Vocational attainment of adults with CF: success in the face of adversity.

Cystic Fibrosis (CF), an autosomal recessive disease, affects 1 in every 3,419 live births among whites and 1 in 12,163 among non-whites (Kosorok, 1996), making CF the most commonly inherited disease with a fatal outcome in the industrialized nations (Staab, 1998). It affects approximately 30,000 people in the US (Cystic Fibrosis Foundation, 2002). Between 1969 and 1998, the adult proportion of CF patients in the United States increased from 8% to 37% (Cystic Fibrosis Foundation, 1999).

Inherited genetically through both parents, CF affects multiple systems in the body, with its greatest impact upon the lungs and pancreas (Hamlett, Murphy, Hayes, & Doershuk, 1996). There are more than 900 known mutations of the CF gene, resulting in a wide range in the severity of the gene's expression (Cystic Fibrosis Mutation Database, July 2000). Lung abnormalities are the most common complications for CF patients, and occur in 50% of cases (Cystic Fibrosis Foundation, 1995). The buildup of secretions, infection, and inflammation can lead to permanent scar tissue, thereby reducing lung function. Poor lung function is a major obstacle for most CF patients, and typically decreases as people mature. On average, seven-year-olds with CF have near normal lung function. Lung function capacity decreases to approximately 69% in 18-year-olds and to 50% in 30-year-olds. Adults with CF face an increased likelihood of producing excess sputum, and they are more likely to develop hemoptysis (coughing up blood), pneumothorax (collapsed lung), and infections requiring intravenous antibiotic treatment (Orenstein, 1997).

Unfortunately, complications of CF are common. CF patients commonly experience chronic nasal congestion due to hyperactive mucus-secreting glands, nasal polyps, and sinus disease. Blockage of the pancreatic ducts leads to inadequate digestion and absorption of nutrients, especially fats, and requires daily enzyme supplements to aid digestion. Persons with CF often experience delayed growth and maturation, even with digestive enzymes and nutritional supplements. Fertility is an important issue because men with CF are typically sterile due to obstructive azoospermia, and women may suffer from progression of lung disease while pregnant. These issues and complications are both physically and emotionally taxing, thus adding to the challenges in daily living that people with CF experience (Hamlett, Murphy, Hayes, & Doershuk, 1996).

Management of CF is time consuming and can significantly burden the individual. Management may include chest physiotherapy two to three times daily, increased caloric intake, and routine use of medications such as bronchodilators and antibiotics (Hamlett et al., 1996). With the onset of chronic, progressive bronchial infection, the lungs of an individual with CF become compromised. The infecting bacteria usually become resistant to oral antibiotics, and most individuals require intravenous (IV) antibiotics at some point (Hamlett et al., 1996). Although many patients are now able to receive IV antibiotics at home, hospital care is often still required for such treatment (Cystic Fibrosis Foundation, 1999).

While the survival rates of persons with CF has increased significantly, morbidity continues to be high because of progressive lung disease, chronic airflow obstruction, and progressive deterioration of respiratory function (Congleton, Hodson, & Duncan-Skingle, 1996). Furthermore, individuals with CF continue to have complications from other related medical conditions such as diabetes, intestinal obstruction, cirrhosis, hemoptysis, and pneumothorax.

Medical advancements in the diagnosis and treatment of CF have contributed to prolonged life (Goldbeck, Schmitz, Henrich, & Herschbach, 2003). The median length of survival increased from one year in the 1940's to 30 years in 1993 (Fiel & Fitzsimmons, 1997). It is projected that children born with CF in 2001 will have a median life expectancy of more than 40 years (Doull, 2001) and more than 90% of children born in 1990 will reach adulthood (Elborn, Shale, & Britton, 1991). Clearly, medical advancements have had a tremendous impact on the survival of individuals born with CF. Now that more individuals with CF are living into adulthood, vocational attainment is an important consideration. Given the medical burden imposed by CF, what happens to these individuals vocationally when they reach adulthood? Shepherd et al. (1990) found that adults with CF did not differ from their healthy peers on measures of emotional social support, self-esteem or life satisfaction, however, they were less likely to be employed. Mungle, Burker and Yankaskas (2002) made the argument that individuals with CF can be employed and can be assisted in that goal by vocational rehabilitation counselors. Descriptive studies have reported that despite the unpredictable prognosis of CF, daily time-consuming self-care therapies, and the potential for job discrimination, adults with CF are able to effectively function in society and maintain employment (Arehart-Treichel, 1984; Nicholson, 1993).

Despite the increased number of working-age individuals with CF, research on career choice and work disability in this population has received very little attention (Gillen, Lallas, Brown, Yelin, & Blanc, 1995). In 1980, Goldberg, Shwachman, and Isralsky noted that, "Although the medical outlook for cystic fibrosis has improved considerably, there is apparently a cultural lag between the new advances known to a group of specialists and the lack of acceptance by the rehabilitation community of this group of patients" (p. 218). Despite this call to action for rehabilitation professionals 25 years ago, little has yet to be done to address documentation of work status or vocational issues among CF adults. Using both ERIC and PsychInfo, we searched the major journals in the field of rehabilitation and vocational rehabilitation: Journal of Applied Rehabilitation, Journal of Rehabilitation, Rehabilitation Counseling Bulletin, Rehabilitation Psychology, Rehabilitation Education, and the Journal of Vocational Behavior. In the past 15 years, only one article focused on vocational rehabilitation counseling for adolescents and adults with cystic fibrosis (Mungle, Burker, & Yankaskas, 2002).

In the recent past, what information existed about the vocational potential of individuals with CF indicated a limited expectation of significant vocational success. The 7th edition of the Certified Rehabilitation Counselor (CRC) Exam Guide to Success stated,

   Vocational considerations include support services to the
   parents so that their child's disability does not interfere
   with their own vocational stability. Need frequent breaks
   for "beatings" for lung percussion to dislodge mucus.
   Coughing can interfere with talking as a condition of
   employment (e.g., telemarketing). Need sedentary job
   due to limited pulmonary function and fatigue (Weed &
   Hill, 2001, p. 110).

In addition, it appears that few persons with CF are being referred for and are receiving state Vocational Rehabilitation services. Out of 55,505 persons with disabilities served in a southeastern state for the fiscal year ending June 30, 2003, only 36 had CF as their primary diagnosis (North Carolina Division of Vocational Rehabilitation, 2003). Out of 1,279,682 clients served in the Department of Vocational Rehabilitation nation-wide, 486 or .038% had the diagnosis of Cystic Fibrosis (Rehabilitation Services Administration, 2003).

This paper provides data about the vocational attainment of a group of adults with CF. The purpose is to address a gap in the literature about patients with this disease and to correct stereotypes about CF in the rehabilitation community. It is hoped that the data in this paper will inspire rehabilitation professionals to better serve this population through research and direct service provision.

Methods

Subjects

After institutional review board approval and written informed consent, 91 males and 92 females with CF who were seen at University of North Carolina (UNC) Hospitals for routine care in the UNC Adult CF Center or who were being evaluated for their candidacy for lung transplant surgery agreed to participate in this study. Participants were recruited from both clinics to ensure a wide range of disease severity in adults with CF.

Procedures

A research assistant who was not a member of the adult CF care team or the lung transplant team recruited participants. For those individuals who expressed an interest in participating, an informed consent form was provided and explained, and the study was described in detail. Participants were given an informed consent form that described the study. Those who agreed to participate signed the consent form and were given a packet of questionnaires, each of which was explained by the research assistant. The questionnaires assessed work status and other demographic factors, including educational status.

Dictionary of Occupational Titles (DOT) (U.S. Department of Labor, 1991) job classifications and strength ratings were identified for each type of work participants reported doing both prior to and at the time of evaluation. The standard vocational preparation (SVP) (U.S. Department of Labor, 1991) and consequent skill-levels (U.S. Social Security Administration Office of Hearings and Appeals as cited in Szymanski, 1996) were identified for each job.

Results

Demographic Factors

A total of 183 individuals (92 females and 91 males) with CF participated in this study. Consistent with the presentation of CF, the majority of participants (98%) were Caucasian, two were Hispanic, one was African American, and one was American Indian. Fifty-six percent of participants were single, 37% were married, 6% were divorced, and 1% were either separated or living with a loved one. Participants had completed an average of 14.25 years of education (SD = 2.5, Range = 4 years to 22 years). The vast majority of individuals (94%) had at least a high school education. The majority of participants (73%) had at least some college education, with 38% having a college degree and 13% having studied at the post-baccalaureate level. At the time of their participation in this study, 13.3% (n = 23) were in school and 86.7% (n = 150) were not. Information about school was unavailable for 10 individuals.

Vocational Factors

Approximately half (48%) of the participants were working at the time of evaluation. Of those who were working, 84% (n = 73) were employed outside of the home and 16% (n = 14) were working inside the home. Data on work status was missing for five subjects. The average number of hours worked by those who were currently working was 32.7 hours/week (SD = 12.6, range = 5 to 60). An additional 16% of participants (n = 29) had worked in the past, but had stopped working prior to the evaluation. Participants who were recruited from the Adult CF Care Center and those who were recruited at the time of their evaluation for lung transplant did not differ on work status (p = .38), so participants were combined for the remainder of the analyses.

Dictionary of Occupational Titles (DOT) (U.S. Department of Labor, 1991) job classifications for all participants who were employed at the time of this assessment or at some point in the past are presented in Table 1. Also identified are the specific job titles held by participants. Jobs were labeled as sedentary, light, medium, heavy, and very heavy identifying the strength rating which is the intensity and duration of standing, walking, sitting, lifting, carrying, pushing, and pulling a person does on the job (U.S. Department of Labor, 1991). The strength ratings indicated that 26% of patients held sedentary jobs (n = 33), 45% were in light jobs (n = 57), 17% were in medium jobs (n = 22), and 2% held heavy jobs (n = 3).

Jobs held by the participants varied according to required training time. The Standard Vocational Preparation (SVP), a U.S. Department of Labor designation for participants' occupations, was identified for each occupation held by the participants. The SVP indicates the amount of training time it takes to prepare for a particular job. The classifications are as follows:

1. Short demonstration

2. Anything beyond short demonstration up to and including 1 month

3. Over 1 month up to and including 3 months

4. Over 3 months up to and including 6 months

5. Over 6 months up to and including 1 year

6. Over 1 year up to and including 2 years

7. Over 2 years up to and including 4 years

8. Over 4 years up to and including 10 years

9. Over 10 years (Dictionary of Occupational Titles, 1991, p. 1009).

Once SVP's were recorded, the jobs were placed into "unskilled," "semi-skilled" and "skilled" categories according to the U.S. Social Security Administration Office of Hearings and Appeals. Unskilled jobs include SVP levels 1 and 2, semi-skilled jobs refer to levels 3 and 4, and skilled jobs include levels 5 through 9 (Szymanski, 1996). The majority of participants who were working at the time of evaluation were in skilled (73%) or semi-skilled (21%) jobs, only 6% were employed in unskilled jobs. Interestingly, a greater number of those who had worked in the past, but who were unemployed at the time of the study had worked in unskilled jobs (31%). A Chi Square analysis revealed significant relationships between the work status at evaluation and skill level of work ([C.sup.2] = 13.7 (2), p. = .001). Participants who were working at the time of evaluation held a substantially higher percentage of skilled jobs (73%) than those who had worked prior to evaluation (48%). Participants who worked prior to but not at the time of evaluation held a higher percentage of unskilled jobs (31%) than those who worked at the time of evaluation (6%). These findings suggest that CF patients with skilled jobs were more likely to maintain their positions than those with unskilled jobs.

Discussion

Twenty-five years ago, Goldberg, Shwachman, and Isralsky (1980) said that the time was right for vocational services and research for individuals with CF. Despite this call, no rehabilitation journal has published a research article on CF and work in the past 15 years. Misperceptions of working with patients with CF existed in the older CRC exam study guides that many rehabilitation professionals utilized, thus perpetuating the belief that individuals with CF are not good candidates for vocational rehabilitation services. The findings in this paper indicate that despite the extensive nature of the effects of CF, the majority of the participants in this study were either working or in school. Of these individuals, slightly more than half were working in professional, technical, or managerial jobs. Another 30% were employed in clerical and sales occupations. The next largest group was working in the service industry. These numbers are impressive given that the majority of these individuals were sick enough to be evaluated for their candidacy for lung transplant. These data suggest that, as for most people, vocation is an important part of life, and the majority of individuals with CF go to school and achieve in careers and work despite declining health.

Contrary to the recommendation by Weed and Hill (2001) that individuals with CF need sedentary jobs, the jobs held by participants with CF in the present study varied in physical demands and strength ratings. The Dictionary of Occupational Titles (U.S. Department of Labor, 2003) describes strength ratings for jobs ranging from sedentary work to very heavy work. Jobs held by CF patients in the present study included: heavy work requirements (construction worker, truck unloader); medium work (ski instructor, housekeeper); light work (restaurant worker, fitness trainer) and sedentary work (telemarketer, social worker, teacher). Consequently, it appears that patients with CF may not only be able to hold jobs, but some may be able to maintain positions that require frequent lifting, walking, standing, pushing, pulling and other physical demands.

The majority of patients were working in skilled or semiskilled jobs. Participants who were working at the time of evaluation held a higher percentage of skilled jobs than those who had worked only prior to evaluation suggesting that CF patients with skilled jobs were more likely to maintain their positions than those with unskilled jobs. Given the results of this study, rehabilitation counselors should be aware of the differences between CF patients who hold skilled versus unskilled positions. Persons with CF in unskilled jobs may require vocational rehabilitation services to assist them in finding new positions they can enjoy and maintain since they are more likely than skilled workers to discontinue employment. Persons in unskilled jobs may also require vocational assistance and advocacy in working with employers to make job modifications so that they can continue working.

The educational attainment of these participants was impressive. The vast majority of individuals had at least a high school education (94%). Most (73%) had at least some college education, 38% had a college degree and 13% had studied at the post-college graduate level. When appropriate, rehabilitation counselors should encourage education in CF adults. Having a higher level of education is more likely to result in skilled employment, which may enhance employability for individuals with CF. This is supported by the present study which found that adults with CF working in skilled jobs were more likely to sustain employment despite declining health.

In conclusion, the goal of vocational rehabilitation services is job placement. Potential clients must show the ability to achieve and maintain employment in order to be eligible for services (NC Division of Vocational Rehabilitation, 2003). CF patients are at risk for being viewed as ineligible for vocational rehabilitation services if the disease is seen as imminently terminal. Research has shown that CF patients are living longer due to medical advances (Elborn, Shale, & Britton, 1991; Fiel & Fitzsimmons, 1997; Goldbeck, Schmitz, Henrich, & Herschbach, 2003; Goldberg, Isralsky, & Shwachman, 1979). With research showing increased life expectancy of individuals with CF and our study's findings, it is clear that CF patients can and do maintain jobs and are therefore, viable candidates for vocational rehabilitation services and career counseling. It is hoped that the jobs attained by individuals with CF reported in the current paper will provide rehabilitation counselors working in the field with innovative avenues to pursue and will spur further research with this population of individuals.

Future research should address the effectiveness of specific counseling strategies for career exploration, job placement and job maintenance. Given that individuals with skilled jobs were more likely to maintain their positions than those with unskilled jobs, future research needs to assess whether unskilled positions are less likely to provide a smoke-free environment or breaks for chest physical therapy, factors that may assist individuals in maintaining employment. Finally, and very importantly, research should assess the needs of the third of patients with CF in this study with no history of employment.

The results of the current study justify the provision of vocational rehabilitation services for persons with CF. It is important for rehabilitation counselors to be aware of the current statistics on the life expectancy of and the issues facing CF patients and consequently be prepared to offer appropriate job placement strategies. We hope the results of this study will encourage rehabilitation counselors to consider patients with CF as good candidates for successful job placement.

Table 1. Job Classifications and Job Titles for Participants
who were Employed and Unemployed at the Time of Evaluation

DOT Job            * % of participants        * % of participants
Classification     working at the             with previous work
and Type of Work   time of evaluation         experience, but
                   and job titles (n=88).     not working at time
                                              of evaluation and
                                              job titles (n=29).

Professional,               53.4%                     37.9%
technical,         accountant, computer       architect,
managerial:        consulting,                teacher, marketing
                   computer programmer,       rep, realtor,
                   network analyst,           librarian,
                   social worker, teacher,    medical resident,
                   lawyer, florist,           trader
                   recreational therapist,
                   speech therapist,
                   administrative
                   assistant, engineer,
                   fitness trainer,
                   ski instructor, modeling
                   instructor, graphic
                   designer, systems
                   analyst, business
                   owner, insurance
                   broker, engineer,
                   interviewer,
                   pharmacist,
                   psychological examiner,
                   public relations
                   specialist

Clerical                    29.5%                     27.6%
and sales          customer care              secretary, cashier,
                   rep, bookkeeper,           retail sales
                   insurance broker,          representative,
                   secretary, cashier,        shipping and receiving
                   retail sales rep, court    supervisor
                   clerk, advertising
                   rep, telemarketer

Service:                     6.8%                     27.6%
                   hotel manager,             fast food worker,
                   house worker,              restaurant worker,
                   undercover shopper,        police dispatcher
                   fast food worker,
                   restaurant worker,
                   babysitter

Agricultural                 1.1%                       0
and Forestry:      lawn service
                   technician

Processing:                   0                         0

Machine                      2.3%                       0
Trades:            mechanic,
                   automated
                   machine tech

Benchwork:                  1.l %                       0
                   dental technician

Structural:                  1.1%                      3.4%
                   construction worker               plumber

Miscellaneous:               4.5%                      3.4%
                   artist, driver manager,      school bus driver
                   photo laboratory
                   technician,
                   truck unloader

* Two jobs could not be categorized due to
incomplete data. Three positions that did
not fall within the Dictionary of Occupational

Titles Classification systems were, "volunteer (n=1),"
"student (n=l)," and "homemaker (n=2)."

References

Arehart-Treichel, J. (1984). Lengthened survival raises problems in cystic fibrosis. JAMA, 252, 2526-2527.

Congleton, J., Hodson, M.E., & Duncan-Skingle, F. (1996). Quality of life in adults with cystic fibrosis. Thorax, 51(9), 936-940.

Cystic Fibrosis Foundation. (2002). Patient Registry 2002 Annual Report. Bethesda, MD: Author.

Cystic Fibrosis Foundation. (1999). CF Facts. Retrieved November 1999, from http://www.cff.org/facts.htm.

Cystic Fibrosis Foundation. (1995). Patient Registry 1994 Annual Data Report. Bethesda, MD: Author.

Cystic Fibrosis Mutation Database. (2000). Retrieved July 2000, from http://www.genet.sickkids.on.ca/cftr-cgi-bin/

Doull, I.J. (2001). Recent advances in cystic fibrosis. Archives of Disease in Childhood, 85(1), 62-66.

Elborn, J.S., Shale, D.J., & Britton, J.R. (1991). Cystic fibrosis: current survival and population estimates to the year 2000. Thorax, 46(12), 881-885.

Fiel, S.B. & Fitzsimmons, S. (1997). Pregnancy in patients with cystic fibrosis. Pediatric Pulmonology, 16, 111-112.

Gillen, M., Lallas, D., Brown, C., Yelin, E., & Blanc, P. (1995). Work disability in adults with cystic fibrosis. American Journal of Respiratory and Critical Care Medicine, 152, 153-15.

Goldbeck, L., Schmitz, T., Henrich, G., & Herschbach, P. (2003). Questions on life satisfaction for adolescents and adults with cystic fibrosis. Development of a disease-specific questionnaire. Chest, 123, 42-48.

Goldberg, R.T., Isralsky, M., & Shwachman, H. (1979). Vocational development and adjustment of adolescents with cystic fibrosis. Archives of Physical Medicine and Rehabilitation, 60, 369-374.

Golberg, R. T., Shwachman, H., & Isralsky, M. (1980). Rehabilitation with cystic fibrosis: From utopia to reality. Rehabiliation Literature, 41 (9-10), 218-228.

Hamlett, K.W., Murphy, M., Hayes, R., & Doershuk, C. F. (1996). Health independence and developmental tasks of adulthood in cystic fibrosis. Rehabilitation Psychology, 41(2), 150-160.

Kosorok, M.R., Wei, W.H., & Farrell, P.M. (1996). The incidence of cystic fibrosis. Statistics in Medicine, 15(5), 449-462.

Mungle, J., Burker, E.J., & Yankaskas, J.R. (2002). Vocational rehabilitation counseling for adolescents and adults with cystic fibrosis. Journal of Applied Rehabilitation Counseling, 33, 15-21.

Nicholson, B. (1993). My experience as an adult with cystic fibrosis. Journal of the Royal Society of Medicine, 86, 30-33.

North Carolina Division of Vocational Rehabilitation. (2003). State-wide service statistics. Raleigh, NC: Author

North Carolina Division of Vocational Rehabilitation. (2003). Goal of the Division of Vocational Rehabilitation. Retrieved July 2003, from http://dvr.dhhs.state.nc.us.

Orenstein, D.M. (1997). Cystic fibrosis and adulthood. In Orenstein, D.M. (Ed.), Cystic Fibrosis: A guide to patient and family (pp. 243-260). Philadelphia, PA: Lippincott-Raven.

Rehabilitation Services Administration. (2003). 2001 Fiscal Year Statistics. Washington, DC: Author.

Shepherd, S.L., Hovell, M.F., Harwood, I.R., Granger, L.E., Hofstetter, C.R., Molgaard, C. & Kaplan, R.M. (1990). A comparative study of the psychosocial assets of adults with cystic fibrosis and their healthy peers. Chest, 97, 1310-1316.

Staab, D., Wenninger, K., Gebert, N., Rupprath, K., Bisson, S., Trettin, M., Paul, K.D., Keller, K.M. & Wahn, U. (1998). Quality of life in patients with cystic fibrosis and their parents: what is important besides disease severity? Thorax, 53(9), 727-731.

Szymanski, E.M., & Parker, R.M. (1996). Work and disability: Issues and strategies in career development and job placement. Austin, Texas: Pro-ed.

U.S. Department of Labor. (1991). Dictionary of Occupational Titles (Rev. 4th ed.). Washington, DC: U.S. Government Printing Office.

U.S. Department of Labor. (2003). Dictionary of Occupational Titles. Retrieved July 2003 from www.occupationalinfo.org/ appendix_1.html#II.

Weed, R., & Hill, J. (2001). CRC Exam Guide to Success. Athens, GA: E & F Vocational Services.

Eileen J. Burker

University of North Carolina at Chapel Hill

Christy Trombley

University of North Carolina at Chapel Hill

Jan Sedway

University of North Carolina at Chapel Hill

Beth Parker Yeatts

University of North Carolina at Chapel Hill

Stacia Carone

University of North Carolina at Chapel Hill

Eileen J. Burker, Ph.D., Division of Rehabilitation Psychology and Counseling, CB #7205, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599-7205 Email: [e-mail]