Newborns with myelomeningocele: their health-related quality of life and daily functioning 10 years later.

OBJECTIVE: Ten years ago, the authors reported on the outcome of their study investigating the degree of discomfort and pain in newborns with myelomeningocele (MMC), using the parameters of unbearable and hopeless suffering. In the current study, they investigated the quality of life, daily functioning, pain and fatigue, ability to communicate, and number of surgeries in the same cohort of patients. They subdivided their study population into severe (Lorber) and less severe (non-Lorber) cases and compared these cases with a healthy population (non-MMC group) and with each other. METHODS: The parents of 22 of 28 patients gave informed consent for this study. The KIDSCREEN-27 and PEDI-CAT (Pediatric Evaluation of Disability Inventory) were used to assess quality of life and daily functioning. Pain and fatigue were self-reported on a 10-point numeric rating scale. Communication and ambulation levels were determined using the Communication Function Classification System (CFCS) and the Hoffer ambulation scale. Using reference data from the KIDSCREEN-27 and PEDI-CAT, the authors created a healthy population comparison group. RESULTS: There was no significant difference in health-related quality-of-life (HRQOL) scores between Lorber and non-Lorber patients, except that school environment domain scores were lower in the Lorber group. When comparing the HRQOL of MMC patients with that of the non-MMC group, the physical well-being and parent relations and autonomy domains scored significantly lower. The daily functioning of MMC patients was lower on all domains of the PEDI-CAT compared with the non-MMC group. Lorber MMC patients scored lower on all domains of the PEDI-CAT when compared with non-Lorber patients. All patients were capable of communicating effectively; most patients (n = 18) were considered CFCS level I, and 4 patients were considered CFCS level II. CONCLUSIONS: This study shows that MMC is a severe, lifelong condition that affects patients' lives in many domains. All the patients in this study are capable of effective communication, irrespective of severity of MMC. Overall, the data show that in newborn MMC patients, future unbearable suffering with respect to pain, mobility, cognition, and communication is hard to predict and may not always occur.

Entering the labor market: increased employment rates of young adults with chronic physical conditions after a vocational rehabilitation program.

PURPOSE: Employment of young adults with chronic physical conditions entering the labor market after finishing post-secondary education remains behind compared to typically developing peers. The aim of this study is to evaluate changes in their paid employment levels after following a vocational rehabilitation intervention ('At Work'). MATERIALS AND METHODS: Participants aged between 16 and 27 years (n = 90) were recruited via rehabilitation physicians and a jobcoach agency and participated in a vocational rehabilitation program. Cochran's Q and McNemar tests served to test the development of intervention participants' paid employment over time. Chi-square tests were used to compare intervention participants' paid employment level with national reference data selected on age and having a self-reported chronic physical condition. RESULTS: Paid employment level of the intervention cohort significantly increased from 10.0% at baseline to 42.4% at 2-years follow-up (p < 0.001). At 2-years follow-up, their employment rates approached the employment rates of national reference data (42.4% versus 52.9%, p = 0.17). CONCLUSION: Starting from a disadvantaged position, the paid employment rate of the intervention cohort substantially increased over time, approaching the employment rate of reference data. 'At Work' seems to be appropriate for supporting this specific group who face obstacles to enter the labor market, to find competitive employment.IMPLICATIONS FOR REHABILITATION'At Work' is a vocational rehabilitation intervention for young adults with chronic physical conditions who experience problems with finding and maintaining competitive employment after finishing post-secondary education.The 'At Work' intervention entails a combination of group sessions and individual coaching sessions based on the supported employment methodology.Paid employment rates of the intervention cohort substantially increased on the short- and long term, and approached employment rates of reference data of persons with chronic physical conditions.The 'At Work' intervention seems appropriate to support young adults with chronic physical conditions who experience barriers for work participation, to enter the labor market and find competitive and sustainable employment.

Fatigue Profiles in Patients with Multiple Sclerosis are Based on Severity of Fatigue and not on Dimensions of Fatigue.

Fatigue related to Multiple Sclerosis (MS) is considered a multidimensional symptom, manifesting in several dimensions such as physical, cognitive, and psychosocial fatigue. This study investigated in 264 patients with severe primary MS-related fatigue (median MS duration 6.8 years, mean age 48.1 years, 75% women) whether subgroups can be distinguished based on these dimensions. Subsequently, we tested whether MS-related fatigue consists of a single common unidimensional factor. Subscale scores on four self-reported fatigue questionnaires, including the Checklist of Individual Strength, the Modified Fatigue Impact Scale, the Fatigue Severity Scale and the SF36 vitality, were used in a cluster analysis to identify patients with similar fatigue characteristics. Next, all 54 items were included in exploratory factor analysis to test unidimensionality. Study results show that in patients with a treatment indication for primary MS-related fatigue, fatigue profiles are based on severity and not on the various dimensions of fatigue. The three profiles found, suggested one underlying fatigue dimension, but this could not be confirmed. Factor analysis of all 54 items resulted in 8 factors, confirming the multidimensional construct of the included fatigue questionnaires.

Teenage predictors of participation of adults with cerebral palsy in domestic life and interpersonal relationships: A 13-year follow-up study.

BACKGROUND: Adults with cerebral palsy (CP) may experience problems with participation in domestic life and interpersonal relationships. AIMS: To identify teenage predictors of adult participation in domestic life and interpersonal relationships. METHODS AND PROCEDURES: This 13-year follow-up of the PERRIN 16-24 cohort included 53 adults with CP without intellectual disability [current age 31.7 (SD = 1.4) years]. Participation performance was assessed as attendance (Vineland Adaptive Behavior Scales), and difficulty/assistance with participation (Life Habits questionnaire). 56 teenage factors were categorized in ICF components. Stepwise multiple linear regression analyses explored predictors of participation. OUTCOMES AND RESULTS: Lower gross motor capacity, following special education, having protective parents and a rigid personality predicted less participation in domestic life. Having rejective parents, receiving little daily support, having a socially avoidant personality or coping style and the male gender predicted less participation in interpersonal relationships. Lower activity and participation levels as a teenager predicted less participation in both domestic life and interpersonal relationships of adults with CP. CONCLUSIONS AND IMPLICATIONS: Environmental and personal factors, gross motor capacity and teenage participation were predictors of participation of adults with CP. These factors help identify subgroups at risk for suboptimal adult participation and provide targets for rehabilitation.

Contemporary management and outcome of myelomeningocele: the Rotterdam experience.

OBJECTIVE: Myelomeningocele (MMC) is the most common form of spina bifida, with a lifelong impact on the quality of life for infants born with this condition. In recent decades, fetal surgery has evolved from an experimental therapy to standard of care for many centers in the world. In this study, the authors aimed to provide an overview of the current management and outcomes for infants with MMC managed at their institution. This then provides a center-specific historical cohort for comparison with future antenatal-treated MMC cases. METHODS: This is a retrospective, single-institution cohort study including all consecutive MMC cases between January 1, 2000, and June 1, 2018, at Erasmus MC. Outcome data included closure of the defect (location, timing, and surgical parameters), hydrocephalus management, Chiari malformation type II (CMTII) management, incidence of spinal cord tethering and outcome, motor outcomes, and continence. RESULTS: A total of 93 patients were included with predominantly lumbosacral lesions. Two patients died during follow-up. Hydrocephalus was present in 84%, with a 71% ventriculoperitoneal shunt reoperation rate. Surgery was performed in 12% for a tethered spinal cord at a mean age of 8 years. Decompression surgery was performed in 3 patients for CMTII. Special education in 63% was significantly associated with hydrocephalus (p < 0.015). Nineteen percent of patients were able to walk independently, and 47% were nonambulators. Social continence for urine was obtained in 75% of patients, 4% had fecal incontinence. CONCLUSIONS: This study provides an overview of current MMC outcomes at the authors' center and will serve as a historical cohort for comparison with future fetal surgery cases operated on at the center in the coming years. Apart from a relatively low surgical untethering rate, the authors' outcome data are comparable to those in the literature. Hydrocephalus is highly prevalent in postnatally treated MMC patients; in this study as in much of the literature, hydrocephalus is correlated with a low cognitive function. Fetal surgery for MMC halves the need for shunt treatment in a select group of MMC pregnancies, constituting a major indication for us to undergo the transition to a fetal surgery center. The fetal benefits of open antenatal surgery for MMC are well established, yet long-term data on especially tethered spinal cord are eagerly awaited.

Energy Conservation Management for People With Multiple Sclerosis-Related Fatigue: Who Benefits?

OBJECTIVE: We investigated whether demographic, disease-related, or personal baseline determinants can predict a positive response to energy conservation management (ECM). METHOD: We conducted a secondary analysis of a single-blind, two-parallel-arms randomized controlled trial that included ambulatory adults with severe MS-related fatigue. Therapy responders and nonresponders were categorized by Checklist Individual Strength fatigue change scores between baseline and end of treatment. Logistic regression analyses were used to assess the determinants of response. RESULTS: Sixty-nine participants were included (ECM group, n = 34; control group, n = 35). In the ECM group, fatigue severity, perception of fatigue, illness cognitions about MS, and social support discrepancies were related to the probability of being a responder. CONCLUSION: The results suggest that people with MS-related fatigue who had a less negative perception of fatigue and who perceived fewer disease benefits and a higher discrepancy in social support had the best response to ECM treatment.

Physical behaviour is weakly associated with physical fatigue in persons with multiple sclerosis-related fatigue.

BACKGROUND: Fatigue affects 80% of persons with multiple sclerosis and is associated with daily physical functioning. Both fatigue and physical behaviour are multidimensional concepts. OBJECTIVE: To study the association between the dimensions of physical behaviour and multiple sclerosis-related fatigue. METHODS: Cross-sectional analysis of 212 persons with multiple sclerosis. Participants were severely fatigued, with a Fatigue Severity Scale median (interquartile range): 5.4 (4.8-5.9) and were minimally to moderately neurologically impaired, based on the Expanded Disability Status Scale: 2.5 (2.0-3.5), 73% had relapsing-remitting multiple sclerosis. Fatigue was measured by questionnaires (i.e. Checklist Individual Strength, Modified Fatigue Impact Scale), and the dimensions subjective, physical, cognitive and psychological fatigue were distinguished. Physical behaviour was measured using an Actigraph GT3X+, and outcomes were categorized into the dimensions of activity amount, activity intensity, day pattern, and distribution of activities. RESULTS: The physical behaviour dimensions were significantly associated with only the physical fatigue dimension (omnibus F-test: 3.96; df1 = 4, df2 = 207; p = 0.004). Additional analysis showed that the amount of activity (unstandardized beta coefficient (ß) = -0.16; 95% confidence interval (CI) -0.27 to -0.04; p = 0.007), activity intensity (ß = -0.18; 95% CI -0.31 to -0.06; p = 0.004) and day pattern of activity (ß = -0.17; 95% CI, -0.28 to -0.06; p = 0.002) were the physical behaviour dimensions that were significantly associated with physical fatigue. CONCLUSION: Physical behaviour is weakly associated with physical fatigue and is not associated with other dimensions of fatigue.

Effectiveness of energy conservation management on fatigue and participation in multiple sclerosis: A randomized controlled trial.

BACKGROUND: Fatigue is a frequently reported and disabling symptom in multiple sclerosis (MS). OBJECTIVE: To investigate the effectiveness of an individual energy conservation management (ECM) intervention on fatigue and participation in persons with primary MS-related fatigue. METHODS: A total of 86 severely fatigued and ambulatory adults with a definite diagnosis of MS were randomized in a single-blind, two-parallel-arm randomized clinical trial to the ECM group or the information-only control group in outpatient rehabilitation departments. Blinded assessments were carried out at baseline and at 8, 16, 26 and 52 weeks after randomization. Primary outcomes were fatigue (fatigue subscale of Checklist Individual Strength - CIS20r) and participation (Impact on Participation and Autonomy scale - IPA). RESULTS: Modified intention-to-treat analysis was based on 76 randomized patients (ECM, n = 36; MS nurse, n=40). No significant ECM effects were found for fatigue (overall difference CIS20r between the groups = -0.81; 95% confidence interval (CI), -3.71 to 2.11) or for four out of five IPA domains. An overall unfavourable effect was found in the ECM group for the IPA domain social relations (difference between the groups = 0.19; 95% CI, 0.03 to 0.35). CONCLUSION: The individual ECM format used in this study did not reduce MS-related fatigue and restrictions in participation more than an information-only control condition.

Longitudinal Association Between Gross Motor Capacity and Neuromusculoskeletal Function in Children and Youth With Cerebral Palsy.

OBJECTIVE: To examine associations over longitudinal measurements between neuromusculoskeletal function and gross motor capacity in children and youth with cerebral palsy (CP). DESIGN: A prospective cohort study. SETTING: Rehabilitation departments of university medical centers and rehabilitations centers. PARTICIPANTS: A sample (N=327) consisting of 148 children (aged 5-9y) and 179 youth (aged 11-20y) with CP, Gross Motor Function Classification System level I (n=180), level II (n=44), level III (n=36), level IV (n=34), and level V (n=33). INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Gross motor capacity was assessed with the Gross Motor Function Measure-66 over a period of 2 to 4 years in different age cohorts. Neuromusculoskeletal function included selective motor control (SMC), muscle strength, spasticity, and range of motion (ROM) of the lower extremities. RESULTS: Multilevel analyses showed that SMC was significantly associated with gross motor capacity in children and youth with CP, showing higher values and a more favorable course of gross motor capacity in those with better SMC. Strength was only associated with gross motor capacity in youth. Reduced ROM of hip (children) and knee extension (youth) and spasticity of the hip adductors (youth) were additionally-but more weakly-associated with lower values and a less favorable course of gross motor capacity. CONCLUSIONS: Results indicate that children and youth with more severely impaired SMC and youth with reduced muscle strength have a less favorable course of gross motor capacity, while spasticity and reduced ROM are less determinative.

Cost-utility of a lifestyle intervention in adolescents and young adults with spastic cerebral palsy.

OBJECTIVE: To evaluate the cost-utility of a lifestyle intervention among adolescents and young adults with cerebral palsy. DESIGN: Single-blind, randomized controlled trial. SETTING: Six university hospital/clinics in the Netherlands. PARTICIPANTS: Fifty-seven adolescents and young adults with spastic cerebral palsy classified as Gross Motor Functioning Classification System (GMFCS) level I-IV. INTERVENTION: A 6-month lifestyle intervention consisting of physical fitness training combined with counselling sessions focusing on physical behaviour and sports participation. MAIN OUTCOME MEASURES: Data on quality of life, direct medical costs and productivity costs were collected using standardized questionnaires. Quality adjusted life years (QALYs) were derived from the Short-Form 36 questionnaire using the Short-Form 6D. RESULTS: Quality of life remained stable over time for both groups. No significant differences between groups were found for direct medical costs or productivity costs. A cost-utility ratio of -€23,664 per QALY was found for the lifestyle intervention compared with no treatment. CONCLUSION: The results of this study are exploratory, but indicate that implementing a lifestyle intervention for the cerebral palsy population might be cost-effective or cost-saving compared with offering no intervention to improve physical behaviour and fitness. However, the large range of uncertainty for the cost-utility ratio should be taken into account and the results interpreted with caution.

Is physical behavior affected in fatigued persons with multiple sclerosis?

OBJECTIVE: To study physical behavior in detail in fatigued persons with multiple sclerosis (MS). DESIGN: Case-control explorative study. SETTING: Outpatient rehabilitation department and participants' daily environment. PARTICIPANTS: Fatigued persons with MS (n=23) were selected from a randomized controlled trial. Cases were matched by age and sex to healthy, nonfatigued controls (n=23). Eligible persons with MS were severely fatigued (Checklist Individual Strength fatigue domain mean score, 43.2±6.6) and ambulatory (Expanded Disability Status Scale mean score, 2.5±1.5). INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Measurements were performed using an accelerometer over 7 days. Outcomes included the following: amount of physical activity expressed in counts per day, counts per minute (CPM), and counts per day period (morning, afternoon, evening); duration of activity intensity categories (sedentary, light physical activity, moderate-to-vigorous physical activity [MVPA]); and distribution of MVPA and sedentary periods over the day. RESULTS: Persons with MS had fewer counts per day (mean difference, -156×10(3); 95% confidence interval [CI], -273×10(3) to -39×10(3); P=.010), had fewer CPM (mean difference, -135; 95% CI, -256 to -14; P=.030), and were less physically active in the morning (mean difference, -200; 95% CI, -389 to -11; P=.039) and evening (mean difference, -175; 95% CI, -336 to -14; P=.034) than controls. Persons with MS spent a higher percentage of their time sedentary (mean difference, 5.6; 95% CI, .1-11.1; P=.045) and spent less time at the higher MVPA intensity (mean difference, -2.4; 95% CI, -4.7 to -0.09; P=.042). They had fewer MVPA periods (mean difference, 29; 95% CI, -56.2 to -2.6; P=.032) and a different distribution of sedentary (mean difference, .033; 95% CI, .002 to .064; P=.039) and MVPA periods (mean difference, -.08; 95% CI, -.15 to -.01; P=.023). CONCLUSIONS: Detailed analyses of physical behavior showed that ambulatory fatigued persons with MS do differ from healthy controls not only in physical activity level, but also in other physical behavior dimensions (eg, day patterns, intensity, distribution).

Long-term trajectories of health-related quality of life in individuals with cerebral palsy: a multicenter longitudinal study.

OBJECTIVES: To (1) determine the long-term trajectory of health-related quality of life (HRQOL) for the dimensions of physical complaints and motor, psychological, and social functioning for groups of individuals with cerebral palsy (CP) aged 1 to 24 years; (2) assess the variability in HRQOL within individuals with CP over time; (3) assess the variability in HRQOL between individuals with CP; and (4) compare the HRQOL in individuals with CP to reference data of typically developing individuals. DESIGN: Multicenter prospective longitudinal study. SETTING: Rehabilitation departments of 3 university medical centers and various rehabilitation centers in The Netherlands. PARTICIPANTS: Dutch individuals with CP (N=424; age, 1-24y). INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: The HRQOL dimensions of physical complaints and motor, psychological, and social functioning. Each individual visited the rehabilitation department for 3 or 4 measurements. The time between measurements was 1 or 2 years. RESULTS: Individuals with CP experience an HRQOL that, on average, remains fairly stable over time. Variability in HRQOL within individuals with CP was similar to that within typically developing individuals. Variability between individuals with CP could be explained by type of CP (motor functioning), Gross Motor Function Classification System level (physical complaints and motor and social functioning), and intellectual disability (physical complaints and social functioning). Finally, individuals with CP experienced a lower HRQOL than did typically developing individuals, especially for the dimensions of motor and social functioning. CONCLUSIONS: Many changes take place in the psychosocial development of the individual with CP, which accordingly change their expectations and those of their caregivers, peers, and professionals. As a result, perceived physical complaints and motor, psychological, and social functioning remain fairly stable over many years.

The effectiveness of aerobic training, cognitive behavioural therapy, and energy conservation management in treating MS-related fatigue: the design of the TREFAMS-ACE programme.

BACKGROUND: TREFAMS is an acronym for TReating FAtigue in Multiple Sclerosis, while ACE refers to the rehabilitation treatment methods under study, that is, Aerobic training, Cognitive behavioural therapy, and Energy conservation management. The TREFAMS-ACE research programme consists of four studies and has two main objectives: (1) to assess the effectiveness of three different rehabilitation treatment strategies in reducing fatigue and improving societal participation in patients with MS; and (2) to study the neurobiological mechanisms of action that underlie treatment effects and MS-related fatigue in general. METHODS/DESIGN: Ambulatory patients (n = 270) suffering from MS-related fatigue will be recruited to three single-blinded randomised clinical trials (RCTs). In each RCT, 90 patients will be randomly allocated to the trial-specific intervention or to a low-intensity intervention that is the same for all RCTs. This low-intensity intervention consists of three individual consultations with a specialised MS-nurse. The trial-specific interventions are Aerobic Training, Cognitive Behavioural Therapy, and Energy Conservation Management. These interventions consist of 12 individual therapist-supervised sessions with additional intervention-specific home exercises. The therapy period lasts 16 weeks. All RCTs have the same design and the same primary outcome measures: fatigue - measured with the Checklist Individual Strength, and participation - measured with the Impact on Participation and Autonomy questionnaire. Outcomes will be assessed 1 week prior to, and at 0, 8, 16, 26 and 52 weeks after randomisation. The assessors will be blinded to allocation. Pro- and anti-inflammatory cytokines in serum, salivary cortisol, physical fitness, physical activity, coping, self-efficacy, illness cognitions and other determinants will be longitudinally measured in order to study the neurobiological mechanisms of action. DISCUSSION: The TREFAMS-ACE programme is unique in its aim to assess the effectiveness of three rehabilitation treatments. The programme will provide important insights regarding the most effective treatment for MS-related fatigue and the mechanisms that underlie treatment response. A major strength of the programme is that the design involves three almost identical RCTs, enabling a close comparison of the treatment strategies and a strong overall meta-analysis. The results will also support clinical practice guidelines for the treatment of MS-related fatigue. TRIAL REGISTRATIONS: Current Controlled Trials ISRCTN69520623, ISRCTN58583714, and ISRCTN82353628.

A new intervention to improve work participation of young adults with physical disabilities: a feasibility study.

AIM: The aim of the study was to evaluate the feasibility of a new intervention to improve work participation of young adults with physical disabilities, addressing (1) implementation and costs and (2) preliminary effectiveness. METHOD: Twelve young adults with physical disabilities (six males, six females; age 19-28 y, median age 21 y 6 mo) participated in a 1-year multidisciplinary vocational rehabilitation intervention. In a pre-post intervention design, we assessed implementation and costs as well as preliminary effectiveness in terms of employment and occupational performance using questionnaires and interviews. We tested pre-post differences with the McNemar test for proportions and the Wilcoxon signed-rank test for scores on occupational performance; p-values less than 0.05 were considered statistically significant. Additionally, we assessed work participation at follow-up after 2 years and 3 years. RESULTS: The intervention was implemented in an outpatient rehabilitation clinic for young adults. The median cost per participant for 1 year was €3128, which is an equivalent to the cost of 72 contact hours per participant. Post intervention, and at 2 years and 3 years follow-up, a significantly higher proportion of participants were employed (8/12 post vs 2/12 pre-intervention; p<0.05), with the ratio of those in paid to unpaid employment being 4:4, 5:3, and 7:1 respectively. Participants showed improved occupational performance in work, self-care, and leisure. INTERPRETATION: Feasibility and preliminary effectiveness of the intervention are promising. Employed participants seemed to have achieved suitable and continuous employment.

Effectiveness of energy conservation treatment in reducing fatigue in multiple sclerosis: a systematic review and meta-analysis.

OBJECTIVES: To systematically review the effects of energy conservation management (ECM) treatment for fatigue in multiple sclerosis (MS), and to study the effect of ECM treatment on restrictions in participation and quality of life (QoL). DATA SOURCES: PubMed, CINAHL, Embase, and Web of Knowledge were searched to identify relevant randomized controlled trials (RCTs) and controlled clinical trials. STUDY SELECTION: To select potential studies, 2 reviewers independently applied the inclusion criteria. DATA EXTRACTION: Two reviewers independently extracted data and assessed the methodologic quality of the studies included. If meta-analysis was not possible, qualitative best-evidence synthesis was used to summarize the results. DATA SYNTHESIS: The searches identified 532 studies, 6 of which were included. The studies compared the short-term effects of ECM treatment and control treatment on fatigue and QoL; 1 study reported short-term and midterm effects on participation, but found no evidence for effectiveness. Meta-analyses (2 RCTs, N=350) showed that ECM treatment was more effective than no treatment in improving subscale scores of the (1) Fatigue Impact Scale: cognitive (mean difference [MD]=-2.91; 95% confidence interval [CI], -4.32 to -1.50), physical (MD=-2.99; 95% CI, -4.47 to -1.52), and psychosocial (MD=-6.05; 95% CI, -8.72 to -3.37); and (2) QoL: role physical (MD=17.26; 95% CI, 9.69-24.84), social function (MD=6.91; 95% CI, 1.32-12.49), and mental health (MD=5.55; 95% CI, 2.27-8.83). Limited or no evidence was found for the effectiveness of ECM treatment on the other outcomes in the short-term or midterm. None of the studies reported long-term results. CONCLUSIONS: The systematic review results provide evidence that in the short-term, ECM treatment can be more effective than no treatment (waiting controls) in reducing the impact of fatigue and in improving 3 QoL scales-role physical, social function, and mental health-in fatigued patients with MS. More RCTs that also study long-term results are needed.

Promoting physical activity in an adolescent and a young adult with physical disabilities.

BACKGROUND: We sought to describe the design of the Active Lifestyle and Sports Participation (ALSP) intervention for adolescents and young adults with physical disabilities, and to present the first 2 cases. METHODS: A 17-year-old boy with myelomeningocele and hydrocephalus and a 23-year-old woman with unilateral cerebral palsy were enrolled into the ALSP intervention, a personalized intervention designed to improve physical activity and fitness levels. Main outcome measures were self-reported physical activity and aerobic fitness. Fitness was determined by submaximal 6-minute walk or wheel test and by maximal cycle or arm ergometer-exercise test. Participants rated satisfaction with the intervention on a Likert-type numeric scale from 1 to 10. RESULTS: Improvements in self-reported physical activity were 51% and 75% for the male and female participant, respectively. Respective improvements in submaximal exercise were 16% and 9%. Maximal exercise increased 39% in the male participant but did not increase in the female participant. Satisfaction with the intervention was rated moderate-good to excellent. CONCLUSION: Data for the first 2 cases suggested that ALSP intervention seemed feasible to offer in an outpatient rehabilitation department, and the effectiveness may be promising. Future studies should determine the short- and long-term effectiveness of the intervention.

Learn 2 Move 16-24: effectiveness of an intervention to stimulate physical activity and improve physical fitness of adolescents and young adults with spastic cerebral palsy; a randomized controlled trial.

BACKGROUND: Persons with cerebral palsy (CP) are at risk for developing an inactive lifestyle and often have poor fitness levels, which may lead to secondary health complications and diminished participation and quality of life. However, persons with CP also tend not to receive structural treatment to improve physical activity and fitness in adolescence, which is precisely the period when adult physical activity patterns are established. METHODS: We aim to include 60 adolescents and young adults (16-24 years) with spastic CP. Participants will be randomly assigned to an intervention group or a control group (no treatment; current policy). The intervention will last 6 months and consist of three parts; 1) counselling on daily physical activity; 2) physical fitness training; and 3) sports advice. To evaluate the effectiveness of the intervention, all participants will be measured before, during, directly after, and at 6 months following the intervention period. Primary outcome measures will be: 1) physical activity level, which will be measured objectively with an accelerometry-based activity monitor during 72 h and subjectively with the Physical Activity Scale for Individuals with Physical Disabilities; 2) aerobic fitness, which will be measured with a maximal ramp test on a bicycle or armcrank ergometer and a 6-minute walking or wheelchair test; 3) neuromuscular fitness, which will be measured with handheld dynamometry; and 4 body composition, which will be determined by measuring body mass, height, waist circumference, fat mass and lipid profile. CONCLUSIONS: This paper outlines the design, methodology and intervention of a multicenter randomized controlled trial (LEARN 2 MOVE 16-24) aimed at examining the effectiveness of an intervention that is intended to permanently increase physical activity levels and improve fitness levels of adolescents and young adults with CP by achieving a behavioral change toward a more active lifestyle. TRIAL REGISTRATION: Dutch Trial Register; NTR1785.

Using the manual ability classification system in young adults with cerebral palsy and normal intelligence.

PURPOSE: The study aimed to establish whether the manual ability classification system (MACS), a valid classification system for manual ability in children with cerebral palsy (CP), is applicable in young adults with CP and normal intelligence. SUBJECTS: The participants (n = 83) were young adults with CP and normal intelligence and had a mean age of 19.9 years. METHOD: In this study, inter observer reliability of the MACS was determined. We investigated relationships between the MACS level and patient characteristics (such as the gross motor function classification system (GMFCS) level, limb distribution of the spastic paresis and educational level) and with functional activities of the upper extremity (assessed with the Melbourne assessment, the Abilhand questionnaire and the domain self-care of the functional independence measure (FIM)). Furthermore, with a linear regression analysis it was determined whether the MACS is a significant determinant of activity limitations and participation restrictions. RESULTS: The reliability was good (intraclass correlation coefficient 0.83). The Spearman correlation coefficients with GMFCS level, limb distribution of the spastic paresis and educational level were 0.53, 0.46, and 0.26, respectively. MACS level correlated moderately with outcome measures of functional activities (correlations ranging from -0.38 to -0.55). MACS level is, in addition to the GMFCS level, an important determinant for limitations in activities and restrictions in participation. CONCLUSION: We conclude that the MACS is a feasible method to classify manual ability in young adults with CP and normal intelligence with a good manual ability.

Lifestyle, participation, and health-related quality of life in adolescents and young adults with myelomeningocele.

This study aimed to describe participation and health-related quality of life (HRQoL) in adolescents and young adults with myelomeningocele and to explore their relationships with lifestyle-related factors. Fifty-one individuals with a mean age of 21 years 1 month (SD 4y 6mo) years participated (26 males, 25 females; 82% hydrocephalus, 55% wheelchair-dependent). Participation was assessed using the Life Habits Questionnaire, and HRQoL was assessed using the Medical Outcomes Study 36-item Short-form Health Survey. Physical activity was measured using an accelerometry-based activity monitor, fitness (peak oxygen uptake) was measured during a maximal exercise test, and the sum of four skin-folds was assessed to indicate body fat. Relationships were studied using logistic regression analyses. Of the participants, 63% had difficulties in daily activities and 59% in social roles. Participants perceived lower physical HRQoL than a Dutch reference population. Participants with higher levels of physical activity and fitness had fewer difficulties in participating in daily activities (odds ratio [OR]=8.8, p=0.02 and OR=29.7, p=0.02 respectively) and a higher physical HRQoL (OR=4.8, p=0.02 and OR=30.2, p=0.006 respectively), but not mental HRQoL. Body fat was not related to participation or HRQoL. In conclusion, a large proportion of individuals with myelomeningocele had difficulties in participation and perceived low physical HRQoL. Higher levels of physical activity and fitness were related to fewer difficulties in participation and higher physical HRQoL.

Transition to adulthood: validation of the Rotterdam Transition Profile for young adults with cerebral palsy and normal intelligence.

The aim of this study was to investigate the validity of the Rotterdam Transition Profile (RTP) to describe the transition process from childhood to adulthood in young adults with cerebral palsy (CP). Participants were recruited from rehabilitation centres and hospital departments of rehabilitation. In total, 81 young adults (47 males, 34 females) with CP and normal intelligence participated (mean age 20y 5mo [SD 1y 4mo] range 18-22y; 95% spastic CP, 48% hemiplegia, 38% diplegia, 14% quadriplegia; 78% Gross Motor Function Classification System Level I, 83% Manual Ability Classification System Level I). The RTP and the Assessment of Life Habits questionnaire are used to measure transition and functioning in daily activities and participation. Almost all participants were in the transition process or had reached an independent adult lifestyle (ranging from 60-100%, housing 42%). Compared with able-bodied peers, young adults with CP lagged behind in their development in housing (25 vs 36%; p<0.05), employment (33 vs 49%; p<0.05), and intimate relationships (37 vs 76%; p<0.01). Associations were found between the phase of transition and age, parents' level of education, gross motor functioning, manual ability, level of education, and level of functioning in daily activities and participation. The RTP is a valid tool to gain more insight into the transition process, at the individual as well as at group level.