Evidence-Based Practice with Community Participation: Select experiences with patients with spina bifida and sickle cell disease

Robin R. Leger, Ph.D., RN, Linda Wagner, Ed.D., RN, and Victoria Odesina, MS, PHCNS-BC, APNG

Abstract

This article will use select experiences in patients with spina bifida and sickle cell disease to illustrate sources of evidence-based practice. Four sources of evidence that can be appraised when developing clinical and/or public policies for individuals with intellectual and developmental disabilities include: published research, clinical knowledge, patient and family experience, and local data. Community-Based Participatory Research is defined and promoted as both a principle and method. A lifespan approach to clinical research in two populations will highlight examples of mutual mentorship, collaboration across disciplines and agencies, and the development of clinical practice guidelines, protocols, and policy development.

Keywords: developmental disabilities , intellectual disabilities , spina bifida , evidence-based practice , community-based participatory research (CBPR) , research methods , instruments , nursing research , sickle cell disease

INTRODUCTION

Nurses with expertise in intellectual and developmental disabilities (I/DD) can play key roles in the design, implementation, and dissemination of evidenced–based research in this area of health care. Nurses gain expertise in I/DD from a wide variety of practice environments and contribute skills and knowledge in collaboration with other disciplines, and across settings and agencies to enhance and improve the care of people living with I/DD. Inter-disciplinary collaboration allows for rich input into the identification of research questions and lends capacity to implement protocols, build data base and registries to support outcomes, and disseminate practice recommendations broadly. Mentoring can provide opportunities for students and colleagues to work together and can be beneficial in identifying new and supporting evidence and further research strategies.

This article supports the premise that there are multiple sources of knowledge that inform evidence-based practice to encourage the development of best practice through the establishment of diverse research teams, mentoring students, promoting community-based participatory research (CBPR), and dissemination efforts. A select review of related concepts and measures used in I/DD research will be identified. The authors will share their experience in collaborating on research programs of study with a lifespan approach to developmental disabilities, in particular with individuals living with spina bifida (SB) and sickle cell disease (SCD). The challenges, opportunities, and use of evidence for changing clinical care, community roles, and policy formation will be highlighted.

Evidence-based practice

Evidence-based practice has emerged in recent years as a way to provide optimal patient care through application of the best available research findings. Research based on clinical issues has continued to inform changes and improvements to patient care. The formal process of examining a body of research, critical review of the findings, and development of clinical guidelines or protocol development has led to improvements and changes at the bedside and into the community over the years. Sigma Theta Tau (1), the International Honor Society of Nursing has established a position paper on evidence-based nursing (EBN). It defines EBN as "an integration of the best evidence available, nursing expertise, and the values and preferences of the individuals, families, and communities who are served." This definition works well within the philosophy of care across the lifespan for individuals and families with I/DD wherein, nursing, family, and community-based organizations (CBOs) expertise are integral to investigation, analysis, dissemination, and provision of the best possible care.

There are a variety of terms that have been used in nursing practice over the years that involve reviewing research for its application to nursing practice. Best practice and clinical practice guidelines are two terms that may be familiar. Professionals who work to advance health care face increasing expectations that the care they provide is clinically effective and cost efficient. Studies are reviewed for their contributions to change and improvement of practice. In evidence-based practice, a clinical question is identified, a search is performed for the best evidence to address clinical problems, and an evaluation/integration of the findings is done along with integration of clinical expertise, patient input, and existing resources (2).

There are several models that can guide utilization of evidence-based practice. The Iowa model (3) and the Stetler model (4) are two of the more common models used in clinical practice settings to guide research utilization at the bedside (2). Both models have been updated over the years and offer specific steps to facilitate research adaptation to bedside practice. A challenge to implementing evidence-based practice is the ability to influence change to improve care. Both the Iowa model and the Stetler model emphasize steps in the process that address applying the research to practice. This involves influencing practitioners and the organization to change well-established procedures. While change may be difficult to implement at the organizational level, it may prove even more of a challenge to individuals and families in the community setting (5).

Rycroft-Malone and colleagues (6) write a compelling piece challenging professionals to consider four sources of evidence when evaluating for best practice and the nature of evidence. These four sources are listed as research, professional knowledge/clinical experience, "local" data and information, and patients' and caregivers' experience and preferences. They state that "[t]he delivery of individualized evidence-based health care not only requires professional craft knowledge and reasoning, but requires such knowledge and reasoning to integrate the four different types of knowledge discussed here within the contextual boundaries of the clinical environment" (p.84). Rycroft-Malone and colleagues defines craft knowledge as knowledge gained through clinical and life experience (6) The examples provided in this discussion are drawn from the expertise of the client/family and community resources as well as the expert knowledge of the professionals and available research to create the context of "person/patient-centred" evidence-based care.

Sensitivity to the cultural, social, and economic contexts that affect individuals with developmental disability is imperative to synthesizing the empirical evidence used to improve care. Nurses whose focus is caring for children have developed a clinical expertise that, when integrated with best practice research, are in a unique position to advocate for optimal care for individuals with I/DD and their families in the context of their day-to-day life. The authors have found that a consideration of the four aspects of the model described by Rycroft-Malone and colleagues offers an alternative to the more standard models such as the Stetler and Iowa models described above. A key to increasing the application and dissemination of research findings is to involve key constituents. This is where community-based participatory research (CBPR) may prove beneficial.

BACKGROUND AND SIGNIFICANCE

Individuals and families whose lives are impacted by disability are under-studied as noted in the Americans with Disability Act, 1990, PL 101-336 and by the World Health Organization (7). There has been a growing need for a lifespan approach to pediatric onset disability with a framework that is continuous (8) and assessment tools that carry forward from the pediatric culture and are understood by adult health care providers to whom they will be referred (9). As more people with I/DD survive into adulthood, it has become clear that for many this transition, including the transference of care to adult health and community-based services, is problematic. For many people, this period results in a potential loss of follow-up care, leading to exacerbation of conditions or new secondary disabilities that co-exist with the adjustments to new sources of services and funding.

Spina bifida and sickle cell disease are conditions with high risks for intellectual and developmental disabilities. Both are genetic, chronic health conditions with risk for secondary health problems (such as neurological, orthopaedic, renal, and pulmonary complications), unanticipated exacerbations of conditions, hospitalizations, and the need for complex management that may delay growth and psycho/social/educational development. Approximately 85% of people living with SB have associated hydrocephalus, which often requires shunting that can lead to associated learning disabilities and/or cognitive impairments (10). Motor disabilities in SB are due to spinal cord impairments (potentially requiring adaptive equipment such as crutches, braces, wheelchairs, etc.), and issues of bowel and bladder incontinence lead to difficulties in achieving psycho-social-recreational and vocational goals.

SCD is a condition where the risk of associated disabilities increases with age. Available evidence suggesting that the quality of health care and prevention is substandard due to lack of knowledge and challenges accessing services. Sensory, mobility, and/or cognitive impairments create barriers to the chronic care that people with SCD and disabilities often need. The incidence of stroke in children with sickle cell disease due to vasooclusive episodes is 8%, and unknown silent infarcts or micro strokes may lead to decreased neurocognitive development and declining IQ scores in those living with SCD (11). Despite the need for early recognition and management, cognitive impairments, neuro-psychological dysfunction, or abnormal findings on magnetic resonance imaging were noted in nearly two-thirds of 138 adults, with one third having IQ scores below 86. This was identified as a major contributing factor for non-adherence to treatment regimens (11). Pubertal changes with increased physical demands, school absenteeism, social isolation, and stigma of illness are among other components that make living well and growing into adulthood with SCD or SB a challenge.

The Spina Bifida Association; the Sickle Cell Disease Association of America; the Genetic Alliance; the National Heart, Lung, and Blood Institute; the U. S. Department of Health and Human Services, Health Resources and Service Administration (HRSA); Maternal and Child Health Bureau; and consumer, family, and professional organizations, along with other associated professional and consumer organizations worldwide have made the understanding of the health and social needs of young adults with chronic disabling conditions a priority. The American Academy of Pediatrics, the American Academy of Family Physicians, and the American College of Physicians-American Society of Internal Medicine published a position paper in 2002 on the transition issues of adolescents with special health care needs (12). In a consensus statement, the three medical boards contend that adolescents and young adults "with severe medical conditions and disabilities that limit their ability to function and result in complicating social, emotional, or behavioral sequelae experience difficulty transitioning from child to adult health care" (p. 1304). These position statements, though limited in empirical data or specific strategies, are valuable as they identify a priority for health care research and delivery.

RESEARCH STRATEGIES

Interdisciplinary and Interagency Collaboration and Mentorship

Whether looking at research in the literature to help appraise current practice, implement new practice guidelines or policies, or formalize research questions in the practice arena, individuals or teams need to reflect on the opportunities, resources, potential partners, and obstacles in their environment. Strategies for evidence-based practice can rely on the universal and timeless questions:

• Who? - research team
• For whom? - individuals, families, or communities; sample or population
• What? - evidence: problem, best practice, phenomenon, experience, etc.
• So What? or Why? - Will the results obtained matter or make a difference? Is the study worthy of time, resources, or effort?
• Where? - clinical environment(s) or community(ies)
• When? - timelines
• How? – methods, finances, and dissemination

Collaboration with different disciplines - among nurses using a consortium model (13), with students, or across agencies - brings together individuals with a variety of skills and resources who can share the work, the joys, and the frustrations. Meleis and colleagues (14) posit that "collaborative mentorship is an active, dynamic approach that demonstrates solidarity …the features of collaborative mentorship are: negotiated relations, mutual interactions, facilitative strategies, and empowerment" (p. 179). Mutual mentoring across disciplines and agencies allows for true collaboration and for evidence to be shared and validated. Using this collaborative model is a complex activity that necessitates awareness of the multiple ways of considering evidence to improve care. The following sections will discuss some of these considerations.

Community-Based Participatory Research (CBPR): A guiding principle and methodology

The community is a group of people sharing a common health interest but not necessarily a particular geographic association (15). To an outsider, it may seem that patients with chronic and developmentally disabling conditions receive most of their care in the health care setting; however, the patients' communities provide the majority of the health care management and support services. The patients' communities in this context are the people providing care to the patient around the clock and over extended periods: care givers; family members; school systems; advocacy groups; social workers; community-based organizations; advocates; home care/health assistants; and religious/spiritual support members.

Community-Based Participatory Research principles and methods are particularly well suited to nurses interested in EBP in I/DD because the approach aids in addressing the problems of health care disparities and breaks down the barriers between researchers and those being researched (16). It is an approach that empowers participants, enhances the capacity of CBOs to function as co-investigators, and potentially leads to sustainability of initiatives. CBPR also positively affects the quality of evaluations because community input leads to the development of culturally appropriate measurements and instruments and fosters a mutual trust that enhances the quantity and quality of data (17). A variety of quantitative, qualitative, and/or mixed methods can be used in both undertaking research and in evaluating the CBPR process (18).

The National Institute of Health has recently promoted a new roadmap, "Blue-Highways," identifying that practice-based research; the use of practice-based networks may be the best setting and process to study questions related to chronic health conditions (19). Enhancing treatment services across health care delivery settings; emergency departments, hospitals, primary and specialty care clinics or community environments will only occur on a sustained basis if the community affected by I/DD is empowered to play a leadership role in the planning, implementation, and evaluation of services.

RESEARCH METHODS

Qualitative Inquiry

Qualitative research is a primary tool that may be used to explore the experience of adolescents or adults with I/DD and their significant others/caregivers. Grounded theory as a research methodology seeks to understand a social process in the participant's own terms and has direct application to the participants involved (20). In addition, factors such as focus on human experience, careful attention to the context of human behavior, and data-rich description yield additional support for a qualitative approach (20). It is not the intent of a qualitative study to test a hypothesis, but rather to generate a conceptual or theoretical model that explains the experience under study (2).

Qualitative research occurs in the environments in which clients and families live. Collaboration with community-based organizations affords the opportunity for health care professionals to develop relationships, build trust, and illicit support for participation. Interviews and observation often take place in the clients' home or community. Qualitative research in the area of I/DD informs practice through attainment of a greater understanding of what children, adults, and families' experience. Phenomenology is another qualitative methodology whose focus is the understanding of one's experience. Since children with I/DD are often overlooked by clinical researchers for a variety of reasons, both grounded theory and phenomenology as qualitative methods provide health care professionals with a richer understanding of what families and individuals with a disability experience on a day-to-day basis.

The current trend toward the use of evidence-based practice in health care settings has called into question the significance of qualitative research in the hierarchy of different levels of clinical evidence Several authors (5, 21, 22) have begun to address the complexity of several "ways of knowing," one of which includes qualitative research. Parse (21) recognizes that findings from qualitative research studies "offer different information from the standardized information that counts as evidence for the extant evidence-based practice scenarios" (p.47), while Jack (5) advocates for both quantitative and qualitative research in the public health setting to improve practice and address complex social and health issues.

Wagner and colleagues are currently conducting qualitative research in CBOs about the experiences of young women with sickle cell disease as they transition to adulthood and the effects of the transition on the women's families. Similarly, medical students under the guidance of Leger and Wagner are conducting a study about the transition experiences of youth with chronic physical and developmental disability. The CBOs' collaboration to identify clients and work with researchers to support the design and successful completion of the studies will help to ensure relevant results.

Quantitative Concepts and Measures for Use in I/DD

A number of approaches and databases are available to those who are conducting literature searches to identify potential research instruments or to support practice (23). One useful database, Health and Psychosocial Instruments (HAPI)© under Ovid Technologies Field Guide, will, for example, search the literature only for articles that have used measurement instruments such as questionnaires, surveys, checklists, rating scales, etc. (24). The search will identify the instruments, authors of the instruments (original source and year first published), means of locating the instruments, and key elements such as sample characteristics and other related measures. One can also search by concept ("hope"), condition ("spina bifida"), and/or run a combined search to cross reference for measures that may then be evaluated for applicability to one's research project or current practice. The following are lists of concepts that can be entered as keywords or phrases (with and without specific I/DD conditions) for use in conducting a search to support clinical practice or a new clinical investigation.

Physiologic Measures (select):

• Vital signs
• Growth and development
• Improved functional status; ADLs; range of motion; strength (power); gait; ambulation
• Clinical measures; laboratory values; function studies (pulmonary, renal, orthopaedic, neurological); diagnostic imaging; pain; oral and dental health; etc.
• Secondary conditions and comorbidities (as confounding or contributing variables)

Quantitative Measures of psychosocial patient and family concepts (select):

• Quality of Life (QOL) or Health-Related Quality of Life ( HRQOL)
• Functional status
• Health status or Severity of illness
• Emotional health status or wellbeing
• Family functioning; stress; resources; social support
• Pain
• Symptom management
• Self-concept
• Satisfaction
• Attitudes (individual, family, or staff)
• Performance indicators: academic; vocational; employment; athletic and/or recreational
• Stigma
• Uncertainty
• Hope

Select Measures: Examples and Applications of Evidence-Based Practice

Example 1. Latex allergy survey in spina bifida: From rare phenomenon to global policy and practice changes

A nursing consortium, the North East Myelodysplasia Association (NEMA), discussed anecdotal accounts of latex allergy experienced by patients in their clinics and one published report of anaphylaxis in two patients (25), which prompted five nurses to survey their spina bifida clinic populations on the incidence, experiences, and symptoms related to latex allergy. The results of this survey indicated the phenomenon was not just a few anecdotal experiences or case reports but a far more prevalent and life-threatening condition needing immediate dissemination and action (26). The nature and prevalence of latex allergy among children and adults with spina bifida, items in the hospital and community environments containing latex and safe alternatives, routes of exposure, and risks for health care providers, informed latex allergy policies and practice changes (27, 28). Nurses were key leaders in the evidence-based practice changes that prompted additional research and the implementation of latex-safe products in hospitals and community environments worldwide.

Example 2. Stigma in adults with sickle cell disease and as experienced by their families

Growing up with a disability that may be "visible" or often "invisible," which may affect an individual's growth and development (29); motor speech or sensory function; ability to perform activities of daily leaving; or risk of hygiene or incontinence problems can lead to stigmatization. Young people with I/DD often display social and developmental delays as they may be more isolated from their able-bodied peers due to multiple hospitalizations, absences from school or work, or feelings of being different. The developmental delays or physical conditions may impair one's ability to take part in the full range of activities of social, sexual/reproductive, and vocational mores. Issues surrounding stigma may also lead to problems with accessing services and/or providers. If stigma is defined as a mark of shame, disgrace, disease, or abnormality (30), then the goal for individuals is to receive support in overcoming the characterization of being discounted and achieve self-acceptance and a place in society (31).

The few literature studies that explore the concept of stigma discuss stigmas related to chronic health, disfigurement, and/or potentially life-threatening conditions such as in epilepsy (32, 33, 34). Panter's investigation (34) describes the stigma of epilepsy as "often a daily companion." The investigation results showed a significant negative relationship between stigma perception and an individual's quality of life. In a recent study of 232 adults with SCD, the researchers refer to the condition as "stigmatizing" as they describe the impact of living with SCD leading to higher levels of depression (35). Adults with SCD who frequently experience acute or chronic pain identify that pain episodes are the leading cause for their experience of stigma, barriers to care, limitations in independence, and isolation, warranting the need for and dissemination of best practice guidelines (36, 37, 38). The authors have recently piloted a quantitative, self-reported study, Stigma in Living with Sickle Cell Disease Survey(s), of adults living with SCD and their family care givers in the United States and Nigeria. The data collected from these surveys are being used to implement screening, education, advocacy, and service projects in both countries (39).

Example 3. Health status, functional status, risks for secondary conditions, and health-related quality of life (HRQOL) in spina bifida and sickle cell disease

People living with I/DD may have corresponding complex conditions with multi-system manifestations requiring a lifespan approach to maintain their health status. As they age and go through physical growth and puberty, conditions may worsen, appear, or become life threatening, requiring new medical interventions and increased monitoring to prevent squealea (40, 41). The literature is inconclusive in defining health status or disease severity in many I/DD conditions. Some authors define health status or disease severity as relevant to a patient's current status and or severity of one system (i.e., neurological, pulmonary, seizure status, or pain episodes in SCD). Others use a lifespan or predictive model with a holistic focus that defines health status or disease severity as a multi-system condition with further risks for disability.

Recently, initiatives have focused on a lifespan approach to living well with a disability. The Centers for Disease Control and Prevention (CDC) has reintroduced this mission to promote health and quality of life by preventing and controlling disease, injury, and disability by opening The National Center for Birth Defects, Developmental Disabilities, and Disability and Health in the fall of 2002. The center uses a lifespan model and provides an integrated forum for organizations and researchers concerned with disability. The two primary goals of the United States national health promotion and disease prevention framework, Healthy People 2010 are to increase the quality and years of healthy life, and to eliminate health disparities (42). Specific objectives within this framework address risk for youth with disabling conditions and the need for research on preventing secondary conditions, promoting health, and improving or maintaining HRQOL. Several prediction models of health and functional status, and health-related quality of life are examined in the nursing literature (43, 44), medical literature (45, 46, 47, 48, 49), and dental/oral health literature (50, 51, 52, 53, 54).

Despite these recent initiatives, a number of comprehensive programs and nursing roles have been eliminated or downsized with health care redesign efforts. Healthcare costs associated with supporting multidisciplinary clinics that provide specialty care to I/DD populations have resulted in the closure of many clinics and the loss of nurse specialists' or nurse coordinators' roles (55). The establishment of transition programs to comprehensive, coordinated and a lifespan approach to care for youth and adults living for with spina bifida was undertaken by a team of nurses at three clinics in New England based on evidence in the literature and their clinical experience (56). Ongoing implications for research, development of systems for transitions across the lifespan, promotion of best practice models for caring for people with I/DD with goals of "aging well" and improving quality of life remain a priority for people living with spina bifida and sickle cell disease (56, 57).

SUMMARY AND CONCLUSION

Perry and Weiss (58) sum it up best when they state, "At the heart of it, evidence-based practice means that we question what we do so that we can help clients be the best they can be" (p.171). As health care services continue to demand accountability and quality for the care provided, it is even more imperative for a group of individuals who are often misunderstood (59) for nurses to advance interventions based on research and review of practice; to collaborate with other disciplines and communities of care; and to mentor our future colleagues in order to deliver excellent care and services.

The previous examples highlight that evidence was sought from a variety of sources such as research, professional knowledge/clinical experience, patient and caregiver experience, and local data information/communities of care (6). Likewise, the methodologies used to obtain further data and promote change in both clinical practice and policy formation are a mix of quantitative, qualitative, and community-based participatory research. This article has presented several venues for advancing best practices in the area of care for individuals with intellectual and developmental disabilities. It offers suggestions for a variety of ways professionals and communities can begin to work together to initiate practice guidelines, develop research proposals, or mentor and guide others. Decisions for care should be based on the collaborative, empirical efforts of individuals and communities whose ethic is to provide for the best quality of life possible for their clients and families.