Quality of Life, Coping, Support Systems and Chronic Pain in. Ehlers Danlos Syndrome

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2 Quality of Life, Coping, Support Systems and Chronic Pain in Ehlers Danlos Syndrome A Thesis Submitted to the Office of Graduate and Professional Studies of University of Cincinnati in partial fulfillment of the requirements for the degree of Master of Science in the Health Promotion and Education Program School of Human Services College of Education, Criminal Justice & Human Services by Shawna Southwick Fox BA in Communication University of Cincinnati 1997 Chair of Committee Liliana Rojas-Guyler, PhD, CHES March 2014

3 ABSTRACT Background: Chronic pain is a common and often disabling manifestation of Ehlers Danlos Syndrome (EDS). This disease is a relatively rare inherited connective tissue disorder characterized by joint hypermobility, skin hyperextensibility, chronic joint and limb pain and both blood vessel and tissue fragility The current study examined adults perceptions of EDS and their perceptions of quality of life, coping, social support and chronic pain. Methods: An observational cross-sectional design was used to assess chronic pain experience, coping behaviors and perceived support descriptive in patients with EDS through a quantitative online questionnaire. There were 1,245 participants who completed the survey and after excluding participants who did not meet the study criteria, total of 949 completed surveys were included for data analysis. Results: A multiple linear regression model was run to assess whether Quality of Life was predicted by Coping and or Support. Findings indicated that perceptions of Coping did not predict Quality of Life, however, Support was a significant predictor. A second model was developed to evaluate if Support and Coping could predict Chronic Pain. Results showed a significant model; however, further analyses indicated that the individual predictors were not significantly related to reports of pain. A stepwise multiple regression was executed to calculate if Quality of Life was predicted by Support, Coping or Demographic factors. Support predicted 26.8% of the variance and Coping an additional 2%. ii

4 Conclusions: Findings of this study indicated that participants who reported higher levels of coping skills and more support reported higher quality of life. Future directions for research should focus on increasing patient support and coping. Investigating the impact of interventions to enhance social support and their impact on patient perceptions will provide important information to facilitate coping of patients with EDS. iii

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6 ACKNOWLEDGEMENTS I would like to thank my committee chair, Dr. Liliana Rojas-Guyler, and my committee members, Dr. Amy Bernard and Dr. Laura Nabors for their guidance and support throughout the course of this research project. Thanks also go to my friends, colleagues and the department faculty and staff for making my time at the University of Cincinnati a truly wonderful experience. I also want to extend my gratitude to the Ehlers Danlos community who were both supportive and willing to participate in the study. v

7 NOMENCLATURE EDS EDS-HT RA MS AD SOC QOL HRQOL IOM NIH GCPS FACT-G FACIT CPCI PSEQ Ehlers Danlos Syndrome Ehlers Danlos Syndrome Hypermobility Type Rheumatoid Arthritis Multiple Sclerosis Acceptance of Disability Sense of Coherence Quality of Life Health Related Quality of Life Institutes of Medicine National Institutes of Health Graded Chronic Pain Scale Functional Assessment of Cancer Therapy General Functional Assessment of Chronic Illness Therapy Chronic Pain Coping Inventory Pain Self-Efficacy Questionnaire vi

8 TABLE OF CONTENTS ABSTRACT... II ACKNOWLEDGEMENTS... V NOMENCLATURE... VI TABLE OF CONTENTS... VII LIST OF TABLES... IX INTRODUCTION... 1 Chronic Pain... 1 Ehlers Danlos Syndrome & Chronic Pain... 2 Coping and Chronic Pain... 4 Support Networks & Quality of Life... 7 Other Factors Impacting Pain Perceptions... 9 Purpose of the Study... 9 METHODS Recruitment Participants Procedures Instrumentation Data Analysis RESULTS Ehlers Danlos Syndrome Diagnoses Chronic pain Experience Quality of Life Support Coping Inferential Statistical Analyses DISCUSSION Implications for Health Education Practice REFERENCES APPENDIX A vii

9 APPENDIX B APPENDIX C APPENDIX D APPENDIX E... 1 APPENDIX F viii

10 LIST OF TABLES Table 1: Demographic Characteristics Table 2: Education, Employment, Income, & Insurance Characteristics Table 3: Living Environment Table 4: EDS diagnoses Table 5: Chronic Pain Experience Table 6: FACT-G Quality of Life Measure Table 7: Total Quality of Life Scale Scores Table 8: Support Scale Scores Table 9 Coping inventory- Guarding, Resting, Asking and Relaxing Subscale Scores.. 48 Table 10 Task Persistence, Exercise and Stretching, Seeking Social Support, and Coping Self-Statements Subscale Scores Table 11 List of Organizations invited to participate in recruitment process ix

11 INTRODUCTION Chronic Pain Chronic pain affects an estimated 116 million American adults, more than the total affected by heart disease, cancer and diabetes combined and costs the nation up to $635 billion dollars each year in medical treatment and lost productivity (IOM, 2011, p. 367). The National Center for Health Statistics estimates that one out of four persons in the U.S. report some type of chronic pain (Meghani & Cho, 2009). Chronic pain is defined in the Anesthesia Guideline for Chronic Pain Management as episodic or persistent pain of a duration or intensity adversely affecting the function or well-being of a person (Chodosh et al., 2004). There is a distinct difference between chronic pain and acute pain. Chronic pain is persistent in nature, not something that easily goes away, and generally lasts for more than 6 months. Acute pain, which generally lasts 6 months or less, can be relatively easily defined as serving some useful function (Meisler, 1999). In the case of chronic pain, it no longer serves any useful function and is typically unresponsive to most interventions as opposed to acute pain which has a shorter duration and often responds to given therapies (1999). Interestingly, women are at significantly greater risk for chronic pain than men. Women appear to experience more pain than their male counterparts for the same pathological condition, a greater number of pain sites as well as an increased risk for developing chronic pain and greater likelihood of using analgesics (Poleshuck, Giles & Tu, 2006). Meisler (1999) reported that 93% of those seeking relief for chronic pain are 1

12 women. Other studies have also had a majority of participants who were female. For example, in one Spanish study of musculoskeletal pain, 100% of the members of one group were female while 92% of the members of the second group were female (Rollman & Lautenbacher, 2001). An individuals experience of pain can be influenced by genes, attitudes toward difficult situations, depression, stress, the ability to understand health information as well as other behavioral, emotional and cultural factors (IOM, 2011). In general, pain is the leading reason that people seek out care from their health care providers (Cowan, 2011). Cowan goes on to state, with regard to chronic pain, that the magnitude of the problem is overshadowed by the lack of education, awareness and general understanding surrounding pain and how to manage it on a daily basis (2011). There are three things that EDS sufferers desperately need to feel supported, understood and empowered. Patients, healthcare providers and society as a whole, need to overcome biases and misapprehensions about chronic pain (IOM, 2011). Ehlers Danlos Syndrome & Chronic Pain Chronic pain is a common and often disabling manifestation of Ehlers Danlos Syndrome (EDS). A relatively rare inherited connective tissue disorder, EDS is characterized by joint hypermobility, skin hyperextensibility, chronic joint and limb pain and both blood vessel and tissue fragility (Berglund, Mattiasson, & Nordström, 2003). Reduced elasticity secondary to abnormal collagen in EDS results in joint hypermobility, skin hyperextensibility, tissue fragility and possible ruptures of visceral organs and blood vessels (Voermans, Knoop, Bleijenberg, & Van Engelen, 2010, p. 371). Individuals with EDS have a defect in their connective tissue, the tissue that 2

13 provides support to many body parts such as the skin, muscles and ligaments throughout the body. The fragile skin and unstable joints found in EDS are the result of faulty collagen (EDNF.org, 2012). Collagen is a protein that acts as "glue" in the body, adding strength and elasticity to connective tissue. To date, EDS has been classified in to nine subtypes: Classical (type I or II), Hypermobility (type III), Vascular (type IV), Kyphoscoliosis (type VI), Arthrochalasia (type VIIA or VIIB), Dermatosparaxis (type VIIC), Unspecified (type V), Peridontosis (type VIII) and Occipital Horn Syndrome (type IX). Hypermobile type III (EDS-HT) is the most prevalent and the most potentially disabling while the vascular type IV is the most life threatening as a result of weak internal connective tissue and the inherent risk of aortic dissection. The hypermobility and classic forms are most common; the hypermobility type may affect as many as 1 in 10,000 to 15,000 people, while the classic type probably occurs in 1 in 20,000 to 40,000 people (NIH.gov, 2006). Other estimates state that as many as 1 in 5,000 may be affected by EDS-HT. There seems to be no racial or ethnic predisposition with regard to Ehlers Danlos Syndrome. EDS appears to be more frequent in the female population. Per leading EDS Geneticist Dr. Brad Tinkle, approximately 80-90% of the EDS population is female (Indiegogo.com, 2013). According to Voermans et al. (2010) pain in Ehlers Danlos Syndrome is often chronic and multifactorial in nature and is suggested to have several causes such as being secondary to frequent dislocations, as a result of repeated soft tissue injury or due to numerous operations resulting in peripheral nerve injury (2010). Musculoskeletal pain in EDS can be chronic, severe and debilitating with muscle weakness, muscle cramping and 3

14 fatigue as common associated features (Rombaut et al., 2011). Sacheti et al. (1997) reported that chronic, frequent debilitating pain of early onset and diverse distribution of this pain is a constant feature in individuals affected with different types of EDS. Coping and Chronic Pain EDS is a lifelong condition for which there is no cure. Affected individuals may face social obstacles related to their disease on a daily basis. Some people with EDS have reported living with fears of significant and painful skin ruptures, becoming pregnant (especially those with the vascular type), their condition worsening, losing their job,becoming unemployed due to physical and emotional issues and social stigmatization in general (Medical Dictionary, 2013). All too often, prevention and treatment of pain are delayed, inaccessible, or inadequate. Nearly all EDS patients experience some degree of pain from non-specific to crippling, disabling pain. The complexity of the disease itself, the use of different diagnostic labels and the inadequate education of healthcare professionals regarding EDS-HT may contribute to poorer functional status in patients with EDS-HT (Rombaut et al., 2011, p. 1986). Rombaut et al. goes on to state that often EDS-HT patients experience a delay in diagnosis or a complete misdiagnosis which often results in incorrect treatments, a needless worsening of the disease and further decline in daily function (2011). Differences in clinical expression amongst those with EDS within a family and a difficulty in diagnosing the condition because of skin and joint hyperelacticity are graded traits that are quite likely related to the under diagnosis of Ehlers Danlos Syndrome (Sacheti et al., 1997). It is this under diagnosis of EDS that 4

15 leads to the worsening of symptoms and increase in chronic pain at multiple sites which may inevitably lead to a decline in a person s overall physical function. It is well known that prolonged untreated or under-treated chronic pain can have substantial negative physical, psychological, and social effects, and can also have a disruptive impact on a person s day-to-day life (Tuzun, 2007). Psychosocial difficulties appear to result from chronic pain and disability, avoidance of social activities and relationships, sexual difficulties and reproductive concerns as well as frustration with the medical system (Berglund, Nordstrom, & Lutzen, 2000, p. 112). Berglund et al. also stated that those with EDS experience life-long pain that affects their physical activity, sleep, work and social relations and these individuals tend to report more depression, anxiety, anger and interpersonal problems. Living with a chronic disease can create serious psychological burden for those attempting to cope. Thus, positive coping strategies become a critical skill for people living with chronic pain, as is the case for many people who have EDS. As Peres and Lucchetti (2010) report, Coping is not restricted to one dimension of functioning; it involves virtually every dimension of human functioning: cognitive, affective, behavioral and psychological (p. 331, p. 332). Over time, a high degree of chronic pain and impairment may progress into pain-related fear and anxiety, activity avoidance and depression that further diminish daily functioning and quality of life (Tuzun, 2007). The presence of pain, in association with the absence of a systematic approach for chronic pain management, impacts dramatically on the psychosocial well-being of affected individuals (Sacheti et al., 1997, p. 89). It is for these reasons that the health related 5

16 quality of life (HRQOL) for the vast majority of those who suffer from the chronic pain of EDS is quite poor at present. Health educators can play an important role and have a positive effect on the outcomes these patients experience. Other health related professions have started to address these issues in the professional literature. As Berglund et al. stated, For health promotion in nursing practice, it is important to have insight into the problem areas which may influence the overall life situation for an individual with EDS (2003, p. 776). Specifically health educators can improve quality of life among EDS patients by addressing health educational needs such as condition specific knowledge about their disease, increasing community awareness and support networks, increasing awareness, and self-efficacy of coping mechanisms in the management of chronic pain due to EDS. Planned health education activities and campaigns should provide information to the public and also contribute to a better understanding of how to manage and even prevent musculoskeletal complications of EDS. Besides general public education activities, patient education should be a key area in the priority of pain management (Tuzun, 2007). Further, Tuzun states that it is well known that patients who have accurate information about their health problem are much more likely to involve themselves in disease management and adhere to treatment recommendations. Patients who empower themselves are more likely to achieve a sense of self-actualization and control over their circumstances compared to patients who feel they are at the mercy of their pain and the health care system. It has been shown that the greater the acceptance of the chronic pain condition, the better the quality of life in the form of improved physical, emotional and 6

17 psychological functioning as well as improved work status. To rise above the pain, sufferers must focus on what they can do rather than what they cannot do (Tuzun, 2007). Health educators, through a multi-disciplinary approach, can play an important role in facilitating education programs that lead to self-empowerment over pain for the patient and help improve their overall quality of life. Support Networks & Quality of Life Another way patients can empower themselves is through the development of support networks. People with EDS often report feeling alone, marginalized, strange and misunderstood. In this regard psychotherapeutic interventions in a group context and making contact with patient associations are especially useful (Baeza-Velasco, Gély- Nargeot, Bulbena, Vilarrasa, & Bravo, 2011, p. 1134). Support networks can increase social support and encourage a sense of understanding, which can decrease feelings of isolation and stigma (2011). Support systems have also been found to improve overall quality of life. It can be posited then that increasing quality of life among EDS patients suffering from chronic pain, and the accompanying potential psychosocial ramifications, could also improve quality of life among this population. Innovative community-based strategies are needed to improve early identification and referral for chronic pain. For instance, extending the clinical concept of pain as the fifth vital sign to the community setting, that is, making an assessment of pain as a component of all community health assessment programs regardless of their primary focus, public health professionals may partner in the early identification and referral to resources available in the community to minimize the burden of pain (Meghani, 2009, p. 315). 7

18 The World Health Organization defines Quality of Life as, an individuals perception of their position in life in the context of the culture and value systems in which they live and in relation to their goals, expectations, standards and concerns. It is a broad ranging concept affected in a complex way by the person's physical health, psychological state, level of independence, social relationships, personal beliefs and their relationship to salient features of their environment (WHO, 1997, p. 1). HRQOL is considered to include three functions, physical, emotional, and also social functions; which are measured on how they affect the health status of a person (Rombaut et al., 2011). Rombaut et al. goes on to state that quality of life can be an important indicator of burden of disease, specifically as it comprises subjective perceptions and feelings the patient has about their well-being, their satisfaction, level of functioning and of impairment. It appears the higher the level of pain and the less control the patient has over their daily life, the worse the overall health status and the lower the quality of life. In a study comparing EDS pain to Rheumatoid Arthritis (RA) pain, Rombaut et al. (2011) found that the EDS-HT group had significantly higher levels of pain severity, significantly more interference on daily activities of pain with daily activities, and a significantly lower amount of control over pain and life events compared to the RA group. The results of this study are a clear illustration of the enormous impact of chronic pain on quality of life and its impediments to leading a life of self-actualization. It appears that acceptance of disability has a positive outcome on the overall health status of an individual with Ehlers Danlos Syndrome. According to a 2003 study by Berglund et al., the greater the acceptance of EDS, the better the functional health status, the better the physical health status and the better psychosocial health status. It was found that a 8

19 greater Acceptance of Disability was associated with a higher level of Sense of Coherence and a better functional health status (Berglund et al., 2003). These studies illustrate the link between quality of life issues and pain experience amongst individuals with EDS. Other Factors Impacting Pain Perceptions Two large survey studies demonstrated that socioeconomic status, as assessed by income, education, and housing status, was significantly associated with chronic pain (Poleshuck et al., 2006). Women are at a significantly greater risk for chronic pain than men, which may be explained by the difference in willingness to report pain symptoms due to the differences in socialization of men and women (Poleshuck (2006), Meisler, 1999, Rollman, 2001). Poleshuck also found that those with lower SES levels were more likely to experience chronic pain (2006). Additionally, minorities experience more severe pain when compared with Whites in the general population and African Americans were more likely than Whites and Hispanics to use prescription pain medications, visit a health care practitioner for any type of pain, and were less likely than Whites but more likely than Hispanics to use non-prescribed treatments for pain (Meghani et al., 2009). Based on these findings, one would expect to find higher levels of pain amongst minority women of low socioeconomic status. Purpose of the Study The purpose of the study was to assess the relationship between demographic characteristics, quality of life, coping techniques, social support levels and chronic pain as reported by Ehlers Danlos Syndrome patients. Further, this study aimed to examine the relations among demographic factors, support and coping as predictors of respondent 9

20 perceptions of quality of life. Due to the dearth of professional literature about EDS, specifically relating to quality of life and chronic pain coping strategies, results from this study are predicted to impact the body of knowledge significantly. The aim of this study was to better understand the quality of life, chronic pain experience, coping strategies and support levels within the EDS population in order to identify the role that health education professionals may play in improving quality of life among this population. Three research questions are posited. 1) Do participants who report positive coping strategies and healthy support systems also report higher quality of life? 2) Do participants who report positive coping strategies and healthy support systems also report lower levels of chronic pain? 3) Can quality of life be statistically predicted by demographic characteristics, level of support or coping strategies reported? METHODS A cross-sectional design was used to assess quality of life, chronic pain experience, coping behaviors and perceived support descriptive data in patients with EDS through a quantitative online questionnaire. Recruitment An online search was conducted to identify organizations that provide information and serve as resources for people affected by EDS. The list of organizations can be found in Appendix A. Each organization was sent a letter asking for their involvement by providing a brief scripted announcement about the research opportunity to their membership and the web address of the online survey portal for those interested in 10

21 participating. Participants saw an announcement directly on the organizational website, received the scripted announcement, learned about the study via an announcement from their support group, or by word of mouth. Recruitment flyers sent to organization members can be found in Appendix B. Participants Study participants were people reporting having been diagnosed with chronic pain and EDS, who were 18 years of age or older, who had access to the Internet and were members of a Connective Tissue Disorder serving organization or support group. Participants were able to read and respond in English and agreed to participate voluntarily. Procedures All procedures were reviewed and approved by the University of Cincinnati Institutional Review Board for the protection of Human Subjects. A copy of the letter of approval can be found in Appendix C. People interested in the study visited the survey website and were presented with the Human Subjects Study Sheet (Appendix D). All participants were advised to read all of the information and then if they were in agreement their consent was given by selecting yes and participating in the survey. Once data was collected, responses were coded to remove any identifiers and data was kept in a locked office on a password protected computer. Only the primary researchers had access to participant responses. The data collection period lasted two weeks, during which the announcement of the study opportunity was posted twice, each one week apart. There is no data currently on prevalence of EDS in the USA but utilizing the conservative 11

22 ratio of 1 in 10,000 (NIH, 2006) it was approximated that nearly 32,000 people in the country have EDS. Many of them are not currently diagnosed. Utilizing the power calculator from Statistical Solutions (2013) it was found that reaching a sample of at least 380 would be ideal to maintain a 95% confidence interval. The number of total survey participants was 1,245. Instrumentation The survey was composed of seven sections measuring demographics, general health status, diagnoses, chronic pain, quality of life, support, coping, perceived difficulties and self-efficacy for an overall 171 total items. A copy of the final survey instrument and a description of all study measures can be found in Appendix E. Select sections assessing demographic factors, coping, support, quality of life, and pain experience were used in analyses for the current study. Preliminary pilot tests with a small convenience snow ball sample of EDS patients showed that the length of time to take the survey ranged from 9 to 20 minutes on a paper version. Actual duration of online survey values varied from 12 minutes to just over 1 hour. However the majority of participants completed the online survey between 22 and 35 minutes (55.2%) and had overall average survey duration of 37 minutes. Section one entailed demographic data, consisting of 16 questions. This section captured age, birthdate, country of residence, gender, what year chronic pain was first experienced, and marital status. Additionally, items measured type of residence (own home or apartment, home of a friend, nursing home, etc.) for most of the year, number of members in the household, education, employment status, annual household income, and 12

23 type of insurance coverage. These questions were adapted from the Diabetes Care Profile (DCP 2.0) instrument (Michigan Diabetes Research and Training Center, 1998). Section two addressed general health status and consisted of five questions. The first four questions were selected from the RAND 36-Item Health Survey (Hays, 1994). The first question measured perceived general health status. The participant rated his/her overall health (1= excellent, 2 = very good, 3 = good, 4 = fair, and 5 = poor ). The next three questions provided a brief quality of life measure based on the past four weeks. These three questions are measured on a 6-point Likert scale with 1 = all the time and 6 = none of the time. A fourth question was added to measure perceived level of anxiety in the prior four weeks and participants recorded their responses on the same 6-point scale. Section three referred to diagnoses and asked the participant if he or she had ever been told by a health care provider he or she had any of the 25 listed diagnoses (as well as an open-ended box listed other ) and to check all that apply. The second question in this section asked which of the following types best describes your EDS diagnosis and lists the nine types of EDS as well as, I may have EDS but not sure of what type, I may have EDS but the diagnosis has yet to be confirmed, I don t have EDS, and also lists other (please specify). Section four addressed perceptions of chronic pain. This section had three subsections measuring the participant s pain experience with a total of 12 questions. The first subsection assessed if the participant has had chronic pain and the length of time they had experienced chronic pain. The second subsection identified areas of the body 13

24 where participants experience pain. Next, participants ranked their top three most painful areas. The last pain experience subsection utilized The Graded Chronic Pain Scale (GCPS), which had seven items measuring level of pain and interference with daily activities (Von Korff et al., 1998). Utilizing the Graded Chronic Pain Severity Scale by Von Korff, Ormel Keefe et al. (1992) participants could be categorized into four Pain Grade Level Classifications (Grade I, Grade II, Grade III and Grade IV). Grade I includes people who experience low intensity and low disability; Grade II, high intensity and low disability; Grade III, High Disability and Moderately Limiting Chronic Pain; and Grade IV includes High Disability and Severely Limiting. Assignment of participants into one of these chronic pain grade level classifications was carried out by following the scoring criteria outlined by the authors. Six of the questions are scored on a 10-point Likert scale. Items 1, 2 and 3 measure level of pain with answer choices ranging from 0 = no pain at all to 10 = pain as bad as it could be. Item 4 measures interference on daily activities with 0 = no interference and 10 = unable to carry on any activities. The next two items (5 and 6) measured level of change in daily life due to pain on a scale ranging from 0 = no change to 10 = extreme change. The last item is an open-ended response item asking the participant how many days they had been kept from their usual activities. The scale scores are then computed by conducting a series of calculations such as averaging scores for each participant in each subscale and then assigning a point value to their score. A final assignment to one of four categories is then conducted (Grade 1, Grade 2, Grade 3, or Grade 4 Chronic Pain Classification). The GCPS has been widely utilized and 14

25 its reliability and validity has been well established (Von Korff et al., 1992). One study looking at persons with spinal cord injury showed internal reliability consistency of the GCPS by demonstrating Cronbach s alpha >.90 (Raichle, Osborne, Jensen, and Cardenas, 2006). To further elucidate the validity and reliability of this scale, another study looking at the reliability and validity of pain interference measures in persons with MS found Cronbach s Alpha to be at >.94 (Osborne, Raichle, Jensen, Ehde, & Kraft, 2006). Section five examined perceptions of quality of life. To measure quality of life, we used the Functional Assessment of Cancer Therapy General (FACT-G ) (Webster, Cella & Yost, 2003). The FACT-G measures physical, social/family, emotional and functional well-being. FACIT questionnaires are described as among the most used instruments in national and international research settings. The majority of FACIT measures go through standard scale development and validation processes. These include four specific phases: item generation, item-reduction, scale construction, and psychometric evaluation (Webster, Cella, & Yost, 2003). This section was comprised of a total of 26 items grouped into four subscales; physical well-being, social family wellbeing, emotional well-being and functional well-being. Answers are obtained using a 4- point scale (with a from rage of 0 = Not at all to 4 = Very much ). All FACT-G scores are obtained by summing and averaging individual subscale scores and like the FACIT scales, a high score is good. Section six assessed support factors. This section has a total of ten questions. The first three were written by the authors and asked the participants to report whether they 15

26 had a) shared their chronic pain experience with others; b) with whom they had shared their chronic pain experience and c) who helped/help them the most in caring for their chronic pain. This was followed by an adapted subscale of six questions. The support subscale was adapted from the Diabetes Care Profile (Michigan Diabetes Research Center, 1998). These items are on a 5-point Likert scale and ask the participant to select the best answer for each statement with regard to family and friends in relation to their chronic pain. Per the Likert scale, 1 = strongly disagree and 5 = strongly agree. To ensure that a higher score was equivalent with higher levels of support by family and friends, some questions were reverse coded. Scale scores were computed by summing across all items. The final question, also written by the authors, was what type of health care providers the participants had found most helpful in treating EDS related chronic pain, lists 27 different providers, asked them to select the top five and in an open-ended question then asked which one specialty has been the most helpful. This question was presented with Click and Drag formatting where participants would click on the specialty and drop it toward the top of the list with the intent that only the top five specialties would be ranked. The reliability of the results of this question may be skewed as participants were required to rank five but some participants under the other open ended question explained that they have only seen one or two specialties in total. Thus only the top one or two ranked specialties can be considered as valid responses. Section seven evaluated coping, and asked the participants what things help with pain and consisted of a 42 question Chronic Pain Coping Inventory or CPCI. (Romano 16

27 et al., 2002). To measure coping, we used The Chronic Pain Coping Inventory -42: Reliability and Validity. Certain types of coping, such as the use of coping selfstatements, have been found to be associated with better physical and psychological functioning among patients with chronic pain (Romano et al, 2002, p. 65).This section asked the participant to indicate how many days they used each coping strategy for pain whether or not they were experiencing pain at the time. This section is based on a 7 point Likert scale with 0= no days and 7 = 7 days. As reported by Romano, there is strong validity and reliability within this particular inventory. Looking at the test-retest, evidence was present of acceptable reliability. Pearson s correlation coefficients were <0.91, indicating a high degree of association between the original and 42-item CPCI scales. All correlations were <0.91 with over three-fourths of these coefficients <0.95 (Romano et al., 2002, p. 70). A panel of reviewers was assembled to evaluate the survey for face and content validity. Reviewers included three researchers experienced in psychometric measurements including one with extensive chronic pain research experience. Additionally two medical doctors with expertise in EDS reviewed the instrument and provided feedback which was incorporated into the final instrument. Lastly nine EDS community members tested the instrument and provided valuable feedback. Data Analysis Data was downloaded from Qualtrics (online survey platform) into the Statistical Package for Social Sciences software version 21 for Windows. The data set was reviewed for missing data points, and these were coded as such. Descriptive statistics (frequency, 17

28 percent, mean, standard deviation, etc.) were utilized to evaluate the sample. Reverse coding was done as necessary prior to computing scale scores. Interaction variables were also created. Subscale and scale scores were computed and observed variables were compared using inferential statistics. Specifically, research questions were assessed by running regression analyses. Correlations were utilized to assess variable relationships but also to identify possible confounding variables or interaction effects. All inferential statistical analyses utilized a confidence interval of 95% and an alpha <.05 significance level. RESULTS The results of data analyses presented here include descriptive and inferential analyses related to select: a) demographic variables (age, gender, marital status, education, residence, insurance, and income level); b) chronic pain experience; c) support; d) coping and e) quality of life. Results are presented as they related to answering the three research questions in the present study. A total of 1,245 people followed the online survey link and read the study information sheet, of which 1,241 (99.7%) agreed to be part of the study following the consent process. The completion rate was computed to be 84% (n=1,045). Survey respondents were eligible to be a part of the study if they were 18 years of age, met the criteria and self-identified as having chronic pain and had a physician diagnosis of Ehlers 18

29 Danlos Syndrome. After excluding participants not meeting the criteria above, a total of 949 completed surveys were included for data analysis. Following descriptive analysis, it was confirmed that 100% of the participants were English-speaking. Table 1 details demographic characteristics of the sample. It was also noted that the overwhelming majority were female (n = 902, 95.7%) versus their male counterparts (n = 41, 4.3%). The age range of the respondents was between 18 and 76 with a mean of years (SD = 11.40). More than half of the respondents reported being married or having a life partner (n = 497, 52.7%), however; there were a number of respondents who reported never having been married (n = 318, 33.7%). The largest proportion of respondents had graduated from college or technical school (n = 334, 35.3%), followed by those who completed some college or technical school (n = 279, 29.5%). In terms of employment, the largest proportion of respondents stated that they were disabled by their condition and unable to work (n = 347, 36.6%). The following group reported being out of the paid workforce whether due to being unemployed, laid-off, being a homemaker or being a full-time student (n = 205, 21.6%). It iss worth noting the proportion of those who worked full-time were very close to that of this group (n = 200, 21.1%). The average household income was reported to be between $40-60,000 with the mean income at approximately $50,000. The average respondent reported living with two people (n = 182, 19.2%) while most reported living with one person (n = 282, 29.7%) (See Table 3). The primary other 19

30 reported places of residence included university campus dorms (n = 12, 1.2% or with their parents (n = 11, 1.1%). With residence as well as other selected items, an other open-ended answer option was also presented. Selected quotations or citations are presented in this results section to illustrate data that may otherwise not be captured. For example, to demonstrate the length to which some people living with chronic pain may resort in order to maintain housing arrangements, the following quote is presented: I live in a camper on my farm, parents assist in maintaining so I can live independently. Had to sell my house (Female, age- 51, divorced/separated). Lastly, when asked to select all of the types of insurance they had during the prior 12 months two major categories were selected. Most respondents reported having had group plan insurance through an employer (n = 440, 46.4%), however many had no insurance at all over the past 12 months (n = 144, 15.2%). Ehlers Danlos Syndrome Diagnoses The most common type of EDS reported was EDS III - Hypermobile type (n = 705, 74.5%). This is also the type of EDS that research has found to be the most painful out of all the types of Ehlers Danlos Syndrome (EDNF.org, 2012). Analysis of respondents who had not selected a specific type showed that they either have a crossover of symptoms, are waiting on laboratory confirmation of specific type after receiving a clinical diagnosis (see Table 4). 20

31 Chronic pain Experience All participants in this analysis (n = 949) reported having chronic pain per the operational definition and 61.2% (n = 581) reported having chronic pain for ten years or longer. Physical Location of Pain. The most often reported location of pain was the lower back (n = 162, 17.2%) followed by the neck (n = 144, 15.2%), with the third most often reported area of pain being the hips (n = 118, 12.5%). Table 13 shows other areas and participant ranking. Pain Intensity. Participants were asked to rate their pain and pain interference in life activities based on a scale of 1-10, with ten being the highest level of pain, currently and within the past six months. When asked to rate their current level of pain (pain right now), 19.7% (n = 187) reported a pain level of a 7 (See Table 5). The remainder of pain intensity and interference questions asked about pain over the prior six months. When asked to report their most intense pain 47.1% (n = 446) reported a pain level of 10. The highest average pain intensity was reported to be 7 (n = 233, 24.6%) and when asked how much pain interfered with daily activities, 23.9% (n = 227) rated it as a 7. Disability. This section included a question asked how much has pain changed their ability to take part in recreational, social or family activities; 17.6% (n = 167) reported a level of 10 and 16.9% (n = 160) reported a level of 9. Lastly, respondents were asked, How much has pain changed your ability to work? to which 22% (n = 208) reported a level of 10. The previously reported high reliability coefficient held true with 21

32 this sample (Cronbach s alpha =.856). Participants were also asked how many days they have been kept from their usual activities due to pain. The answers ranged from 0 to 180 and the average number of days missed was (SD = 14.17). A series of subscales are computed based on the total scores from the items above. The first subscale is Pain Intensity (average score items 1-3 multiplied by 10). Scores on this subscale can range from 0 to 100 (where 100 is the highest pain intensity). Scores 0-50 are categorized as Low Intensity and scores >50 are categorized as High Intensity. In this sample, 53.6% (n = 507) of respondents scored between 81 and 100 with a mean score of (SD = 14.17), a mean score that falls in the High Intensity range. A second subscale is the Disability Score (average score items 4-6 multiplied by 10) which has a range of scores from 0-100, which are then categorized into Disability Points (e.g. Score 0-29 = 0 disability points and a score of 70 or greater = 3 Disability Points). A third subscale, the Disability Days ranges from days, where the number of disability days is then also categorized into Disability Points. Graded Chronic Pain Categories. All Disability Points are then added and in conjunction with the Disability Score and the Intensity Score a Graded Chronic Pain Category is then assigned to each participant. The complete breakdown of Graded Pain Categories for study participants can be seen in Table 5. Nearly 9 out of 10 participants who report Chronic Pain and an Ehlers Danlos diagnosis has high pain intensity and scored in the High Disability and Severely Limiting Graded Pain Category with 88.4% (n = 839) out of 949 respondents classified as Grade IV. 22

33 Quality of Life Physical well-being. To measure physical well-being, the FACT-G, or the Functional Assessment of Cancer Therapy General was adopted for this study. The scores on this section ranged between 0-26 with the mean at 9.67 (SD = 5.452). Two hundred and fifty eight of the respondents (31.5%) scored between a 5-8 where only 36 (3.7%) respondents scored between This indicates a low level of physical wellbeing. This assessment was highly reliable with a Cronbach at.801. Functional well-being. Looking at respondent s functional well-being, scores fell between 0-24 with the mean at (SD = 4.896). Three hundred and twenty one (33.7%) of the respondents scored between a where only 39 (4.1%) scored between a Since the majority of the participants scored relatively low on the scale, this reflects a lower functional quality of life. This assessment is highly reliable with Cronbach s at.838. Emotional well-being. The FACT-G was also modified and used to measure emotional well-being. Most of these questions first had to be reverse coded to ensure that again, a higher score was associated with higher level of quality of life. The scores ranged from Three hundred and eighty (40.1%) scored between 6 and 10 with a mean of (SD = ). This puts the respondents in the low range of middle on the emotional well-being scale with high reliability as Cronbach s was at.795. Social/family well-being. Lastly, a modified version of the FACT-G was again used to measure the functional quality of life amongst participant and again, a high score would reflect a high functional well-being. On this scale, the scores ranged from

34 Two hundred and eighteen (17.9%) participants scored between a with a mean of (SD = 6.469). Only 72 (7.5%) of respondents scored between a This puts the respondents in the low range of middle on the social/family scale a well-being scale as well. Reliability was high with Cronbach s at.824 (See Table 6). Overall quality of life. The overall mean FACT-G score (combining social/family well-being with functional, emotional and physical well-being averaged scores) was (SD = ) out of a possible 108 points. This indicates an average score of 42.23%. The Cronbach s Alpha score of.756 illustrates that this too was a reliable scale (see Table 7). Support To measure support, participants were asked a series of questions on a scale of 1-5 with 1 being strongly disagree and 5 being strongly agree. Questions such as, Friends and family accept me and my chronic pain, who helps you most in caring for your chronic pain and who have you told about your chronic pain, were asked of the respondents. Nine hundred and twenty nine (98.2%) had told others about their chronic pain. Eight hundred and eighty one (92.8%) told their immediate family while 703 (74.1%) told their spouse/partner. Two hundred and sixty four (27.8%) answered a 4 (somewhat agree) to the question, Friends and family think I exaggerate or overstate my pain. 300 (31.7%) answered a 4 to, Friends and family feel uncomfortable about me because of my chronic pain, however, 353 (37.2%) responded with a 4 to the question, Friends and family accept me and my chronic pain. In looking at the open-ended question, many revealed their condition through social media and at church. Scores 24

35 ranged from 6-30 with a mean of (SD = 5.859) out of a possible 30 points. Of the respondents, 280 (29.8%) scored between 16 and 20 which puts them just over the middle of the support scale, at 63.47%. Cronbach s Alpha was reported at.851 giving this scale high reliability (see Table 8) Coping To measure coping amongst participants, the Chronic Pain Coping Inventory-42 or, CPCI, was utilized. The CPCI was developed to assess behavioral and cognitive pain coping strategies such as exercise, guarding, resting and coping self-statements. The CPCI guarding and seeking social support scales have previously been established to statistically explain unique amounts of variance in disability scores; and the Guarding and Resting subscales have made unique contributions to the explanation of variance in pain severity (Romano et al., 2002). The CPCI scales were chosen as they were shown to have adequate to excellent internal consistency and test retest stability. Participants were given 42 questions and asked to rate how many days in the past week they used each strategy to cope with their chronic pain. For example, question 30, asked how many days they had limited their standing time to which 542 (58.2%) participants answered 7 days. The 42 items are divided into 8 subscales. The first four, Guarding, Resting, Asking others for help and Relaxing are presented here first and relevant score details can be found in table 9. Looking at the guarding scale, answers ranged from 0 to 7 with a mean of 4.59 (SD = 1.814) (See Table 9). Cronbach s Alpha was measured at.712 which makes this test highly reliable. The resting scale also ranged from 0-7 with a mean of 2.45 (SD =.97). This test is found to be reliable with 25

36 Cronbach at.686. The next scale in this inventory is asking for help. With the same range as other Coping Subscales, the mean score for this scale was 3.68 (SD = 2.34). The majority of respondents 32.7% (n = 299) report using this strategies 3 to 4 days per week. The relaxation scale followed with respondents having reported an average of just over 2 days using some sort of relaxation technique. The largest group 48.6% (n =380) report using relaxation strategies 1 to 2.99 days per week with a mean score for this scale of (SD = 1.733). This is a reliable test as Cronbach alpha was found to be.660. The remainder of the subscales includes Task Persistence, Exercise and Stretching, Seeking Social Support, and Coping Self-Statements Information related to participants responses is presented in Table 10. The Task Persistence scale made statements such as, I didn t let the pain interfere with my activities and again, asked the respondent how many days did they use this strategy to cope with their chronic pain. This category carried a mean of 4.06 (SD = 1.87); 299 (32.71%) of participants stated that they used task persistence 3 to 4.99 days per week in coping with their condition. Since Cronbach s was found to be.683, this test is considered reliable. Following the task persistence scale is the Exercise and Stretching scale. 373 (44.5%) of respondents stated that they used exercise 1 to 2.99 days per week to cope with their chronic pain. The mean for this score was 2.40 (SD = 1.89) and the test was highly reliable with Cronbach s at.777. Seeking Social Support was the next scale. An example of seeking social support would be, I talked to someone close to me. The mean for this scale was 2.52 (SD = 1.92). 340 (41.7%) respondents stated that they sought social support 1 to 2.99 times per 26

37 week in an effort to cope with their chronic pain. This scale is also highly reliable with Cronbach s Alpha scoring at.730. The final scale in CPCI coping inventory is Coping Self-Statements. Statements such as, Told myself things will get better. Measured how often participants utilized self-talk to cope with their chronic pain. 267 (30.3%) of the respondents stated that they use such coping self-statements 3 to 4.99 days per week with a mean of 3.75 (SD = 2.15). This scale has good reliability with Cronbach s at.691. A total Coping Scale score was computed by averaging the eight subscale scores for each participant. Participants who did not answer at least 50% of each subscale were not included in this calculation. The total Coping Scale scores ranged from 1.76 to 7 days and had a mean of 5.07 (SD =.863). Cronbach for the total Coping scale was.871. Additional tables (Tables 11 and 12) are presented in Appendix F. Inferential Statistical Analyses Correlation analyses were conducted to assess the relationship between demographic variables and scale scores (total quality of life [FACT-G], chronic pain [GCPS], support, and coping). Results of a Pearson correlation analysis showed that age was correlated with coping (r =.152, p <.001, n = 805). Marital status was correlated with chronic pain, support, coping (r =.101, p =.004, n = 803) and quality of life. Level of education was correlated with chronic pain (r = -.088, p =.007, n = 942) and quality of life (r =.130, p <.001, n = 945). Income level was correlated with chronic pain (r = -.097, p =.004, n = 905), support (r =.111, p <.001, n = 896) and quality of life (r =.255, p <.001, n = 909). 27

38 Correlational analysis also showed interactions between the scaled scores. Namely, quality of life was correlated with support (r =.508, n = 935, p <.001), coping (r = -.150, n = 805, p <.001) and chronic pain (r = -.129, n = 931, p <.001). Additionally, chronic pain was correlated with support in (r = -.129, n = 931, p <.001). Regression analyses were conducted to evaluate the research questions. First, multiple linear regression was run to assess if Quality of Life was predicted by Coping and or Support, F(3,793) = , p <.001. The main effect for Coping was not significant. The main effect for Support was significant, in that as Support levels increased, Quality of Life scores became higher, standardized Beta =.532, t = 3.312, p <.001. This model predicted 28% of variance in Quality of Life scores. Second, multiple linear regression was conducted to evaluate if Support and Coping could predict Chronic Pain. Results showed a significant model with F(3,791) = 3.79, p =.010. Although the model was statistically significant as the R 2 value was diminutive. The main effects for Support and Coping were not significant. Third, stepwise multiple regression was executed to calculate if Quality of Life was predicted by Demographics, Support or Coping. Specifically; age, gender, education, marital status, Support and Coping were entered in to the equation as predictors. Results for the model were significant, F(1,896) = , p <.001. The main effects for marital status, age and gender were not significant. The main effect for education was significant, standardized Beta =.085, t = 3.014, p =.003. The main effects for Support (standardized Beta =.0510, t = 17.76, p <.001) and Coping (standardized Beta = -.149, t = -5.23, p < 28

39 .001) were also significant. Support predicted 26%, Coping an additional 2.2% and education 0.7% of the variance in Quality of Life. DISCUSSION The purpose of this study was to determine the relationship between demographic characteristics, quality of life, coping, support systems and chronic pain as reported by patients with Ehlers Danlos Syndrome as well as to identify significant demographic, support and coping predictors of quality of life. Demographically, income was a predictor of quality of life. Most cross-sectional studies report a positive and significant association between income and at least some aspects of health-related QOL (Short & Mallonee, 2006). In this study, a correlation was found between income and quality of life. It was determined that the higher the income, the higher the quality of life is found to be and the lower the income, the lower the quality of life. Studies indicate that women suffer more chronic pain in most Western countries than men, with considerable physical, social and mental consequences for the sufferers (Skuladottir & Halldorsdottir, 2011). The majority of respondents for the current study were female (95.7%) and EDS appears to be found more frequently in the female population. EDS-HT (Hypermobility Type III) is not only the most painful form of EDS, it was also proven to be the most common as 705 of the 949 (74.5%) participants suffer from this form of Ehlers Danlos Syndrome. As Tuzun (2007) previously stated, it is well known that prolonged untreated or under-treated chronic pain can have substantial negative physical, psychological, and social effects and can also have a disruptive impact on a person s day-to-day life. 29

40 Looking further at chronic pain, it was reported that 100% of respondents suffered from chronic pain and pain significantly interfered with social activity levels, daily activities and the ability to work. Highest pain intensity over a six month period was rated at a 7/10. Results from the Graded Chronic Pain Scale reflect that those suffering with EDS have high pain intensity and fell into the graded pain category of High Disability and Severely Limiting, Grade IV. Regardless of living with chronic, intense and debilitating pain, somehow there are positive areas of the participants quality of life that remain intact which is reflected in some of the support and coping scales. Support and coping were positively related to quality of life. The higher the level of coping and support on the part of the respondent, the better the quality of life outcome. The first step of support lies in telling people about your condition in the first place and 946 out of our total 949 respondents had told someone that they had EDS and nearly 98% had told someone that they suffered from chronic pain. While most told immediate family, others reached out using social media and support groups to reveal their EDS and experience with chronic pain. As Baeza-Velasco et. al. (2011) stated earlier, support networks can increase social support and encourage a sense of understanding, which can decrease feelings of isolation and stigma. 929 said they got support from a friend. The mean score for support was which is above average of the range 6-30, which, means that more than half of the respondents feel supported which is reflected in the Quality of Life scores. As reported earlier, Support was predictive of Quality of Life accounting for 28% of the variance. 30

41 Level of Coping is measured by using several scales and respondents scored high on several of these. The scores reflected that respondents reported relatively good coping skills based on the Chronic Pain Coping Inventory. Those with EDS know how to ask for help, make positive coping-self statements and maintain task persistence. When a person becomes used to living a life of chronic pain, it is hard to learn how to stop and give in to it. It is this drive that may be responsible for improving the patient s quality of life; however, according to one of the scales respondents also know when to relax. The only scale that reflected a low score was Seeking Social Support which, again, may have something to do with stigma and the fear of reaching out due to having been marginalized in the past. Besides its harmful consequences on patients physical and psychological functioning, chronic pain also can be detrimental to the social functioning of sufferers (Tunzun, 2007). This is where support groups can be crucial for those with EDS to seek social support and validation. As Baeza-Velasco et al. (2011) previously stated, supports systems have also been found to improve overall quality of life, which in turn has the potential for improving the overall quality of life in EDS patients suffering from chronic pain and the accompanying psychosocial ramifications. As this study utilized on-line support groups as a platform to disseminate the survey, a whole on-line network of EDS support groups and organizations was discovered with the aim of supporting and improving the quality of life for those who suffer with any type of EDS. Quality of Life was determined by utilizing the FACT-G measure. Functional well-being and physical well-being scores were quite low. Emotional well-being and family/social well-being scores were around mid-range on the scale which indicates an 31

42 average quality of life in these areas. Employment was associated with higher total QOL. With higher family and psychological/spiritual quality of life, work can serve as a distraction from the pain and help patients cope more successfully on a daily basis (Gerstle et. al., 2001). Fifty-eight percent of the respondents in this survey were either disabled or not working versus the 33.0% who were still working at least part-time. The answer to this problem may lie in staying active and engaged in something such as volunteer work or some other community-serving interest as the pain permits. In reviewing the other comments throughout the survey, there was a theme of resiliency amongst participants despite their chronic pain due to EDS. One respondent was quoted as saying, I m a fighter. To rise above the pain, sufferers must focus on what they can do rather than what they cannot do (Tuzun, 2007). The general limitations of this study consist of the following: the study utilized a convenience sample; participants may have gotten fatigued and not completed the entire survey; results are limited to the validity of self-report data and only participants with Internet access were able to complete the survey. Specific limitations must also be noted. The insurance item proved to be focused toward US participants in that we did not provide international measures for different types of insurance in other countries. That is, we did not have the correct language to capture strong insurance coverage indicators. Many international respondents may have answered other due to the fact that the choices were not appropriate or recognizable to them. It is also impossible to know how many people received the recruitment flyer but did not follow the link to view the consent form. 32

43 Implications for Health Education Practice According to Cowan (2011), with regard to chronic pain, the magnitude of the problem is overshadowed by the lack of education, awareness and general understanding surrounding pain and how to manage it on a daily basis. As previously avowed by Berglund et al. (2003) in regards to health promotion efforts in health settings, it is important to have insight into the problem areas which may influence the overall life situation for an individual with EDS (p.776). This is where health educators must start. Health educators must first understand the physical and psychosocial impact of living with chronic pain and EDS before they can effectively develop health education programs to address the problem. Those in chronic pain, especially women, may be seen in any clinical setting and health professionals need to be able to recognize their needs in order to be able to give effective care, to cooperate with them and empower them (Skuladottir et al., 2011). Tuzun (2007) has stated that although efforts in the health sector are obviously of crucial importance in the management of chronic pain, a high level of patient and societal involvement is key to reducing the burden of this condition. Patients should work in partnership with health-care professionals, actively participating in their care. Providing access to exercise facilities, walking and cycle ways increases the opportunities for, and quality of life in chronic pain reduces barriers to, physical activity. Innovative community-based strategies are needed to improve early identification and referral for chronic pain. For instance, extending the clinical concept of pain as the fifth vital sign to the community setting, that is, making an assessment of pain as a component of all 33

44 community health assessment programs regardless of their primary focus, public health professionals may partner in the early identification and referral to resources available in the community to minimize the burden of pain (Meghani, 2009, p. 315). This strategy itself could eventually reduce the isolation and stigma felt by chronic pain patients. They may finally feel heard and understood. Health educators are not only in a position to help connect those suffering with EDS to community resources, but they can become a resource as well. Initiating campaigns addressing chronic pain may decrease the innate stigma associated with this condition. Planned health education activities and campaigns should provide information to the public and also contribute to a better understanding of how to manage and even prevent musculoskeletal complications of EDS. Besides general public education activities, patient education should be a key area of pain management interventions. Patients need to become their own best advocates and work alongside the health educators to create a sense of empowerment. Through this research, it has been established that Support and Coping predict Quality of Life. Specifically, Health Educators could use this information to create programs that will enhance quality of life by taking steps to ensure that support and coping techniques are the underpinnings of future educational programs. It has also been proven that Income was found to be correlated with Quality of Life. Health Educators could create chronic pain support groups and EDS coping skill workshops on a sliding scale that will attract those of low income to receive the support and coping skills they need. Since Support was the biggest indicator of Quality of Life, Health Educators could intervene by launching public 34

45 educational campaigns about living with chronic pain and help implement pain as the fifth vital sign amongst community health center clinic doctors and nursing staff to better capture the occurrence the chronic pain and to help reduce stigma while helping the patient feel understood and supported. We currently operate off of a four vital sign system pulse, respiration, blood pressure and temperature. Implementing pain as the fifth vital sign could also serve as early detection in pain management. Finally, we know that the scores for Seeking Social Support were low. Something health educators could do to intervene here would be to hold workshops for both the chronic pain sufferer and their loved ones in an effort to improve communication, thereby, leading to increased levels of social support for patients.. Participating in support programs may improve individuals abilities to live with the chronic pain of Ehlers Danlos Syndrome, pain may become manageable with the development of instrumental coping skills and a high level of patient-focused support. 35

46 REFERENCES Baeza-Velasco, C., Gély-Nargeot, M., Bulbena Vilarrasa, A., & Bravo, J. (2011). Joint hypermobility syndrome: problems that require psychological intervention. Rheumatology International, 31(9), doi: /s Berglund, B., Nordstrom, G. & Lutzen, K. (2000). Living a restricted life with Ehlers- Danlos Syndrome (EDS). International Journal of Nursing Studies, 37(2), Berglund, B., Mattiasson, A., & Nordström, G. (2003). Acceptance of disability and sense of coherence in individuals with Ehlers Danlos syndrome. Journal of Clinical Nursing, 12(5), Chodosh, O., Solomon, D., Roth, C., Chang, J., MacLean, C., Ferrell, B., Shekelle, P., & Cowan, P. (2011). Living with chronic pain. Quality of Life Research, 20(3), doi: Ehlers Danlos National Foundation (EDNF). (2012). What is EDS? Retrieved from id= Gerstle, D. S., All, A. C., & Wallace, D. C. (2001). Quality of life and chronic nonmalignant pain. Pain Management Nursing : Official Journal of the American Society of Pain Management Nurses, 2(3), p doi: /jpmn

47 Hays, RD (1994). The Medical Outcomes Study (MOS) Measures of Patient Adherence. Retrieved November 10, 2013, from the RAND Corporation web site: Indiegogo. My EDS - Ehlers Danlos Syndrome Journey by Dr. Brad Tinkle (2013). Retrieved from Meghani, S. and Cho, E. (2009). Self-reported pain and utilization of pain treatment between minorities and nonminorities in the United States. Public Health Nursing, 26(4), doi: /j x Meisler, J. (1999). Chronic pain conditions in women. Journal of Women s Health, 8(3), Nicholas, M. (2005). The pain self-efficacy questionnaire: taking pain into account. European Journal of Pain, 11(2), Osborne, T., Raichle, K., Jensen, M., Ehde, D., and Kraft, G. (2006). The reliability and validity of pain interference measures in persons with multiple sclerosis. Journal of Pain and Symptom Management, 32(3), Peres, M, and Lucchetti, G. (2010). Coping strategies in chronic pain. Current Pain and Headache Reports, 14(5), DOI /s Poleshuck, E., Giles, D., & Tu, X. (2006). Pain and depressive symptoms among financially disadvantaged women's health patients. Journal of Women's Health, 15(2), doi: /jwh

48 Rollman, G., and Lautenbacher, S. (2001). Sex differences in musculoskeletal pain. The Clinical Journal of Pain, 17(1), Romano, J., Jensen, M., & Turner, J. (2003). The chronic pain coping inventory 42: reliability and validity. Pain, 104(1-2), Rombaut, L., Malfait, F., De Paepe, A., Rimbaut, S., Verbruggen, G., De Wandele, I., & Calders, P. (2011). Impairment and impact of pain in female patients with Ehlers- Danlos syndrome: A comparative study with fibromyalgia and rheumatoid arthritis. Arthritis & Rheumatism, 63(7), DOI: /art Rombaut, Malfait, Coois, DePaepe, & Calders (2010). Musculoskeletal complaints, physical activity and health-related QOL amongst patients with ehlers-danlos syndrome hypermobility type. Disability and Rehabilitation, 32(16), p Sacheti, A., Szemere, J., Bernstein, B., Tafas, T., Schechter, N., & Tsipouras, P. (1997). Chronic pain is a manifestation of the Ehlers-Danlos syndrome. Journal of Pain and Symptom Management, 14(2), Short, P. and Mallonee, E., (2006). Income disparities in the quality of life of cancer survivors. Medical Care, 44(1), Skuladottir, H. and Halldorsdottir, S. (2011). The quest for well-being: self-identified needs of women in chronic pain. Scandanavian Journal of Caring Sciences, 25(1), Statistical Solutions Sample Size Calculator at pss_calc.php. Accessed on November 26,

49 The Michigan Diabetes Research and Training Center (1998). Diabetes Care Profile 2.0, University of Michigan retrieved from on September 11, Tüzün, E. (2007). Quality of life in chronic musculoskeletal pain. Best Practice & Research Clinical Rheumatology, 21(3), Voermans, N., Knoop, K., Bleijenberg, G. & van Engelen, B. (2010). Pain in Ehlers Danlos syndrome is common, severe, and associated with functional impairment. Journal of Pain and Symptom Management, 40(3), Webster, K., Cella, D., and Yost, K., (2003). The functional assessment of chronic illness therapy (FACIT) measurement system: properties, applications, and interpretation. Health and Quality of Life Outcomes, 1(79). doi: / Retrieved from WHO (1997) World Health Organization. Programme on Mental Health. WHOQOL Measuring Quality of Life. Retrieved from 39

50 APPENDIX A Tables Table 1: Demographic Characteristics Age N % M SD Total Gender Female Male Total Country Of Residence United States United Kingdom Canada Other Total Marital Status Married/Life Partner Never Married Separated/Divorced Widowed Total Note: missing data excluded. 40

51 Table 2: Education, Employment, Income, & Insurance Characteristics Amount Of Schooling N % 8 th Grade Or Less 3.3 Some High School High School Graduate Or GED Some College/Technical School College Graduate Or Technical School Graduate Graduate Degree Total Current Employment Status Working Full-Time (35 Hours Or More) Working Part-Time (Less Than 35 Hours) Not Working Disabled Other Total Household Income 0-20, , , , Total Health Insurance Individual Plan Member Pays Group Plan Employer Pays Us Government Health Plan Medicaid Medicare No Insurance In Past 12 Months Other Total Note: Missing data excluded 41

52 Table 3: Living Environment How many people live with you N % I live alone person people people people or more people Total Where do you live most of the year Your home, apartment, condo Senior citizen apartment, condo 4.4 Home of a relative or friend Retirement home 1.1 Other Total Other places you live most of the year University dorms With parents or family With boyfriend 1.1 Mobile home 1.1 In a camper

53 Table 4: EDS diagnoses Type of Ehlers Danlos Syndrome N % Hypermobile (type III) Classical (type I/II) Vascular (type IV) Kyphoscoliosis (type VI), Arthochalasia (type 9.9 VIIA/VIIB) or Peridontal (type VII) May have EDS but not sure what type May have EDS but diagnosis not yet confirmed I don t have EDS 1.1 Other Total

54 Table 5: Chronic Pain Experience When Chronic Pain Began N % M SD Less than 12 years ago years ago years ago years ago or longer Total Pain Intensity Subscale Total Pain Disability Subscale 0-29 = Grade I = Grade II = Grade III = Grade IV Total

55 Table 6: FACT-G Quality of Life Measure Physical Well-Being Scores N % M SD Total Emotional Well-Being Scores Total Social/Family Well-Being Scores Total Functional Well-Being Scores Total

56 Table 7: Total Quality of Life Scale Scores FACT-G Total Score N % M SD Total

57 Table 8: Support Scale Scores Scores N % X SD Total

58 Table 9 Coping inventory- Guarding, Resting, Asking and Relaxing Subscale Scores Guarding Scale Scores N % M SD 0 days per week to 2.99 "days per week" to 4.99 "days per week" to 6.99 "days per week" "days per week" Total Resting Scale Scores 0 days per week to 2.99 "days per week" to 4.99 "days per week" to 6.99 "days per week" "days per week" Total Asking for Help Scale Scores 0 days per week to 2.99 "days per week" to 4.99 "days per week" to 6.99 "days per week" "days per week" Total Relaxing Scale Score 0 days per week to 2.99 "days per week" to 4.99 "days per week" to 6.99 "days per week" "days per week" Total

59 Table 10 Task Persistence, Exercise and Stretching, Seeking Social Support, and Coping Self- Statements Subscale Scores Task Persistence Scale Score N % M SD 0 days per week to 2.99 "days per week" to 4.99 "days per week" to 6.99 "days per week" "days per week" Total Exercise and Stretching Scale Scores 0 days per week to 2.99 "days per week" to 4.99 "days per week" to 6.99 "days per week" "days per week" Total Seeking Social Support Scale Scores 0 days per week to 2.99 "days per week" to 4.99 "days per week" to 6.99 "days per week" "days per week" Total Coping Self Statements Scale Score 0 days per week to 2.99 "days per week" to 4.99 "days per week" to 6.99 "days per week" "days per week" Total

60 Table 11 List of Organizations invited to participate in recruitment process ORGANIZATIONS Ehlers Danlos National Foundation Center for Ehlers Danlos Syndrome Alliance Cincy Zebras (Facebook support group) Zebras for Life (Facebook support group) Ehlers Danlos Syndrome Research Group (Facebook support group) EDS Head and Neck Group (Facebook support group) 50

61 APPENDIX B 51

62 Figure 1 Recruitment Flyer 52

63 APPENDIX C Figure 2 IRB Letter of Approval 53

64 APPENDIX D Figure 3 Final Study Consent Form 54