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

Abstract

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 Cerebral palsy (CP) is a diagnostic label that embraces a range of disorders of movement and posture. Substantial variation exists with regard to the type, topographical pattern, and severity of the movement disorder, the presence of associated conditions and impairments, and how these combine to impact on function, participation, and quality of life. Since the label 'CP' accommodates such a wide variety of possible clinical manifestations, information about survival with CP based on different combinations of clinical features is needed to counsel families about a child's prognosis, to plan for the provision of medical, educational, and social services, to inform public policy, and for medicolegal services relating to litigation. The aim of this study was to describe the rates, trends, predictors, and causes of mortality in individuals with nonpostneonatally acquired CP who were born in the Australian state of Victoria between 1970 and 2004. A number of groups have previously studied life expectancy in CP samples but many studies have had shortcomings. Recent studies of all CP cases from long-standing registers in Western Australia, 1 the UK, 2 and Denmark 3 have provided useful information on the long-term prognosis of all newly diagnosed children. The UK study noted regional differences in survival, but it was unclear whether these reflected regional variation in survival patterns, risk factor profiles, levels of care, or differences in definition, classification, or ascertainment.2 Severity of disability is the key factor influencing survival in CP. Immobility or severe motor impairment of all four limbs, 1-9 intellectual impairment, 1,2,4-7,10 epilepsy, 3,4,10 severe visual and hearing impairments, 2,7,11 hydrocephalus, 4 and an increasing number of impairments 1,2,5-7 have been associated with poorer survival. Although different severity profiles have been used by ea...

Reid et al. reply

“…

This letter is commented on by Reid et al on page 867-868 of this issue SIR-Reid et al 1 reported on the long-term survival of persons with cerebral palsy in the Australian state of Victoria. They found that rates, predictors, and causes of mortality were in line with reports from other countries.

…”

Prevalence of orofacial dysfunction in cerebral palsy and its association with gross motor function and manual ability

“…1 Impaired eating is associated with poor growth 2,3 and problems with chewing and swallowing may jeopardize respiration. 4,5 Notably, the most common causes of death in young individuals with CP are secondary respiratory diseases. 4,5 Hence, dysfunction in the face, tongue, palate, and throat, generically termed orofacial dysfunction (OFD), 6 has a strong impact on health in individuals with CP.…”

Morbimortalidad asociada al estado nutricional y vía de alimentación en niños con parálisis cerebral

“…This is important, because despite being weakened, the FM of CP patients may be similar to normal, by proportional decrease of body weight components 39 . The main cause of morbidity and mortality were respiratory infections in our study, according to what was published 4,33 . These patients have an increased risk of pulmonary aspiration, secondary to the coexistence of GER and dysphagia 6 , and the presence of WB is an independent risk factor for recurrence of hospitalizations 34 .…”

“…Most of the patients had GMFCS V, coming from a referral center that serves patients with greater severity and comorbidity. This severity is greater than other studies and is the main determinant of survival in these patients 4,27 . The most frequent etiology reported was perinatal (asphyxia and prematurity), although it has been shown that the main one should be prenatal 26 .…”

“…It is the most common physical disability in children, and it affects 2.11 from each 1000 live births 2 , being more frequent in preterm infants 3 . Its mortality is fifty times greater than other causes in the pediatric population, according to its severity and associated comorbidities 4,5 , rating a survival of 60% at 19 years, at date 5 . These patients need a complex multidisciplinary care 6 , in which the nutritional treatment is fundamental, since they present high prevalence of malnutrition 7 , deficient staturo-ponderal rates and even overweight or obesity 8 , as well as deficits of micronutrients 9,10 , osteopenia and osteoporosis 12 .…”