The aim of this study is to assess the evolution of respiratory and feeding support in children with spinal muscular atrophy (SMA) type 1 after 24 months of nusinersen treatment.

Data on SMA type 1 children treated with nusinersen between 2017 and 2023 from the Canadian Neuromuscular Disease Registry were extracted. The cohort was divided into two groups based on age at treatment initiation: ≤2 years and >2 years. The primary outcome was the (i) time to death or needing full-time (≥16 hours/day) ventilation and (ii) time to needing feeding tube support. The secondary outcomes were differences in respiratory and feeding support requirements between the two groups at 24-month follow-up.

Thirty-two children were included, and the median age (range) for treatment initiation was 3.2 months (0.8– 13.1) in children who initiated treatment at ≤2 years and 51.2 (28.7–183.8) in those who initiated at >2 years of age. The median age of death or full-time ventilation was 8.6 months (6–22.4) and 10.5 months (4–24) for the two groups, respectively. The median age for initiation of feeding support was 5.1 (1.7–26.4) and 14.5 months (3.9–130.6), respectively. At 24 months (n = 23), there were no significant differences between the need for respiratory or feeding tube support between the two treatment groups.

Most children with SMA type 1 treated with nusinersen across Canada have continued need for respiratory and feeding support over time when initiated after symptom onset.

Radiation dermatitis (RD) is a frequent toxicity during radiotherapy (RT) for head and neck cancer (HNC). We report the first use of KeraStat® Cream (KC), a topical, keratin-based wound dressing, in patients with HNC receiving RT.

This pilot study randomized HNC patients treated with definitive or postoperative RT (≥60 Gy) to KC or standard of care (SOC), applied at least twice daily during and for 1-month after RT. Outcomes of interest included adherence to the assigned regimen (at least 10 applications per week of treatment), clinician- and patient-reported RD, and skin-related quality of life.

24 patients were randomized and completed the study. Most patients had stage III-IV disease and oropharynx cancer. Median RT dose was 68 Gy; the bilateral neck was treated in 19 patients, and 18 patients received concurrent chemotherapy. Complete adherence was observed in 7/12 (SOC) vs. 10/12 (KC, p = 0.65).  Adherence by patient-week was 61/68 versus 64/67, respectively (p = 0.20). No differences in RD were observed between groups.

A randomized trial of KC versus SOC in HNC patients treated with RT is feasible with good adherence to study agent. An adequately powered randomized study is warranted to test the efficacy of KC in reducing RD.

Newborn screening (NBS) identifies infants with severe, early-onset diseases, enabling early diagnosis and treatment. In Canada, decisions regarding disease inclusion in NBS programs occur at the provincial level, which leads to variability in patient care. We aimed to determine whether important differences exist in NBS programs across provinces and territories. Given that spinal muscular atrophy (SMA) is the most recent disease added to NBS programs, we hypothesized that its inclusion would show interprovincial variability and be more likely in provinces already screening for a greater number of diseases.

We conducted a cross-sectional survey of all NBS labs in Canada to understand: 1) what conditions were included in their program; 2) what genetic-based testing was performed and; 3) if SMA was included.

All NBS programs (N = 8) responded to this survey by June 2022. There was a 2.5-fold difference in the number of conditions screened (N = 14 vs N = 36) and a 9-fold difference in the number of conditions screened by gene-based testing. Only nine conditions were common to all provincial NBS programs. NBS for SMA was performed in four provinces at the time of our survey, with BC recently becoming the fifth province to add SMA to their NBS on October 1, 2022. Currently, 72% of Canadian newborns are screened for SMA at birth.

Although healthcare in Canada is universal, its decentralization gives rise to regional differences in NBS programs which creates inequity in the treatment, care, and potential outcomes of affected children across provincial jurisdictions.

Research has shown a strong relationship between hallucinations and suicidal behaviour in general population samples. Whether hallucinations also index suicidal behaviour risk in groups at elevated risk of suicidal behaviour, namely in individuals with a sexual assault history, remains to be seen.

We assessed whether hallucinations were markers of risk for suicidal behaviour among individuals with a sexual assault history.

Using the cross-sectional 2007 (N = 7403) and 2014 (N = 7546) Adult Psychiatric Morbidity Surveys, we assessed for an interaction between sexual assault and hallucinations in terms of the odds of suicide attempt, as well as directly comparing the prevalence of suicide attempt in individuals with a sexual assault history with v. without hallucinations.

Individuals with a sexual assault history had increased odds of hallucinations and suicide attempt compared to individuals without a sexual assault history in both samples. There was a significant interaction between sexual assault and hallucinations in terms of the odds of suicide attempt. In total, 14–19% of individuals with a sexual assault history who did not report hallucinations had one or more suicide attempt. This increased to 33–52% of individuals with a sexual assault history who did report hallucinations (2007, aOR = 2.85, 1.71–4.75; 2014, aOR = 4.52, 2.78–7.35).

Hallucinations are a risk marker for suicide attempt even among individuals with an elevated risk of suicidal behaviour, specifically individuals with a sexual assault history. This finding highlights the clinical significance of hallucinations with regard to suicidal behaviour risk, even among high-risk populations.

Community studies have found a relatively high prevalence of hallucinations, which are associated with a range of (psychotic and non-psychotic) mental disorders, as well as with suicidal ideation and behaviour. The literature on hallucinations in the general population has largely focused on adolescents and young adults.

We aimed to explore the prevalence and psychopathologic significance of hallucinations across the adult lifespan.

Using the 1993, 2000, 2007 and 2014 cross-sectional Adult Psychiatric Morbidity Survey series (N = 33 637), we calculated the prevalence of past-year hallucinations in the general population ages 16 to ≥90 years. We used logistic regression to examine the relationship between hallucinations and a range of mental disorders, suicidal ideation and suicide attempts.

The prevalence of past-year hallucinations varied across the adult lifespan, from a high of 7% in individuals aged 16–19 years, to a low of 3% in individuals aged ≥70 years. In all age groups, hallucinations were associated with increased risk for mental disorders, suicidal ideation and suicide attempts, but there was also evidence of significant age-related variation. In particular, hallucinations in older adults were less likely to be associated with a cooccurring mental disorder, suicidal ideation or suicide attempt compared with early adulthood and middle age.

Our findings highlight important life-course developmental features of hallucinations from early adulthood to old age.

Self-harm is a common presentation to acute hospitals, associated with increased risk of completed suicide. Safety plans are increasingly recommended to help patients recognise and prevent escalation of self-harm behaviours.

This project aimed to improve quality and documentation of safety planning for patients admitted at an acute general hospital due to self-harm, who were assessed by Liaison Psychiatry. We aimed to increase the number of patients given written safety plans on discharge by 50%.

The PDSA cycle model of quality improvement was used. A retrospective audit of clinical records was conducted over 3 months to establish baseline documentation of safety planning (n = 51). A template for a self-harm crisis plan, used in other areas of the Trust, was adopted, to be adapted to each patient. A leaflet for sources of crisis support and patient feedback form were developed and distributed to clinicians in the team. Data collection was repeated one month later (n = 48). The second set of interventions involved a training session for clinicians on developing safety plans in collaboration with patients, and a poster highlighting the process to be undertaken when discharging a patient admitted with self-harm.

Following initial interventions, 20% of patients had completed safety plans and 50% received advice, an increase of 20% and 40% respectively. The second PDSA cycle showed increase in numbers to 38% and 67% respectively.

Creating a crisis plan with a hospital-specific leaflet for the Liaison Psychiatry team increased the number of patients discharged with safety plans in place. 86% of patients who participated in safety-planning found the process helpful and felt likely to use the plan in future crises. This is an area of ongoing quality improvement which can be implemented in other hospitals to better equip patients with skills and support to reduce self-harm/suicide attempts.

The general health of children of parents with mental illness is overlooked.

To quantify the difference in healthcare use of children exposed and unexposed to maternal mental illness (MMI).

This was a retrospective cohort study of children aged 0–17 years, from 1 April 2007 to 31 July 2017, using a primary care register (Clinical Practice Research Datalink) linked to Hospital Episodes Statistics. MMI included non-affective/affective psychosis and mood, anxiety, addiction, eating and personality disorders. Healthcare use included prescriptions, primary care and secondary care contacts; inflation adjusted costs were applied. The rate and cost was calculated and compared for children exposed and unexposed to MMI using negative binomial regression models. The total annual cost to NHS England of children with MMI was estimated.

The study included 489 255 children: 238 106 (48.7%) girls, 112 741 children (23.0%) exposed to MMI. Compared to unexposed children, exposed children had a higher rate of healthcare use (rate ratio 1.27, 95% CI 1.26–1.28), averaging 2.21 extra contacts per exposed child per year (95% CI 2.14–2.29). Increased healthcare use among exposed children occurred in inpatients (rate ratio 1.37, 95% CI 1.32–1.42), emergency care visits (rate ratio 1.34, 95% CI 1.33–1.36), outpatients (rate ratio 1.30, 95% CI 1.28–1.32), prescriptions (rate ratio 1.28, 95% CI 1.26–1.30) and primary care consultations (rate ratio 1.24, 95% CI 1.23–1.25). This costs NHS England an additional £656 million (95% CI £619–£692 million), annually.

Children of mentally ill mothers are a health vulnerable group for whom targeted intervention may create benefit for individuals, families, as well as limited NHS resources.

The Zero Suicide framework is a system-wide approach to prevent suicides in health services. It has been implemented worldwide but has a poor evidence-base of effectiveness.

To evaluate the effectiveness of the Zero Suicide framework, implemented in a clinical suicide prevention pathway (SPP) by a large public mental health service in Australia, in reducing repeated suicide attempts after an index attempt.

A total of 604 persons with 737 suicide attempt presentations were identified between 1 July and 31 December 2017. Relative risk for a subsequent suicide attempt within various time periods was calculated using cross-sectional analysis. Subsequently, a 10-year suicide attempt history (2009–2018) for the cohort was used in time-to-recurrent-event analyses.

Placement on the SPP reduced risk for a repeated suicide attempt within 7 days (RR = 0.29; 95% CI 0.11–0.75), 14 days (RR = 0.38; 95% CI 0.18–0.78), 30 days (RR = 0.55; 95% CI 0.33–0.94) and 90 days (RR = 0.62; 95% CI 0.41–0.95). Time-to-recurrent event analysis showed that SPP placement extended time to re-presentation (HR = 0.65; 95% CI 0.57–0.67). A diagnosis of personality disorder (HR = 2.70; 95% CI 2.03–3.58), previous suicide attempt (HR = 1.78; 95% CI 1.46–2.17) and Indigenous status (HR = 1.46; 95% CI 0.98–2.25) increased the hazard for re-presentation, whereas older age decreased it (HR = 0.92; 95% CI 0.86–0.98). The effect of the SPP was similar across all groups, reducing the risk of re-presentation to about 65% of that seen in those not placed on the SPP.

This paper demonstrates a reduction in repeated suicide attempts after an index attempt and a longer time to a subsequent attempt for those receiving multilevel care based on the Zero Suicide framework.

Patient registries represent an important method of organizing “real world” patient information for clinical and research purposes. Registries can facilitate clinical trial planning and recruitment and are particularly useful in this regard for uncommon and rare diseases. Neuromuscular diseases (NMDs) are individually rare but in aggregate have a significant prevalence. In Canada, information on NMDs is lacking. Barriers to performing Canadian multicentre NMD research exist which can be overcome by a comprehensive and collaborative NMD registry.

We describe the objectives, design, feasibility and initial recruitment results for the Canadian Neuromuscular Disease Registry (CNDR).

The CNDR is a clinic-based registry which launched nationally in June 2011, incorporates paediatric and adult neuromuscular clinics in British Columbia, Alberta, Ontario, Quebec, New Brunswick and Nova Scotia and, as of December 2012, has recruited 1161 patients from 12 provinces and territories. Complete medical datasets have been captured on 460 “index disease” patients. Another 618 “non-index” patients have been recruited with capture of physician-confirmed diagnosis and contact information. We have demonstrated the feasibility of blended clinic and central office-based recruitment. “Index disease” patients recruited at the time of writing include 253 with Duchenne and Becker muscular dystrophy, 161 with myotonic dystrophy, and 71 with ALS.

The CNDR is a new nationwide registry of patients with NMDs that represents an important advance in Canadian neuromuscular disease research capacity. It provides an innovative platform for organizing patient information to facilitate clinical research and to expedite translation of recent laboratory findings into human studies.

Perceived causes of depression can affect treatment preferences and outcomes. Men and women may have somewhat differing views about the causes of their depression, but there is a paucity of research on gender differences in hospitalised patients with depression.

We examined potential gender differences in hospitalised patients’ perceived causes for their depression and their relationship with treatment beliefs and preferences.

A sample of 52 psychiatric inpatients hospitalised with depression was recruited and completed self-report measures of reasons for depression, depression severity, treatment beliefs and preferences.

Biological reasons for depression were associated with more severe depression and more positive medication beliefs. In addition, results showed that women were significantly more likely to endorse physical and biological reasons for their depression compared with men. Gender moderated the relationship between physical reasons for depression and medication beliefs, such that men endorsing physical reasons found antidepressants less acceptable and had more negative beliefs about medication compared to women.

Findings indicate that depressed men and women in the hospital may have differing views about the causes of their depression and this may affect the acceptability of treatments. Depression treatment in inpatient settings should be better personalised to match the beliefs of individual patients.

Food cost is an important factor influencing the consumption of nutritious foods and subsequent chronic disease risk. The present study compared the cost of branded food products with their generic equivalents across a range of food categories.

The survey was conducted within two major supermarket chains across six locations in Sydney, Australia (n 12).

Price differences were calculated for ‘core’ (nutrient dense and low in energy) and ‘extra’ (high in undesirable nutrients and/or energy) packaged foods (n 22) between generic and branded items.

A cost saving of 44 % was found by purchasing generic over branded products across all food categories. The most significant savings were for core foods, such as bread and cereals, and the smallest cost savings were seen for fruit products. There was little variation in cost saving between branded and generic products by socio-economic status of the supermarket location.

The large price differential between branded and generic food products implies that consumers, particularly those on lower incomes, could benefit financially from purchasing generic items. The promotion of core generic products may be an effective strategy to assist people on lower incomes to meet dietary guidelines.

This paper describes findings from a study that evaluated the implementation and impact of case management for long-term conditions (CMLTC) in 10 primary care trusts (PCTs).

Patients who have long-term conditions and complex health and social needs may require case management to deliver and coordinate their care from a range of agencies.

A cross-sectional postal survey of managers with lead responsibility for CMLTC in each PCT is adopted to describe the implementation of services. A retrospective cohort analysis of longitudinal routinely collected admission data for patients enrolled within the CMLTC service (nine months before and nine months after the entry; n = 867) is used to measure their impact.

The organisation of case management varied between PCTs in some aspects despite a high level of coordination across the geographical area. Mean emergency admissions and associated length of stay (LOS) for patients reduced significantly in the nine months after the service entry. There were a number of fairly robust positive and negative influences on these outcome measures in the regression analysis. Most patients with a history of emergency admissions experienced a marked improvement over time. However, most of those without any or with few admissions experienced an increase in admissions and corresponding LOS. Furthermore, a proportion of frequent service users with particular diagnoses also experienced an increase or remained at a high level. A very modest effect was shown with regard to the features of case management arrangements. For each day spent in hospital before service entry, patients are predicted to experience a reduction of nearly one day after. The main contributor explaining increases in LOS for emergency admissions was the number of primary and secondary diagnoses. Each added diagnosis is associated with a 2.4-day increase in LOS, everything else being equal.

Sudden, out-of-hospital cardiac arrest (OHCA) has an annual incidence of approximately 50 per 100,000 population. Public access defibrillation is seen as one of the key strategies in the chain-of-survival for OHCA. Positioning of these devices is important for the maximization of public health outcomes. The literature strongly advocates widespread public access to automated external defibrillatiors (AEDs). The most efficient placement of AEDs within individual communities remains unclear.

A retrospective case review of OHCAs attended by the South Australia Ambulance Service in metropolitan and rural South Australia over a 30-month period was performed. Data were analyzed using Utstein-type indicators. Detailed demographics, summative data, and clinical data were recorded.

A total of 1,305 cases of cardiac arrest were reviewed. The annual rate of OHCA was 35 per 100,000 population. Of the cases, the mean value for the ages was 66.3 years, 517 (39.6%) were transported to hospital, 761 (58.3%) were judged by the paramedic to be cardiac, and 838 (64.2%) were witnessed. Bystander cardiopulmonary resuscitation (CPR) was performed in 495 (37.9%) of cases. The rhythm on arrival was ventricular fibrillation (VF) or ventricular tachycardia (VT) in 419 (32.1%) cases, and 315 (24.1%) of all arrests had return of spontaneous circulation (ROSC) before or on arrival at the hospital. For cardiac arrest cases that were witnessed by the ambulance service (n = 121), the incidence of ROSC was 47.1%.

During the 30-month period, there only was one location that recorded more than one cardiac arrest. No other location recorded recurrent episodes.

This study did not identify any specific location that would justify defibrillator placement over any other location without an existing defibrillator. The impact of bystander CPR and the relatively low rate of bystander CPR in this study points to an area of need. The relative potential impact of increasing bystander CPR rates versus investing in defibrillators in the community is worthy of further consideration.