences in the TME between ILC and IDC identifying differences in matrix organization and growth factor signaling pathways