NPHSNC Stakeholder Synthesis Report

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INTERIM VERSION – NOT FOR PUBLICATION National Population Health Study of Neurological Conditions Synthesis Report to Stakeholders Interim Version: October 31, 2013 For input by NHCC Stakeholders Prepared by Garth Bray on behalf of The NPHSNC Synthesis Panel

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Transcript of NPHSNC Stakeholder Synthesis Report

Page 1: NPHSNC Stakeholder Synthesis Report

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National Population Health Study of

Neurological Conditions

Synthesis Report to Stakeholders

Interim Version: October 31, 2013

For input by NHCC Stakeholders

Prepared by Garth Bray

on behalf of

The NPHSNC Synthesis Panel

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Contents Projects and Surveys of the NPHSNC ......................................................................................iii

Glossary ..................................................................................................................................v

Acronyms ............................................................................................................................. vii

Introduction .......................................................................................................................... 1

Report Summary .................................................................................................................... 3 1. Impact of Neurological Conditions 3 2. Health Services for Neurological Conditions 4 3. Scope of Neurological Conditions 4 4. Risk Factors for Neurological Conditions 5 5. Knowledge Gaps 6

NPHSNC Project Findings........................................................................................................ 7

1. Impact ............................................................................................................................... 7 1.1 Impacts of Neurological Conditions on Affected Individuals 7 1.2 Some impacts of neurological conditions differed for men (M) and women (W) 8 1.3 Impact of Neurological Conditions on Children and their Families 9 1.4 Impacts of Neurological Conditions on Aboriginal Canadians 10 1.5 Impacts of Neurological Conditions on Informal Caregivers 10

Knowledge Gaps regarding the Impact of Neurological Conditions 11 Themes Emerging from the NPHSNC regarding Impact of Neurological Conditions 11

2. Health Services..................................................................................................................12 2.1 Individuals with neurological conditions used more health care services across the continuum of care than those with other chronic conditions. 12 2.2 Health Services Provision for Persons with Neurological Conditions 13 2.3 Costs of services for persons with NCs are greater than those without an NC at all stages of care. 14 2.4 Informal Health Care Services 15 2.5 Mental Health Service Needs of Persons with Neurological Conditions 16 2.6 Health Services for Children with Neurological Conditions 16 2.7 Health Services for Aboriginal Canadians with Neurological Conditions 16 2.8 Predicting and Evaluating Health Care Needs in Long-term Care Settings 17

Gaps in Understanding Health Services for Persons with Neurological Conditions 18 Themes Emerging from the NPHSNC regarding Health Services for Persons with NCs 19

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3. How Common are Neurological Conditions in Canada? ......................................................20 3.1 Incidence and Prevalence Estimates from Different Sources 20 3.2 More than half of the Canadians in continuing care (home care, long-term care residences) have a neurological condition as their primary diagnosis. 22 3.3 The epidemiological features of several NCs differed according to sex and age 22 3.4 Common Co-morbidities associated with Neurological Conditions 22 3.5 Surveillance Capacity Building 22

Gaps Regarding the Occurrence and Extent of Neurological Conditions in Canada 26 Research Gaps: 26 Gaps in Infrastructure: 26 Themes Emerging from the NPHSNC Regarding Scope 26

4. Risk Factors .......................................................................................................................28 4.1 Potentially Modifiable Risk Factors for Onset of Neurological Conditions 28 4.2 Identified Genetic Factors for Neurological Conditions 29 4.3 Contribution of the NPHSNC to Risks for Progression of Neurological Conditions 30

Knowledge Gaps regarding Risk Factors for Neurological Condition 31 Themes emerging from the NPHSNC regarding Risk Factors 31

5. Microsimulation ................................................................................................................32

Tables ...................................................................................................................................33 Synthesis Report (SR) Tables 33

Appendices ...........................................................................................................................34 SR Appendix 1: Synthesis Panel Members 34 SR Appendix 2: Implementation Committee Members 36 SR Appendix 3: Synthesis Panel Comments on Risk Factors for Neurological Conditions 37

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Projects and Surveys of the NPHSNC Projects and surveys in this report are identified by a number (assigned by the order in which each was first mentioned in the text) or its short title.

[1] Nathalie Jetté, Tamara Pringsheim: Understanding the Epidemiology of Neurological Conditions and Building the Methodological Foundation for Surveillance.

Short title: Jetté-Pringsheim Systematic Reviews Project.

[2] Claudia Lagacé et al, PHAC: Canadian Community Health Surveys-2010-2011. Short title: CCHS-2010, 2011

[3] Kim Reimer: Neurological Conditions in British Columbia. Short title: Reimer BC Administrative Data Project.

[4] Karen Tu, Liisa Jaakkimainen, Debra Butt: Validation of Administrative Data Algorithms to determine Population Prevalence and Incidence of Alzheimer's disease and other dementias, multiple sclerosis, epilepsy and Parkinson's disease (parkinsonism).

Short title: Tu-Jaakkimainen-Butt Ontario Administrative Data Project.

[5] Neil Drummond, Richard Birtwhistle: Canadian Primary Care Sentinel Surveillance Network - Neurodegenerative Conditions.

Short title: Drummond-Birtwhistle EMR Project.

[6] Claudia Lagacé et al, PHAC: Survey on Neurological Conditions in Institutions in Canada. Short title: SNCIC, 2011-2012.

[7] John Hirdes, Colleen Maxwell, Nathalie Jetté: Innovations in Data, Evidence and Applications for Persons with Neurological Conditions (ideas PNC).

Short title: Hirdes Chronic Care Resident Assessment Project.

[8] Christina Wolfson, Parminder Raina: Canadian Longitudinal Study on Aging: Neurological Conditions Initiative (CLSA-NCI).

Short title: Wolfson-Raina CLSA-NCs Project.

[9] Lawrence Korngut: Neurological Registry Best Practice Guidelines and Implementation Toolkit. Short title: Korngut Registry Guidelines Project.

[10] Claudia Lagacé et al, PHAC: Survey on Living with a Neurological Condition in Canada. Short title: SLNCC, 2011-2012.

[11] Joan Versnel, Tanya Packer: The Everyday Experience of Living With and Managing a Neurological Condition (LINC).

Short title: Versnel-Packer LINC Project.

[12] Carrie Bourassa: Understanding from Within: Developing Community-Driven and Culturally Relevant Models for Understanding and Responding to Neurological Conditions among Aboriginal People.

Short Title: NWAC (Native Women's Association of Canada) Project.

[13] Daniel Krewski: Systematic Review of Factors Influencing the Onset of Neurological Conditions. Short title: Krewski Onset Risk Factors Project.

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[14] Daniel Krewski: Systematic Review of Factors Influencing the Progression of Neurological Conditions. Short title: Krewski Progression Risk Factors Project.

[15] Susan Jaglal: Use and Gaps in Health and Community-Based Services for Neurological Populations: A Systems Analysis.

Short title: Jaglal Health Services Project.

[16] Michael Shevell, Maryam Oskoui: Expansion of a Canadian Multi-Regional Population-Based Cerebral Palsy Registry.

Short title: Shevell-Oskoui Cerebral Palsy Registry Project.

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Glossary While the authors have attempted to reduce and simplify the technical language in this report, several terms are essential to understanding project outcomes.

Health Care Terms UAdministrative Health DataU: collections of information on the delivery of health care services. In Canada, the health care databases of the provincial and territorial governments collect and store information relevant to the administration of universal medical care insurance. The main sources of administrative data pertain to hospital services, physician billings, and prescription drugs. Depending on how data concerning specific health conditions are captured, they can be used for surveillance purposes, such as estimating disease incidence and prevalence.

UAlgorithmU: a step-by-step procedure for calculations (see Case Definition).

UAlternate Level of Care (ALC)U: care provided in an acute care hospital for patients deemed well enough by their care team to be cared for elsewhere. Patients receiving this level of care are usually awaiting transfer to a long-term care facility or some other form of supported living.

UCaregivers - informal and formalU: as used throughout the NPHSNC project and its reports, the term informal care refers to care provided by family, friends and neighbours while formal care refers to home care providers or health care professionals.

UCase definitionU: the method by which the subjects of a condition under investigation are identified for the surveillance of public health conditions. When case-definitions require more complex decision-rules than simply equating a diagnosis with one or more administrative data code(s), the case definitions are sometimes referred to as algorithms. Examples of such algorithms in this report are the case definitions that can be fulfilled by more than one condition; e.g. a case definition that requires one hospital admission for a condition or (alternatively) three or more physician codes during a specified time interval.

UContinuing CareU: health, social and support services, including respite and palliative care, offered on a prolonged basis through home care programs or in long-term care facilities such as nursing homes.

UCo-morbidityU: traditionally referred to as diseases, disorders or conditions that occurred at the same time as another disorder but were not related to it. As understanding of the biology of specific diseases has advanced, the meaning of some associations has changed. For example, mood disorder, once considered a co-morbidity of Parkinson's disease (as well as several other NCs), is now interpreted as a possible early manifestation of these conditionsP0F

1,1F

2P. In this report, the term is used in both its traditional

sense (e.g. the SLNCC report) as well as more broadly as "other diagnoses" recorded in databases along with targeted neurological conditions (e.g. the Reimer BC Administrative Data Project).

UHealth Utilities Index (HUI) scoresU: a set of weights that quantify the “utility” (perceived value, under conditions of uncertainty) of various health states, based on a survey of what people in the population (not necessarily with any health condition) say they would prefer in terms of health states, each relative to the othersP2F

3,3F

4P. The HUI assesses 8 attributes of health status - vision, hearing, speech, ambulation,

1 Schuurman, A.G. et al., Increased risk of Parkinson's disease after depression: a retrospective cohort study. Neurology 58:1501-1504, 2002. 2 Leentjens, A.F. et al., Higher incidence of depression preceding the onset of Parkinson's disease: a register study. Mov. Disord. 18:414-418,

2003. 3 Feeny, D. Approach to the Assessment of Health-related Quality of Life, Medical Care, 2000; 9: II 151-II 154.

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dexterity, emotion, cognition and pain with 5 or 6 levels per attribute ranging from normal to severely impaired.

UInternational Classification of Disease (ICD )U: a health care classification system that assigns diagnostic codes to diseases. The tenth revision of the ICD, known as 35TICD-1035T, is currently used to code data on hospital separations (discharge, transfers and death) in all provinces and territories of Canada. The ICD-9 is still used as the basis for physician billing codes in many jurisdictions.

UPatient RegistriesU: collections of data related to patients with a specific diagnosis or condition. When appropriately managed, registries are able to follow many patients across large geographical areas to obtain epidemiological data, assessments of disease burden and evaluation of therapeutic measures.

Statistical Terms UConfidence interval (CI)U: a statistical measurement of the reliability of an estimate. Narrow confidence intervals indicate greater reliability than those that are wide. The 95% confidence intervals used in this report indicate the ranges of values that are likely to include the true rate 19 times out of 20.

UEstimateU: an approximation of the true value of a characteristic of a population. As used in this report, estimates derived from a sample of the entire population, represent the best available information.

UIncidenceU: the number of new cases of a disease or condition occurring in a given time period. Incidence can be expressed as Uincidence proportionU (the number of new cases within a specified time period divided by the size of the population initially at risk) or Uincidence rateU (the number of new cases per population in a given time period, expressed as new cases per number of person-years).

45TUMeta-analysisU45T: the statistical synthesis of data from several studies that address a similar question.

UPrevalenceU: in this report, prevalence is expressed as the number of individuals with a neurological condition per 100,000. Prevalence is a better measure of the burden of the disease on society while incidence is more useful in understanding the cause of a disease.

UQualitative synthesisU: the drawing together of the findings of individual studies of systematic searches for research on a topic without doing a statistical procedure such as meta-analysis.

URangeU: the difference between the largest and smallest value of a group of observations

URisks and Risk FactorsU: risk is the chance that an event will occur and is usually expressed as a proportion or frequency with a value between 0 (no risk) and 1 (definite risk). As used in relation to health and disease, a risk factor is a variable (e.g. a behaviour, condition, or exposure) that affects the risk of disease occurrence. (See Appendix 4.)

USurveillanceU: as used in public health, surveillance is the ongoing systematic collection, analysis, and interpretation of data on population health that is used by ministries of health to develop health-care policies.

USystematic ReviewU: a critical assessment and evaluation of all research studies that address a particular clinical issue. A systematic review may also include a quantitative pooling of data, called a meta-analysis.P4F

5

4 Feeny, D. et al. Medical Care, 2002; 40: 113-128. 5 Source: Agency of Healthcare Research and Quality, U.S. Department of Health and Human Services, website accessed 2013-Sep-24.

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Acronyms AD/dementia Alzheimer’s Disease and other dementias

ALC Alternate Level of Care ALS Amyotrophic lateral sclerosis (ALS) CCDSS Canadian Chronic Disease Surveillance System CCHS Canadian Community Health Survey CI Confidence Interval CLSA Canadian Longitudinal Study of Aging CP Cerebral Palsy CPCSSN Canadian Primary Care Sentinel Surveillance Network HD Huntington’s Disease HUI Health Utilities Index interRAI International Resident Assessment Instrument LINC The Everyday Experience of Living With and Managing a Neurological Condition M Men MD Muscular Dystrophy MS Multiple Sclerosis NC Neurological Condition NHCC Neurological Health Charities Canada NPHSNC National Population Health Study of Neurological Conditions NWAC Native Women’s Association of Canada PD Parkinson’s Disease PHAC Public Health Agency of Canada PI Principal Investigator RAI Resident Assessment Instrument – see “interRAI” SAC Scientific Advisory Committee SCI Spinal Cord Injury SLNCC Survey on Living with Neurological Conditions in Canada SNCIC Survey of Neurological Conditions in Institutions in Canada SP Synthesis Panel SR Synthesis Report SOD Superoxide dismutase TBI Traumatic Brain Injury TS Tourette Syndrome W Women

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Introduction The National Population Health Study of Neurological Conditions (NPHSNC)P

5F

6P was undertaken jointly by

Neurological Health Charities Canada (NHCC) and the Public Health Agency of Canada (PHAC) in 2009 to document the extent of targeted neurological conditionsP6F

7P (NCs) in Canada as well as their impact on

affected individuals, families and the health care system.

Neurological diseases, disorders and injuries are major causes of disability in Canada. The symptoms of these conditions are paralyzed or weakened muscles, impaired coordination, loss of sensation, seizures, confusion, pain, and altered memory and capacity to think. As a result, Canadians living with neurological conditions face many long-term challenges with reduced ability to function and activity limitations. Because the incidence of some of the more common neurological conditions increases with age, the number of people facing these challenges is expected to rise as Canada’s population ages. Consequently, the direct and indirect costs associated with these conditions will also increase over the next 20 years.

Within the ultimate goal of supporting efforts aimed at reducing the burden of NCs, the pan-Canadian NPHSNC investigated these conditions in terms of four focus areas:

• their impact on affected individuals, their families, caregivers and communities, • related health services needs, • how common NCs are and how frequently they occur in Canada, and • risk factors for the onset and progression of these conditions.

The increased understanding of the broad range of effects of NCs in these four areas is expected to assist governments, healthcare providers and neurological health charities in developing policies and strategies to meet the health and social needs of affected Canadians and their families.

The NPHSNC used a variety of approaches to achieve its goals in the four focus areas: 13 external projects funded through contribution agreements, three national surveys conducted by Statistics Canada, and an expansion of the PHAC's Chronic Disease Surveillance System (CCDSS) to gather provincial and territorial administrative data on NCs. Continuing efforts include a model that will project the economic and human impacts of NCs over the next 5, 10, 15 and 20 years.

This report describes the initial results that emerged from this work. These findings have undergone a comprehensive review process involving several meetings with both the Scientific Advisory Committee for the project and a Synthesis Panel (Appendix 1). The Synthesis Panel reviewed the Scientific Advisory Committee report and identified the key themes and gaps outlined in this report.

Stakeholders were consulted by the NHCC during the planning stages of the NPHSNC and their involvement continued during the Study through their NHCC representatives. As the Study nears its completion, the neurological health charities and individual stakeholders are being consulted for their feedback and to ensure that the final report on the NPHSNC reflects the perceptions, needs and experiences of those affected by NCs. The final version of the report will be reviewed by the project Implementation Committee (Appendix 2) prior to its release by NHCC and the PHAC. It is anticipated that

6 For additional background information about the Study and its findings, please visit the Neurological Health Charities Canada Website: http://www.mybrainmatters.ca/ or the Public Health Agency of Canada website: http://recherche-search.gc.ca/rGs/s_r?st=s&s5bm3ts21rch=x&num=10&st1rt=0&langs=eng&cdn=phac&q=NPHSNC

7 Targeted initially: Amyotrophic lateral sclerosis (ALS), Alzheimer's disease and other dementias (AD/dementia), brain tumour, cerebral palsy, dystonia, epilepsy, Huntington's disease, hydrocephalus, multiple sclerosis, muscular dystrophy, Parkinson's disease, spina bifida, spinal cord injury, traumatic brain injury, Tourette syndrome. Some projects also investigated migraine, spinal cord tumour, and stroke.

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NHCC and its stakeholders will use this report, as well as the more detailed data in the final report of the NPHSNC Scientific Advisory Committee, to support the development of a National Brain Strategy for Canada.

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Report Summary The projects and surveys of the NPHSNC were jointly planned and managed by Neurological Health Charities Canada (NHCC) and the Public Health Agency of Canada (PHAC) between 2009 and 2013. The long-range goal of this project was to support efforts to reduce the burden of neurological conditions (NCs) in Canada.

A Scientific Advisory Committee reviewed the final reports of the 13 external projects carried out in universities or government departments across Canada as well as the 3 national surveys conducted by Statistics Canada. Subsequently a Synthesis PanelP7F

8P, identified the following key findings for the four

focus areas of the Study.

1. Impact of Neurological Conditions NPHSNC projects documented the diverse and debilitating impacts of NCs on the lives and well-being of affected individuals, their families, informal caregivers and communities. Considered collectively, individuals with NCs reported [10,11]P

8F

9P that these impacts:

a. Affected their general health, mental health, financial security as well as their ability to work and participate in other activities;

b. Produced feelings of stigmatization;

c. Included a range of functional impairments that affected their memory, thinking ability, speech, vision, hearing, hand/finger dexterity, mobility, bladder and bowel control to varying extents;

d. Resulted in pain and discomfort.

Other findings on the impact of NCs:

e. The severity of global disability (as measured by the Health Utilities Index - HUI score) due to migraine, multiple sclerosis, and stroke was worse in men while for AD/dementia the score was worse in women [10].

f. Children with NCs had impairments of quality of life and limited educational opportunities [11]. Caring for a child with an NC affected the parents' general and mental health; 35% of parents reported that the impact of the child's illness on their family was "extreme" [11].

g. In addition to the impacts of NCs experienced by other Canadians, Aboriginal Canadians described unique impacts related to culture and spirituality as well as disparities in their social determinants of health [12].

h. Informal caregivers, who are often family members, provide extensive services for individuals in continuing care (home care and institutions). The levels of distress experienced by NC caregivers were greater than that of non-NC caregivers, particularly if the NC manifested cognitive impairment or behavioral issues [7].

8 The members of the Synthesis Panel are listed in Appendix 4. 9 The number in square brackets refers to the projects listed on pages iii, iv.

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2. Health Services for Neurological Conditions Health services for those with NCs were analyzed from several perspectives in different NPHSNC projects.

a. Individuals with NCs were shown to use more health care services than those with other chronic conditions whether they: were living in the community [10,11], acute care hospitals [3], alternate levels of care in acute hospitals [7], or nursing homes [3,7]; or were receiving formal home care [7] or the services of physicians [3] and other health care professionals [7].

b. A literature review and interviews with key informants [15] identified two main deficiencies among service providers: (i) a lack of knowledge about NCs, and (ii) the limited availability of services for education, employment, caregiver support, housing and transportation. These deficiencies were accentuated for those living in rural areas.

c. Although a high proportion of care providers offered services for patients with more common NCs (stroke, Parkinson’s disease, Alzheimer’s disease, traumatic brain injury, and multiple sclerosis), only a small proportion reported services for individuals with less common conditions such as dystonia, Tourette syndrome and Rett syndrome [15].

d. Most service providers had exclusion criteria, particularly for patients with psychiatric diagnoses, severe behavioural disorders, or substance abuse/dependence [15].

e. Patients rated the quality of a spectrum of health services lower than did the service providers.

f. Costs of services for persons with NCs are greater than those without an NC in both acute [3] and continuing care [7].

g. Out-of-pocket expenses, particularly for medications (including co-payments), were more than $1000/year for many NC patients [6,11].

h. Several projects documented the mental health service needs of individuals with NCs [2,6,11] but a survey of service providers indicated that these needs may not be being met [15].

i. Aboriginal Canadians cited many health services challenges including ambiguity about which level of government was responsible for care provision; the need for better cultural competence among care providers; and the need for Aboriginal people to understand the stigma associated with NCs.

3. Scope of Neurological Conditions Estimates of the prevalence and incidence of NCs can be determined from a variety of sources, each of which has strengths and weaknesses. While recognizing these limitations, the Synthesis Panel has highlighted the following observations.

a. The Usystematic reviewsU of the literature [1] produced estimates of prevalence and incidence for many of the targeted NCs. These estimates were used to compare data from other NPHSNC projects. Few Canadian studies of prevalence or incidence were identified.

b. Prevalence estimates derived from Uadministrative dataU [3,4] were statistically closest to the estimates of the systematic reviews for ALS, cerebral palsy, Huntington's disease, hydrocephalus, and Parkinson's disease. However, for the other targeted conditions, prevalence estimates from this source were outside the 95% CI, although some were within the ranges of the studies subjected to meta-analysis.

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c. The Uself-report surveysU [2,8] produced estimates of prevalence that were within or close to the 95% CI of the systematic reviews for dystonia, hydrocephalus (infants and children), and Parkinson's disease but only within the ranges of the studies of the systematic reviews for 7 other NCs and outside the ranges of the studies of the systematic reviews for 5 NCs, including AD/dementia. This latter finding underscores the need for caution when interpreting some data from self- or proxy-report surveys.

d. Incidence estimates available from one Uadministrative dataUbase [3] were within or close to the 95% CI of the systematic reviews for AD/dementia, ALS, brain tumours, Huntington's disease, Parkinson's disease, and spinal cord injury (pre-hospital mortality excluded), within the ranges of the studies of the systematic reviews for 3 NCs, and outside these ranges for 3 others. (Due to missing or incomplete data, comparisons were not possible for cerebral palsy, dystonia, migraine, muscular dystrophy, spina bifida, spinal cord tumour, stroke, and Tourette syndrome.)

e. The prevalence estimates of three NCs (dementia, epilepsy and parkinsonism) derived from the Uelectronic medical recordsU (EMRs) of a network of family physicians [5] were within or close to the 95% CI of the estimates of the systematic reviews.

f. Other main findings pertaining to the scope of NCs:

• More than half of the Canadians in continuing care (home care, nursing homes) had a NC as their primary diagnosis [7].

• The epidemiological features of several NCs differ according to sex and age, findings that can have major impacts on affected individuals and society [2].

• Heart disease, diabetes mellitus, hypertension, and mood disorder were common co-morbidities for the overall population with an NC [10].

• Several projects contributed to the development of a surveillance system for NCs in Canada by PHAC.

4. Risk Factors for Neurological Conditions a. The Krewski research team [13] identified many factors associated with the onset of 14 NCs. These

associations, which fulfilled criteria for statistical significance, are currently being investigated by the research team to determine their impact on the Canadian population as well as their clinical relevance.

b. Because they could be the basis for prevention strategies, the potentially modifiable associations identified as risk factors were highlighted by the Synthesis Panel. Environmental factors and maternal or prenatal health issues were potential risk factors for several NCs.

c. The risk factors project [13] also tabulated NCs caused by mutations of single genes that are known causes of certain NCs (e.g. Huntington's disease, muscular dystrophy) as well as susceptibility genes whose relationship to disease causation is more complex.

d. The determination of risk factors for progression of NCs is difficult to carry out. To be meaningful, these studies need to follow the course of individuals at risk of, or affected by, the NC over many years.

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5. Knowledge Gaps Several key knowledge gaps became obvious when synthesizing the project reports:

a. More and better information on impact and extent of NCs for:

• the less prevalent conditions such as ALS, dystonia and Huntington's disease, • children, • Aboriginal Canadians (First Nations, Métis, Inuit), • members of the Armed Forces, • new immigrants, • the homeless, and • those who are incarcerated;

b. Information on the impact of NCs on formal caregivers of individuals with severe, debilitating NCs;

c. The need to express potential risk factors in ways that relate their impact to the overall Canadian population as well as their relative risk (see Appendix 3), and evaluations of the clinical or public health relevance of statistically-validated risk factors by condition-specific experts;

d. Health care costs for all jurisdictions regarding direct and indirect costs, out-of-pocket expenses, particularly medication co-payments, and the costs sustained by informal caregivers;

e. The availability of multidisciplinary care, including mental health care, for those with NCs.

Conclusions

The information collected or consolidated on NCs in terms of impact, health services, the extent of their occurrence and the recognition of potentially correctable risk factors for their onset are substantial contributions to the understanding of these debilitating conditions. The findings reported by the various projects underscore the commonalities shared by individuals with different specific conditions. Addressing the knowledge gaps identified by the NPHSNC projects will be the next steps toward achieving the goal of reducing the burden of neurological conditions.

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NPHSNC Project Findings Deliberations by the Synthesis Panel (Appendix 1 ) resulted in useful conclusions related to each of the project’s four focus areas as well as the identification of knowledge and research gaps and key themes.

1. Impact The neurological conditions addressed in the NPHSNC are chronic, often progressive disorders that impose a range of diverse and debilitating impacts on the lives and well-being of the affected individuals, their families, informal caregivers and communities. Five NPHSNC projects and surveys investigated the impacts of neurological conditions in quantitative and qualitative terms.

Summary of Key Findings 1.1 Impacts of Neurological Conditions on Affected Individuals

Statistics Canada conducted follow-up interviews with respondents to the 2010 and 2011 Canadian Community Health Surveys who had indicated that they or a family member living in the same private dwelling had one of 18 NCsP9F

10P. The response rate for this new survey, the Survey on Living with a Neurological Condition in Canada

(SLNCC), was 81.6%. The findings of the SLNCC were based on structured interviews about their conditions with 4,409 Canadians. When transposed to the Canadian population by Statistics Canada procedures, this random sample represented 1,737,888 Canadians with an NC. SLNCC results were reported for all 18 NCs together and for each condition individually. Another NPHSNC project, the Versnel-Packer LINC project, conducted structured interviews with about 1300 volunteers concerning the impacts of their NC. These interviews, the results of which were reported for the group as a whole rather than by NC, identified many of the impacts noted in the SLNCC. Considered together, the main findings were:

• Having an NC adversely affected Ugeneral healthU. 26% of the SLNCC respondents with NCs reported "fair or poor" general health compared with 10% for the general Canadian population. For those with Parkinson's disease, stroke, and brain or spinal cord tumour, the rate of fair or poor general health was more than 50%.

• Several Uco-morbid conditionsU such as heart disease, diabetes mellitus, hypertension and mood disorder occurred more frequently in those with NCs than in those without.

• ULimitations of their usual activitiesU were experienced by nearly 90% of individuals with an NC, ranging from 66% for Tourette syndrome to 95% for spinal cord injury or tumour. (The number of individuals with ALS or Huntington's disease was too small to assess this and other impacts.)

• Neurological conditions had an impact on Uwork statusU: The 2010 CCHS documented that 36% of people 17 years and older with one or more NCs (excluding migraine) did not have a job compared to 27.5 % among those 17 years and older without NCs (but including migraine alone). 23% of those with NCs were permanently unable to work compared to 2.2% of those without NCs.

• The proportion of those permanently unable to work was highest for individuals with brain injuryP10F

11P, brain or

spinal cord tumour, multiple sclerosis, muscular dystrophy, and stroke.

10 Amyotrophic lateral sclerosis (ALS), Alzheimer's disease and other dementias (AD/dementia), brain tumour, cerebral palsy, dystonia, epilepsy, Huntington's disease, hydrocephalus, migraine, multiple sclerosis, muscular dystrophy, Parkinson's disease, spina bifida, spinal cord injury, spinal cord tumour, stroke , traumatic brain injury, and Tourette syndrome.

11 When the respondents to the CCHS 2010,2011 who self-reported a "brain injury" were asked additional screening questions by the SLNCC interviewers, 25% (150/611) stated that their "brain injury" was not due to trauma; the nature of the NC that these individuals were describing is unknown.

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INTERIM VERSION – NOT FOR PUBLICATION • UHaving an NC contributed to financial insecurityU: 35% of adults with an NC surveyed in the Versnel-Packer LINC

Project reported that their family had experienced a financial crisis in the previous year and 14% of those who were employed had been demoted or had taken a cut in pay.

• UIndividuals with NCs felt stigmatizedU: Between 10 and 30% of SLNCC respondents with an NC reported that they perceived that others felt uncomfortable around them or avoided them, that they were left out of activities, or that they were embarrassed by their condition.

• USide effects ofU UMedicationsU: Of the 51.9% of SLNCC population who were taking a medication for their NC, 29.7% reported medication side effects that affected their lives moderately (23.8%), quite a bit (17.5%) or severely (7.0%).

• UHealth Utilities Index (HUI) ScoresU: The mean overall HUI scores (see Glossary) for AD/dementia, brain tumour, epilepsy, migraine, multiple sclerosis, Parkinson's disease, stroke, traumatic brain injury and spinal cord injury were less than 0.7, which is generally taken as indicating significant impairment of quality of lifeP11F

12P (SR Table 5).

Among the SLNCC sample analyzed by condition, the lowest score for an individual component of the HUI was 0.37 for the cognition impairment of AD/dementia (SR Table 7).

The economic burden of NCs is discussed in section 2 - Health Services.

1.2 Some impacts of neurological conditions differed for men (M) and women (W) (* statistically significant difference based on the CI; P

E P interpret with caution: data with a coefficient of variation of 16.7-33.3%)

• General health "fair" or "poor" (all NCs): M - 34.3%, W - 22.4% *

• Stress level “quite a bit” or “extremely” (NC caused by a spinal cord injury): M - 30.4%, W - 54.6% * • Prevalence of common co-morbid conditions:

- heart disease (all NCs): M - 12.8%, W - 7.7%P

EP *

- hypertension (all NCs): M - 34.6%, W - 25.4% * - mood disorders (for MS): M - 11.2%P

EP, W - 26.6% *

- urinary/bowel incontinence (for stroke): M - 36.8%, W - 58.8% *

• Impact on work status - permanently unable to work: - All NCs: M - 20.5%, W - 9.1% * - Migraine headaches: M - 17.4%P

EP, W - 6.3%P

E P*

• Impact on work status - experienced changes to work activities: - All NCs: M - 41.6%, W - 27.9% *

• Stigma - perceived that some people felt uncomfortable around them: - All NCs: M - 26.3%, W - 16.8% *

• Use of assistance: - Accessed formal assistance (all NCs): M - 13.4%, W - 8.1% * - Used formal assistance daily (all NCs): M - 5.7 %, W - 2.6%P

EP *

- Used informal assistance less than once a month (all NCs): M - 4.3%P

EP, W - 11.2%P

EP *

12 Y. Feng, J. Bernier, C. McIntosh, H. Orpana, Health Rep. 2009, 20:43-50.

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- Used informal assistance at least once a month (all NCs): M - 2.8%P

EP, W - 7.2%P

E P*

- Used informal assistance daily (all NCs): M - 21.4%; W 13.7% *

• Global HUI scores indicating severe disability (less that 0.7) for: - All NCs combined: M - 0.5, W - 0.6 - AD/dementia: M - 0.3, W - 0.2 - Migraine: M - 0.5, W - 0.6 - Multiple sclerosis: M - 0.5, W - 0.6 - Stroke: M - 0.5, W - 0.4

• Age at symptom onset and diagnosis: The mean age at symptom onset and/or diagnosis was different between men and women for certain NCs, although most differences were not statistically significant. Mean age of onset for -

- AD/dementia: M - 70, W - 74 - Parkinson's disease: M - 63, W - 65 - Spinal cord tumour: M - 50, W - 34P

E - Tourette syndrome: M - 8, W - 11

Mean age of diagnosis for - - AD/dementia: M - 72, W - 76 * Years between symptom onset and diagnosis for - - Multiple sclerosis: M - 3, W - 6 - Tourette syndrome: M - 5, W - 1 - Dystonia: M - 3, W - 8

1.3 Impact of Neurological Conditions on Children and their Families

The Versnel-Packer LINC Project was the only NPHSNC project to investigate the impact of NCs on parent-child relationships. This project report provided the following limited information on the impacts of an NC on the child and her/his parents:

• Impaired quality of life, as measured by a quality of life score (PedsQL), which was approximately 52 for children in the LINC Project compared with 70 for children with other chronic conditions, and 80 for healthy children;

• The parents of 24% of children in the LINC study considered that their children hadS Slimited educational opportunities because of their NC.

• 14% of children with a neurological condition were described by their parents as housebound.

Parents (who are usually the informal caregivers of children with NCs) reported the following impacts of having a child with an NC:S

• They used generalist physician services at a level similar to Canadians overall but were more likely to have a hospital stay, to contact a specialist physician, to receive mental health services or to consult a social worker or counsellor;

• 18% rated their mental health as "poor" or "fair" and 35.6% were receiving mental health assistance. although most parents expressed satisfaction with life in general and described their general health as "good" or better;

• 35% rated the impact of their child's NC on the family as "extreme".

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INTERIM VERSION – NOT FOR PUBLICATION 1.4 Impacts of Neurological Conditions on Aboriginal Canadians

The Native Women’s Association of Canada (NWAC) Project team did a qualitative analysis of the material from interviews with 17 NC patients or caregivers, 22 key informants and 41 participants in four research circles. The four interconnected groups of impacts that affected peoples’ day-to-day lives, were:

• UPhysical Uimpacts: pain, exhaustion, mobility impairment, dependence, and other physical symptoms such as dizziness, nausea, and tremors;

• UEmotionalU impacts: fear, anger, anxiety, loneliness, guilt and helplessness;

• UMental healthU impacts: confusion and disorientation, word mis-association, short-term memory loss and depression;

• USpiritualU impacts were less about the spiritual being of individuals and more about how individuals used their spiritual being to cope with other impacts. In some cases their spiritual needs were not met and therefore it was more challenging to use spirituality as a coping mechanism. Important aspects of that spirituality, which helped some participants cope with the impacts of the NC either as an affected person or a caregiver, were: the teachings from their traditions; a positive attitude; access to traditional ceremonies, noting that some participants reported difficulty getting such needs met at health care facilities; seeking the advice and guidance of Elders (some participants noting difficulty in finding Elders to help them with their journey (NWAC Project Report, section 8 - Discussion and Conclusions).

Aboriginal people, and women in particular (as few men were included in the data collection), were impacted by disparities in their social determinants of health and in their health status. Many participants suffered from multiple conditions (e.g., diabetes, cardiovascular disease, etc.) in addition to their NC, and this tended to amplify the many impacts they had to endure. This research team concluded that culturally relevant, patient-centred care that looks at the whole person, including their other ailments – rather than specialized reductionist approaches to healing NCs – would likely result in better outcomes for Aboriginal peoples.

1.5 Impacts of Neurological Conditions on Informal Caregivers

The Hirdes Chronic Care Resident Assessment Project based on interRAI and other data sources investigated the extent and impact of NCs on the informal caregivers of individuals in long-term care programs (home care and long-term institutions). This project documented the following results:

• UCaregiver ProfileU: For 1/3 of individuals in a home care program, the caregiver (who often lived with the affected NC individual and was the spouse or child) provided assistance with daily living support of 22 or more hours/week; 58% of those with ALS received this level of care.

• UCaregiver distressU was higher for NC than non-NC caregivers:

- Distress was experienced by 28% of NC caregivers but only by 13% of non-NC caregivers (Hirdes, Appendices B-3,4: data from 541,515 Home Care clients in Ontario and Manitoba, 211,331 of whom had an NC.);

- Caregiver distress was greater if the NC manifested cognitive impairment or behavioral issues: more than 1/3 of AD/dementia and Huntington disease caregivers reported distress;

- Caregiver distress was predictable by the priority level (as determined by the MAPLe algorithm) of the NC individual, and was therefore measureable with settings collecting RAI data.

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INTERIM VERSION – NOT FOR PUBLICATION Knowledge Gaps regarding the Impact of Neurological Conditions Data are lacking or deficient pertaining to the impact of NCs on:

• Those affected with less common NCs, such as ALS, Huntington's disease and dystonia whose sample sizes in surveys such as the SLNCC are too small for valid analysis;

• Those affected by NCs not addressed by the NPHSNC; • Professional caregivers for individuals with severe, debilitating NCs; • A broader spectrum of First Nations, Métis and Inuit Canadians; • Residents of the three territories; • Children, both those affected with an NC and those living with an affected parent or sibling; • Canadians who are excluded from surveys such as the CCHSP12F

13P.

Some of the existing gaps in pan-Canadian data on the impacts of NCs could be filled by maximizing the use of existing data collection processes (e.g. the interRAI or the Système de mesures d’automonie fonctionnelle [SMAF]) or by incorporating data on the impacts of more NCs into existing national data resources (e.g. the CLSA, administrative data collections, EMR networks). S

Themes Emerging from the NPHSNC regarding Impact of Neurological Conditions a. Persons with different NCs share many of the same impacts of these conditions.

b. NCs impose significant impacts on the well-being and financial security of individuals with the condition, their caregivers, and the community.

c. Perceptions of stigmatization are substantial additional burdens for those with an NC.

d. Financial insecurity and cognitive impairment can be major barriers to self-efficacy/management for persons with NCs.

e. The manifestations of NCs, which are often progressive, vary with the stage of the conditions as well as the age of the affected individual.

f. The sex-specific differences in some of the impacts of NCs (e.g. the longer interval between onset of symptoms and diagnosis in women than in men with multiple sclerosis) need to be explored further.

g. The capacity of affected individuals to respond to the disabilities imposed by NCs is affected by such social determinants of health as economic situation, educational status and community attitudes.

h. It is necessary to consider ethnic and aboriginal differences, including culture and language, regarding the impacts of NCs.

i. Aboriginal Canadians experienced unique impacts of NCs, in addition to having to deal with the impacts encountered by others.

13 Persons living on reserves and other Aboriginal settlements in the provinces; full-time members of the Canadian Forces; the institutionalized population; and persons living in the Quebec health regions of Région du Nunavik and Région des Terres-Cries-de-la-Baie-James.

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2. Health Services Several projects of the NPHSNC explored the need for, and adequacy of, health services along the continuum of health care provision from the perspectives of the affected individuals, their informal caregivers, health care providers, and policy makers.

Summary of Key Findings Health services for those with neurological conditions were analyzed in terms of utilization, costs, the role of informal caregivers, perceptions of administrators and patients or caregivers, delivery of continuing care, care of children, and the special needs of Aboriginal Canadians.

2.1 Individuals with neurological conditions used more health care services across the continuum of care than those with other chronic conditions.

• The Versnel-Packer LINC Project survey of 754 volunteers with NCsP13F

14P living in the community found that these

individuals used more universally insured health services than Canadians with other chronic health conditionsP14F

15P,

particularly specialist physician services, as well services that are not always accessible through public funding, e.g. nurses, physiotherapists, psychologists, social workers and counselors. Twenty-five per cent of the LINC population made extensive use of formal care giving, mainly for personal care and housework.

• The SLNCC, a larger survey of Canadians living in private dwellings, determined that 9.7% of those surveyed reported receiving formal assistance during the previous year that included general help with activities, personal care, medical care, transportation or meal preparation and delivery. (This rate is substantially lower than that of the Versnel-Packer LINC Project survey, probably due in part to the high proportion [20% unweighted; 62% weighted] of persons with migraine in the SLNCC population.)

• UAcute hospitalU utilization, expressed as per capita hospital days, was 3.5 to 110 times higher, depending on the specific condition, for persons with NCs than for persons without the NC in British Columbia in 2010P15F

16P. Per

capita hospitalization days were highest for persons with spinal cord injury and traumatic brain injury, findings that probably reflect the length of time that individuals with these conditions spend in rehabilitationP16F

17P.

• Per capita Uphysician servicesU, a reflection of utilization of services by persons both in the community and in acute care hospitals, were 1.45 to 5.6 times higher among persons with NCs than among those without these conditions in BC in 2010. Spinal cord injury, traumatic brain injury and AD/dementia had the greatest numbers of per capita physician visits.

• Per capita days in Uresidential careUP17F

18P were approximately 3-200 times higher among persons with NCs in British

Columbia in 2009-2010. Days in residential care were highest for persons with AD/dementia, Huntington's disease and parkinsonism.

• Persons in registered Uhome careU programs (in Ontario) used more health care services than did those receiving home care for other conditions.

14 A convenience sample recruited through their NHCC-affiliated or other organization. 15 Compared to respondents to the 2009,2010 CCHS who reported having two or more chronic conditions. 16 These proportions were calculated by dividing the costs associated with for each NC by the costs for those without the specific condition. 17 The BC administrative data for acute hospitals include rehabilitation hospitals and day surgery. 18 Described as "home and continuing care" (Reimer BC Administrative Data Project, Year 3 Report, page 6)

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INTERIM VERSION – NOT FOR PUBLICATION • Persons with NCs (AD/dementia ~ 50%, Stroke ~ 30%, Parkinson's disease ~ 5%) accounted for a majority

of patients designated as Ualternate level of careU (ALC) in acute care hospitals. Abusive behaviours were present in ~ 3/4 of ALC patients with AD/dementia.

2.2 Health Services Provision for Persons with Neurological Conditions Provision of health services was described from the perspectives of health care administrators and persons with neurological conditions or their caregivers.

2.2.1 Perceptions of Health Care Administrators

To identify needs, gaps, current policies and best practices, the Jaglal Health Services Project did a scoping review of 723 articles, held structured interviews with 180 key informants from across Canada (39% health care professionals, 47% non-health care professionals, 14% policy makers) and conducted an online survey that was completed by administrators in 645 of the 2783 publically-funded, Canadian health care institutions (137 acute care hospitals, 131 long-term care facilities, and 171 community outpatient centres).

The scoping review and key informant interviews documented:

• The lack of knowledge/awareness about NCs among service providers;

• The limited availability of much needed services for education/return to school, employment, family/caregiver support, housing and transportation, particularly for those living in rural areas.

The survey of administrators in the Jaglal Health Services Project determined that:

• A high proportion of care providers offered services for patients with stroke (71%), Parkinson’s disease (65%), Alzheimer’s disease (65%), TBI (59%), and multiple sclerosis (57%);

• Only a small proportion of the providers reported services for individuals with less common conditions such as dystonia (28%), Tourette Syndrome (17%), or Rett syndrome (13%);

• There was a paucity of both information on, and services for, education/return to school, employment, family/caregiver support, housing, and transportation;

• Most service providers had exclusion criteria: 33% (n=153) for psychiatric diagnosis, severe behavioural disorders, substance abuse or substance dependence; 32% (n=147) for medical instability, degenerative medical conditions, and/or presence of co-morbidities; and 21% (n=97) on the basis of age. Only 28% (n=130) of service providers reported no exclusion criteria.

• 47% (n=222) of service providers cited staff ratios and 37% (n=173) cited physical environment as having an impact on the ability to provide services.

2.2.2 Perceptions of Persons with Neurological Conditions and their Informal Caregivers

The perspectives of 100 patients with NCs concerning the health care system in the Versnel-Packer LINC Project were surveyed and compared with the perspectives of 100 care providers in the Jaglal Health Services Project. Individuals from each group were questioned about the perceived adequacy of various aspects of health care servicesP18F

19P.

Service adequacy could be rated as optimal (9-11), advanced (6-8), basic or intermediate (3-5), or providing little or no support (0-2). Overall, the patients surveyed rated these services lower than did the service providers.

19 Community partnerships and resources, continuity of care, evidence-based guidelines, self-management support, practice team function and leadership, provider education, informing patients about guidelines, specialist involvement and follow-up.

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INTERIM VERSION – NOT FOR PUBLICATION • Mean scores given by patients were all in the "basic/intermediate" or "little to no support ranges" while

providers’ scores were "advanced" or "basic/intermediate";

• Goal setting and follow-up/coordination received the lowest ranking by patients, with mean scores in the "little or no support for chronic illness" range; providers also gave follow-up their lowest score but their ratings were in the "basic or intermediate" range;

• Neither group rated any aspect of service provision as "optimally integrated for chronic illness" (Jaglal Health Services Project Report, Appendix 1, Slide Deck P4).

Other NPHSNC projects provided patient or caregiver comments on health services for NCs:

• 30% of the LINC respondents requiring a specialist appointment reported access difficulties.

• 30% of the LINC project respondents used community-based services, for which they expressed high levels of overall satisfaction.

• A small sample (n=104) of parents of children with cerebral palsy rated the services their children had received quite highly using the Measure of Process of Care questionnaireP19F

20P (Shevell-Oskoui Cerebral Palsy

Registry Project report).

2.3 Costs of services for persons with NCs are greater than those without an NC at all stages of care.

Total and per capita direct health care costsP20F

21P for 13 NCs investigated in British Columbia for fiscal year 2010-2011

(Reimer Administrative Data Project) , were higher among persons with each of the NCs, compared to age-standardized data for persons without that condition:

SR Table 2 - Health Care CostsP21F

22P, British Columbia, 2010-2011

Neurological Condition Total for BCP22F

23 Per capita with the NC Per capita without the NC

AD/dementia $527,494,451 $ 5,180 $1,7636 Anterior horn cell

diseaseP23F

24 $ 8,405,080 $12,956 $1,827

Brain tumour, malignant $ 25,624,373 $13,247 $1,850 Cerebral palsy $ 50,520,339 $ 6,131 $1,822 Epilepsy $208,678,615 $ 5,776 $1,798 Huntington's disease $ 2,159,876 $10,825 $1,854 Hydrocephalus $ 44,923,392 $12,035 $1,822 Multiple sclerosis $ 70,461,943 $ 6,907 $1,843 Muscular dystrophyP24F

25 $ 4,256,892 $11,284 $1,827 ParkinsonismP25F

26 $120,358,004 $11,083 $1,833 Spina bifida $ 7,008,611 $ 9,174 $1,827

20 King, S., Rosenbaum, P., & King, G. (1996). Parents' perceptions of care-giving: Development and validation of a measure of processes. Developmental Medicine and Child Neurology, 38, 757-772.

21 Available for acute hospital costs, physician services (billed in and out of hospital), Pharmacare and Pharma Net (self-paid or 3rd party-paid costs not covered by Pharmacare).

22 See footnote 33 23 Adjusted for inflation 24 Includes ALS

25 Includes muscular dystrophies and myopathies 26 Includes Parkinson's disease

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Spinal cord injury $ 17,720,325 $75,201 $1,850 Traumatic brain injury $ 86,838,932 $26,905 $1,837

Large as these figures are, they undoubtedly underestimate the true costs for each condition because this project only had access to direct costs and some out of pocket expenses (prescription drugs not covered by BC Pharmacare). Services provided by salaried physicians and many indirect costs (such as time away from work, caregiver time away from work) could not be determined from the available database.

• The costs of caring for individuals with NCs in Uhome careU programs and in Uchronic care institutionsU is greater than that for individuals with other conditionsP26F

27P (Hirdes Chronic Care Resident Assessment Project).

• UOut-of-Pocket ExpensesU: 60% of the LINC sample population reported out-of-pocket costs for prescribed medication, despite the fact that 85% of the sample reported having a drug insurance plan. Nearly 20% had paid more than $1,000 in out-of-pocket expenses in the previous year (Versnel-Packer LINC Project Report. Questions about out-of-pocket expenses were also part of the SLNCC (survey). Average annual out-of-pocket costsP27F

28 Pper patient were reportableP28F

29 Pfor the following SLNCC populations by neurological conditions: 21TP29F

30:P21T

- AD/other dementias: $1,004 - Epilepsy: $ 466 - Multiple sclerosis: $ 990P

EP

- Parkinson's disease: $1,119 - Spinal cord injury: $1,169 - Stroke: $ 844 - Traumatic brain injury: $ 781

• The BC PharmaNet data-base provided information about the costs of medications that are not covered by BC Pharmacare although self-paid and 3rd party payments are not separated. For three NCs in 2010-2011, these (out-of-pocket, at least in part) costs were more than $1000: brain tumours - $1095 (age-standardized comparison group without the condition - $313); multiple sclerosis - $1,270 (comparison group - $311); and, parkinsonism - $1,071 (comparison group - $311) (Reimer BC Administrative Data Project).

2.4 Informal Health Care Services

Care provided by family, friends and neighbours, termed "informal care" throughout the NPHSNC, is an important service required and received by many persons with a neurological condition.

• 45% of the Versnel-Packer LINC Project sample population reported that they received informal care.

• 39.6% of respondents to the SLNCC had received informal assistance that included general help with activities, personal care, medical care, transportation or meal preparation and delivery. The proportion receiving informal assistance for emotional support was high at 78.8%.

27 Data submitted to the Microsimulation project but not provided to the SAC/Synthesis Panel. 28 The denominator of these means included those patients who reported zero costs. 29 For other conditions, the coefficient of variation was greater than 33.3% and therefore did not meet Statistics Canada's quality standards.

E Coefficient of variation 16.6-33.3%; Statistics Canada recommends caution. 30 Data provided by Christina Bancej, PHAC

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INTERIM VERSION – NOT FOR PUBLICATION 2.5 Mental Health Service Needs of Persons with Neurological Conditions

Several projects documented the mental health service needs of individuals with NCs.

• 33.9% of the SLNCC 2011-2012 population overall reported that most days were "quite a bit" or "extremely" stressful, compared with 23.6% of the general population (CCHS 2011).

• Mood disorder (type not specified) was reported as being diagnosed in 23.0% of the SLNCC population overall; the frequency was higher in subjects with a NC due to a brain or spinal cord tumour.

• 9.5% of the LINC population reported a diagnosis of depression that was moderate or severe.

• 34.8% of the SLNCC population reported having received formal emotional supportP30F

31P because of their NC; the

receipt of emotional support was highest at 53.2% among those 15-34 years old.

In contrast to these documented needs for mental health services, the results of the Jaglal Health Services Project suggest that such needs are not being adequately met for persons with neurological conditions. The online survey completed by the administrators of publicly-funded acute care (n=137), long-term care (n=141) hospitals, and Community Outpatient Centres (n=171) from all regions of Canada found that 33% of the respondents indicated that their facility did not accept patients with psychiatric diagnoses or severe behavioral disorders. Only 9% (n=44) of these service providers had a neuropsychologist, and only 3% (n=15) had a neuropsychiatrist.

2.6 Health Services for Children with Neurological Conditions

Although data on health services for children with NCs was extremely scarce in the NPHSNC projects, the findings of a few projects suggested that health services for some children may be sub-optimal.

• Based on interviews with 74 parents, 20% of the LINC children with an NC had not seen a general practitioner or a pediatrician in the previous year and only two thirds had received specialist physician care (Versnel-Packer LINC Project Report.

• Children with newly-diagnosed cerebral palsy utilize a range of rehabilitation services (Shevell-Askoui Cerebral Palsy Registry Project report).

• The survey of health services providers found that fewer offered services for children (0-17 years of age) than for adults (Jaglal Health Services Project report).

2.7 Health Services for Aboriginal Canadians with Neurological Conditions

Based on individual interviews, key informant interviews, and research circles involving 80 individuals (69 women, 11 men; 65 First Nations, 7 Metis, 8 non-Aboriginal or not specified), the NWAC Project team identified several challenges that confront Aboriginal Canadians requiring health care services for NCs:

• The lack of accessible specialized health care and diagnostic services in northern, rural, or remote locations;

• Transportation to health-care services, especially for people living in remote areas;

• Difficulties navigating the health system in relation to which level of government was responsible;

• Lack of support and training for families with a member affected by an NC;

• The need for better cultural competence among health care providers;

31 The nature of the "formal emotional support" is not known.

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INTERIM VERSION – NOT FOR PUBLICATION • The lack of “easy to understand” information about NCs; and

• The need for greater awareness in Aboriginal (First Nations, Inuit and Métis) populations about NCs to address stigma associated with these conditions.

Overcoming these challenges would entail actions such as: working more closely with Aboriginal people; bringing more services into Aboriginal communities; instituting holistic approaches to healing; providing opportunities for traditional ceremonies and gatherings; and making advocates or guides available for Aboriginal persons accessing the health care system.

The health services challenges enumerated by this project could serve used as guide posts for the evolution of new policies for the provision of health care for Aboriginal Canadians.

2.8 Predicting and Evaluating Health Care Needs in Long-term Care Settings

The Hirdes Chronic Care Resident Assessment Project provided information related to predicting needs for, and evaluating, continuing care.

• Care needs in chronic care settings are better defined by the type of functional impairment than by the diagnostic category.

• Assessment tools such as interRAI are able to define care needs and to predict outcomes of persons with chronic NCs.

• The combination of an NC and cognitive impairment may be an important barrier to access to appropriate rehabilitative services. Persons with cognitive impairment were less likely to receive occupational therapy and life skills training than persons without cognitive impairment. Thus, the combination of an NC and intellectual disability may be an important barrier to access to appropriate rehabilitative services (Project Report, Appendix B-15).

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INTERIM VERSION – NOT FOR PUBLICATION Gaps in Understanding Health Services for Persons with Neurological Conditions a. The acute care utilization and cost information concerning individuals with NCs (provided by the analysis of the

BC administrative data) only provide a picture of costs for a single province. An attempt to determine a national picture has not been attempted since 2007 when the Canadian Institute for Health Information (CIHI), in collaboration with the CBANHCP31F

32P, estimated direct (hospital care, physician services and drug expenditures)

and indirect costs for the 11 most prevalent NCs for fiscal year 2000-2001P32F

33P.

b. Given that all provincial and territorial drug plans involve co-payments, it would be valuable to have these data to better appreciate the cost of medications to individuals for each NC.

c. The actual costs, both direct and indirect, of providing care for individuals with NCs in continuing care (mainly home care programs and chronic care institutions) are not yet available so an understanding of the global costs of NCs remains incomplete. Cost data from the Hirdes Resident Assessment Project are being processed for the Microsimulation Project which will produce cost projections into the future.

d. The limited information on the provision of health care for First Nations, Inuit, Métis, other vulnerable populations and children with NCs are significant gaps.

e. In addition to the opinions of administrators and patients, it would be informative to survey formal health-care providers (e.g. physicians, nurses, social workers, physiotherapists, occupational therapists and speech therapists) on their perceptions of the accessibility, timeliness and quality of health services for persons with NCs.

f. There is a paucity of services and service providers to address the coexistence of mental health disorders and NCs, in spite of the various lines of evidence documenting the need for mental health services by individuals with NCs.

g. Information about the availability of multidisciplinary care, which is particularly important for those with NCs, was not addressed by any of the NPHSNC projects.

h. There is a lack of information on the distribution and quality of health services across the various regions and jurisdictions of Canada.

32 Canadian Brain and Nerve Health Coalition, the advocacy program of the Canadian Neurological Sciences Federation. 33 Canadian Institute for Health Information, The Burden of Neurological Diseases, Disorders and Injuries in Canada (Ottawa: 2007).

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INTERIM VERSION – NOT FOR PUBLICATION Themes Emerging from the NPHSNC regarding Health Services for Persons with NCs a. Many of those with NCs receive extensive services from informal caregivers, who often require emotional and

financial support.

b. There is a need to recognize and address the impact and complexities of the mental health co-morbidities in individuals with NCs and their caregivers. Given the proportion of service providers who report restriction on the provision of care for persons with psychiatric disorders, it will be important to determine how eligibility criteria are applied and if a disproportionate amount of restriction is being put on those with NCs.

c. Functional ability, as well as diagnosis need to be considered when developing care plans for those with NCs.

d. Individuals with NCs need transition support for as they move along the continuum of care, often because of progression of their condition.

e. Individuals with milder forms of neurological conditions have special needs that require recognition and support.

f. The availability and cost of drugs, including co-payments, is becoming an increasingly significant component of the overall health care costs both for patients and the health care system.

g. Inconsistencies in care across regions and jurisdictions exist but have yet to be comprehensively identified.

h. Individuals with neurological conditions will benefit from the current moves in some jurisdictions to modify chronic care, such as the development of a revised chronic care model that incorporates environmental, social, and educational factors are incorporated into the revised model.

i. The culture and language of ethnic and aboriginal populations must be considered in the delivery of healthcare services for persons with NCs.

j. Community-based services and supports as well as traditional programs and facilities are both important components of an effective health service delivery.

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3. How Common are Neurological Conditions in Canada? Studies of the occurrence and extent of neurological conditions (NCs) in Canada were carried out to document the size of the burden of these conditions. At the outset, it is important for readers to appreciate several points concerning incidence and prevalence - the two principal measurements of occurrence and extent, respectively.

• It is appropriate to refer to incidence and prevalence as "estimates" because their calculation is usually based on samples of the population - not the entire population.

• Each of the sources for incidence or prevalence estimates has its strengths and limitations. • Although "perfect" values for incidence and prevalence remain elusive, confidence in a given estimate is

strengthened if it is confirmed by more than one source. • By combining data from multiple published studies, meta-analyses (see Glossary) can potentially generate

a more precise estimate of true incidence or prevalence. The main limitation of such estimates is their dependence on the quality and uniformity of the studies involved.

• When comparing other sources of incidence or prevalence estimates with those of a meta-analysis of several smaller studies, the confidence interval (see Glossary) usually indicates a better "match" than the range of the smaller studies.

• Other statistical features of the confidence interval provide an indication of the precision of the incidence or prevalence measurement.

Summary of Key Findings 3.1 Incidence and Prevalence Estimates from Different Sources

The incidence and prevalence estimates of NCs from different sources are shown in SR Tables 1,2,3. Considered together, the NPHSNC projects identified the best sources of incidence and prevalence for certain NCs as well as for those NCs for which other methods of measurement are required (SR Table 5 on the following page). The comparisons of the prevalence and incidence estimates from the different sources used in the NPHSNC projects, summarized in Table 5 (next page), also support several conclusions concerning data sources for the surveillance of NCs.

• Systematic reviews and meta-analyses produced estimates on the prevalence and incidence of NCs that are useful comparisons for new data from the NPHSNC projects (SR Tables 1,2,3). The systematic reviews also documented the limited number of such studies in Canada (SR Table 6).

• Estimates of incidence and prevalence from administrative databases typically agreed with those of the systematic reviews, making them promising sources for future surveillance development.

26TSynthesis Panel Comment26T: For surveillance purposes, administrative data have the advantage of capturing real patient encounters with health care providers rather than relying on potentially biased self-reports from surveys. They are limited, however, in the amount of information they capture about people and their experiences living with NCs beyond basic demographics (e.g., age, sex, geography) and their use of health services. National-level surveillance using administrative data is only possible when each jurisdiction captures administrative data in a consistent way. Administrative data based on physician billing codes have the added limitations of being dependent on codes that are often outdated in terms of contemporary understanding of NCs. They are also dependent on the diligence with which the examining physician applies the codes.

• Self-report surveys can be used to estimate prevalence but the results need to be interpreted with caution, particularly for NCs that are rare, can interfere with survey participation (e.g. dementia) or may be denied because of perceived stigma (e.g. Huntington's disease). Although their dependence on correct and complete

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responses by participants can limit their accuracy, surveys also have advantages in terms of their ability to provide consistent national coverage relatively quickly for surveillance purposes.

• The electronic medical records (EMRs) of family physicians are potential sources of readily accessible epidemiological data for NCs.

SR Table 6 - Prevalence and Incidence of Neurological Conditions from Different Sources

Estimates from Different Data Sources compared to Systematic Reviews and Meta-analysesP

1

Data source Within, or close to, 95% CI Within range, outside 95% CI

Above range Below range

Prevalence Estimates Administrative dataP

2 AD/dementia P

also 3

Cerebral palsy Huntington's disease Hydrocephalus Parkinson's disease P

also 3

ALS Epilepsy Multiple sclerosis P

also

3

Muscular dystrophy EpilepsyP

3 Brain tumours, malignant Spina bifida Spinal cord injury Traumatic brain injuryP

4

Self-report surveyP

5 Dystonia Hydrocephalus - infants, children Parkinson's disease

Brain tumours Cerebral palsy Epilepsy Huntington's disease Multiple sclerosis Spina bifida Tourette syndrome

ALS Muscular dystrophy

AD/dementia Hydrocephalus - adults Spinal cord injury Traumatic brain injury

Electronic medical recordsP

6 AD/dementia Epilepsy Parkinson's disease

Incidence Estimates Administrative dataP

2,7 AD/Dementia ALS Brain tumours, malignant Huntington's disease Parkinson's disease Spinal cord injuryP

8P

Epilepsy Hydrocephalus - infants, children Multiple sclerosis

Hydrocephalus - adults

Traumatic brain injuryP

9

P

1 P Jetté-Pringsheim Systematic Reviews Project

P

2P Reimer British Columbia Administrative Data Project

P

3P Tu-Jaakimainen-Butt Ontario Administrative Data Project

P

4P Compared to a single study

P

5P Claudia Lagacé et al., PHAC: Canadian Community Health Surveys, 2010 and 2011

P

6P Drummond-Birtwhistle Electronic Medical Records Project

P

7P Due to the absence of studies or incomplete data, comparisons of incidence were not possible for cerebral palsy,dystonia, migraine,

muscular dystrophy, spina bifida, spinal cord tumour, stroke, and Tourette syndrome. P

8P Pre-hospital mortality excluded

P

9P Within the range of 2 Canadian studies but not 35 others

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INTERIM VERSION – NOT FOR PUBLICATION 3.2 More than half of the Canadians in continuing care (home care, long-term care residences) have a

neurological condition as their primary diagnosis.

It is no surprise that NCs are more prevalent among those in long term care facilities and in home care programs than among those living in the community at large, but the actual figures are striking. Two NPHSNC projects (SNCIC, 2011-2012 and the Hirdes-Jetté-Cameron Continuing Care Resident Assessment Project) documented the high proportions of individuals in continuing care settings who have a NCP33F

34P. For the jurisdictions

where data are available, more than 50% of individuals in formal home care programs (Ontario) or long-term care facilities (Ontario and several other provinces or territories) have at least one NC (SR Table 4). Nearly 70% of patients in long-term care facilities had a diagnosis of AD/dementia (SNCIC Report).

Although there were variations in the prevalence estimates between the two studies (SR Table 4), they are probably a reflection of their different data sources. The regional differences may reflect a combination of true population variability, differences in admission policies, and differing availability of other support services between regions.

3.3 The epidemiological features of several NCs differed according to sex and age

Data from the 2010, 2011 CCHS confirmed that multiple sclerosis and migraine headaches affect more women than men while Tourette syndrome, Parkinson's disease, effects of a brain or spinal cord injury, and cerebral palsy occur more frequently in men.

The overall prevalence of NCs increased with age, peaking at 80 years and over. This peak was driven mainly by AD/dementia, stroke and Parkinson’s disease – NCs that are more prevalent in older age groups. Other conditions tend to present in childhood (e.g. cerebral palsy, spina bifida, hydrocephalus) while others primarily affect young adults (e.g. multiple sclerosis, neurotrauma). Epilepsy was one of the three most prevalent NCs in all age groups (CCHS).

The figures from the Reimer BC Administrative Data Project Report that depict the age-specific incidence and prevalence of neurological conditions illustrate age and sex differences quite strikingly. These differences have major impacts on affected individuals (e.g. the ability of young adults to work) and society (e.g. to provide care for the disabled elderly).

3.4 Common Co-morbidities associated with Neurological Conditions

The Survey on Living with a Neurological Condition in Canada (SLNCC) [6] identified heart disease, diabetes mellitus, hypertension, and mood disorder as common co-morbidities for the overall population with an NC. The Reimer BC Administrative Data project also listed as co-morbidities the eight most common "other diagnoses" in the previous five years associated with each of the 13 NCs identified in the BC administrative databases.

3.5 Surveillance Capacity Building

One of the goals of the NPHSNC was to provide basic information to inform the development of a surveillance system for NCs in Canada. Progress towards the achievement of this goal was advanced through four interdependent approaches: the systematic reviews of Jetté and Pringsheim; preliminary developments at PHAC to add NCs in the Canadian Chronic Disease Surveillance System; the estimates of incidence and prevalence based on different data sources by the projects and surveys of the NPHSNC; and the development of guidelines for the creation and management of registries.

34 Data Sources: The SNCIC data were derived from an on-line survey of the administrators of registered chronic care institutions across Canada; the Hirdes-Maxwell-Jetté ideas-PNC project based its data on the diagnosis recorded through the periodic resident assessments (interRAI). The NPHSNC Scientific Advisory Committee had concerns about the validation of the diagnoses for both these studies.

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3.5.1 Evidence from a Systematic Review of the Literature

To determine the best sources of ascertainment for 15 NCs, Jetté and Pringsheim: • Identified 30 studies by systematic review that validated International Classification of Disease (ICD)

coding of case-definitions in administrative data; • Evaluated the sources of ascertainment of the more than 1,200 studies in their systematic reviews of the

incidence and prevalence of neurological conditions; • Prepared an inventory of neurological registriesP34F

35P; and

• Consulted more than 50 experts.

The conclusions of these investigations are summarized in SR Table 6.

SR Table 7 - Sources and Quality of Ascertainment Methods for Neurological Conditions in Studies Identified by Systematic Reviews P

1

Neurological Condition

Description of the eligible studies identified by the systematic reviews Sources

recommended for surveillance Canadian

studiesP

2 Validation of ICD coding

accuracy Most frequent source of data

AD/dementia Prevalence: 9 Incidence: 2

Broad range of diagnostic accuracy of ICD coding

Door to door surveys common

Multiple

ALS/MND/AHCD Prevalence: 0 Incidence: 1

Excellent coding accuracy for MNDP35F

36P

Administrative data

Multiple

Brain tumour Prevalence: 0 Incidence: 1

Lack of appropriate coding algorithms for specific tumours

Registries common Existing cancer registries

Cerebral palsy Prevalence: 2 Incidence: 0

No ICD-based validation studies

Prospective birth cohorts

Multiple; registries

Dystonia Prevalence: 0 Incidence: 0

No ICD-based validation studies

No data Population-based random sampling or door-to-door study

Epilepsy Prevalence: 4 Incidence: 0

Excellent coding accuracy Door-to-door surveys

Administrative data ± EMRs.

Huntington's disease Prevalence: 0 Incidence: 1

No ICD-based validation studies

No data

Multiple

Hydrocephalus Prevalence: 1 Incidence: 0

No ICD-based validation studies

Infant surveillance programs, chart reviews, registries

Multiple

Migraine Not investigated Multiple sclerosis Prevalence: 11

Incidence: 7 Excellent coding accuracy Administrative

data Multiple

35 Registries are collections of data related to patients with a specific diagnosis or condition.

36 MND - motor neuron disease, the item in the ICD-9 coding system that includes ALS.

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Muscular dystrophy Prevalence: 0 Incidence: 1

No ICD-based validation studies

Chart reviews Multiple

Parkinson's disease Prevalence: 1 Incidence: 0

Broad range of diagnostic accuracy of ICD coding

Multiple sources Multiple

Spina bifida Prevalence: 5 Incidence: 0

No ICD-based validation studies

Registries Anomaly surveillance programs

Spinal cord injury Prevalence: 0 Incidence: 1

Broad range of diagnostic accuracy of ICD coding

Administrative data

Administrative data, Rick Hansen registry

Spinal cord tumour Not investigated No data No data No recommendation Stroke Not investigated No data No data No recommendation Tourette syndrome Prevalence: 0

Incidence: 0

No data School based studies

Children: school based studies; adults: random sampling or door-to-door surveys.

Traumatic brain injury Prevalence: 0, Incidence: 1

Broad range of diagnostic accuracy of ICD coding

Administrative data

Multiple sources

1 From the Jetté-Pringsheim Systematic Reviews Project Report 2 Most were single studies not included in the meta-analysis

3.5.2 Addition of Neurological Conditions to the CCDSS36F

37

The CCDSS is a collaborative network of provincial and territorial surveillance systems supported by PHAC. As the projects and surveys of the NPHSNC were progressing, the CCDSS Neurological Conditions Working Group was conducting development work to add 4 NCs to the CCDSS, in addition to the chronic conditions already included in this pan-Canadian system (e.g. diabetes, hypertension). The inclusion of stroke to the CCDSS has been ongoing, and is led by the CCDSS Stroke Working Group. Progress to data includes:

• The development of NC case definitions for multiple sclerosis, Parkinsonism, epilepsy, Alzheimer disease and other dementia, and stroke that apply across provinces and territories;

• The completion of validation studies of the algorithms developed for these conditions;

• The completion of feasibility studies testing the best performing algorithms in several provinces;

• The launch of a CCDSS national pilot study in 2013/2014, with 2015/2016 identified as the target date for formal inclusion of these NCs in the CCDSS.

3.5.3 Potential Sources of Ascertainment from the NPHSNC Projects and Surveys

The estimates of incidence and prevalence (based on different data sources that were produced by several of the NPHSNC projects and surveys) also contributed to the investigation of the best ascertainment sources for surveillance purposes for each of the NCs under consideration. The findings of these projects are summarized in SR Table 5.

37 Expansion of the Canadian Chronic Disease Surveillance System (CCDSS) for National Surveillance of Neurological Conditions by Catherine Pelletier (Project Lead), Asako Bienek, Sulan Dai, Jay Onysko, Chris Waters, all of whom are members of the Surveillance and Analysis Division of PHAC.

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3.5.4 Guidelines for the Development and Maintenance of Registries

Disease or patient registries are collections of data related to patients with a specific diagnosis or condition. The NPHSNC Registry Guidelines Project (L. Korngut, PI) carried out extensive literature reviews, held 3 structured focus group meetings and 2 consensus conferences, and used a modified Delphi consultation to produce comprehensive guidelines for the development, implementation and maintenance of registries of NCs in Canada. These guidelines have been publishedP37F

38P and will assist investigators to plan future registries for NCs.

This project delineated the key elements of:

• Good registry design: participant informed consent, transparency, an advisory council, clear data ownership, appropriate data security, data release procedures, multi-modal patient recruitment, patient follow-up, planned data linkage, standard operating procedures, a data management plan, and appropriate data collection.

• High quality registries: a quality management plan, standardized methodologies to address data collection and source inconsistencies, the use of iterative or pilot-tested data collection method, rigorous, consistent and documented processes for data cleaning and correction, trained personnel, and a defined audit system with initialization triggers.

• Registries that have a high impact: advance planning, adequate human and financial resources, regular communication with participants and stakeholder, efficient data collection, and transparent operation.

The structured focus groups determined that although Canadian patients are in general supportive of the concept of disease registries and are willing to participate in such registries, they have certain qualifications.

• Before providing their consent to participate in a registry, patients wanted to know certain features of the registry: its purpose; its capacity to facilitate ethical research with meaningful results; that it is well managed and sustainable; that participation will not be onerous; and, that they may withdraw at any time.

• Patients also had difficulty understanding the need to share other information such as: personal directives, age, occupation, income, provincial health number, and marital status with disease registries. They also did not wish to share their Social Insurance Number with a disease registry, even though that information is the only nationally applicable individual identifier.

38 Neurological Registry Best Practice Guidelines, Can. J. Neurol. Sci., 2013, 40: Suppl. 2.

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INTERIM VERSION – NOT FOR PUBLICATION Gaps Regarding the Occurrence and Extent of Neurological Conditions in Canada There are two main categories of information gaps concerning the scope of neurological conditions: research gaps and gaps in infrastructure.

Research Gaps:

a. The limited data on the extent of NCs in children;

b. The lack of data concerning NCs for those who are not included in the annual CCHSP38F

39P;

c. Precise data on the less prevalent conditions such as ALS, dystonia and Huntington's disease;

d. Benign brain tumours remain under-reported in provincial/territorial cancer registries, though efforts are underway to improve data capture;

e. Ascertainment of some NCs (e.g. multiple sclerosis, Parkinson's disease) at their earliest stages when symptoms are often non-specific and physicians use codes (e.g. "numbness") that describe symptoms but do not indicate a diagnosis.

Gaps in Infrastructure:

a. If the diagnostic codes for physician billing claims are to be a useful component of administrative data sets, they need to be upgraded (e.g. to the ICD-10-CA codes that are now used for hospital separations in all provinces and territories), standardized across jurisdictions and incentives found to improve accuracy in their use.

b. For the 45 and older age group, incidence data may eventually become available from the Wolfson-Raina CLSA-NCI project, recognizing the inherent limitations of the project's reliance on volunteer participants. The CLSA contribution to the understanding of NCs would also be enhanced if the comprehensive cohort of this longitudinal, long-term study were expanded to include other conditions that affect the aging population, particularly stroke.S

c. As electronic medical records become more widespread and administrative data become more sophisticated (e.g. pharmaceutical, costing data), it will be important to promote standardized algorithms for estimating prevalence and other epidemiological factors.

Themes Emerging from the NPHSNC Regarding Scope a. Multiple sources of data, each with varying quality and different methodological limitations, are used to

estimate prevalence and incidence of neurological conditions. When the estimates from different sources differ significantly, they need to be interpreted with caution and the divergent estimates carefully balanced and critically appraised.

b. The precision of prevalence or incidence estimates from various sources can be judged statistically from their 95% CIs. For estimates based on meta-analyses, the ranges of the component studies can also be informative although they are generally wider than the 95% CI.

c. Statistics on the incidence and prevalence of NCs by region, age group, sex, and other key variables have not been routinely generated in Canada.

39 See footnote #13

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INTERIM VERSION – NOT FOR PUBLICATION d. Estimates of prevalence and incidence compiled periodically and regularly from administrative data can be

used to trace the course of NCs over time (as was done in the Reimer BC Administrative Data Project) and across geographic regions.

e. Pan-Canadian data on the scope of NCs would be enhanced by the standardization across jurisdictions of case-definitions, algorithms and data collection methods using broad, stable categories that can accommodate changes in diagnostic criteria and the classification of diseases over time. For some conditions, more precise case-definitions are required.

f. If confirmed in annual collections of data over a number of years, the variability among provinces and territories in terms of incidence and prevalence estimates of NCs could be a useful means of studying the effectiveness of policies and interventions in different jurisdictions or regions.

g. To determine the prevalence of severely debilitating NCs in the whole population, it is necessary to include data from both the community and institutions.

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4. Risk Factors Two NPHSNC projects (Krewski Risk Factors for Onset and Risk Factors for Progression Projects) analyzed several thousand articles and selected only systematic reviews, meta-analyses and observational studies that met specific criteria to assess risk factors for the onset and progression of 14 neurological conditions. The selected articles were used for a qualitative synthesis (see Glossary). The results presented here are sets of associations for each NC that were considered meaningful by statistical analysis; these results may not include other known associations because of the methodology and criteria employed.

Summary of Key Findings 4.1 Potentially Modifiable Risk Factors for Onset of Neurological Conditions

Given the disproportionately high level of disability caused by NCs, the potential prevention of these conditions is an important strategy for alleviating their burden. Potentially modifiable risk factors for 14 NCs were extracted by the Synthesis Panel from the catalogue of statistically significant associations as determined by the Krewski risk factors team. They are listed here by NC.

It is important to note that an association does not mean that the presence of the risk factor is a cause of the NC, or that absence of the risk factor would guarantee that an individual would not develop the NC. Furthermore, these risk factors may only be associated with a small percentage of the cases of a specific NC or specific populations affected by a NC.

AD/dementia: Head injury in males, depression, diabetes mellitus, conjugated equine estrogen use with medroxyprogesterone acetate, pesticides, electromagnetic field exposure, current smoking and lower social engagement.

27TSynthesis Panel Comment:27T Depression, mild cognitive impairment and social isolation may be early manifestations of AD/dementia rather than risk factors.

ALS: Exposure to pesticides.

Brain tumours: Birth weight >4,000g, maternal consumption of cured meats during pregnancy (children); pesticide exposure (children and adults); hairdressers and related workers. Protective effects of asthma, eczema and overall allergy for glioma. No consensus on association between brain tumour and mobile phones.

Cerebral Palsy: Low arterial cord pH, clinical chorioamnionitis, late preterm gestation age, gestational age, birth weight and multiple gestation/births.

Dystonia: A group of genetic disorders; see 4.2

Epilepsy: Children - Maternal infection, eclampsia and preeclampsia, maternal smoking, preterm birth; Adults - alcohol abuse, traumatic brain injury.

27TSynthesis Panel Comment: 27TStroke, brain tumours, CNS infections as well as several metabolic and degenerative brain diseases are also risk factors for recurrent seizures.

Huntington's Disease: A genetic disorder; see 4.2

Hydrocephalus: Congenital onset: maternal obesity, multiple births, maternal parity, S,S maternal thyroid disease, preterm birth. Adult onset: hypertension, ischemic heart disease.S

Multiple Sclerosis: low serum 25(OH)D levels/sunlight exposure, Epstein-Barr virus infection and smoking.

Muscular Dystrophy: A group of genetic disorders; see 4.2.

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INTERIM VERSION – NOT FOR PUBLICATION Parkinson's Disease: Pesticide exposure. Protective effect of coffee drinking and cigarette smoking. SP Comment: Rural living, drinking well-water, and farming occupations were not included in the list of

potentially preventable factors because they are considered to be related to pesticide exposure.

Spina bifida: Lack of folic acid intake during pregnancy, paternal exposure to Agent Orange, maternal obesity prior to pregnancy, mothers exposed to chlorinated solvents, high levels of magnetic fields, anaesthetic gases, or organic pollutants, and fathers exposed to welding fumes.

Tourette syndrome: Family studies support a genetic cause; see 4.2. The severity of the tic disorder correlates with the occurrence of a range of pregnancy complications.

Traumatic Brain Injury: The use of helmets in bicycling, skiing, snowboarding, ice hockey and motorcycling is protective.

4.2 Identified Genetic Factors for Neurological Conditions NCs that are caused by mutations of single genes can be identified by highly predictive genetic tests so their recognition as genetic risk factors has considerable prognostic value. Other NCs result from the interplay of susceptibility genes and toxic or environmental factors (often unidentified). The systematic reviews of the Krewski Risk Factors project identified the following genetic factors that are associated with the onset of NCs:

AD/dementia: Genetic risk factor for susceptibility - APOE e4 allele. Synthesis Panel Comment: Mutations in 3 genes (APP, PSEN1, PSEN2 or TREM2) or duplication of the APP

gene are found in less than 1% of all AD patients

ALS: Genetic risk factors of uncertain role - SOD1 and C9ORF72 mutations, ATAXIN-2 gene variants. Synthesis Panel Comment: Even for the familial forms of ALS, identified gene abnormalities are rare: only about 20% of patients with familial ALS, which make up 10% of all ALS patients, have the SOD1 [superoxide dismutase] mutation.

Dystonia: More than twenty different monogenic forms of dystonia have been identified.

Epilepsy: Family history of epilepsy or seizures. Synthesis Panel Comment: Mutations of ion channel or neurotransmitter receptor component genes are found in about 1% of primary generalized epilepsy.

Huntington's Disease: A dominantly inherited disorder cause by a mutation of the Huntingtin gene that results in excessive trinucleotide (CAG) repeats. There is an inverse correlation between the age at onset and the length of the CAG repeats.

Multiple Sclerosis: Genetic risk factor for susceptibility - HLA-DRB1*1501 allele. Synthesis Panel Comment: As many as 25% of patients with multiple sclerosis have a relative with the condition; about 5% are first-degree relatives.

Muscular Dystrophy: A group of inherited muscle diseases with genotypically and phenotypically distinct characteristics. Duchenne muscular dystrophy is caused by mutations of the dystrophin gene, which is transmitted by sex-linked recessive inheritance.

Parkinson's Disease: Family history and several genes listed in the PDGene database were positively associated with PD. Synthesis Panel Comment: Mutations in the Parkin2 gene, which cause a familial form of 35TParkinson's disease35T known as autosomal recessive juvenile Parkinson's disease, were not mentioned in the Krewski Report.

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INTERIM VERSION – NOT FOR PUBLICATION Tourette syndrome: The familial nature of Tourette syndrome is well established, but the precise genetic

mechanisms have not been determined. While genetic studies have found rare mutations that appear responsible for the phenotype in certain families, a single genetic mutation that causes Tourette syndrome in a large proportion of individuals has not been found. Tourette syndrome is likely a genetically heterogenous disorder with many possible susceptibility genes.

4.3 Contribution of the NPHSNC to Risks for Progression of Neurological Conditions

It is recognized that studies of risk factors for disease progression are exceedingly difficult to do. It is often impossible to disentangle risk factors for onset from determinants of progression and rate of progression. To do so requires serial measurements of patients over time, starting from before they developed the condition and lasting until the condition is advanced. Most studies are cross-sectional and some NCs may have an insidious onset so that they identify cases at different stages in the natural history of the condition. When a study searches for risk factors, therefore, it may conflate those for onset with those for progression to the stage at which each person was diagnosed.

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INTERIM VERSION – NOT FOR PUBLICATION Knowledge Gaps regarding Risk Factors for Neurological Conditions 39F

40 a. To support advocacy for preventative actions, the potentially modifiable risk factors for the onset of NCs need

to be expressed in terms of population attributable risk as well as relative risk (see Glossary).

b. The clinical or public health relevance of the statistically validated risk factors needs to be evaluated by condition-specific clinicians and scientists.

c. To provide information that could slow or halt the progression of NCs, further studies of the risk factors for progression are necessary.

Themes emerging from the NPHSNC regarding Risk Factors

a. The statistical analysis of risk factors for the onset of NCs is only the first stage in the process of identifying risk factors that are potentially responsive to preventive measures.

b. There is a complex interplay between genetic and environmental factors that needs to be recognized when assessing risks for individuals and their family members.

c. There are substantial methodological challenges involved in the identification of risk factors related to disease causality. In many instances, the appropriate studies have not been done.

d. Risk factors for disease progression require detailed and expensive longitudinal studies of "at risk" individuals throughout the earliest and advanced stages of the condition will be required.

40 The Krewski Risk Factors Project team is carrying out the analyses and consultations to address the first two gaps listed.

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5. Microsimulation Because the occurrence of the more common neurological conditions (NCs) increases with age (e.g. Alzheimer's disease, dementia, Parkinson's disease, stroke), the number of people with these conditions is projected to rise as Canada’s population ages. Consequently, the direct and indirect costs associated with these conditions will also increase. To estimate the extent and the costs of these increases over the next 20 years, a Microsimulation Project is underway at the Public Health Agency of Canada (PHAC). When completed, this study will provide projections pertaining to the burden and cost of several NCs over the next 5, 10, 15, and 20 years.

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Tables Synthesis Report (SR) Tables SR Table 1 - HUI Scores for Neurological Conditions in the SLNCC Population (appended)

SR Table 2 - Health Care Costs in British Columbia, 2010/2011 (in text)

SR Table 3 - Prevalence Estimates of Neurological Conditions from Three Sources (appended)P40F

41P

SR Table 4 - Incidence Estimates of Neurological Conditions from Two Sources (appended)

SR Table 5 - Prevalence Estimates for Four Neurological Conditions from Different NPHSNC Projects

SR Table 6 - Prevalence and Incidence of Neurological Conditions from Different Sources (in text)

SR Table 7 - Sources and Quality of Ascertainment Methods Identified for Neurological Conditions by Systematic Reviews

SR Table 8 - Prevalence Estimates of Neurological Conditions in Long-term Care Facilities (appended)

41 Confirmation of some data in this table by the PIs is pending.

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Appendices SR Appendix 1: Synthesis Panel Members Garth M. Bray, MD, Professor Emeritus, Departments of Medicine and of Neurology & Neurosurgery, McGill University Expertise: Clinical neurology, laboratory investigation of neuronal responses to injury Chair, Synthesis Panel

Elizabeth Donner, MD, Associate Professor, Department of Pediatrics, University of Toronto Pediatric neurologist, Hospital for Sick Children Expertise: epilepsy and other neurological conditions in children

Lesley K. Fellows, MD, Associate Professor, Department of Neurology and Neurosurgery, McGill University Neurologist and investigator, Montreal Neurological Institute and Hospital Expertise: cognitive disorders

Ian D Graham, PhD, Professor, School of Nursing, University of Ottawa Senior Scientist, Centre for Practice-Changing Research, The Ottawa Hospital Research Institute Expertise: clinical epidemiology

Colleen Harris, RN, MN Nurse-coordinator, nurse practitioner, Multiple Sclerosis Clinic, Foothills Hospital, Calgary, Alberta Expertise: multiple sclerosis

Carley Hay, MHSc Senior Specialist, Chronic Disease Management and Prevention Unit, Ontario Ministry of Health and Long- Term Care Expertise: epidemiology; policy and research related to chronic disease management and prevention; health system performance measurement

David Hogan, MD, Professor and Chair, Geriatric Medicine, University of Calgary Expertise: clinical aspects of aging

Katharina (Kathy) Kovacs-Burns, PhD, Professor, University of Alberta Director, Interdiscilinary Health Research Academy, Edmonton Expertise: interdisciplinary health research, qualitative health research

Lisa Lix, PhD, Professor, Department of Community Health, University of Manitoba Director of the Data Science Unit, the George and Fay Yee Centre for Healthcare Innovation Co-chair of the Science Committee of PHAC’s Canadian Chronic Disease Surveillance System. Expertise: health services research and methodology

Ian McDowell, PhD, Professor of Epidemiology and Community Medicine, University of Ottawa Expertise: health research methodology, epidemiology of dementia

Thomas J. Murray, MD, Professor Emeritus, Dalhousie University Former Dean of Medicine, Dalhousie University Director, Dalhousie Multiple Sclerosis Research Unit Expertise: multiple sclerosis, community health

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INTERIM VERSION – NOT FOR PUBLICATION Scott B. Patten, MD, Professor, Departments of Community Health Sciences and Psychiatry, University of Calgary Expertise: epidemiology of mood disorders Vice-Chair, Synthesis Panel

Christel Renoux, MD, PhD, Assistant Professor, Department of Neurology and Neurosurgery, McGill University Expertise: neuro-epidemiology, clinical neurology

Christopher A. Shaw, PhD, Professor, Department of Ophthalmology & Visual Sciences, University of British Columbia Expertise: genetic and environment risk factors for neurological conditions

A. Jonathan Stoessl, MD, Professor & Head, Neurology, University of British Columbis Director, Pacific Parkinson's Research Centre & National Parkinson Foundation Center of Excellence Expertise: Parkinson's disease

Robyn Tamblyn, PhD, Professor, Departments of Medicine and of Epidemiology & Biostatistics, McGill University Scientific Director, Clinical and Health Informatics Research Group, McGill University Scientific Director, Institute of Health Services and Policy Research, CIHR Expertise: health policy research, determinants of prescription drug use, interventions to improve drug safety

Charles Tator, MD, PhD, Professor of Neurosurgery, University of Toronto Founding Director, Think-First Canada, an injury-prevention advocacy organization Expertise: brain and spinal cord injuries, laboratory investigation of spinal cord injury

Terry Lynn Young, PhD, Associate Professor, Discipline of Genetics, Memorial University of Newfoundland Expertise: genetics of hearing loss and pediatric epilepsy

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INTERIM VERSION – NOT FOR PUBLICATION SR Appendix 2: Implementation Committee Members Celina Rayonne Chavannes Director, Research Initiatives, NHCC Implementation Committee Co-Chair

Sulan Dai Senior Epidemiologist, Chronic Disease Surveillance Division, PHAC

Frances Gardiner Program Officer, Chronic Disease Surveillance Division, PHAC

Nathalie Gendron Assistant Director, Institute of Neurosciences, Mental Health and Addiction, CIHR

Joyce Gordon President and CEO, Parkinson Society Canada President, NHCC

Deanna Groetzinger Vice-President, Government Relations and Policy, MS Society of Canada

Deanna Huggett Senior Policy Analyst, Chronic Care Unit, Health Canada

Susan Latter Stakeholder Representative

Suzanne Nurse Executive Director, Canadian League Against Epilepsy

Jay Onysko Manager, Chronic Disease Surveillance and Monitoring Division, PHAC Co-Chair, Implementation Committee

Nalini Sen Director, Research Programs, Alzheimer Society of Canada

David Taylor Director of Research, ALS Canada

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INTERIM VERSION – NOT FOR PUBLICATION SR Appendix 3: Synthesis Panel Comments on Risk Factors for Neurological Conditions41F

42 In the sense that the term is used in this report, risk is a proportion or frequency that must assume a value between 0 (no risk) and 1 (certainty). In this instance, risk refers to the proportion of people with a certain characteristic (or ‘risk factor’) who develop a neurological condition. Like all proportions, risk has a numerator (the number of individuals developing the disease during a specified interval of time) and a denominator (the total number of individuals at risk, i.e. limited to individuals who don’t already have the outcome at the start of the specified risk interval).

The impact of a risk factor is usually identified by a Urelative riskU (the ratio of risks in those “exposed" to those "not exposed" to the factor) or relative rate (rate ratio) and sometimes by an Uodds ratioU. For both of these ratios, a value close to 1.0 indicates no impact of the risk factor; a value of 2 would indicate a doubling of risk among those exposed, and so forth. For the estimation of the impacts of identified risks on a population (e.g. Canadians), it is important to consider Upopulation-attributableU risks in addition to Urelative Urisks. (A high relative risk may indicate that a factor is devastating when it occurs, yet its occurrence may be so infrequent that its impact on population health may be minimal.)

It is also important to recognize that a characteristic or behaviour identified as being UassociatedU with a disease or condition is not necessarily a UcauseU of that disease or condition. A risk or rate ratio (or difference) may be elevated as a result of artifactual intermixing of effects (e.g. the risk of lung cancer may be elevated in people who drink a lot of coffee, but only because people who drink a lot of coffee also tend to smoke more). Hence, a simple ratio of risks or rates does not necessarily quantify a causal effect. While some would designate such a factor as a risk factor in the sense of identifying a person at elevated risk, others would call this a Urisk indicatorU or marker to distinguish it from a causative factor. Risk indicators are useful for identifying populations at increased risk, which may trigger further studies to identify the actual causal factors.

Various statistical methods can partially help to distinguish between causes and associated factors, but some purely logical criteria for identifying a true cause must also be met. These include the extent to which a study can clarify the temporal sequence of exposures (cause must precede effect), the biological plausibility of a causal explanation, as well as its strength, consistency and coherence with existing literature. Systematic reviews and meta-analyses can address some, but not all, of these criteria for identifying a causal link.

Systematic reviews help to evaluate the consistency of reported findings, and by application of quality review criteria, can eliminate studies with serious methodological flaws. But systematic reviews do not automatically address the challenge of distinguishing risk indicators from causal factors. The advantage of meta-analyses is that by pooling results of homogeneous studies, meta-analysis can improve on the precision of estimates arising from individual studies. However, it is not realistic to expect that a systematic review would provide a final and decisive statement about the etiology of NCs. This requires, in addition to the statistics, a clear conceptual interpretation of the mechanisms underlying the observed relationship.

Following discussions with members of the NPHSNC Scientific Advisory Committee and Synthesis Panel, the Krewski research team agreed to have the clinical and contextual relevance of the statistically identified risk factors assessed by experts for each NC, to indicate those studies that distinguished risk factors (with supposedly etiological connotations) from risk indicators, and to report population attributable risks which are required to indicate the impacts of the identified risks on the Canadian population. The results of these additional

42 This discussion of risk factors was prepared by Scott Patten and other members of the Synthesis Panel

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