IMPLICATIONS FOR PRACTICE: Our findings offer guidance to improve aftercare for bereaved siblings and their families. Additional research is needed to further delineate the needs of bereaved siblings and to develop strategies to promote adaptation to…
BACKGROUND: Progressive neuromuscular disease in children is life limiting and these children and young people would benefit from palliative care services, but data are limited on the number and demography of these children. AIM: To describe the…
BACKGROUND: Life-limiting conditions (LLCs) describe diseases with no reasonable hope of cure that will ultimately be fatal. For children with these diseases, palliative care services should be available but few data are available to estimate the…
OBJECTIVES: To compare peer relationships among bereaved siblings and matched classmates, and to examine gender, grade level, and time since death as moderators. METHODS: Families were recruited from cancer registries at four hospitals 3-12 months…
Frequency and type of incontinence and its association with other variables were assessed in females with Rett Syndrome (RS) ( = 63), using an adapted Dutch version of the 'Parental Questionnaire: Enuresis/Urinary Incontinence' (Beetz et al. 1994).…
CONTEXT: Few studies have compared multiple perspectives of changes experienced by parents after a child's death. OBJECTIVES: This study used interviews with bereaved parents and siblings to examine changes in parents during the first year after the…
Background: Rett syndrome (RTT) and autism disorder (AD) are 2 neurodevelopmental disorders of early life that share phenotypic features, one being hand stereotypies. Distinguishing RTT from AD often represents a challenge, and given their distinct…
Rett syndrome is characterized by loss of motor and social functions, development of stereotypic hand movements, seizures, and breathing disturbances. This study evaluates the presence of overnight respiratory disturbances. Polysomnography in…
Like any new specialty, paediatric palliative medicine is facing challenges as it establishes itself. While many of the required core skills have their roots in adult palliative medicine, its practitioners come from a range of paediatric backgrounds…
We employed grounded theory to explain how Canadian pregnant women and care providers manage birth. The sample comprised 9 pregnant women and 56 intrapartum care providers (family doctors, midwives, nurses, obstetricians, and doulas [individuals…
ObjectiveTo review the experience of the practice of withdrawal of artificial nutrition and hydration (WANH) and to describe parental perspectives on the process.DesignA retrospective chart review and parental survey.SettingTertiary level Neonatal…
Recent studies and consensus statements have expressed the need to involve palliative care services in the care of children with progressive neuromuscular diseases (PMD), yet there have been no reviews of the utilization of palliative care services…
Objective: Degenerative ataxias in children present a rare condition where effective treatments are lacking. Intensive coordinative training based on physiotherapeutic exercises improves degenerative ataxia in adults, but such exercises have…
Abstract Background: Proxy reporting is frequently used to assess symptom distress of patients with advanced chronic organ failure. The aim of the present cross-sectional study was to examine agreement in severity of symptom distress, presence of…
X-linked adrenoleukodystrophy (X-ALD) is the most frequent peroxisomal disease. The two main clinical phenotypes of X-ALD are adrenomyeloneuropathy (AMN) and inflammatory cerebral ALD that manifests either in children or more rarely in adults. About…
BACKGROUND: Pain is common during cancer treatment, and patient self-reporting of pain is an essential first step for ideal cancer pain management. However, many studies on cancer pain management report that, because pain may be underestimated, it is…
BACKGROUND: The purpose of this article is to report on the quality of the existing evidence base regarding the effectiveness of clinical pathway (CPW) research in the hospital setting. The analysis is based on a recently published Cochrane review of…
OBJECTIVE: We conducted a nationwide survey to determine the prevalence of common gastrointestinal and nutritional disorders in Rett syndrome (RTT) based on parental reporting and related the occurrence of these problems to age and methyl-CpG-binding…
BACKGROUND AND PURPOSE: The purpose of this study was to evaluate the mutation status of PANK2 among Korean patients with pantothenate kinase-associated neurodegeneration (PKAN) and to document the outcome of pallidal deep brain stimulation (DBS).…
INTRODUCTION: Rett syndrome (RTT) is a neurological disorder usually associated with a mutation in the MECP2 gene. Conductive Education (CE) is an educational approach that has not yet been explored with regard to children with RTT. OBJECTIVE:…
The prevalence of sleep problems in individuals with intellectual disability (ID) seems to vary between genetic syndromes associated with ID. Different types of sleep disturbances may indicate underlying causes of sleep problems and these types of…
‘Virtual memorials’ intended to memorialize the lives of children imply significant shifts in the conceptualization of death, particularly for grieving parents. Created by parents in memory of their deceased children, on-line memorials constructed…
Palliative care provides a holistic approach to symptom relief using a multidisciplinary team approach to enhance quality of life throughout the entire course of a particular illness. The care team consists of movement disorders neurologist, a…
BACKGROUND: Oropharyngeal dysphagia encompasses problems with the oral preparatory phase of swallowing (chewing and preparing the food), oral phase (moving the food or fluid posteriorly through the oral cavity with the tongue into the back of the…
