Employment and social issues in adults with cerebral palsy

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.

To describe the prevalence of cerebral palsy (CP), subtype distribution, motor and intellectual impairment, and epilepsy in adults with CP compared with children with CP. CP subtype and impairment data from the population-based CP register of western Sweden and population data from Statistics Sweden were used to compare surviving adults (n=581; 244 females, 337 males) born between 1959 and 1978, with the same cohort as children (n=723; 307 females, 416 males), andwiththe most recent cohort, born from 2007 to 2010 (n=205; 84 females, 121 males). Prevalence of CP in adults born between 1959 and 1978 was 1.14 per 1000. The occurrence of impairments differed between CP subtypes. Motor and intellectual impairment were closely related, regardless of subtype. Subtype distribution among survivors differed significantly from the original cohorts (p=0.002), and the most recent cohort (p<0.01), tetraplegia and dyskinetic CP being less common in survivors. Severe motor impairment, intellectual disability, and epilepsy were less common among survivors than in the original cohorts (p=0.004, p=0.002, p=0.037) and the most recent cohort (p=0.004, p=0.008, p<0.01). Data on prevalence, subtype distribution, and impairments in children with CP are not applicable to adults with CP. Population-based studies of adults with CP are needed. Cerebral palsy (CP) subtypes are differently distributed in adults compared to children. The prevalence of impairments in adults with CP is related to CP subtype. Spastic tetraplegia and dyskinetic CP are less common in adults than children. Severe motor impairment, intellectual disability, and epilepsy are less common in adults.

To examine interrater agreement and validity of the Functional Communication Classification System (FCCS) for young children with cerebral palsy (CP) aged 2 or 3 years. Speech-language pathologist (SLP) and parent FCCS ratings for 31 children with CP (aged 2y, n=16; aged 3y, n=15; 18 males, 13 females) were examined for interrater agreement using a weighted Cohen's kappa statistic. Relationships between FCCS (SLP) ratings and: (1) concurrent validity with the Language Use Inventory, a standardized pragmatic assessment for children aged 18 to 47 months, (2) gross motor and fine motor function, (3) associated impairments (visual and intellectual), and (4) primary expressive communication mode were examined using Spearman's correlation coefficients. Almost perfect interrater agreement between SLP and parent FCCS ratings were found (kw =0.94). Correlations with FCCS (SLP) were excellent for pragmatic function (rs =-0.83,p<0.001), intellectual function (rs =0.89, p<0.001), and primary expressive communication mode (rs =0.92,p<0.001). Correlations were good for gross motor function (rs =0.72,p<0.001) and visual impairment (rs =0.70,p<0.001) and fair for fine motor function (rs =0.53,p<0.002). Analysis was unwarranted for epilepsy (n=1 out of 31) and hearing-associated impairments (n=0 out of 31). The FCCS has excellent interrater agreement and validity for communication classification of children with CP aged 2 or 3 years and is highly suitable for surveillance and research purposes. What this paper adds The Functional Communication Classification System (FCCS) is a valid instrument for children with cerebral palsy (CP) aged 2 or 3 years. Excellent agreement exists between speech-language pathologist and parent FCCS ratings. The FCCS has excellent correlation with intelligence, pragmatic function, and primary expressive mode. Stronger correlations with the Gross Motor Function Classification System and vision exist for children aged 2 or 3 years. Weaker correlations with manual ability exist when compared to older children with CP.

BackgroundChildren with cerebral palsy (CP) frequently experience chronic pain. The burden and severity of such pain is often underestimated in relation to their other impairments. Recognition and awareness of this chronic pain among children with CP constitute the cornerstone for caretakers and clinicians to improve the quality of life of those children. This study aimed to determine the prevalence of chronic pain among children with CP, and the factors associated.MethodsA cross-sectional study of children with CP, aged 2–12 years, attending the CP rehabilitation clinic and Pediatric Neurology Clinic at Mulago Hospital, Uganda from November 2017 to May 2018. A detailed history and clinical examination were performed and the co-morbidities were determined. CP was classified using the Gross Motor Function Classification System (GMFCS), Manual Ability Classification System, Communication Function Classification System (CFCS), and the Eating and Drinking Ability Classification System (EDACS) and documented with the level of impairment in the different domains. Pain was assessed by using the revised Face, Legs, Activity, Consolability, Cry pain scale.ResultsA total of 224 children with CP were enrolled. The prevalence of chronic pain was 64.3%. The majority had spastic bilateral CP (77.8%), moderate pain lasting over 6 months, and none of them was on long-term pain management. Epilepsy (60.9%), behavioral problem (63.2%), hearing impairment (66,7%), learning problem (67,6%), dental caries (75%), gastro-esophageal reflux (75%), sleep disorders (79.5%), vision impairment (80%), and malnutrition (90%) were co- morbid conditions of chronic pain in children with CP in this study. The factors independently associated with chronic pain among children with CP were the GMFCS level IV & V, CFCS level IV & V, EDACS level IV & V, female children, and caretaker aged more than 30 years.ConclusionsTwo-thirds of children with CP attending rehabilitation in this hospital had chronic pain. None was receiving pain management. Chronic pain was associated with the presence of multiple co-morbidities and more severe disability. Rehabilitation and care programs for children with CP should include assessment of pain in routine care and provide interventions for pain relief in children with CP even at an early age.

To determine the prevalence of drooling, swallowing, and feeding problems in persons with cerebral palsy (CP) across the lifespan. A systematic review was conducted using five different databases (AMED, CINAHL, Embase, MEDLINE, and PubMed). The selection process was completed by two independent researchers and the methodological quality of included studies was assessed using the STROBE and AXIS guidelines. Meta-analyses were conducted to determine pooled prevalence estimates of drooling, swallowing, and feeding problems with stratified group analyses by type of assessment and Gross Motor Function Classification System level. A total of 42 studies were included. Substantial variations in selected outcome measures and variables were observed, and data on adults were limited. Pooled prevalence estimates determined by meta-analyses were as high as 44.0% (95% confidence interval [CI] 35.6-52.7) for drooling, 50.4% (95% CI 36.0-64.8) for swallowing problems, and 53.5% (95% CI 40.7-65.9) for feeding problems. Group analyses for type of assessments were non-significant; however, more severely impaired functioning in CP was associated with concomitant problems of increased drooling, swallowing, and feeding. Drooling, swallowing, and feeding problems are very common in people with CP. Consequently, they experience increased risks of malnutrition and dehydration, aspiration pneumonia, and poor quality of life. Drooling, swallowing, and feeding problems are very common in persons with cerebral palsy (CP). The prevalence of drooling, swallowing, and feeding problems is 44.0%, 50.4%, and 53.5% respectively. There are limited data on the prevalence of drooling, swallowing, and feeding problems in adults. Higher Gross Motor Function Classification System levels are associated with higher prevalence of drooling, swallowing, and feeding problems. There is increased risk for malnutrition, dehydration, aspiration pneumonia, and poor quality of life in CP.

Prevalence of Venous Thromboembolism in Ambulatory and Non-Ambulatory Patients with Cerebral Palsy

BackgroundThe majority of children with cerebral palsy (CP) experience challenges in functional communication from a young age. A pivotal aspect of functional communication is language comprehension. A variety of classification systems and questionnaires are available to classify and describe functional communication skills in children with CP. A better understanding of the convergent validity of (subsections of) these tools, as well as their relationship with spoken language comprehension, will be valuable in both clinical practice and research.AimsTo investigate the convergent validity of (subsections of) functional communication tools and the relationship with spoken language comprehension in children with CP.Methods & ProceduresCross‐sectional data on 138 children were subdivided into three developmental stages based on (Dutch) educational phases: ages 18 months–3;11y (n = 59), 4;0–5;11 years (n = 37) and 6;0–8;11 years (n = 42). The following functional communication tools were used to classify and describe functional communication: Communication Function Classification System (CFCS), subscales of the Caregivers Priorities and Child Health Index of Life with Disabilities—Dutch Version (CPCHILD‐DV) and the Focus on Communication Under Six‐34 (FOCUS‐34) questionnaire. Spoken language comprehension was assessed with the Computer‐Based instrument for Low motor Language Testing (C‐BiLLT). Correlations between the functional communication tools, and with the C‐BiLLT, were calculated using Pearson's and Spearman's correlation coefficients. It was hypothesized a priori that correlations of at least 0.60 suggest good convergent validity.Outcomes & ResultsAt all developmental stages, a significant ordered decreasing tendency of communication outcomes was found across CFCS levels; lower CFCS levels were associated with lower scores on the CPCHILD‐DV and FOCUS‐34, and with a lower level of spoken language comprehension (C‐BiLLT). Correlation coefficients of the functional communication tools exceeded 0.60 at all developmental stages. Correlations between C‐BiLLT raw scores and the functional communication tools varied between 0.351 and 0.591 at developmental stage 18 months–3;11 years, between 0.781 and 0.897 at developmental stage 4;0–5;11 years, and between 0.635 and 0.659 at developmental stage 6;0–8;11 years.Conclusions & ImplicationsThe functional communication tools assessed in this study showed convergent validity at all developmental stages. The CFCS, currently most widely used in paediatric rehabilitation, is adequate in the classification of functional communication. However, for more detailed clinical goal setting and evaluation of change in functional communication, the additional use of FOCUS‐34 or CPCHILD‐DV is recommended.What this paper addsWhat is already known on the subjectA range of functional communication tools are available that help describe and classify functional communication in children with CP. These include the CFCS, subsections of CPCHILD‐DV and FOCUS‐34. The CFCS classifies functional communication in daily life with familiar and unfamiliar partners. Specific subsections of the CPCHILD‐DV and FOCUS‐34 include items that pertain to communicative participation. The innovative C‐BiLLT provides a standardized method to assess spoken language comprehension in children with CP and significant motor impairments.What this paper adds to existing knowledgeIn the present study, convergent validity was confirmed between CFCS and specific subsections of the CPCHILD‐DV and FOCUS‐34. Correlations between these functional communication tools and the C‐BiLLT were moderate to strong.What are the potential or actual clinical implications of this work?For clinical and research purposes (for instance, accurate prescription of augmentative and alternative communication—AAC), healthcare and educational professionals together with parents need to know how functional communication tools converge and how functional communication levels relate to the comprehension of spoken language. The CFCS provides a valid classification of functional communication abilities in children with CP. However, to measure change in functional communication and to evaluate treatment outcomes, use of additional functional communication tools such as the CPCHILD‐DV and FOCUS‐34 is recommended. When discrepancies are found between communicative abilities and spoken language comprehension, it is strongly recommended that valid tools are used in a more detailed examination of the child's spoken language comprehension skills and functional communication.

Objectives: To analyse the living conditions and social outcomes (housing, engagement in employment or higher education, access to personal assistance and having a partner) in adults with cerebral palsy (CP) relative to their age, sex, communication ability, and motor skills.Methods: Cross-sectional registry-based study of 1,888 adults (1,030 males/858 females) with CP in the Swedish CP follow-up programme, median age 25 years (range 16–78 y). Type of housing, occupation, access to personal assistance and having a partner were analysed relative to their age, sex, and the classification systems for Gross Motor Function (GMFCS) and Communication Function (CFCS). Binary logistic regression models were used to calculate odds ratios (OR) for independent living, competitive employment, and having a partner.Results: Most of the 25- to 29-year olds (55.6%) lived independently, increasing to 72.4% in 40- to 49-year olds, while the majority (91.3%) of those under 20 years lived with their parents. Independent living was almost equal in adults at GMFCS levels I (40.2%) and V (38.6%). This parity was explained by access to personal assistance, which increased with higher GMFCS and CFCS levels. Personal assistance of >160 hours/week was associated with a high probability of independent living (OR 57). In the age span 20–64 years, 17.5% had competitive employment and 45.2% attended activity centres for people with intellectual disabilities. In the younger age group up to 24 years old, 36.9% went to mainstream/higher education and 20.5% went to special schools. In total, 13.4% had a partner and 7.8% lived together. Slightly more women than men had a partner, and most individuals were classified at CFCS level I.Conclusion: Only one in eight adults with CP has a partner, and one in six has competitive employment. Access to personal assistance is the single most important factor for independent living. It is vital to support adults with CP throughout their lifespan to achieve the best possible outcomes in all aspects of life.

This study was conducted to evaluate the clinical profile and predisposing factors of cerebral palsy (CP) in Jordanian children. Two hundred Jordanian children born between 1990 and 2005 with CP were reviewed in three teaching hospitals to study prospectively and retrospectively their clinical profile, possible etiological factors, and associated problems. A data collection sheet was developed to collect information from the children’s parents, or occasionally from the chart regarding the demographic characteristics, detailed history, findings of the physical, developmental and neurological examination. Spastic CP was the predominant type (64.5%) with a quadriplegic CP being the most common (36%). Dyskinetic CP was present in 10.5%, while ataxic in 18%. In congenital CP, birth asphyxia constituted a significant possible cause (33.5%). Epilepsy is one of the most common associated problems among CP children constituting 56%. The results of our magnetic resonance imaging findings showed that only 15.5% of the children were normal. This was the first study which detailed the clinical spectrum of CP in Jordan. We concluded that the clinical spectrum of CP in our developing countries may differ from that of the developed countries.

BackgroundSleep plays a prominent role in the growth and development of children. Children with cerebral palsy (CP) are more prone to sleep disorders (SDs) than their peers. Children with CP, have a higher prevalence of disorders involving; initiation and maintenance of sleep, sleep-wake transition, excessive sleepiness and arousal. These sleep disorders impact on the quality of life of these children. Despite, having a high prevalence of CP in Uganda, there is a paucity of data that focuses on sleep disorders in CP, including a lack of prevalence estimates of sleep breathing disorder (SBD) in CP. Understanding the prevalence and disorders of sleep within this population would help advise on the development of tailored interventions to address the needs of these children and improve their quality of life. This study determined the prevalence and associated factors of sleep disorders among children aged 2 – 12 years with cerebral palsy in Uganda.MethodsThis was a cross sectional study. All participants had a physical examination and screening with the Sleep Disturbances Scale for Children (SDSC) questionnaire to determine the prevalence of sleeps disorders. A total score (TS) ≥ 51 on the Sleep Disturbances Scale for Children was regarded as abnormal.ResultsA total of 135 participants were recruited. The prevalence of sleep disorders was 43/135 (32%) with 95% CI: (24.0-39.7). The most common type of sleep disorders was a disorder of initiating and maintaining sleep 37(27%). The factors associated with sleep disorders among children with cerebral palsy were bilateral spasticity (p = 0.004); OR:(95%CI), 11.193: (2.1 – 59.0), lowest levels of gross motor function V (p = < 0.001); OR:(95%CI), 13.182: (3.7 – 47.0) or IV (p = 0.007); OR:(95%CI), 12.921: (2.0 – 82.3), lowest level of manual ability V (p = 0.004); OR:(95%CI), 11.162: (2.2 – 56.4) and presence of epilepsy (p = 0.011); OR:(95%CI), 3.865: (1.4 – 10.9).ConclusionsThe prevalence of sleep disorders among children with cerebral palsy in Uganda is high. Severe disability and presence of epilepsy were associated with sleep disorders among children with cerebral palsy.

To investigate spoken language comprehension (SLC), single-word comprehension (SWC), functional communication development, and their determinants, in children with cerebral palsy. This was a prospective cohort study in the Netherlands spanning 2 years 6 months. The main outcomes were SLC and SWC, assessed by the Computer-Based instrument for Low motor Language Testing (C-BiLLT) and the Peabody Picture Vocabulary Test-III-NL (PPVT-III-NL) respectively; and functional communication, measured by a subscale of the Focus on the Outcomes of Communication Under Six-34 (FOCUS-34). Linear mixed models were used to determine developmental trajectories, which were compared with norm and reference data. Potential determinants, for example intellectual functions, speech production, functional communication level (classified with the Communication Function Classification System, CFCS), and functional mobility, were added to assess their effects. Children with cerebral palsy (n = 188; mean age 59 months, range 17-110) were monitored for 2 years 6 months. Developmental trajectories for SLC (C-BiLLT) and SWC (PPVT-III-NL) were nonlinear; those for functional communication (FOCUS-34) were linear. Compared with norm and reference groups, significantly delayed SLC, SWC, and functional communication development were found. Determinants for SLC and SWC were intellectual functions and functional communication level (CFCS); and for functional communication development (FOCUS-34), speech production and arm-hand functioning. Children with cerebral palsy showed delayed SLC, SWC, and functional communication development compared with norm and reference groups. Remarkably, functional mobility was not associated with the development of SLC, SWC, or functional communication. Children with cerebral palsy have delayed spoken language comprehension (SLC), single-word comprehension (SWC), and functional communication development, compared to norm and reference data. Determinants for SLC and SWC development are intellectual functions and functional communication level. Determinants for functional communication development are speech production and arm-hand functioning. Functional mobility is not associated with SLC, SWC, or functional communication.

Children with Cerebral Palsy (CP) experience physical motor impairments in their movement and posture, often accompanied by comorbidities such as epilepsy, intellectual disability, behavioural problems and challenges in communication and language. Knowledge about spoken language comprehension and functional communication development and its determinants in children with CP across all functional mobility levels, using standardized and direct assessment tools, is still very limited. Therefore, the Cerebral Palsy–Communication and Language Learning (CP-CaLL) research project was initiated. This research project is a multi-site prospective cohort study investigating (the developmental trajectories of) spoken language comprehension and functional communication, and their determinants in children with CP across all Gross Motor Function Classification System (GMFCS) levels (to classify functional mobility) using standardized and direct assessment tools. The results of the CP-CaLL research project are presented in this PhD thesis. The three aims of this thesis were to investigate: (1) the developmental trajectories of receptive language (spoken language comprehension and single-word comprehension) and functional communication in children with CP; (2) which determinants are relevant for the receptive language development and functional communication development in children with CP, following the International Classification of Functioning, Disability and Health for children and youth (ICF-CY) framework; and (3) the relationship between spoken language comprehension and functional communication in children with CP.

Background Cerebral palsy has been linked to decreased quality of life. However use of self- versus proxy-perspectives and norm-sample comparisons to examine quality of life impact may produce different results. Aims To compare quality of life ratings in children and adolescents with cerebral palsy relative to typically developing peers in consideration of sample and methodological moderators on estimated effects. Methods Eleven independent studies, comprising a pooled sample of 1475 families living with cerebral palsy and 42119 peers, were identified. Study reporting quality was evaluated with the QualSyst tool and standardised mean group differences (Hedges’ g) with associated confidence intervals and p values calculated. Heterogeneity was examined using a random effects model. Results All studies provided good to excellent methodological and statistical detail. Physical quality of life was significantly impaired among those with cerebral palsy (g range: −0.42 to −1.58). However, inconsistent findings were noted in relation to the effect of cerebral palsy on psychological (g range: 0.04 to −0.80) and social quality of life (g range: −0.80 to −0.51), depending on the measurement used. There was a trend for parents to evaluate their child’s physical quality of life lower than child-reported scores. Conclusions Physical quality of life is, invariably, more affected in those with cerebral palsy. The connection between cerebral palsy and psychosocial quality of life is less clear. Noted parent-child discrepancies highlight the value of a multi-informant approach to child quality of life assessment. Implications for rehabilitation Quality of life is an important health-related outcome in cerebral palsy research and practice. Collecting both self-report and proxy data can help to highlight quality of life issues that are salient to the parent and to the child or adolescent with cerebral palsy. Selection of the appropriate quality of life instrument depends on the assessment purpose, with available measures varying in their focus on functionality, subjectivity and illness-specific items. Quality of life assessment of children with cerebral palsy should extend beyond functional abilities to include less obvious, but critical, psychological and social issues.

BackgroundHip dysplasia is common among children with cerebral palsy (CP), particularly in spastic CP. It can result in pain, reduced function and quality of life. However, the burden of hip dysplasia among children with CP in llow-and middle-income countries (LMICs) like Bangladesh is unknown. We aimed to define the burden of hip dysplasia among children with spastic CP in Bangladesh.MethodsThis study includes a subset of the Bangladesh CP Register (BCPR) study cohort who were registered between January and March 2015. The BCPR is a population-based surveillance of children with CP (aged < 18 years) operating in a northern sub-district (Shahjadpur; child population ~ 226,114) of Bangladesh. Community-based key informant’s method (KIM) survey conducted to identify children with CP in the surveillance area. A diagnosis of CP was made based on clinical history and examination by the study physicians and physiotherapist. Study participants had an antero-posterior (AP) X-ray of their pelvis. The degree of subluxation was assessed by calculating the migration percentage (MP).ResultsDuring the study period, 196 children with CP were registered, 144 had spastic CP. 40 children with spastic CP (80 hips) had pelvic X-Rays (mean age 9.4 years, range 4.0–18.0 years) and 32.5% were female. Gross Motor Function Classification System (GMFCS) showed 37.5% (n = 15) with GMFCS level I-II and 62.5% (n = 25) with GMFCS level III-V. Twenty percent (n = 8) of the children had hip subluxation (MP: 33–80%). Osteopenic changes were found in 42.5% (n = 17) children.ConclusionsTo the best of our knowledge this is one of the first studies exploring hip dysplasia among children with spastic CP in Bangladesh. Our findings reflect that hip dysplasia is common among children with spastic CP. Introduction of hip surveillance programmes is imperative for prevention of secondary complications, reduced function and poor quality of life among these children.

Providing important evidence for the major causal contributors to cerebral palsy in Africa.