The experience of family caregivers of ventilator-assisted individuals who participated in a pilot web-based peer support program: A qualitative study.

Introduction: Family caregivers play an important role supporting the day-to-day needs of ventilator-assisted individuals (VAIs) living at home. Peer-to-peer communication can help support these caregivers and help them sustain caregiving in the community. Online peer-support has been suggested as a way to help meet caregivers' support needs. Methods: A qualitative descriptive approach was used to elicit the perspectives of support received from caregivers who participated in a pilot web-based peer support program from October to December 2018. Data were collected through the transcripts of weekly online peer-to-peer group chats. Data were analyzed using an integration of thematic and framework analysis. Results: In total, eight caregivers and five peer mentors participated in the pilot. All five mentors and four of the caregivers participated in the weekly chats. We identified three themes, a) The experience of caregivers is characterized by unique challenges related to the complexity of VAI care including technology; b) Mentors and caregiver participants reciprocally share support; c) Despite hardships, there are things that make caregiving easier and joyful. Discussion: Our results add to the growing body of evidence pointing to the importance of online communities for supporting vulnerable caregivers. The reciprocal element of peer support, where trained mentors and untrained participants both benefit from support, can help sustain peer-support interventions. Despite the challenges of providing care to a VAI, there are facilitators that may help ease the caregiving experience and caregivers can benefit from ongoing support that is tailored to their needs along the caregiving trajectory.

Health Care Use, Costs, and Survival Trajectory of Home Mechanical Insufflation-Exsufflation.

BACKGROUND: Despite expert recommendations for use, limited evidence identifies effectiveness of mechanical insufflation-exsufflation (MI-E) in addressing respiratory morbidity and resultant health care utilization and costs for individuals with neuromuscular disorders. We examined the impact of provision of publicly funded MI-E devices on health care utilization, health care costs, and survival trajectory. METHODS: This is a retrospective pre/post cohort study linking data on prospectively recruited participants using MI-E to health administrative databases to quantify outcomes. RESULTS: We linked data from 106 participants (8 age < 15 y) and determined annualized health care use pre/post device. We found no difference in emergency department (ED) visit or hospital admission rates. Following MI-E approval, participants required fewer hospital days (median [interquartile range] [IQR]) 0 [0-9] vs 0 [0-4], P = .03). Rates of physician specialist visits also decreased (median IQR 7 [4-11] vs 4 [2-7], P < .001). Conversely, rates of home care nursing and homemaking/personal support visits increased. Following MI-E, total costs were lower for 59.4%, not different for 13.2%, and higher for 27.4%. Physician billing costs decreased whereas home care costs increased. Regression modeling identified pre-MI-E costs were the most important predictor of costs after approval. At 12 months, 23 (21.7%) participants had died. Risk of death was higher for those using more medical devices (hazard ratio 1.12, [95% CI 1.02-1.22]) in the home. CONCLUSIONS: Provision of publicly funded MI-E devices did not influence rates of ED visits or hospital admission but did shift health care utilization and costs from the acute care to community sector. Although increased community costs negated cost savings from physician billings, evidence suggests costs savings from reduced hospital days and fewer specialist visits. Risk of death was highest in individuals requiring multiple medical technologies.

Education Experiences of Adult Subjects and Caregivers for Mechanical Insufflation-Exsufflation at Home.

BACKGROUND: In 2014, the Ministry of Health of Ontario, Canada, approved a program of public funding for specialist-prescribed mechanical insufflation-exsufflation (MI-E) devices for home use by individuals with neuromuscular respiratory insufficiency. Since 2014, 1,926 MI-E devices have been provided, exceeding device-use projections. Few studies describe the initial and ongoing education and support needs of home MI-E users and their family caregivers. This study aimed to explore the requirements of initial and ongoing education and support for MI-E device use, user confidence, and barriers and facilitators to home MI-E. METHODS: We conducted semi-structured interviews with new (< 6 months) and established (6-48 months) MI-E users and family caregivers. Device users rated their confidence on a numeric rating scale of 1 (not confident) to 10 (very confident). RESULTS: We recruited 14 new and 14 established MI-E users and caregivers (including 9 dyads), and we conducted 28 interviews. Both new and established users were highly confident in use of MI-E (mean ± SD scores were 8.8 ± 1.2 and 8.3 ± 2.1, respectively). Overall, the subjects were satisfied with their initial education, which consisted of a 1-2 h one-on-one session at home or in the clinic with a device demonstration and hands-on practice. Subjects viewed hands-on practice and teaching of caregivers as more beneficial than written materials. Ongoing support for device use was variable. Most subjects indicated a lack of specific follow-up, which resulted in uncertainty about whether they were using the MI-E device correctly or whether MI-E was effective. Facilitators to device utilization were ease of use, initial training, support from formal or informal caregivers, and symptom relief. Barriers were inadequate education on MI-E purpose, technique, and benefit; lack of follow-up; and inadequate knowledge of MI-E by nonspecialist health providers. CONCLUSIONS: The current model of home MI-E education at initiation meets user and caregiver needs. Better ongoing education and follow-up are needed to sustain the benefits through assessment of MI-E technique and its effectiveness.

Trends in incidence, prevalence, and mortality of neuromuscular disease in Ontario, Canada: A population-based retrospective cohort study (2003-2014).

BACKGROUND: Population trends of disease prevalence and incidence over time measure burden of disease and inform healthcare planning. Neuromuscular disorders (NMD) affect muscle and nerve function with varying degrees of severity and disease progression. OBJECTIVE: Using health administrative databases we described trends in incidence, prevalence, and mortality of adults and children with NMD. We also explored place of death and use of palliative care. METHODS: Population-based (Ontario, Canada) cohort study (2003 to 2014) of adults and children with NMD identified using International Classification of Disease and health insurance billing codes within administrative health databases. RESULTS: Adult disease prevalence increased on average per year by 8% (95% confidence interval (CI) 6% to 10%, P <.001), with the largest increase in adults18-39 years. Childhood disease prevalence increased by 10% (95% CI 8% to 11%, P <.0001) per year, with the largest increase in children 0 to 5 years. Prevalence increased across all diagnoses except amyotrophic lateral sclerosis and spinal muscular atrophy for adults and all diagnoses for children. Adult incidence decreased by 3% (95% CI -4% to -2%, P <.0001) but incidence remained stable in children. Death occurred in 34,336 (18.5%) adults; 21,236 (61.8%) of whom received palliative care. Death occurred in 1,009 (5.6%) children; 507 (50.2%) of whom received palliative care. Mortality decreased over time in adults (odds ratio (OR) 0.86, 95% CI 0.86-0.87, P <.0001) and children (OR 0.79, 95% CI 0.76-0.82, P <.0001). Use of palliative care over time increased for adults (OR 1.18, 95% CI 1.09 to 1.28, P <.0001) and children (OR 1.22, 95% CI 1.20 to 1.23, P <.0001). CONCLUSIONS: In both adults and children, NMD prevalence is rising and mortality rates are declining. In adults incidence is decreasing while in children it remains stable. This confirms on a population-based level the increased survival of children and adults with NMD.

Monitoring Cough Effectiveness and Use of Airway Clearance Strategies: A Canadian and UK Survey.

BACKGROUND: Regular monitoring combined with early and appropriate use of airway clearance can reduce unplanned hospital admissions for patients with neuromuscular disease (NMD) and spinal cord injury (SCI). We aimed to describe and compare knowledge of guidelines, monitoring of cough effectiveness, clinician prescription/provision of airway clearance strategies, and service provision constraints in the United Kingdom and Canada. METHODS: This was a cross-sectional survey of clinicians affiliated with NMD and SCI clinics in Canada, 2016 attendees at the Home Mechanical Ventilation Conference in the United Kingdom, and United Kingdom physiotherapist networks. RESULTS: We received 155 surveys (92 from Canada; 63 from the United Kingdom). More UK respondents (76%) were aware of airway clearance guidelines than Canadian (56%) respondents (P = .02). Routine assessment of cough effectiveness was reported by more UK respondents (59%) than Canadian (42%) respondents (P = .044). Cough peak flow (CPF) was the most common method used in both countries, although it was more commonly used in the UK (96%) than in Canada (81%, P = .02). Fewer Canadian respondents reported using CPF before initiation of airway clearance (81% vs 94%, P = .046), and fewer Canadian respondents showed results to patients for technique feedback (76% vs 97%, P = .007). Similar participant numbers reported using CPF after initiation to ensure adequate technique (73% vs 72%, P = .92). Mechanical insufflation-exsufflation (MI-E) + lung volume recruitment (LVR) + manually assisted cough when CPF ≤ 270 L/min was most routinely recommended (41% overall). Monotherapy was infrequent (LVR 15%, manually assisted cough 7%, and MI-E 4%). More Canadians identified constraints on service provision, specifically insufficient public funding for equipment (68% vs 39%, P = .002) and inadequate community workers' knowledge (56% vs 34%, P = .002). Funding for community support was a common constraint in both countries (49% vs 42%). CONCLUSIONS: The somewhat variable cough effectiveness monitoring and airway clearance practices identified in this survey confirm the need for further work on knowledge translation related to guideline recommendations and the need to address common constraints to optimal service delivery.

Respiratory health service utilization of children with neuromuscular disease.

OBJECTIVES: To quantify health service utilization including assessment, monitoring, and treatment of respiratory complications of children with neuromuscular disease (NMD), identifying practice variation and adherence to guideline recommendations at a population level. METHODS: North American population-based cohort study (2003-2015) of children with NMD using hospital diagnostic and physician billing codes within health administrative databases. RESULTS: We identified 18 163 children with NMD. Mean (SD) age was 7.8 (5.6) years with 40% ≤5; 45% were female. Most common diagnoses were cerebral palsy (50%) and spina bifida (16%); 8% had muscular dystrophy. From fiscal years 2003-2014, 15 600 (86%) children went to an emergency department on average 3.5 times every 3 years; 6575 (36%) for respiratory reasons. 8788 (48%) were admitted to hospital with 2190 (12%) for respiratory reasons and 2451 (13%) required intensive care. Respiratory specialist outpatient visits occurred for 2226 (12%) children on average 6.5 visits every 3 years; 723 (4%) had in-hospital respiratory specialist consultation. Pulmonary function testing was conducted in 3194 (18%) children on average 2.4 times every 3 years; sleep studies in 1389 (8%). CONCLUSION: In this population-based study of children with NMD, healthcare utilization for respiratory complications was considerable. Frequency of respiratory specialist consultation, monitoring of respiratory function and sleep disordered breathing was variable but on average reflected professional society recommendations. Children with NMD are frequent ED users suggesting a need to improve community and social supports. We did not detect reduced access to respiratory monitoring or specialist consultation in adolescents transitioning to adult services.

Patterns of healthcare utilisation for respiratory complications of adults with neuromuscular disease: a population study.

Our objective was to quantify health service utilisation including monitoring and treatment of respiratory complications for adults with neuromuscular disease (NMD), identifying practice variation and adherence to guideline recommendations at a population level.We conducted a population-based longitudinal cohort study (2003-2015) of adults with NMD using hospital diagnostic and health insurance billing codes within administrative health databases.We identified 185 586 adults with NMD. Mean age 52 years, 59% female. 41 173 (22%) went to an emergency department for respiratory complications on average 1.6 times every 3 years; 14 947 (8%) individuals were admitted to hospital 1.4 times every 3 years. Outpatient respiratory specialist visits occurred for 64 084 (35%) with four visits every 3 years, although substantial variation in visit frequency was found. 157 285 (85%) went to the emergency department (all-cause) almost 4 times every 3 years, 100 052 (54%) were admitted to hospital. Individuals with amyotrophic lateral sclerosis/motor neurone disease (ALS/MND) had more emergency department visits compared with other types of NMD (p<0.0001).One-third of adults with NMD received respiratory specialist care at a frequency recommended by professional guidelines, although substantial variation exists. Emergent healthcare utilisation was substantial, emphasising the burden of NMD on the healthcare system and urgent need to improve community and social supports, particularly for ALS/MND patients.

Physician-assisted death: A Canada-wide survey of ALS health care providers.

OBJECTIVE: To survey amyotrophic lateral sclerosis (ALS) health care providers to determine attitudes regarding physician-assisted death (PAD) after the Supreme Court of Canada (SCC) invalidated the Criminal Code provisions that prohibit PAD in February 2015. METHODS: We conducted a Canada-wide survey of physicians and allied health professionals (AHP) involved in the care of patients with ALS on their opinions regarding (1) the SCC ruling, (2) their willingness to participate in PAD, and (3) the PAD implementation process for patients with ALS. RESULTS: We received 231 responses from ALS health care providers representing all 15 academic ALS centers in Canada, with an overall response rate for invited participants of 74%. The majority of physicians and AHP agreed with the SCC ruling and believed that patients with moderate and severe stage ALS should have access to PAD; however, most physicians would not provide a lethal prescription or injection to an eligible patient. They preferred the patient obtain a second opinion to confirm eligibility, have a psychiatric assessment, and then be referred to a third party to administer PAD. The majority of respondents felt unprepared for the initiation of this program and favored the development of PAD training modules and guidelines. CONCLUSIONS: ALS health care providers support the SCC decision and the majority believe PAD should be available to patients with moderate to severe ALS with physical or emotional suffering. However, few clinicians are willing to directly provide PAD and additional training and guidelines are required before implementation in Canada.

Mortality associated with periodic limb movements during sleep in amyotrophic lateral sclerosis patients / Mortalidade associada aos movimentos periódicos de membros durante o sono em pacientes com esclerose lateral amiotrófica

OBJECTIVE: To describe the prevalence and severity of periodic limb movements during sleep in amyotrophic lateral sclerosis patients and to explore this fact as a predictor of severity of the condition with respect to mortality. METHODS: In this case-control study, questionnaire and polysomnographic data were analyzed from 35 amyotrophic lateral sclerosis patients. Controls were matched by age, genre, and body mass index. A Kaplan-Meier curve was used to compare the survival time of patients with periodic limb movements of sleep index below or above 5. RESULTS: The number of amyotrophic lateral sclerosis patients with an index greater than five was higher than controls (19 (53%) versus 4 (11%); p<0.0001), and the mean index was higher (23.55±40.07 versus 3.28±8.96; p=0.0009). Earlier mortality was more common in patients with more than five periodic limb movements per hour of sleep than patients with less than five periodic limb movements per hour of sleep (7/19 (37%) versus 1/16 (6%); p=0.04) in this group of patients that had a mean survival of 33 months. CONCLUSIONS: There were more periodic limb movements of sleep in amyotrophic lateral sclerosis patients than in the control population. The higher number of these movements in amyotrophic lateral sclerosis patients correlates with disease severity and may suggest poor survival.

OBJETIVO:Descrever a prevalência e a severidade dos movimentos periódicos de membros durante o sono nos pacientes com esclerose lateral amiotrófica e explorar isso como um preditor de severidade da doença e mortalidade. MÉTODOS: Estudo caso controle em que foram analisados 35 pacientes por questionários e polissonografia. Os controles foram pareados por idade, gênero, e índice de massa corporal. Uma curva de Kaplan-Meier foi usada para comparar o tempo de sobrevida em pacientes com índice de movimento periódico de membros durante o sono acima e abaixo de 5. RESULTADOS: O número de pacientes com esclerose lateral amiotrófica com índice de movimentos periódicos de membros durante o sono acima de cinco foi maior do que os controles (19 (53%) versus 4 (11%); p<0,0001) e a média do índice de movimentos periódicos de membros durante o sono também foi maior no grupo dos pacientes (23,55±40,07 versus 3,28±8,96; p=0,0009). A mortalidade precoce foi mais comum em pacientes com mais que cinco movimentos durante o sono por hora do que pacientes com menos do que cinco movimentos durante o sono por hora (7/19 (37%) versus 1/16 (6%); p=0,04). Nesse grupo, os pacientes tiveram sobrevida média de 33 meses. CONCLUSÃO:Houve um maior número de movimentos periódicos de membros durante o sono em pacientes com esclerose lateral amiotrófica do que na população controle. O maior número de movimentos periódicos de membros durante o sono em pacientes com esclerose lateral amiotrófica foi correlacionado com severidade da doença e pode sugerir menor sobrevida.

Mortality associated with periodic limb movements during sleep in amyotrophic lateral sclerosis patients.

OBJECTIVE: To describe the prevalence and severity of periodic limb movements during sleep in amyotrophic lateral sclerosis patients and to explore this fact as a predictor of severity of the condition with respect to mortality. METHODS: In this case-control study, questionnaire and polysomnographic data were analyzed from 35 amyotrophic lateral sclerosis patients. Controls were matched by age, genre, and body mass index. A Kaplan-Meier curve was used to compare the survival time of patients with periodic limb movements of sleep index below or above 5. RESULTS: The number of amyotrophic lateral sclerosis patients with an index greater than five was higher than controls (19 (53%) versus 4 (11%); p < 0.0001), and the mean index was higher (23.55 ± 40.07 versus 3.28 ± 8.96; p = 0.0009). Earlier mortality was more common in patients with more than five periodic limb movements per hour of sleep than patients with less than five periodic limb movements per hour of sleep (7/19 (37%) versus 1/16 (6%); p = 0.04) in this group of patients that had a mean survival of 33 months. CONCLUSIONS: There were more periodic limb movements of sleep in amyotrophic lateral sclerosis patients than in the control population. The higher number of these movements in amyotrophic lateral sclerosis patients correlates with disease severity and may suggest poor survival.

The effect of lupus nephritis on pregnancy outcome and fetal and maternal complications.

OBJECTIVE: To evaluate the effect of lupus nephritis on pregnancy with respect to fetal outcome, maternal complications, and lupus activity. METHODS: All pregnancies seen between 1970 and 2003 in the Lupus Clinic were evaluated for the 3 outcomes. Renal disease was defined as the presence of nephrotic syndrome, dialysis, renal transplant, serum creatinine > 120 mmol/l, proteinuria, sterile hematuria and pyuria, or the presence of casts. Fetal complications were evaluated in pregnancies resulting in either live births or stillbirths. Generalized estimating equations were used to test for differences in outcomes between pregnancies with and without the presence of active renal disease. Repeated measures adjustments were made in the model for multiple pregnancies in the same mother. RESULTS: There were 193 pregnancies in 104 women. Of these, 81 occurred in the presence of active renal disease during the study period, defined as 6 months prior to conception until the date of pregnancy outcome. One hundred twelve pregnancies were defined as nonrenal. No statistical difference was found in pregnancy outcome. Fetal complications were not different between the 2 groups with the exception of low birth weight and congenital malformations, which were observed more frequently in the renal group. Pregnancy-induced hypertension was more frequent in pregnancies with renal disease. Lupus flares were also more likely to occur in pregnancies with renal disease compared to those without. CONCLUSION: Lupus nephritis in pregnancy does not lead to worsened pregnancy or fetal outcomes. Active renal disease, however, is associated with pregnancy-induced hypertension, as well as a flare of lupus activity during pregnancy.

The effect of pregnancy on lupus nephritis.

OBJECTIVE: To evaluate the effect of pregnancy on lupus nephritis with respect to renal activity and renal deterioration. METHODS: Seventy-eight pregnancies occurred in 53 women with systemic lupus erythematosus (SLE) and renal disease. Seventy-eight nonpregnant SLE patients with evidence of renal disease were matched to the study population by age at the time of each pregnancy and by the presence of a renal manifestation at the beginning of the study. The nonpregnant controls were seen within 2 years of the assessment dates of the pregnant patients with whom they were matched. Renal activity was defined as the presence of active urine sediment or proteinuria, and changes in these parameters were monitored throughout the study period in both study populations. Renal deterioration was defined as an increase in the serum creatinine level that was >20% above the baseline value or an increase to >120 mmoles/liter. RESULTS: Renal disease activity patterns were available for 74 pairs of pregnancies and controls. Renal disease became active during the study period in 33 pregnancies (44.6%) and 31 controls (41.9%). Serial serum creatinine levels were available for 75 study pairs, among which 62 pregnancies (82.7%) and 57 controls (76.0%) showed no deterioration. Comparison of the treatments received by both the pregnant and the nonpregnant patients showed no significant difference in the amount of steroids taken. A significantly lower amount of immunosuppressive and antimalarial agents were taken during the pregnancies. CONCLUSION: During pregnancy in patients with SLE and renal disease, changes in renal disease activity and deterioration in renal function are similar to those which occur in nonpregnant patients with lupus nephritis.