Zebras in the workplace: Vocational rehabilitation considerations for individuals with Ehlers-Danlos Syndrome

Abstract

BACKGROUND:

In this article, readers are introduced to a rare disorder, Ehlers-Danlos Syndrome (EDS), an emerging disability that can substantially impair vocational functioning.

OBJECTIVE:

An overview of EDS and information about common co-occurring conditions are provided.

CONCLUSION:

Vocational implications of EDS are discussed, including functional limitations and recommended job accommodations. Strategies for illness self-management are explained, as well as potential interventions to improve career self-efficacy. Additional services to include in VR plans (e.g., peer supports, job development and placement services) are also examined.

Keywords

Ehlers-Danlos Syndrome EDS vocational rehabilitation employment

1 Introduction

Due to advances in medicine and technology, chronic health conditions that used to be considered rare or fatal are now being seen more often, especially in health care, educational, and vocational rehabilitation (VR) settings. A disease or disorder is defined as rare when it affects fewer than 200,000 Americans at any given time (Eurodis, 2014). The National Organization for Rare Disorders (2016) has compiled a database of over 1,200 rare diseases or disorders. The Centers for Disease Control (CDC, 2016) have identified over 7,000 rare diseases. By 2020, it is estimated that ten percent of the world’s population will be affected by a rare disorder (CDC, 2016). One of these rare disorders is Ehlers-Danlos Syndrome (EDS). EDS is a group of hereditary connective tissue disorders that can affect multiple functional domains and introduce an array of challenges to carrying out activities of daily living, including employment (Ehlers-Danlos Society, 2016). It is estimated that 1 in 5,000 individuals in the United States has EDS (Ehlers-Danlos Society, 2016); however, members of the EDS community state that EDS is not rare, just rarely properly diagnosed (Ehlers-Danlos Society, 2016).

The purpose of this article is to (a) inform vocational rehabilitation (VR) professionals about the medical and vocational aspects of EDS and (b) present considerations for effective VR planning to improve the employment outcomes of individuals with this rare disorder. The article begins with a case study to illustrate the impact of EDS on vocational functioning and the implications for VR planning.

2 Case study: Jane

Jane is a 25 year old Caucasian female living in the Midwest. She has a very physically demanding job as an early childhood special education teacher, which has been her dream job since she was a small child. For the last several years, she has dealt with many health issues, including adult onset asthma, chronic cough, chronic sinus infections, and multiple orthopedic problems. She has had four surgeries to stabilize “loose joints,” as well as sinus surgery and a tonsillectomy. During a recent round of physical therapy, the physical therapist expressed concern about Jane’s joint hypermobility and excessive range of motion. He recommended that Jane see a rheumatologist. Jane saw the rheumatologist, who told her that she had benign joint hypermobility and that it was nothing to worry about. The rheumatologist recommended that she take ibuprofen for joint pain if needed. After a few more months in physical therapy, Jane decided to get a second opinion and saw another rheumatologist. This rheumatologist ran several blood tests and ordered x-rays. After a physical examination and reviewing the test results, the rheumatologist diagnosed Jane with Ehlers-Danlos Syndrome, hypermobility type. After telling Jane the diagnosis, she explained that there was no cure, nothing the doctor could do for her, and that Jane needed to quit her job and go on disability. Jane was devastated and had no idea how to proceed with her life in the context of this news. After speaking with her employer about implementing the job modifications recommended by both the rheumatologist and the physical therapist, the employer decided that it was too risky for Jane to remain in her position, even with job modifications, and Jane was terminated from her job and paid for the remaining time in her contract. Jane was in shock and was at a loss as to what she should do next. How was she going to support herself? What kind of job was she going to be able to find? Jane did not even know what type of job she would be interested in or suited for. She was only 25 years old; what was she going to do with the rest of her life?

3 Overview of Ehlers-Danlos Syndrome

Jane’s situation is not unique and illustrates the devastating impact that EDS can have on individuals who are diagnosed with this rare disorder. Members of the Ehlers-Danlos community identify themselves as zebras. Initially, this designation arose from the phrase that is taught to medical students throughout their training – ‘When you hear the sound of hooves, think horses, not zebras’.

In medicine, the term “zebra” is used to describe a rare disease or condition and the majority of the types that make up the Ehlers-Danlos syndromes are rare. However, the most prevalent form of the condition, the hypermobile type, is not rare at all but instead highly prevalent, both mis- and under-diagnosed. Yet still the zebra is the perfect mascot across all the EDS types. There is not one type of zebra with the same stripes – but when you see a zebra, you know it’s a zebra. There are so many people living with Ehlers-Danlos syndrome with different stripes. We have different symptoms, different types, different experiences, but we are working towards a time that when a medical professional sees someone with EDS, they know that it is Ehlers-Danlos syndrome. (Ehlers-Danlos Society, 2016, para. 3)

There are six different types of EDS: classical, hypermobility, vascular, kyphoscoliotic, arthrochalasia, and dermatosparaxis (Tinkle, 2008). The most common type, the diagnosis that Jane finally received, is the hypermobility type (H-EDS).

“Ehlers-Danlos Syndrome is known or thought to alter the biology of collagen in the body (the most abundant protein), which can lead to multi-systemic symptoms. Each type has certain physical traits and with notable exception to the most common form, the hypermobile type of Ehlers-Danlos Syndrome, most types have a known disease-causing gene. There are physical characteristics that are common to all types of Ehlers-Danlos Syndrome, including hypermobile joints (i.e., joints that move in greater amounts than expected) and skin involvement, such as any of the following: soft, stretchy, saggy, too thin, easy bruising, easy wounding, poor wound healing and/or atrophic scaring.”(The Ehlers-Danlos Society, para. 2)

Castori, Morlino, Celletti, Celli, Morrone, Colombi, Camerota, and Grammatico (2012, p.1) defined H-EDS as an “underdiagnosed heritable connective tissue disorder characterized by generalized joint hypermobility and a wide range of visceral, pelvic, neurologic, and cognitive dysfunctions. Deterioration of quality of life is mainly associated with pain and fatigue”. EDS can impact almost all of the systems of the body, including but not limited to: orthopedic problems (joints and bones), sleep dysfunction, heart problems, jaw and dental issues, breathing/pulmonary issues, gastrointestinal issues, musculoskeletal issues, issues with the autonomic nervous system, and pain (Tinkle, 2008). People with chronic conditions, including EDS, are prone to anxiety and depression, as well as feeling a loss of control over their bodies and their lives (Tinkle, 2008). Chronic pain is one of the most common issues associated with EDS. Chopra (2015) explained that there are many different types of pain experienced by those with EDS: mechanical or structural pain, inflammatory pain, nerve pain, muscle pain, and headaches.

A growing body of research suggests that certain other chronic health conditions are co-morbid or co-occurring with EDS. One of these conditions is dysautonomia. “Dysautonomia is an umbrella term used to describe several different medical conditions that cause a malfunction of the autonomic nervous system” (Dysautonomia International, 2012). Various forms of dysautonomia such as postural orthostatic tachycardia syndrome (POTS) and neurocardiogenic syncope (NCS) can develop secondary to EDS and can take years to be properly diagnosed because symptoms tend to wax and wane (Dysautonomia Support Network, 2015). According to Dysautonomia International (2012), POTS can cause lightheadness, fainting, tachycardia, chest pains, shortness of breath, gastrointestinal upset, shaking, exercise intolerance, temperature sensitivity and more. Basically, the person’s autonomic nervous system cannot regulate the body’s reactions when changing positions, such as going from laying down to sitting up or sitting to standing causing increased heart rate and lowered blood pressure, These bodily reactions can cause the person to become lightheaded or faint. NCS is the most common form of dysautonomia and ranges from mild to severe. In mild cases, individuals may faint once or twice in a lifetime, and, in severe cases, individuals may faint several times a day (Dysautonomia International, 2012). Symptoms of dysautonomia include abnormally low (i.e., bradycardia) or an abnormally high (i.e., tachycardia) heart rate: extremely low blood pressure; narrow pulse pressure; frequent, large swings in heart rate or blood pressure; frequent bouts of dehydration; chronic fatigue; heart palpitations; dizziness or vertigo; syncope (i.e., losing conciousness) or near syncope; low blood volume; frequent nausea and gastrointestinal issues (motility issues); difficulty swallowing; chest pain; shortness of breath; migraines or frequent headaches; abnormal pupil response and sensitivity to light; and difficulty regulating body temperature (Dysautonomia Support Network, 2015). Jane was eventually diagnosed with POTS, NCS, and inappropriate sinus tachycardia, a few years after her EDS diagnosis. Chronic fatigue syndrome and fibromyalgia are also common co-occuring conditions (Knight, 2011).

Milhorat, Bolognese, Nishikawa, McDonnell, and Francomano (2007) reported an association between Chiari malformation and hereditary connective tissue disorders such as EDS. Jane was eventually diagnosed with Chiari malformation and craniocervical instability, and travelled from the Midwest to the East Coast to have surgery with a top neurosurgeon who specialized in EDS and Chiari.

In Chiari I malformation, the lower part of the cerebellum, known as the cerebellar tonsils, hangs down through the opening at the bottom of the skull, preventing cerebrospinal fluid (CSF) from flowing easily into the spinal canal. The symptoms of Chiari malformation are: dizziness, ringing in the ears, loss of balance, headache, choking, limb weakness and neck pain. Thus far surgery is the only treatment option and it aims to normalize the flow of cerebrospinal fluid (Chiari and Syringomyelia Foundation, 2015, para. 1).

EDS is diagnosed through a physical examination and a thorough review of a person’s medical history. The Beighton scale or the Brighton scale for testing hypermobility is used as a diagnostic tool for determining the type of EDS. The Beighton scale tests different joints and their range of motion. If at least five out of the nine joints tested exceed typical range of motion, then the individual is considered to have joint hypermobility. The diagnosis of EDS is considered a clinical diagnosis because the diagnosis comes from a physical examination instead of a diagnostic test such as a blood test or x-ray. Genetic testing is available for most types of EDS, although it is not available for the hypermobility type, and it is recommended that genetic testing be used conservatively and to confirm a diagnosis rather than establish one. EDS can be diagnosed at any time in life, but it is most commonly diagnosed in late adolescence or early adulthood. It is more common in females than in males and is more common in Caucasian individuals than in individuals of other races. Because there is no cure for EDS, management of the condition is focused on controlling symptoms. A person with EDS will have many members of his or her medical team, which could include a primary care provider, a rheumatologist, one or more orthopedic surgeons, a neurologist, a neurosurgeon, a pain management physician, a gastrointestinal doctor, a cardiologist, and a physical therapist, (Ehlers-Danlos Society, 2016). Jane’s medical team includes a primary care provider, a physiatrist for pain management, a physical therapist, a cardiologist, an acupuncturist, and several orthopedic surgeons.

4 Vocational implications

According to Levy (2016, p.4), “functional and psychosocial impairment are common [in individuals with EDs], manifesting with decreased sport-related physical activity, diminished health-related quality of life, and significant impact on daily function” (p. 4). Because the age of onset and diagnosis of EDS usually happens anywhere from late adolescence to middle adulthood (Levy, 2016), and EDS affects multiple systems in the body with waxing and waning, it is logical to conclude that EDS will have a substantial impact on a person’s career. The impact may range from needing job accommodations to stay in a current job, to needing to change jobs completely, to being unable to work. As was the case with Jane, many individuals are told by their physicians that they should discontinue working, despite the fact that they want to (and may need to) continue their careers.

In terms of work restrictions, people with EDS should avoid repetitive motion and heavy lifting in order to protect their joints from overuse injuries or repetitive motion injuries. Fatigue is common in people with EDS and can impact memory, concentration, and stamina. Chronic pain from EDS means that the individual is in constant discomfort that ranges in severity, and can lead to depression, anxiety, poor concentration, and low self-esteem. Chronic pain and the potential for joint dislocations means that the individual with EDS may have functional limitations in sitting, standing, bending, twisting, climbing, carrying, stooping, kneeling, crouching, reaching, and driving a vehicle for long periods. The person may also have difficulty with strenuous activities for long periods, limited mobility, difficulty with fine hand movements, and meeting strict deadlines. Functional limitations that Jane experienced in her job as an early childhood special education teacher included difficulty lifting students, stooping, kneeling, and bending. There were times that Jane would experience joint dislocations while lifting a student with mobility issues, or when kneeling or crouching on the floor to work with students. Because Jane worked with some students with behavior disorders, she would have to restrain them during outbursts, which usually resulted in Jane having one or more joints dislocated during the restraint process.

To receive job accommodations, individuals must first disclose their disability status to employers. However, individuals with EDS are often reluctant to disclose their need for accommodations. Reasons for non-disclosure of chronic conditions at work include fear of unemployment, mistrust of managers, stigma attached to certain conditions and feeling useless to the organization (Munir, Yarker & Haslam, 2008). These concerns are legitimate as illustrated by Jane’s disclosure to her employer of the need for job accommodations and the employer’s refusal of her request. Varying symptoms create issues with credibility, believability, and one’s reputation at work. Unpredictability regarding remission and flares causes stress and concern over work attendance and job performance (Beatty & Joffe, 2006). “Absenteeism from work often creates additional stress and may interfere with job performance, advancement, and financial security including health insurance” (Tinkle, 2008, p. 117). Dependence on employer-provided group health insurance may keep employees with chronic conditions stuck in a job, which is called “job lock”. Employees with chronic health conditions who rely on this type of health insurance have 40% lower job mobility than other workers (Beatty, 2011). Changes to health insurance legislation (e.g., the Affordable Care Act) in recent years may alleviate some instances of job lock, but group health insurance provided by employers is often still better than private insurance for people with EDS and associated conditions.

5 Vocational rehabilitation considerations

5.1 Counseling, guidance, and referral

Many people with EDS have difficulty trusting professionals because of past negative experiences. Most professionals they encounter know little or nothing about EDS and so the person with EDS is always the expert and has to repeatedly explain the condition. When meeting with a VR professional for the first time, the person with EDS may seem reserved, hesitant, or anxious because he or she is trying to assess the professional and whether he or she is trustworthy and helpful. VR professionals have an ethical obligation to help clients succeed in attaining or maintaining goals relating to employment. The “right” way to carry out this obligation is not always clear. With new chronic health conditions and disabilities continuously emerging, it is important for VR professionals to be aware of these conditions, become familiar with resources available for these individuals, and know where they as professionals can go to learn more about different health conditions and disabilities. VR professionals must:

Become familiar with medical aspects of rare disorders such as EDS

Understand the psychological tolls and stigma associated with rare disorders

Gauge the extent to which medical and psychological factors combine to impede successful employment

Develop knowledge of resources and model programs that can assist people with rare disorders in establishing and/or maintaining careers (Rumrill & Bishop, 2015). See Appendix for a list of resources for learning more about EDS.

Living with a chronic condition (such as EDS) can reshape people’s core ideas and image of themselves (Beatty & Joffe, 2006). “Chronically ill persons frequently experience a crumbling away of their former self-images without simultaneous development of equally valued new ones” (Charmaz, 1983, p.168). VR professionals must be comfortable with making referrals, especially to mental health professionals that can help clients work through any issues surrounding their change in identity, grieving the loss of the life they had planned, accepting new limitations, and learning how to live with a chronic health condition. Jane began seeing a counselor immediately after being diagnosed with EDS and the counselor helped her work through and process her diagnosis, the implications of life with EDS, and the loss of the career that Jane had worked toward for her entire life. Jane’s counselor recommended that she meet with a VR professional at the local state VR agency to explore career options and accommodations. Roessler, Reed, and Brown (1998) found that individuals with chronic health conditions “expressed a need for career counseling to clarify their vocational plans and to secure job training needed to retain or advance in their positions (p. 267). Support services and counseling should be provided to enable workers to identify and rely on personal assets to cope with chronic conditions (Roessler, Rumrill & Fitzgerald, 2004). It is important for clients to believe that they have a sense of personal control over work related events and activities and are satisfied with their abilities to make decisions about their jobs and accommodations (Roessler et al., 1998). Determination, acceptance of pain as a permanent part of life and positive coping representations have been identified as facilitators for returning to work (Kalsi, Turkistani, Sykes, Lucas & Zarnegar, 2016). Tinkle (2008) stated that “emotional and psychological support is as important in the treatment of this disorder [EDS] as physical therapy and pain management” (p. 115). Learning to accept chronic pain as a part of her life and learning positive coping representations was an ongoing process for Jane that she worked on in counseling for several months. Acceptance of limitations was also a reoccurring theme for Jane that she would work on with her counselor when new limitations would arise and lead to feelings of frustration and sometimes denial of limitations.

5.2 Illness self-management

The acceptance of a diagnosis of a chronic health condition, such as EDS, is a process akin to the grieving process one goes through when losing a loved one. In this instance, the person is grieving the loss of the life that he or she had planned, which takes time, and people should be allowed to take the time needed to accept their new life and become ready to move forward with that life (Tinkle, 2008). Initially after her diagnosis, Jane jumped right in to trying to find a new career, and operated on ‘autopilot,’ without taking the time to really acknowledge and process the profound change that had taken place in her life, as well as the future implications of life with EDS. With the help of her counselor, Jane was able to take the time (several months) to process all that she was feeling and experiencing. This would be a process repeated over and over again as new limitations and life changes were experienced. The individual with EDS may also feel a lack of control over his or her life due to the diagnosis of EDS. One way to take back some control is through learning to successfully manage EDS and treatment options. Jane read everything she could find on EDS and how to manage it. “Successful self-management of chronic conditions requires sufficient knowledge of the condition and its treatment, performance of condition management activities and application of the necessary skills to maintain adequate psycho-social functioning” (Barlow, Wright, Sheasby, Turner & Hainsworth, 2002, p. 177). Illness self-management should include developing skills needed to manage medication and symptoms as well as managing psychosocial consequences and lifestyle changes (Barlow et al., 2002). A study conducted by Kralik, Koch, Price and Howard (2004) found that people with chronic illness identify “self-management as a process initiated to bring about order in their lives” (p. 259). Illness self-management had four themes: “recognizing and monitoring the boundaries, mobilizing the resources, managing the shift in self-identity, and balancing, pacing, planning and prioritizing” (Kralik et al., 2004, p. 259). VR professionals should check in with clients with EDS to see how well they think they are managing their illness and encourage them to work with the appropriate medical professionals if they believe their condition(s) and any co-occurring disorders they may have are not being managed well, especially if these are preventing clients from looking for, entering, or maintaining employment.

5.3 Career self-efficacy interventions

For individuals with chronic conditions such as EDS, illness issues and lowered expectations of others can reduce career self-efficacy and lower career goals and expectations (Beatty, 2011). The construct of self-efficacy was developed by Albert Bandura (1994) and refers to one’s belief in his or her ability to successfully accomplish a task or activity. Career self-efficacy refers to how one’s self-efficacy beliefs influence career choices, career performance, and persistence when facing obstacles to obtaining or maintaining a certain career (Sullivan & Mahalik, 2000). By helping clients to modify their faulty self-efficacy beliefs and build self-efficacy, counselors are encouraging clients to become more autonomous and take control over career decision making. When Jane first came in to see the VR counselor, she was very discouraged and her career self-efficacy was very low. She had wanted to be a teacher for her entire life and could not imagine doing anything else with her life.

Betz (2004) provided a two-step guideline to incorporate the concept of self-efficacy in practice. Step one is to include the concept of self-efficacy in the initial discussions with the client. Questions should be asked that help the VR professional to evaluate the client’s self-efficacy beliefs in terms of his or her competence in career decision making, performance, or advancement and to determine the client’s self-imposed limits. These interventions should be based on Bandura’s four sources of self-efficacy information: performance accomplishments, vicarious learning or modeling, verbal persuasion, and emotional arousal (Betz & Shifano, 2000). Performance accomplishments refer to times in one’s past that one was able to successfully perform the behavior or action in question. Vicarious learning or modeling directs one’s attention to others in similar situations who have successfully performed the behavior or action that the individual is attempting to perform. Verbal persuasion is usually found in the form of encouragement, praise, and support from others. Emotional arousal suggests that negative emotions such as anxiety should be lowered, while positive emotions such as hope should be raised. All four of these sources should be considered and addressed when working with clients with EDS who may be dealing with poor self-efficacy beliefs.

Step two is to implement interventions to address domains of behavior that could benefit from an increase in self-efficacy. Luzzo, Funk, and Strang (1996) conducted a study examining the effects of a videotaped career intervention on career decision making self-efficacy. The intervention was referred to as attributional retraining material and was an eight minute video in which “both a female and male college graduate described their career development over the course of their late adolescence and early adult years” (Luzzo et al., 1996, p.382). The graduates described times in their lives when they experienced career obstacles, failures, and indecision and how persistence helped overcome these difficulties. Participants who had an external career locus of control (influenced by external reinforcements) increased their career decision making self-efficacy significantly after the intervention, but participants with an internal career locus of control did not. Criticism of this intervention includes that the intervention (videotape) was very brief and participants were passive observers instead of active participants (Sullivan & Mahalik, 2000). An alternative approach that can be used when working with individuals with EDS who have low career self-efficacy is job shadowing other individuals with EDS or related conditions.

Betz and Shifano (2000) conducted a study to look at the effectiveness of an intervention based on self-efficacy theory to increase women’s confidence and interests in “Realistic” activities from Holland’s theory. The intervention consisted of professional men and women modeling the desired behaviors. The instructors demonstrated each task, ensured the successful completion of each task by the participants, and both the instructors and participants encouraged and supported each other. Results from the study indicated that “self-efficacy expectations of college women with respect to the Realistic domain of Holland’s vocational theory could be significantly increased” (Betz & Shifano, 2000, p. 47) as a result of the intervention.

Interventions do not always need to be strictly structured in the way that the previous interventions were. The VR professional may not have the time to devote to lengthy interventions with clients. However, there are some basic questions and strategies that VR professionals can use to help increase the career self-efficacy of clients. For example, they can ask clients, “What would you do/be if you could do anything?” They can then help clients to brainstorm and use a visualization exercise to consider all possibilities for careers by removing all potential barriers that come up in their visualization. VR professionals can also ask, “When have you successfully made a change or a decision in the past?” and help the client remember times when she or he has been successful in making decisions and changes in the past and how he or she made those changes/decisions. Finally, the question “What barriers are in your way?” can prompt clients to explain real or perceived barriers and brainstorm with the VR professional ways to overcome those barriers.

Jane’s VR counselor used these questions to help Jane begin to think about careers other than teaching that she might be interested in. At first, Jane had a difficult time with the questions, “What would you do/be if you could do anything?” because her answer was an early childhood special education teacher, which was no longer an option for her. With further conversation, Jane said that she wanted to work with people and help them and make a difference in their lives in some way because that is one of the reasons she became a teacher in the first place. Jane and the VR counselor discussed successful decisions Jane had made in her past and eventually the only barrier Jane identified as being left in her way was that she did not know what her options were. After completing some career assessments and further work with the VR counselor, Jane decided to pursue a graduate degree in counseling and become a mental health counselor.

5.4 Peer supports

Living with a rare chronic health condition such as EDS can be isolating, both emotionally and physically. Tinkle (2008) stated that “having a good support system is KEY. Many times, folks can find others with similar conditions or concerns to discuss their thoughts and feelings such as support groups” (p. 115). Because EDS is a rare condition, there was no one in Jane’s area that also had EDS, but Jane was able to find two online support groups that were very helpful in providing support and understanding, as well as education and tips for managing life with EDS. Peer support interventions are both psychoeducational and therapeutic. These interventions are “designed to be supportive and expressive, seek to develop supportive relationships among group members, enhance expression of emotional issues related to their illness, and build meaning in the face of the disease” (Brown, Daly & Rickel, 2007, p.55). Peer support groups, both online and in person, are growing in popularity as an alternative to traditional mental health counseling or psychotherapy (Brown, Daly & Rickel, 2007). Social support and close peer relationships have been shown to promote coping with a chronic illness. The peer relationships that are developed are even more meaningful when all individuals in the peer support group have the same chronic illness because members are perceived as really understanding each other, being easy to talk to, and caring for and supporting each other (Kyngas, 2004). Kyngas (2004) also found that online chat rooms or support groups can be very important part of a support network because communication is anonymous, allowing the individual to feel able to share issues and emotions freely, without fear of judgment. VR professionals should be aware of peer support resources in their communities and encourage clients with EDS to find a local or online support group.

5.5 Job development, placement, and accommodation planning

Beatty (2011) conducted a qualitative study looking at career barriers and career paths for people with chronic illness. She interviewed 23 people that were currently working and lived with at least one chronic illness and found that there were four major career paths experienced by people with chronic illness: plateauing, redirecting, retreating, and self-employment. In the job development and placement process, VR professionals can use the findings from Beatty’s (2011) research to first explore with clients with EDS the career path they wish to pursue and then tailor employment interventions and the job search process to the client’s chosen career path. The first career path, plateauing, is not always voluntary and is defined as “remaining in a job role for such a long time that progression to a higher level is unlikely” (Beatty, 2011, p. 101). Plateauing can happen because people fear discrimination in the job market, loss of employer provided health insurance, and job lock. Plateauing is also associated with low career self-efficacy beliefs (Beatty, 2011).

The second career path, redirecting, happens when a person chooses to change careers, usually because future health problems are anticipated that could interfere with success in the current or desired career path. This is the path that Jane eventually ended up taking, although it was not her choice to change careers. She decided she wanted to go into the field of counseling and applied to and was accepted into a graduate program in counselor education. The third career path, retreating, is different than redirecting and is defined as “a conscious choice to decrease their work effort” (Beatty, 2011, p. 103). This strategy usually means changing to a lower level/less-demanding job in the same field or reducing hours at the current job. The decision to employ the retreating strategy may indicate that the person is experiencing low career self-efficacy and low coping efficacy beliefs. Beatty (2011, p. 104) explained that self-employment, the fourth career path, is a sort of “‘opting out’ of the career game”. The people that pursue this option are usually looking for flexibility and autonomy that may not be found in the traditional workplace or in their current job.

After determining the chosen career path of clients with EDS and identifying employment options, the next step is to assist clients to learn about appropriate job accommodations and how to advocate for these accommodations in the workplace so that they can enter, resume, maintain, or change employment. First, however, VR professionals may need to educate individuals with EDS about the employment provisions of the Americans with Disabilities Act Amendments Act. They may also need to explore with clients with EDS their concerns about disclosure and weigh both the risks and benefits of disclosure so that they can make informed decisions about whether to disclose (Koch & Rumrill, 2017). Then, both the VR professional and the client should consider what information to disclose, when to disclose, and to whom to disclose. The next step is to identify the person’s accommodation needs. The Work Experience Survey (Roessler & Gottcent, 1994) is a helpful tool for identifying accommodations. It is a structured interview meant to help employees self-identify their own reasonable accommodation needs in the areas of accessibility, essential job functions, career mastery, and job satisfaction. Examples of reasonable accommodations that may be needed by employees with EDS include braces and splints to help stabilize weak joints and allow individuals to continue to complete certain job tasks. An ergonomic evaluation and use of an ergonomic workstation design is helpful to eliminate or reduce muscle pain, strain, and stiffness. Environmental demands should be modified as needed for areas of diminished capacity and focus should be placed on increasing capacity in other applicable areas (Gray & Fossey, 2003). The use of assistive technology, such as programs for speech to text dictation, may be beneficial to reduce the strain of typing. Because working full time is often difficult for people with EDS due to constant medical appointments, surgeries, physical issues, and problems with stamina, job sharing and a flexible work schedule may be good options for these individuals. Additional accommodations that the Job Accommodation Network (JAN, 2016) recommends are working from home, providing carts and lifting aids, allowing longer breaks, allowing time off for medical treatment, allowing a self-paced workload, and installing automatic door openers. As a part of her graduate degree process, Jane completed an internship at a community mental health agency and was hired as a mental health counselor by that agency after graduation. With the guidance and support of her VR professional, Jane was able to request and receive appropriate accommodations at work such as an ergonomic workstation, a supportive desk chair, and a flexible work schedule that allowed her to attend medical appointments and rest when needed. With the benefit of these accommodations, Jane was able to successfully gain and maintain employment.

6 Conclusion

The purpose of this article was to inform VR professionals about Ehlers-Danlos Syndrome (EDS) and commonly co-occurring conditions. In collaboration with a VR professional, individuals with EDS can develop and enhance their career self-efficacy and be successful in finding or maintaining employment. Strategies such as illness self-management, connections to resources, peer supports, and appropriate job accommodations can be beneficial in helping these clients to achieve their self-determined career goals. Currently, EDS is considered a rare disease or disorder with no cure; management of symptoms is currently the only treatment goal. With the field of medicine ever evolving and advancing, the hope is that one day there will be a cure for EDS. Until that time, VR professionals should not be surprised to see a ‘zebra’ like Jane appear in their office looking for help, guidance, and support in navigating the world of work with an emerging disability. Although the diagnosis of EDS dramatically changed Jane’s life from the life that she had planned, with the help of her mental health counselor and VR professional, Jane was able to successfully complete a graduate program, become a mental health counselor, and secure gainful employment with proper accommodations. Jane now specializes in working with clients with chronic pain and chronic health conditions, helping others navigate life after diagnosis.

Conflict of interest

None to report.

Footnotes

Appendix: Resources

Resources for Information About EDS
The Ehlers-Danlos Society
http://ehlers-danlos.com
EDS Today
http://www.edstoday.org
Genetic and Rare Diseases (GARD) Information Center
http://rarediseases.info.nih.gov/GARD/
NIH/National Institute of Arthritis and Musculoskeletal and Skin Diseases
http://www.niams.nih.gov/
Joint Hypermobility Handbook – A Guide for the Issues and Management of Ehlers-Danlos Syndrome Hypermobility Type
and the Hypermobility Syndrome by Brad Tinkle
Living Life to the Fullest with Ehlers-Danlos Syndrome: Guide to Living a Better Quality Life While Having EDS by Kevin Muldowney
Zebras of Hope: A Guide to Living with Ehlers-Danlos Syndrome by Ellen Kelleher
A Multidisciplinary Approach to Managing Ehlers-Danlos (Type III) – Hypermobility Syndrome: Working with the Chronic Complex Patient by Isobel Knight and Rodney Grahame

Acknowledgments

Special thanks to Dr. Lynn Koch for her assistance in editing and preparing this manuscript.

References

Barlow

, Wright

, Sheasby

, Turner

, & Hainsworth

(2002). Self-management approaches for people with chronic conditions: A review. Patient Education and Counseling, 48, 177–187.

Beatty

(2012). Career barriers experienced by people with chronic illness: A U.S. study. Employee Responsibility and Rights Journal, 24, 91–110.

Beatty

, & Joffe

(2006). The career effects of chronic illness. Organizational Dynamics, 35(2), 182–195.

Betz

(2004). Contributions of self-efficacy theory to career counseling: A personal perspective. The Career Development Quarterly, 52, 340–353.

Betz

, & Schifano

(2000). Evaluation of an intervention to increase realistic self-efficacy and interests in college women. Journal of Vocational Behavior, 56, 35–52.

Brown

R. T.

, Daly

B. P.

, & Rickel

A. U.

(2007). Chronic illness in children and adolescents. Cambridge, MA: Hogrefe and Huber Publishers.

Castori

, Morlino

, Celletti

, Celli

, Morrone

, Colombi

, & … Grammatico

(2012). Management of pain and fatigue in the joint hypermobility syndrome (a.k.a. Ehlers-Danlos syndrome, hypermobility type): Principles and proposal for a multidisciplinary approach. American Journal of Medical Genetics Part A, 158A(8), 1–16.

Centers for Disease Control and Prevention (CDC). (2016). Chronic diseases: The leading cause of death and disability in the United States. Retrieved from http://www.cdc.gov/chronicdisease/overview/

Charmaz

(1983). Loss of self: A fundamental form of suffering in the chronically ill. Sociology of Health and Illness, 5(2), 168–195.

10.

Chopra

(2015). Pain management in Ehlers-Danlos syndrome [PowerPoint slides]. Retrieved from http://ehlers-danlos.com/2015-annual-conference-files/Chopra.pdf

11.

Dysautonomia International. (2012). What is dysautonomia? Retrieved from http://www.dysautonomiainternational.org/page.php?ID=34

12.

Dysautonomia Support Network. (2015). What is dysautonomia? Retrieved from http://www.dysautonomiasupport.org/#!dysautonomia/fq5ld

13.

Eurodis. (2014). What is a rare disease? Retrieved from http://www.eurordis.org/content/what-rare-disease

14.

Job Accommodation Network. (2016). Searchable online accommodation resource. Retrieved from https://askjan.org/soar/index.htm

15.

Knight

(2011). A guide to living with hypermobility syndrome: Bending without breaking. Philadelphia, PA: Singing Dragon.

16.

Koch

L. C.

, & Rumrill

P. D.

(2017), Rehabilitation counseling and emerging disabilities: Medical, psychosocial, and vocational aspects. New York, NY: Springer Publishing Company.

17.

Kralik

, Koch

, Price

, & Howard

(2004). Chronic illness self-management: Taking action to create order. Patient Involvement in Clinical Nursing, 13, 259–267.

18.

Kyngas

(2004). Support network of adolescents with chronic disease: Adolescents’ perspective. Nursing and Health Sciences, 6, 287–293.

19.

Levy

H. P.

(2016). Ehlers-Danlos syndrome, hypermobility type. GeneReviews [Internet]. Retrieved from http://www.ncbi.nlm.nih.gov/books/NBK1279/#eds3.Chapter_Notes

20.

Luzzo

, Funk

, & Strang

(1996). Attributional retraining increases career decision-making self-efficacy. The Career Development Quarterly, 44, 378–386.

21.

Milhorat

T. H.

, Bolognese

P. A.

, Nishikawa

, McDonnell

N. B.

, & Francomano

C. A.

(2007). Syndrome of occipitoatlantoaxial hypermobility, cranial settling, and chiari malformation type I in patients with hereditary disorders of connective tissue. Journal of Neurosurgery: Spine, 7, 601–609.

22.

National Organization for Rare Disorders. (2007). Ehlers danlos syndrome. Retrieved from: http://rarediseases.org/rare-diseases/ehlers-danlos-syndrome/

23.

Roessler

R. T.

, & Gottcent

(1994). The work experience survey: A reasonable accommodation/career development strategy. Journal of Applied Rehabilitation Counseling, 25(3), 16–21.

24.

Roessler

, Reed

, & Brown

(1998). Coping with chronic illness at work: Case studies of five successful employees. Journal of Vocational Rehabilitation, 10, 261–269.

25.

Rumrill

, & Bishop

(2015). Introduction to the special issue: Vocational rehabilitation issues and outcomes for people with chronic illnesses. Journal of Vocational Rehabilitation, 42, 99–100.

26.

Sullivan

K. R.

, & Mahalik

J. R.

(2000). Increasing career self-efficacy for women: Evaluating a group intervention. Journal of Counseling and Development, 78, 54–62.

27.

Tinkle

(2008). Issues and management of joint hypermobility: A guide for the ehlers-danlos syndrome hypermobility type and the hypermobility syndrome. Greens Fork, IN: Left Paw Press.