Current technologies provide individuals with complex communication needs with a powerful array of communication, information, organization, and social networking options. However, there is the danger that the excitement over these new devices will result in a misplaced focus on the technology, to the neglect of what must be the central focus – the people with complex communication needs who require augmentative and alternative communication (AAC). In order to truly harness the power of technology, rehabilitation and educational professionals must ensure that AAC intervention is driven, not by the devices, but rather by the communication needs of the individual. Furthermore, those involved in AAC research and development activities must ensure that the design of AAC technologies is driven by an understanding of motor, sensory, cognitive, and linguistic processing, in order to minimize learning demands and maximize communication power for individuals with complex communication needs across the life...

https://www.tandfonline.com/doi/pdf/10.3109/07434618.2013.848935

Putting people first: re-thinking the role of technology in augmentative and alternative communication intervention.

Aim The aims were to investigate survival of children with cerebral palsy (CP) and to search for modifiable factors that influence survival in CP. Method The total population of children with CP in southern Sweden born between 1990 and 2005, and followed from 1994 to 2010 comprised 718 children. The study included 708 of these children (297 females, 411 males) participating in a secondary prevention programme. CP subtype, Gross Motor Function Classification System (GMFCS) levels, and comorbidities were described. Kaplan-Meier survival curves were plotted. The following factors were investigated using Cox regression analysis: GMFCS level (co-varies with overall health), size of health care catchment area, gastrostomy feeding, and sex. Results The estimated survival at 19 years of age was 60% in children with the most severe gross motor limitations (GMFCS level V). Death occurred throughout childhood. All children at GMFCS level I or II, and 96% of the whole CP population, survived. The mortality risk in childhood CP was three times higher in catchment areas that covered small populations than in areas with a large population. Gastrostomy feeding was associated with a ninefold increased risk of dying, regardless of GMFCS level and catchment area. Interpretation Fragile children with CP, as indicated by GMFCS level V and gastrostomy feeding, had the lowest chance of surviving childhood. Health care catchment area seemed to influence survival rate. (Less)

https://www.lifeexpectancy.org/articles/westbom2011.pdf

Survival at 19 years of age in a total population of children and young people with cerebral palsy.

Aim This study used data collected prospectively since 1986 from a population-based cerebral palsy registry to explore the rates, predictors, trends, and causes of mortality for individuals born in Victoria, Australia, between 1970 and 2004.



Method  Data were extracted for 3507 individuals (1972 males; 1535 females). The probability of survival before 31 May 2010 was determined using the Kaplan–Meier method; age-specific mortality rates were calculated per 1000 person-years and related to population rates. Using Cox proportional hazards regression, relative risks of mortality were estimated for different categories of chosen demographic and clinical variables. Causes were tabulated according to the direct cause of death.



Results  There were 418 deaths. Crude mortality was 20% at the age of 40 years. Relative to the population, mortality was highest in children aged under 15 years and decreased to twice the population rate at the age of 35 years. The strongest independent predictor of mortality was no independent ambulation (adjusted hazard ratio 6.2 [95% confidence interval 3.3–11.8]); additional predictors were severe intellectual impairment (3.0 [1.7–5.2]), epilepsy (1.4 [1.1–1.9]), deafness (2.6 [1.4–4.7]), and term birth (1.8 [1.3–2.4]). No improvement in survival was seen over time (unadjusted hazard ratio 1.00 [95% CI 0.99–1.01]). Respiratory causes were the most common direct causes of death.



Interpretation  Rates, predictors, and causes of death for individuals with cerebral palsy in Victoria were similar to those found in other population cohorts. Lack of improvement in survival since 1970 was an unexpected finding that warrants further investigation.

https://onlinelibrary.wiley.com/doi/pdf/10.1111/j.1469-8749.2012.04218.x

Survival of individuals with cerebral palsy born in Victoria, Australia, between 1970 and 2004

Aim

The aim of the study was to determine survival probabilities and life expectancies for individuals with cerebral palsy based on data collected over a 28-year period in California.



Method

We identified all individuals with cerebral palsy, aged 4 years or older, who were clients of the California Department of Developmental Services between 1983 and 2010. Kaplan–Meier survival curves were constructed for 4-year-old children, and the estimated survival probabilities were adjusted to reflect trends in mortality by calendar year. For persons aged 15, 30, 45, and 60 years, separate Poisson regression models were used to estimate age-, sex-, and disability-specific mortality rates. These mortality rates were adjusted to reflect trends of improved survival, and life expectancies were obtained using life table methods.



Results

The sample comprised 16 440, 14 609, 11 735, 7023, and 2375 persons at ages 4, 15, 30, 45, and 60 years, respectively. In 1983, 50% of 4-year-old children who did not lift their heads in the prone position and were tube fed lived to age 10.9 years. By 2010, the median age at death had increased to 17.1 years. In ambulatory children the probability of survival to adulthood did not change by more than 1%. Life expectancies for adolescents and adults were lower for those with more severe limitations in motor function and feeding skills, and decreased with advancing age. Life expectancies for tube-fed adolescents and adults increased by 1 to 3 years, depending on age and pattern of disability, over the course of the study period.



Interpretation

Over the past three decades in California there have been significant improvements in the survival of children with very severe disabilities. There have also been improvements to the life expectancy of tube-fed adults, though to a lesser extent than in children.

/pdf/recent-trends-in-cerebral-palsy-survival-part-ii-individual-17r1f2frgs.pdf

Recent trends in cerebral palsy survival. Part II: individual survival prognosis.

Aim

To determine whether the trend of improved survival among individuals with cerebral palsy (CP) in California during the 1980s and 1990s has continued during the most recent decade.



Method

In an observational cohort study we evaluated individuals with CP, aged 4 years and older, who were clients of the California Department of Developmental Services. Medical diagnoses, functional disabilities, and special health care requirements were assessed with Client Development Evaluation Reports made between 1983 and 2010. Trends in birth cohort survival were analyzed with Kaplan‒Meier curves and Cox regression. Calendar year period effects were analyzed with Poisson regression.



Results

A total of 51 923 persons with CP (28 789 males [55%], 23 134 females [45%]; mean age 14y 11mo, SD 14y 1mo, range 4y 0mo to 96y 10mo) collectively contributed 662 268 years of follow-up. There were 7690 deaths for an overall mortality rate of 11.6 per 1000 persons per year. No significant birth cohort effects on survival were observed in 4-year-olds who had no severe disabilities. By contrast, children who did not lift their heads in prone position who were born in more recent years had significantly lower mortality rates (Cox hazard ratio 0.971, p<0.001) than those with comparable disabilities born earlier. With regard to calendar year period effects, we found that age-, sex-, and disability-specific mortality rates declined by 1.5% (95% CI 0.9‒2.1) year-over-year from 1983 to 2010. The estimate increased to 2.5% (95% CI 1.9‒3.1) per year when we additionally controlled for tube-feeding status. Mortality rates in tube fed adolescents and adults, ages 15 to 59 years, declined by 0.9% (95% CI, 0.4‒1.4) per year. No improvement was observed for adolescents or adults who fed orally or for those over age 60. In fact, the ratio of age-specific mortality rates for these latter groups to those in the general population, increased by 1.7% (95% CI 1.3–2.0) per year during the study period.



Interpretation

The trend toward improved survival has continued throughout the most recent decade. Declines in CP childhood mortality are comparable to the improvements observed in the United States general population (i.e. the mortality ratio in childhood has remained roughly constant over the last three decades). In contrast, the mortality ratio for most adolescents and adults with CP, relative to the general population, has increased.

/pdf/recent-trends-in-cerebral-palsy-survival-part-i-period-and-brh1253whx.pdf

Recent trends in cerebral palsy survival. Part I: period and cohort effects.

BACKGROUND: Communication is frequently impaired in young people (YP) with bilateral cerebral palsy (CP). Important factors include motoric speech problems (dysarthria) and intellectual disability. Augmentative and Alternative Communication (AAC) techniques are often employed. The aim was to describe the speech problems in bilateral CP, factors associated with speech problems, current AAC provision and use, and to explore the views of both the parent/carer and young person about communication. METHODS: A total population of children with bilateral CP (n = 346) from four consecutive years of births (1989-1992 inclusive) with onset of CP before 15 months were reassessed at age 16-18 years. Motor skills and speech were directly assessed and both parent/carer and the young person asked about communication and satisfaction with it. RESULTS: Sixty had died, eight had other conditions, 243 consented and speech was assessed in 224 of whom 141 (63%) had impaired speech. Fifty-two (23% of total YP) were mainly intelligible to unfamiliar people, 22 (10%) were mostly unintelligible to unfamiliar people, 67 (30%) were mostly or wholly unintelligible even to familiar adults. However, 89% of parent/carers said that they could communicate 1:1 with their young person. Of the 128 YP who could independently complete the questions, 107 (83.6%) were happy with their communication, nine (7%) neither happy nor unhappy and 12 (9.4%) unhappy. A total of 72 of 224 (32%) were provided with one or more types of AAC but in a significant number (75% of 52 recorded) AAC was not used at home, only in school. Factors associated with speech impairment were severity of physical impairment, as measured by Gross Motor Function Scale level and manipulation in the best hand, intellectual disability and current epilepsy. CONCLUSIONS: In a population representative group of YP, aged 16-18 years, with bilateral CP, 63% had impaired speech of varying severity, most had been provided with AAC but few used it at home for communication.

Speech, communication and use of augmentative communication in young people with cerebral palsy: the SH&PE population study.

Objective To ascertain mortality from 1 to 18 years, and predictors of mortality. Design Long-term follow-up of population cohort born 1989–1992. Setting Births in South East Thames Region. Patients 346 children with bilateral cerebral palsy (CP). Interventions Not applicable. Main outcome measures Mortality; predictors of mortality. Results 98% of the cohort were traced. 61/340 (17.9%) had died by age 16–18 years at a steady mortality. The main predictive factor was severity of impairment of functional ability (hazard ratio 5.7, 95% CI 2.1 to 15.0 for poor hand manipulation; 6.8 (1.9 to 23.9).for severe communication problems). Conclusions Although there were deaths throughout the childhood and teenage years, the majority of children with bilateral CP are likely to survive to adulthood, especially if they do not have major functional impairment at 2 years. This confirms findings of other studies of children with CP.

https://adc.bmj.com/content/archdischild/96/11/1077.full.pdf

Mortality from 1 to 16–18 years in bilateral cerebral palsy

Aim We explored factors associated with pain and its severity in a population cohort of young people with bilateral cerebral palsy, comparing parent/carer and young people self-reports. Method Of 278 survivors (mean age 16y 8mo, SD 1y 4mo, range 13y 8mo-19y 3mo) from the South Thames in the Study of Hips and Physical Experience cohort of 338 young people with bilateral cerebral palsy, 212 parents/carers and 153 young people completed questionnaires on the presence, severity, timing, site, associated factors, impact, and treatment of pain. Results Seventy per cent of parents/carers reported pain within 3 months, 59% the previous week, and 50% the previous day with 56% reporting 'regularly experienced'. Of young people able to do so, 63% reported pain within 3 months, 50% the previous week, and 42% the previous day, with 48% reporting regular pain. There was strong agreement between the parent/carer and young people, reporting pain severity over the previous 3 months. Pain severity was associated with increased motor impairment and comorbidity, particularly constipation, spasticity, equipment use, and higher emotional score, but not sex, intellectual disability, speech, or maternal education. Multiple sites of musculoskeletal pain were reported in two-thirds of individuals. Pain was associated with voluntary movement in individuals with less motor impairment and with being moved in those with severe motor impairment. Greater pain severity had a negative effect on both physical and psychological quality of life. Interpretation Increasing awareness of the comorbidities in cerebral palsy may aid effective treatment, reducing pain experienced by young people with cerebral palsy. What this study adds Regular moderate or severe pain is reported in young people with bilateral cerebral palsy (CP) in all Gross Motor Function Classification System levels. Pain is reported more frequently in young people who are non-ambulant. General ill health is strongly associated with severity of pain after controlling for severity of CP, especially constipation. Pain occurs most often in ambulant young people during voluntary activity and in those who are non-ambulant when being moved. There is strong agreement between parents/carers and young people about pain presence and severity.

/pdf/factors-associated-with-pain-in-adolescents-with-bilateral-fzv50qk9yz.pdf

Elspeth Will

Papers

Speech, communication and use of augmentative communication in young people with cerebral palsy: the SH&PE population study.

Mortality from 1 to 16–18 years in bilateral cerebral palsy

Factors associated with pain in adolescents with bilateral cerebral palsy.