Effects of Interdisciplinary Therapy in A Patient with Severe Dystonic Cerebral Palsy: a 12-Year Follow-up Case Report

Respiratory problems have a significant impact on morbidity and mortality in patients with cerebral palsy (CP). In particular, recurrent aspiration, impaired airway clearance, spinal and thoracic deformity, impaired lung function, poor nutritional status, and recurrent respiratory infections negatively affect respiratory status. Bronchopulmonary dysplasia may contribute to pulmonary problems, but asthma is not more common in CP than in the general population. We discuss treatment options for each of these factors. Multiple coexisting and interacting factors that influence the respiratory status of patients with CP should be recognized and effectively addressed to reduce respiratory morbidity and mortality. WHAT THIS PAPER ADDS: Respiratory problems are a significant cause of morbidity in patients with cerebral palsy (CP). Respiratory status in patients with CP is influenced by recurrent aspiration and impaired airway clearance. Spinal and thoracic deformity, impaired lung function, poor nutrition, and respiratory infections also negatively affect respiratory status. These factors should all be addressed to reduce respiratory problems in patients with CP.

Epidemiology and risk factors for sleep disturbances in children and youth with cerebral palsy: An ICF-based approach

BackgroundThe WHO International Classification of Functioning, Disability and Health (ICF) is now the dominant model for exploring the social consequences of a health condition. This paper investigates the association of...

Halliwick aquatic therapy is a rehabilitation intervention that is gaining popularity for people with disabilities. This scoping review provides an overview on the state of research about the impact of Halliwick aquatic therapy for children with disabilities. Four electronic databases were searched to obtain research on the use of the Halliwick method for paediatric rehabilitation: Medline, CINAHL, Embase and PsycINFO. Potential citations were first screened by title and abstract, and full texts were then examined on the second round of screening. We analyzed the demographic details of their study population, how therapy was implemented (e.g., lesson frequency or structure), and what measurements were used, with measured variables mapped onto the domains of the framework for health of the WHO's International Classification of Functioning, Disability and Health (ICF). Twenty-four publications met the inclusion criteria for this review. The majority of research included children with cerebral palsy (n = 12) or autism spectrum disorder (n = 8), with very few studies including other disabilities (n = 5). There was a wide variation in the number of lessons per study and how each lesson was structured. Fourteen studies lacked a control group. Looking at ICF domains, all 24 articles measured variables pertaining to body structure and functions, four looked for changes in performance of daily activities, four into ability to participate in social roles, and seven into changes in personal factors. There is no consensus on how the Halliwick method should be structured for participants, leaving a gap for future research on programme implementation. To shift our viewpoint beyond what a disability prevents to what one's level of health and functioning allows, it is important to broaden the scope of research into the other ICF domains.

Clinical measures in health and rehabilitation settings are often used to examine child functioning to better support the diverse needs of children with neurodevelopmental disorders (NDD) and their families. The WHO's International Classification of Functioning, Disability, and Health (ICF) framework reflects a focus of health beyond biomedical deficits, using the concept of functioning to create opportunities for measurement development involving this construct. In the measures developed in the field of childhood NDD, it is unclear whether and how these tools measure and incorporate the ICF framework and its domains within health care contexts. Understanding how these measures utilize the ICF will enable researchers and clinicians to operationalize function-focused concepts in studies and clinical practice more effectively. This narrative review aims to identify and describe function-focused measures that are based on the ICF for children with NDD, as described in the peer-reviewed literature. This review used a systematic search strategy with multiple health-focused databases (Medline, PsycInfo, EMBASE, EMCARE), and identified 14 clinical measures that provide direct support for children (aged 0-21) with NDD in pediatric health (and other) settings. Results described the measures that were primarily developed for three main diagnostic populations [cerebral palsy, autism spectrum disorder, and communication disorders]; had varying contextual use (clinical-only or multiple settings); and for which authors had conducted psychometric tests in the measure's initial development studies, with the most common being content validity, interrater reliability, test-retest reliability. Participation (79%, n = 11) & Activities (71%, n = 10) were the most common ICF domains captured by the set of measurement tools. Overall (71%, n = 10) of the identified measures utilized multiple ICF domains, indicating that the “dynamic nature” of the interactions of the ICF domains was generally evident, and that this result differentiated from “linking rules,” commonly used in research and clinical practice. The implications of these findings suggest that clinical measures can be an effective application of the ICF's defined concepts of functioning for children with NDD.

There is a lack of accurate information on the prevalence and causes of musculoskeletal impairment (MSI) in low income countries. The WHO prevalence estimate does not help plan services for specific national income levels or countries. The aim of this study was to find the prevalence, impact, causes and factors associated with musculoskeletal impairment in Malawi. We wished to undertake a national cluster randomized survey of musculoskeletal impairment in Malawi, one of the UN Least Developed Countries (LDC), that involved a reliable sampling methodology with a case definition and diagnostic criteria that could clearly be related to the classification system used in the WHO International Classification of Functioning, Disability and Health (ICF). A sample size of 1,481 households was calculated using data from the latest national census and an expected prevalence based on similar surveys conducted in Rwanda and Cameroon. We randomly selected clusters across the whole country through probability proportional to size sampling with an urban/rural and demographic split that matched the distribution of the population. In the field, randomization of households in a cluster was based on a ground bottle spin. All household members present were screened, and all MSI cases identified were examined in more detail by medical students under supervision, using a standardized interview and examination protocol. Data collection was carried out from 1st July to 30th August 2016. Extrapolation was done based on study size compared to the population of Malawi. MSI severity was classified using the parameters for the percentage of function outlined in the WHO International Classification of Functioning (ICF). A loss of function of 5-24% was mild, 25-49% was moderate and 50-90% was severe. The Malawian version of the EQ-5D-3L questionnaire was used, and EQ-5D index scores were calculated using population values from Zimbabwe, as a population value set for Malawi is not currently available. Chi-square test was used to test categorical variables. Odds ratio (OR) was calculated with a linear regression model adjusted for age, gender, location and education. A total of 8,801 individuals were enumerated in 1,481 households. Of the 8,548 participants that were screened and examined (response rate of 97.1%), 810 cases of MSI were diagnosed of which 18% (108) had mild, 54% (329) had moderate and 28% (167) had severe MSI as classified by ICF. There was an overall prevalence of MSI of 9.5% (CI 8.9-10.1). The prevalence of MSI increased with age, and was similar in men (9.3%) and women (9.6%). People without formal education were more likely to have MSI [13.3% (CI 11.8-14.8)] compared to those with formal education levels [8.9% (CI 8.1-9.7), p<0.001] for primary school and [5.9% (4.6-7.2), p<0.001] for secondary school. Overall, 33.2% of MSIs were due to congenital causes, 25.6% were neurological in origin, 19.2% due to acquired non-traumatic non-infective causes, 16.8% due to trauma and 5.2% due to infection. Extrapolation of these findings indicated that there are approximately one million cases of MSI in Malawi that need further treatment. MSI had a profound impact on quality of life. Analysis of disaggregated quality of life measures using EQ-5D showed clear correlation with the ICF class. A large proportion of patients with moderate and severe MSI were confined to bed, unable to wash or undress or unable to perform usual daily activities. This study has uncovered a high prevalence of MSI in Malawi and its profound impact on a large proportion of the population. These findings suggest that MSI places a considerable strain on social and financial structures in this low-income country. The Quality of Life of those with severe MSI is considerably affected. The huge burden of musculoskeletal impairment in Malawi is mostly unattended, revealing an urgent need to scale up surgical and rehabilitation services in the country.

Cerebral palsy occurs in up to 2.1 of every 1000 live births and encompasses a range of motor problems and movement disorders. One commonly occurring movement disorder amongst those with cerebral palsy is dystonia: sustained or intermittent involuntary muscle spasms and contractions that cause twisting, repetitive movements and abnormal postures. The involuntary contractions are often very painful and distressing and cause significant limitations to activity and participation.Oral medications are often the first line of medical treatment for dystonia. Trihexyphenidyl is one such medication that clinicians often use to treat dystonia in people with cerebral palsy. To assess the effects of trihexyphenidyl in people with dystonic cerebral palsy, according to the World Health Organization's (WHO) International Classification of Functioning, Disability and Health (ICF) domains of impairment, activity and participation. We also assessed the type and incidence of adverse effects in people taking the drug. We searched CENTRAL, MEDLINE, Embase, eight other databases and two trials registers in May 2017, and we checked reference lists and citations to identify additional studies. We included randomised controlled trials comparing oral trihexyphenidyl versus placebo for dystonia in cerebral palsy. We included studies in children and adults of any age with dystonic cerebral palsy, either in isolation or with the associated movement disorders of spasticity, ataxia, chorea, athetosis and/or hypotonia. We included studies regardless of whether or not the study authors specified the method used to diagnose dystonia in their study population. Primary outcomes were change in dystonia and adverse effects. Secondary outcomes were: activity, including mobility and upper limb function; participation in activities of daily living; pain; and quality of life. We used standard methodological procedures expected by Cochrane. We identified one study, which was set in Australia, that met the inclusion criteria. This was a randomised, double-blind, placebo-controlled, cross-over trial in 16 children (10 boys and 6 girls) with predominant dystonic cerebral palsy and a mean age of 9 years (standard deviation 4.3 years, range 2 to 17 years). We considered the trial to be at low risk of selection, performance, detection, attrition, reporting and other sources of bias. We rated the GRADE quality of the evidence as low.We found no difference in mean follow-up scores for change in dystonia as measured by the Barry Albright Dystonia Scale (BADS), which assesses eight body regions for dystonia on a 5-point scale (0 = none to 4 = severe), resulting in a total score of 0 to 32. The BADS score was 2.67 points higher (95% confidence interval (CI) -2.55 to 7.90; low-quality evidence), that is, worse dystonia, in the treated group. Trihexyphenidyl may be associated with an increased risk of adverse effects (risk ratio 2.54, 95% CI 1.38 to 4.67; low-quality evidence).There was no difference in mean follow-up scores for upper limb function as measured by the Quality of Upper Extremity Skills Test, which has four domains that collectively assess 36 items (each scored 1 or 2) and produces a total score of 0 to 100. The score in the treated group was 4.62 points lower (95% CI -10.98 to 20.22; low-quality evidence), corresponding to worse function, than in the control group. We found low-quality evidence for improved participation (as represented by higher scores) in the treated group in activities of daily living, as measured by three tools: 18.86 points higher (95% CI 5.68 to 32.03) for the Goal Attainment Scale (up to five functional goals scored on 5-point scale (-2 = much less than expected to +2 = much more than expected)), 2.91 points higher (95% CI 1.01 to 4.82) for the satisfaction subscale of the Canadian Occupational Performance Measure (COPM; satisfaction with performance in up to five problem areas scored on a 10-point scale (1 = not satisfied at all to 10 = extremely satisfied)), and 2.24 points higher (95% CI 0.64 to 3.84) for performance subscale of the COPM (performance in up to five problem areas scored on a 10-point scale (1 = not able to do to; 10 = able to do extremely well)).The study did not report on pain or quality of life. At present, there is insufficient evidence regarding the effectiveness of trihexyphenidyl for people with cerebral palsy for the outcomes of: change in dystonia, adverse effects, increased upper limb function and improved participation in activities of daily living. The study did not measure pain or quality of life. There is a need for larger randomised, controlled, multicentre trials that also examine the effect on pain and quality of life in order to determine the effectiveness of trihexyphenidyl for people with cerebral palsy.

BackgroundThere is a lack of accurate information on the prevalence and causes of musculoskeletal impairment (MSI) in low income countries. The WHO prevalence estimate does not help plan services for specific national income levels or countries. The aim of this study was to find the prevalence, impact, causes and factors associated with musculoskeletal impairment in Malawi. We wished to undertake a national cluster randomized survey of musculoskeletal impairment in Malawi, one of the UN Least Developed Countries (LDC), that involved a reliable sampling methodology with a case definition and diagnostic criteria that could clearly be related to the classification system used in the WHO International Classification of Functioning, Disability and Health (ICF)MethodsA sample size of 1,481 households was calculated using data from the latest national census and an expected prevalence based on similar surveys conducted in Rwanda and Cameroon. We randomly selected clusters across the whole country through probability proportional to size sampling with an urban/rural and demographic split that matched the distribution of the population. In the field, randomization of households in a cluster was based on a ground bottle spin. All household members present were screened, and all MSI cases identified were examined in more detail by medical students under supervision, using a standardized interview and examination protocol. Data collection was carried out from 1st July to 30th August 2016. Extrapolation was done based on study size compared to the population of Malawi. MSI severity was classified using the parameters for the percentage of function outlined in the WHO International Classification of Functioning (ICF). A loss of function of 5–24% was mild, 25–49% was moderate and 50–90% was severe. The Malawian version of the EQ-5D-3L questionnaire was used, and EQ-5D index scores were calculated using population values from Zimbabwe, as a population value set for Malawi is not currently available. Chi-square test was used to test categorical variables. Odds ratio (OR) was calculated with a linear regression model adjusted for age, gender, location and education.ResultsA total of 8,801 individuals were enumerated in 1,481 households. Of the 8,548 participants that were screened and examined (response rate of 97.1%), 810 cases of MSI were diagnosed of which 18% (108) had mild, 54% (329) had moderate and 28% (167) had severe MSI as classified by ICF. There was an overall prevalence of MSI of 9.5% (CI 8.9–10.1). The prevalence of MSI increased with age, and was similar in men (9.3%) and women (9.6%). People without formal education were more likely to have MSI [13.3% (CI 11.8–14.8)] compared to those with formal education levels [8.9% (CI 8.1–9.7), p<0.001] for primary school and [5.9% (4.6–7.2), p<0.001] for secondary school. Overall, 33.2% of MSIs were due to congenital causes, 25.6% were neurological in origin, 19.2% due to acquired non-traumatic non-infective causes, 16.8% due to trauma and 5.2% due to infection. Extrapolation of these findings indicated that there are approximately one million cases of MSI in Malawi that need further treatment. MSI had a profound impact on quality of life. Analysis of disaggregated quality of life measures using EQ-5D showed clear correlation with the ICF class. A large proportion of patients with moderate and severe MSI were confined to bed, unable to wash or undress or unable to perform usual daily activities.ConclusionThis study has uncovered a high prevalence of MSI in Malawi and its profound impact on a large proportion of the population. These findings suggest that MSI places a considerable strain on social and financial structures in this low-income country. The Quality of Life of those with severe MSI is considerably affected. The huge burden of musculoskeletal impairment in Malawi is mostly unattended, revealing an urgent need to scale up surgical and rehabilitation services in the country.

Background: This cross-sectional study assessed both family and individual quality of life (QOL), and their association with self-esteem, optimism, chronic psychological stress, anxiety, and depression in parents of children with chronic conditions. Methods: Parents of children with Down syndrome (DS), autistic spectrum disorder (ASD), cerebral palsy (CP), diabetes mellitus type 1 (DMT1), and parents of children without chronic diseases with typical development (TD) were included. Multivariate linear regression analysis was used to assess parental characteristics associated with the domains of individual and family QOL. Results: Compared to the parents of TD children, parents of children with ASD and DS were more likely to report reduced family QOL in all domains, while parents of children with DMT1 had lower parental perception. Self-esteem was positively associated with all domains of individual QOL, while optimism was associated with the overall individual QOL perception and health. Higher stress perception was negatively associated with most of the domains of individual and family QOL. Conclusions: This study confirmed that parents of children with chronic conditions are more likely to have lower perception of both individual and family QOL, which were associated with self-esteem, chronic stress, anxiety, and depression. Interventions should focus not only on the child with a chronic condition but on parents too.

Long-term therapy with intrathecal baclofen improves quality of life in children with severe spastic cerebral palsy.

Aim Major abdominal surgery (MAS) can have a diverse impact on patient’s life, including physical, emotional and socio-economic effects. At present, there is no patient reported outcome (PRO) instrument that was specifically developed for MAS population. The aim of this study was to develop a hypothesised conceptual framework of the key domains that underpin the process of recovery following major abdominal surgery in the adult population. This framework will serve as a foundation for developing a valid PRO instrument for the target population of interest. Method The development of the hypothesised conceptual framework included the following stages: 1) defining major abdominal surgery and verification of its discriminative properties, 2) systematic review of currently available PRO instruments, 3) mapping of the domains within identified PRO instruments to WHO International Classification of Functioning, Disability and Health (ICF) conceptual framework, 4) derivation of the hypothesised conceptual framework by linking of the ICF domains and the health-related quality of life theoretical framework. Results Fourteen PRO instruments were identified by the systematic review. The individual PRO items within each instrument collectively covered 25 ICF domains and 53 sub-domains. A hypothesised conceptual framework was derived based on the “conceptual model of health-related quality of life” and the ICF framework, applied within three principles of exposure, moderating factors and outcomes. Conclusions This study proposed a hypothesised conceptual framework for the development of PRO instruments to evaluate recovery following major abdominal surgery. However, its relevance, content validity and comprehensiveness remain to be examined in future studies.

BackgroundRehabilitation is an integral component of palliative care. An understanding of functional goals can help tailor interventions and support the evaluation of services. This study examined the nature and timescale of functional goals in palliative care, attainment of goals following personalised rehabilitation, responsiveness relative to health-related quality of life across, and factors associated with goal achievement.MethodsProspective, observational cohort study of adults with advanced progressive illness from 10 UK hospices referred for rehabilitation assessment. Urgency of care needs and functional status were assessed using the palliative Phase of Illness (stable, unstable, deteriorating) and Australia-modified Karnofsky Performance Status (AKPS, ≥ 60,60 − 50, ≤ 40) respectively. Health-related quality of life was assessed using EuroQoL 5-Dimension 5-Level (EQ-5D-5 L) utility score. Functional goals were set collaboratively with patients using SMART goal statements, mapped onto the WHO International Classification of Functioning, Disability and Health (ICF). Goal Attainment Scaling (GAS) was used to evaluate achievement against an anticipated outcome using a T-score. Ordinal logistic regression was sued to identify factors associated with goal achievement.Results364 patients (54% female, mean (SD) age 68 (14) years, 71% cancer, 71% stable Phase, median AKPS 60) took part. They set a median (range) of 2 (1–4) goals; 645 in total. Goals had a median (range) timeframe of 28 (1-196) days and spanned 13/30 ICF domains; most frequently mobility, general tasks and demands, mental functions, community, social and civic life, and self-care. The majority focused on activity (51%) and participation (20%). Following personalised rehabilitation, GAS T-scores improved overall (mean (SD) change 8.9 (13.4)) and for each subgroup by Phase and AKPS (all p < 0.01). EQ-5D scores improved overall, but not for those with a deteriorating Phase or AKPS ≤ 40. Living alone or receiving multiple interventions increased the likelihood of goal achievement, whereas being wheelchair or bedbound, receiving a generic exercise intervention, or having goals rated as very difficult reduced it.ConclusionsFunctional goals in palliative care typically focus on optimising activity and participation in the short term. Progress towards personalised goals can be achieved through personalised rehabilitation, including among people with deteriorating health or largely confined to bed. Goal Attainment Scaling can help direct and evaluate rehabilitation interventions in this setting.

Background: The aim was to investigate the application of radiopotentiation which was initiated by the fact that in patients with locally advanced non-small cell lung cancer there is a high risk of relapse due to failure in local control of the disease and the risk of systemic micrometastases. The other aim was the evaluation of quality of life. Methods: A study group consisted of 67 patients. Patients were first treated with TD30 Gy in 10 fractions during 2 weeks (5 fractions per week) with a potentiation by 20 mg/m 2 of carboplatinol intravenous bolus infusion just prior to each radiotherapy fraction. After a 2-week pause, additional radiation of 25 Gy in 10 fractions was applied during 2 weeks (5 fractions/week) with a potentiation by 20 mg/m 2 of carboplatinol. Total tumor dose (TTD) was 55 Gy (30+25 Gy) in 20 fractions, total duration of the therapy was 6 weeks, and total dose of carboplatinol was 600 mg. A control group consisted of 70 patients and they were treated with a radical radiotherapy with a conventional fractionation (60 Gy in 30 fractions, 2 Gy per day, 5 fractions per week). Quality of life was evaluated by Karnofsky performance scale (KI), at the beginning of the treatment, after the completion of the whole course of treatment, and during follow-up at regular check-ups. Results After the completion of the whole course of treatment most of patients had KI 80%. Analysis made during the last check-up showed statistically significant low KI. In both study and control groups KI was significantly higher at the beginning of the treatment than KI after the completion of the whole course of treatment (p=0.001). However, the analysis of KI at the time of the completion of the whole course of treatment showed significantly better KI in study group (p=0.036) than in control group. Conclusion: These results showed that the combined radiochemotherapy treatment is well tolerated.

Cerebral Palsy (CP) is a leading cause of childhood motor disability, making independent walking a crucial therapeutic goal. Robotic assistive devices offer potential to enhance mobility, promoting community engagement and quality of life. This is an observational report of 22 cases of children with CP in which we evaluated the Moonwalker exoskeleton (a dynamic moving aid system) usability, functional changes, and caregivers' perspectives based on the International Classification of Functioning (ICF). All children (aged 2-8 years, with a severe gait impairment and inability to use a conventional walker) underwent Moonwalker training for 20 sessions, followed by home use for five months. Post-treatment, majority of children showed improved endurance assessed by the 10-m walk test with a notable involvement of the upper trunk and arm movements for gait assistance. Many of them achieved rather remarkable results reaching a velocity of ≥0.5 m/s given the constraints of the walking exoskeleton and the children's size, while at admission all children walked at a speed of less than 0.5 m/s. Several positive environmental factors and family adherence were noted, as assessed by ICF in a subgroup of children. This study on a sample of children demonstrated that the Moonwalker exoskeleton allows walking and training at home in children with severe CP, enhancing development, social interaction, and endurance, while being well-received by families.

The associations between quality of life and clinical symptoms in individuals with an at-risk mental state and first-episode psychosis