Perspectives on Palliative Care Among Duchenne Muscular Dystrophy Patients and Their Families in Singapore.

INTRODUCTION: With better medical care, patients with Duchenne muscular dystrophy (DMD) now live longer but face more complex medical and social needs. This study described the perceptions of DMD patients and their families of disease-specific palliative care services in Singapore. MATERIALS AND METHODS: A multicentre, cross-sectional study involving DMD patients and their families was carried out. Structured questionnaires were administered to them to collect data on their understanding of palliative care, health services accessed and desired by them and quality of life. RESULTS: A total of 30 pairs of DMD patients and their caregivers responded. Most patients were >13 years old (70%) and non-ambulant (86%). Most of them and their families (70%) were also not aware of palliative care and support services that were available to them in Singapore. Additionally, they perceived greater financial assistance and better transport services as resources that could better meet their care needs. The presence of scoliosis and need for ventilatory support were associated with lower quality of life in patients. CONCLUSION: There is a need to improve awareness and provision of palliative care services for DMD patients in Singapore where discussion of end-of-life care is often considered taboo. Prevention and correction of scoliosis and provision of appropriate ventilatory support may improve quality of life in DMD patients.

Sleep Patterns and Dysfunctions in Children with Learning Problems.

INTRODUCTION: This study aimed to determine the sleep patterns and dysfunctions in children with learning problems in comparison against a local population-based sample. MATERIALS AND METHODS: Parents of 200 children with learning problems and 372 parents of a local population-based sample of typically developing (TD) children were recruited to complete a questionnaire on their child's sleep patterns and sleep problems. The Children's Sleep Habits Questionnaire (CSHQ) is a validated parent-reported sleep screening questionnaire that contains 54 items identifying sleep behaviours in children. RESULTS: The mean age of the sample was 4.2 years (SD: 1.4; range, 2 to 6 years). Sleep duration was similar between the 2 groups. The difference in mean CSHQ subscale scores between children with learning problems and TD children was significant for sleep-disordered breathing (1.3 vs 1.2, P = 0.001). Among children with learning problems, 36.5% snored (vs 26.6% of TD children), 30.5% had noisy breathing (vs 18.8%), and 9.0% (vs 4.6%) experienced difficulty breathing 2 or more times a week. Children with learning problems woke up in a more irritable mood (P = 0.01), had more difficulty in getting out of bed (P <0.001), and took a longer time to be alert (P <0.001). They exhibited fewer behaviours of daytime drowsiness (P = 0.009). Among this group of children, 15.0% of parents reported that their child had a sleep problem compared to 9.0% in the TD group. CONCLUSION: Sleep breathing disorders and symptoms of morning sleepiness are more prevalent in children with learning problems. Symptoms of daytime lethargy are similar between the 2 groups. We suggest that a simple outpatient screening targeted at these problems be instituted in the initial workup of any child with learning difficulties.

Sleep behaviour in a sample of preschool children in Singapore.

INTRODUCTION: Sleep problems are common in all ages, but may be particularly acute in urban Singapore. This study aims to describe the sleep behaviour of, and to identify any sleep problems in, preschool children. MATERIALS AND METHODS: This was a cross-sectional questionnaire survey of 372 children attending local childcare centers. The questionnaire was based on the Children's Sleep Habits Questionnaire (CSHQ), a validated parent-report sleep screening questionnaire that contains 54 items identifying sleep behaviours in children. RESULTS: A total of 372 (40.0%) children participated. The mean age was 4.1 (SD 1.3) years (range, 2 to 6 years). Average total sleep duration was 10.8 hours (SD 1.1) with average night-time sleep duration of 8.5 hours (SD 0.6) and average nap duration of 1.6 hours (SD 1.0). Co-sleeping was common; 80.9% of children shared a room with someone else. The most common sleep problems were in the domains of sleep resistance and morning behaviour; namely: requiring company to fall asleep (n = 272, 73.1%), being afraid to sleep alone (n = 228, 61.6%) and diffi culty in waking up (n = 165, 44.4%). Among parents, 84.1 % (n = 313) perceived that their child's sleep duration was adequate. CONCLUSION: The duration of sleep in the Singaporean preschool population sampled is signifi cantly lower than recommended values and that of previously described Caucasian populations. Parental perception of sleep adequacy deviates from current recommendations. Given the clear relation of sleep duration with cognitive functioning, learning, and physical growth, this sleep deprivation should be addressed with parental education and opportunistic screening of sleep in well-child follow-ups.

Acute starvation in C57BL/6J mice increases myocardial UCP2 and UCP3 protein expression levels and decreases mitochondrial bio-energetic function.

Associations between uncoupling protein (UCP) expression and functional changes in myocardial mitochondrial bio-energetics have not been well studied during periods of starvation stress. Our aim was to study the effects of acute starvation, for 24 or 48 h, on combined cardiac mitochondrial function and UCP expression in mice. Isolated heart mitochondria from female mice starved for 48 h compared to that from mice fed revealed a significantly (p < 0.05) decreased adenosine diphosphate-to-oxygen ratio, a significantly increased proton leak and an increased GTP inhibition on palmitic acid-induced state 4 oxygen consumption (p < 0.05). These bio-energetic functional changes were associated with increases in mitochondrial UCP2 and UCP3 protein expression. In conclusion, our findings suggest that increased UCP2 and UCP3 levels may contribute to decreased myocardial mitochondrial bio-energetic function due to starvation.