Maximizing quality of life in adults with cystic fibrosis: The critical role of certified rehabilitation counselors

Cystic fibrosis (CF) is a recessive genetic disorder which occurs in approximately 1 per 3,500 births per year (Cystic Fibrosis Foundation, 2011). Cystic fibrosis is a multi-system disorder in which exocrine gland dysfunction causes an abnormally thick viscosity of exocrine gland secretions including mucus, saliva, and sweat, consequently obstructing the respiratory and digestive systems (Berge & Patterson, 2004). In the past, poor food absorption and chest infection greatly increased CF morbidity (Kepron, 2003). Fortunately, new and more effective treatments continue to improve life expectancy. Whereas a few decades ago children with CF rarely survived past two years of age, many individuals are now surviving into their 40s (Cystic Fibrosis Foundation). The longer life span comes with symptoms and required treatments that can interfere with everyday activities and social interactions and the financial burdens of the illness and treatments can exact a toll on the entire family (Harrop, 2007). Despite extensive treatment routines and health challenges, recent studies indicate that individuals with CF generally lead active, age-appropriate social lives into adulthood (Berntsson, Berg, Brydolf, & Hellstrom, 2007; Pfeffer, Pfeffer, & Hodson, 2003; Pizzignacco & de Lima, 2006). In the Cystic Fibrosis Foundation's (2011) 2009 Patient Registry Annual Data Report, 25% of adults with CF were students, 35% were working full-time, 11% worked part-time and 39.2% were married or living with a partner. Of these 26,477 individuals, 47% were age 18 or older (Cystic Fibrosis Foundation). With a longer expected lifespan, CF now affects more stages of the life cycle as individuals with CF attend college, start careers, marry, and decide whether or not to have children (Berge & Patterson, 2004). Adequate information, skill training, and resources appropriate to both typical and CF-related challenges during stages of life development can help ensure that individuals with CF have the support necessary to live active, fulfilling lives into adulthood. Certified rehabilitation counselors (CRCs) are well-suited to assist individuals with CF as they maximize their quality of life. The purpose of this paper is to present information on the biopsychosocial aspects of CF and propose ways in which CRCs are qualified to provide helpful individual and family support throughout adulthood. Knowledge domains and areas of skill demonstration required to become a CRC qualify CRC professionals to implement appropriate interventions. Examples illustrate how CRCs could be a beneficial addition to treatment teams for individuals with CF in a variety of ways. Review of the Literature Information related to biopsychosocial aspects of cystic fibrosis, human development, and the field of rehabilitation counseling was reviewed, using peer-reviewed journal articles dating from 1984 to 2011. Sources consisted primarily of journal articles located using the PubMed, PsychInfo, CINHAL, and PsychArticles databases in addition to journals and books available in UNC-Chapel Hill libraries. Information from the Council on Rehabilitation Education (CORE) and the Commission on Rehabilitation Counselor Certification (CRCC) websites were used to provide evidence that Certified Rehabilitation Counselors (CRCs) are sufficiently qualified to assist individuals with CF and would be beneficial members of the comprehensive treatment team provided through recognized cystic fibrosis treatment centers in the United States. Health & Psychological Issues in CF In general, most adults with CF contend with diminishing ability to engage in activities due to worsening health (Orenstein, 2004). This can be a particularly difficult adjustment for individuals who are accustomed to being very active. As lung function declines in adulthood, performance on pulmonary function tests decline. The forced expiratory volume in one second (FEV1) measure is a common test used to determine lung functioning compared to what is predicted of a healthy individual of the same age, sex, and height. …