Tertiary paediatric hospital admissions in children and young people with cerebral palsy.

BACKGROUND: Many previous studies that have investigated hospital admissions in children and young people with cerebral palsy lack information on cerebral palsy severity and complexity. Consequently, little is known about factors associated with the frequency and type of hospital admissions in this population. This study used hospital admission data available for all children and young people known to a population-based cerebral palsy register to describe the patterns of use of tertiary paediatric hospital services over a 5-year period. METHODS: This was a retrospective cohort analysis of routinely collected admission data from the two tertiary paediatric hospitals in the Australian state of Victoria. Data on admissions of individuals born between 1993 and 2008 registered on the Victorian Cerebral Palsy Register were analysed (n = 2183). RESULTS: Between 2008 and 2012, 53% of the cohort (n = 1160) had at least one same-day admission, and 46% (n = 996) had one or more multi-day admissions. Those with a moderate to severe motor impairment and those with a co-diagnosis of epilepsy had more admissions, and for multi-day admissions, longer lengths of stay, P < 0.05. Across all severity levels, respiratory and musculoskeletal diseases were the most frequently reported reasons for medical and surgical admissions, respectively. All-cause readmission rates for urgent multi-day stays within 7, 30 and 365 days of an index admission were 10%, 23% and 63%, respectively. CONCLUSIONS: The reasons for hospital admissions reported here reflect the range of comorbidities experienced by children and young people with cerebral palsy. This study highlights priority areas for prevention, early diagnosis and medical management in this group. Improved primary and secondary prevention measures may decrease non-elective hospital admissions and readmissions in this group and reduce paediatric inpatient resource use and healthcare expenditure attributable to cerebral palsy.

One-year follow-up of the outcome of a randomized controlled trial of a home-based intervention programme for children with autism and developmental delay and their families.

INTRODUCTION: There is debate about the type and intensity of early childhood intervention that is most helpful for children with developmental problems. The aim of the study was to determine whether a home-based programme provided over 12 months resulted in sustained improvement in development and behaviour 12 months after the intervention ceased. The characteristics of the children and families who benefited most from the intervention were also studied. METHOD: Randomized controlled trial. Participants A total of 59 children, aged 3-5 years, attending two early childhood intervention centres in Melbourne, Australia. Intervention Half of the subjects received an additional home-based programme consisting of 40 weekly visits. MAIN OUTCOME MEASURES: Bayley Scales of Infant Development and Wechsler Preschool and Primary Scale of Intelligence Revised, Preschool Behaviour Checklist, Bayley Behaviour Rating Scale and Behaviour Screening Questionnaire. All tests administered pre-intervention, following the intervention and 12 months later. Secondary outcome measures Family stress, support and empowerment. RESULTS: Fifty-four children completed the assessments 12 months after conclusion of the intervention. Compared with the control group, improvement in aspects of cognitive development in the children who received the extra intervention was sustained 1 year later (P= 0.007) while significant behavioural differences post intervention were not. Analyses of the data by the Reliable Change Index indicated improvement of clinical significance occurred in non-verbal areas. In contrast to the control group who deteriorated, language skills in the intervention group remained stable. Improvements were significantly associated with higher stress in the families. CONCLUSION: Improvements following the provision of a home-based programme to preschool children with developmental disabilities were sustained 1 year later. Children from highly stressed families appeared to benefit most, reinforcing the importance of involving families in early childhood intervention programmes.

The relationship between cerebral palsy and cryptorchidism.

This study examined the reported association between cerebral palsy and cryptorchidism. A comparison was made among 25 boys with cerebral palsy under the age of 2 years and 6 months, 25 boys with cerebral palsy aged between 5 and 10 years, and age-matched controls. The testes remained in the same position with age in boys with cerebral palsy, whereas in normal children the testes were slightly lower initially (P less than .005) and became lower still with growth (P less than .001). This result, taken in conjunction with previous studies, casts doubts on the theories of early damage to the hypothalamic-pituitary-gonadal axis as the cause of maldescent in cerebral palsy. It is postulated that any apparent increase in cryptorchidism in older patients with cerebral palsy may be caused by spasticity of the cremaster muscle leading to pathologic retraction of the testis out of the scrotum.

Asthma: the doctor's failure in communication.

Forty-one children with asthma and their parents were interviewed to assess their level of understanding of the disease, drug therapy and prognosis, to define their fears and anxieties and to elucidate the extent to which asthma had interfered with activities and family life. Knowledge of the disease and its natural history was poor, understanding of drug therapy inadequate and anxieties relating to death, separation, permanent lung damage, curtailment of activities were common.

Dental health of children with cerebral palsy following sialodochoplasty.

Drooling occurs commonly in children with cerebral palsy (CP). Surgical procedures, known as slalodochoplasties, are often performed for the control of drooling. These include major salivary gland excision, parasympathetic nerve section, duct ligation, and duct re-routing. Alterations in saliva amount, flow, and consistency occur following sialodochoplasty, and the resultant effect on dental homeostasis requires further investigation. This controlled study investigated 19 children with CP following sialodochoplasty (surgery group) and 75 children with CP treated nonsurgically (control group) who attended our hospital. Dental caries experience-including dmft, DMFT, and partial DMFS scores of mandibular incisors and canines only-plaque index, and enamel developmental defects index were recorded. Saliva buffering capacity and bacterial counts were assessed. The surgical group (median DMFT = 5.00) had significantly more dental caries when compared with the control group (median DMFT = 0.00), Wilcoxon Signed-rank Test, P < 0.0001. This study has shown that children with CP following sialodochoplasty have increased risk of dental caries when compared with those treated nonsurgically for drooling. Although no caries predictors were identified, alterations to the caries-protective role of saliva are considered the likely cause. Children who undergo this procedure should receive intensive pre- and postsurgical preventive dental therapy.

Randomized trial of botulinum toxin injections into the salivary glands to reduce drooling in children with neurological disorders.

The primary aim of this randomized, controlled trial was to assess the effectiveness of botulinum toxin A (BoNT-A) injections into the submandibular and parotid glands on drooling in children with cerebral palsy (CP) and other neurological disorders. Secondary aims were to ascertain the duration of any such effect and the timing of maximal response. Of the 48 participants (27 males, 21 females; mean age 11y 4mo [SD 3y 3mo], range 6-18y), 31 had a diagnosis of CP and 15 had a primary intellectual disability; 27 children were non-ambulant. Twenty-four children randomized to the treatment group received 25 units of BoNT-A into each parotid and submandibular gland. Those randomized to the control group received no treatment. The degree and impact of drooling was assessed by carers using the Drooling Impact Scale questionnaire at baseline and at monthly intervals up to 6 months postinjection/baseline, and again at 1 year. Maximal response was at 1 month at which time there was a highly significant difference in the mean scores between the groups. This difference remained statistically significant at 6 months. Four children failed to respond to the injections, four had mediocre results, and 16 had good results. While the use of BoNT-A can help to manage drooling in many children with neurological disorders, further research is needed to fully understand the range of responses.

Cerebral palsy: what do medical students know and believe?

OBJECTIVES: Negative attitudes toward people with disabilities, including cerebral palsy, may be related to misunderstandings or lack of knowledge about the disability. If held by medical practitioners, they can have detrimental implications for the care of people with disabilities. The purposes of this study were to examine the knowledge and attitudes of medical students regarding cerebral palsy and to examine the effects of the videotape 'Understanding Cerebral Palsy' on these two areas. METHODS: The attitudes and knowledge regarding cerebral palsy of 54 medical students in their penultimate year were measured before and after watching a video produced to educate health professionals about cerebral palsy. They were assessed using a self-administered questionnaire constructed specifically for the study, adapted from previously validated questionnaires. RESULTS: These medical students generally had limited knowledge about cerebral palsy and displayed negative attitudes toward people with cerebral palsy. It was also found that males had less positive attitudes than females (P = 0.014) and that students educated mainly in Asia had less positive attitudes than students educated mainly in Australia (P = 0.012). The videotape was shown to be effective in improving the students' knowledge about cerebral palsy. A small but significant improvement in attitudes was also shown (P = 0.014), with the attitudes of some students improving dramatically. However, negative attitudes remained in many. CONCLUSIONS: Based on the findings, structured teaching about cerebral palsy is necessary within the medical curriculum at the University of Melbourne. Greater promotion of positive attitudes toward people with cerebral palsy and other disabilities is required.

Agreement of aspiration tests using barium videofluoroscopy, salivagram, and milk scan in children with cerebral palsy.

To study the agreement between three tests for aspiration, barium videofluoroscopy, salivagram, and milk scan we studied 63 children with severe non-ambulant spastic quadriplegic cerebral palsy (CP) aged 14 months to 16 years (32 males, 31 females). The salivagram was most frequently positive (56%, 95% confidence interval 43 to 68%); the next most frequently positive was barium videofluoroscopy when aspiration was defined as the presence of either laryngeal penetration of material or frank aspiration (39%, 95% confidence interval 26 to 53%). The milk scan was rarely positive (6%, 95% confidence interval 2 to 16%). Agreement between the tests of aspiration was poor. The maximum agreement (kappa=0.20) was between aspiration as diagnosed with the salivagram and by barium videofluoroscopy. Positive tests for aspiration are frequent in children with severe CP. Frequency varies widely depending on the investigation used. There is poor agreement between tests used for the diagnosis of aspiration. This information is of importance in assessing the significance of test results.

The health care of young adults with cerebral palsy.

OBJECTIVE: To evaluate the health status and provision of health services to young adults with cerebral palsy. RESULTS: Ninety-seven per cent had ongoing health problems. Consultations with most health professionals declined markedly after leaving school. CONCLUSIONS: Young adults with cerebral palsy have considerable, continuing impairment and disability. Lack of contact with health services after they leave school may adversely affect their health status.

A pilot evaluation of conductive education-based intervention for children with cerebral palsy: the Tongala project.

OBJECTIVE: To perform an objective evaluation of a programme based on conductive education (CE). METHODOLOGY: Two groups of children with cerebral palsy were studied over 6 months. Eleven children participated in a CE-based programme and nine children received traditional early intervention programmes. Videotapes of the children performing items from the Vulpe Assessment Battery (VAB) were scored by assessors blind to the treatment group. The questionnaire on resources and stress (QRS-F) was administered to the primary caregiver. RESULTS: Few statistically significant results were obtained. There was a trend for the conductive education-based group to make slightly greater gains. The videotaped measures yielded high Chronbach coefficients and high interrater correlation coefficients suggesting that this is a useful method to evaluate progress. CONCLUSIONS: The study was limited by small sample size, the nature of the control group and the lack of random assignment to CE and control groups. It did provide a basis for the development of further research in the area.

Six month follow-up: the crucial test of multidisciplinary developmental assessment.

Participation of parents in the developmental assessment process is thought to be beneficial in promoting understanding of their child's disability, and improving consensus between parents and professionals about appropriate intervention programmes. If costly multidisciplinary assessments are to be justified, it is necessary to establish long-term benefits for the child. This highlights a need for research identifying how families use services after diagnostic assessment and what they understand to be important for their child. Poor parent-professional agreement about diagnosis may be a factor contributing to low compliance with recommendations. The major purpose of the current study was to follow-up families 6 months after developmental assessment, in order to investigate use of recommended intervention services. In addition, mothers' opinions about diagnostic findings, recommendations and early intervention services were examined. Subjects were 40 pre-school children who presented for developmental assessment, and their mothers. The majority were diagnosed with developmental problems in multiple domains. Results indicated that most mothers recalled and agreed with their child's diagnosis, but underestimated the severity of developmental delay. Families had not accessed the range of multidisciplinary intervention programmes recommended, given the complexity of their children's disabilities. Speech therapy was considered the service of highest priority by mothers, and was the treatment most frequently received. Mothers recognized a need for more therapeutic interventions for their child. An unexpected finding was the high prevalence of families who organized nonprescribed therapies. Possible explanations of the findings and implications for service delivery are discussed.

Efficacy of programmes based on Conductive Education for young children with cerebral palsy.

Conductive Education (CE)-based programmes have been introduced to Australia over the past 10 years. The aim of this project was to compare these programmes with traditional neurodevelopmental programmes of rehabilitation for young children (12 to 36 months, mean age 22 months 3 weeks) with cerebral palsy. Thirty-four children were matched by age, motor impairment, and cognitive ability and randomly allocated to CE-based or equivalent-intensity control programmes. Those unwilling to be randomized (32) were still studied but their outcomes were treated separately. Our results indicate that children involved in CE-based programmes made similar progress to those involved in traditional programmes. Statistically significant changes were spread among the four groups of subjects. The findings also endorsed the measures used and the expected maturational effect was detected. Although there were major difficulties in conducting this randomized trial, it was achieved by gathering parental and professional support, and adequate funding.

Cerebral palsy in Victoria, Australia: mortality and causes of death.

OBJECTIVE: To study the causes of death and the characteristics of children with cerebral palsy that had died over a 25-year period in Victoria, Australia. METHODOLOGY: Names of children that had died were collected from the Victorian Cerebral Palsy Register. Their hospital records were studied and information was gathered about age of death, motor impairment, the presence or absence of associated disabilities and cause of death. RESULTS: One hundred and fifty-five children had died during the period 1970-95. The majority of children had severe spastic quadriplegia, intellectual disability and epilepsy. The predominant cause of death was pneumonia, although for many children who died at home the cause was unknown. CONCLUSIONS: Children with cerebral palsy are a diverse group and those with a severe motor deficit have a reduced life expectancy. Lung disease remains an important cause of morbidity and mortality for this group. Further information about the causes of death is needed, particularly for those children that die at home.

Children with sialorrhoea in the absence of neurological abnormalities.

OBJECTIVE: To study the outcome of 21 typically developing children who had been referred to the Saliva Control Clinic at the Royal Children's Hospital, Melbourne. METHOD: Information was collected on various aspects of the child's health, oromotor function and severity of drooling. A follow-up telephone survey was completed by the parents of these children after a mean period of 3.4 years. Information was gathered regarding their child's drooling, current health, oromotor status and the usefulness of the clinic. RESULTS: There was a statistically significant improvement (P < 0.01) in the saliva control measures analysed on follow-up. Thirteen children ceased to drool, three still drooled occasionally, and five continued to drool. The recommendations, mainly advice and referrals to other health professionals, were generally reported to be helpful by the parents. CONCLUSIONS: This cohort of children was established retrospectively, and the study is therefore limited in both numbers and design. The results indicate that some typically developing children may be delayed in the development of saliva control. Parents and professionals can be reassured that this problem is likely to resolve.

Gastro-oesophageal reflux and feeding problems after gastrostomy in children with severe neurological impairment.

This study evaluated the effect of percutaneous endoscopic gastrostomy (PEG) on the feeding problems and gastro-oesophageal reflux (GOR) of 30 consecutive children with severe neurological impairment who had PEG between October 1990 and March 1993. Evaluation was by questionnaire, clinical history, examination, 24-hour oesophageal pH monitoring and endoscopy. Gastrostomy placement significantly reduced feeding time, feed-related choking episodes and frequency of chest infections. Family stress was significantly reduced in two-thirds of cases. Significant weight-gain occurred. The clinical severity of GOR was significantly increased in eight patients and fundoplication was required in five. 24-hour oesophageal pH measurements before PEG did not reliably predict subsequently increased GOR. Seven patients died, but their deaths were apparently unrelated to GOR. PEG effectively provides nutrition, improves feed-related stresses, but may exacerbate GOR.

Objective assessment of limb movement in children with cerebral palsy.

A preliminary report of a method for assessing the quality of movement in children with cerebral palsy, using an accelerometer, is presented. Able-bodied and disabled children performed a simple task, and there were marked differences between the two groups. Whilst the technique requires further development, it is hoped that accelerometry may eventually provide an objective tool to enable rigorous testing of various therapeutic procedures in cerebral palsy.

Long-term outcome of saliva-control surgery.

Thirty-nine patients who had undergone transposition of the submandibular ducts and unilateral ligation of a parotid duct for saliva control were followed up six years after surgery. Overall improvement was documented according to a drooling quotient, drooling severity and drooling frequency measures. Despite this superficially favourable outcome, a significant proportion of patients (39 per cent) or their caregivers had not found the surgery helpful. Complications included ranula formation, complaints of dry mouth, difficulty with swallowing, and changes in the consistency of oral secretions. More knowledge is needed of the likely outcomes of this procedure in individual patients, so that appropriate advice can be given and complications minimised.

Evaluation of an early childhood programme based on principles of conductive education: the Yooralla project.

OBJECTIVE: To perform an objective evaluation of a programme based on conductive education (CE) for preschool and early school-aged children with cerebral palsy. METHODOLOGY: The progress of 17 children participating in a CE-based programme (the Yooralla programme) was compared with 17 children enrolled in traditional early childhood services. Videotapes of the children performing items from the Vulpe Assessment Battery (VAB) were scored by assessors blind to the treatment group; three standardized tests of cognitive ability were administered; and the questionnaire on resources and stress (QRS-F) was given to the primary caregiver at the beginning and conclusion of 6 months. RESULTS: The Yooralla group improved in motor performance and parental coping variables. Both groups showed improvement on the cognitive measures, with the control group demonstrating slightly greater gains. CONCLUSIONS: Conductive education may benefit the motor development of children with cerebral palsy and provide greater parental support. A randomized control study with adequate subject numbers is necessary to confirm this tentative conclusion.

Early intervention: professional views and referral practices of Australian paediatricians.

OBJECTIVE: To gather information from paediatricians concerning their attitudes to early intervention services for young children with developmental disabilities and to study their referral practices. METHODOLOGY: Two hundred and ninety-five Australian paediatricians completed a postal questionnaire. RESULTS: Paediatricians presented a positive view of early intervention, particularly for its beneficial impact on families, and perceived the quality of services to be comprehensive or adequate. There were few differences between States, or between country or metropolitan areas. Most paediatricians make prompt referrals to early intervention services when a child has an established disability. Referral is much less likely with suspected delay. CONCLUSIONS: Paediatricians are aware of the benefits of early intervention but additional information concerning the role of these services and their significant impact on families and children should be provided.