What Deficits in Sexual and Reproductive Health Knowledge Exist among Women with Cystic Fibrosis? A Systematic Review

When female patients with cystic fibrosis (CF) become pregnant, numerous complications and long-term implications need to be considered; thus, knowledge of CF's impact on sexual and reproductive health is important for quality care. As the lifespan of people with CF continues to expand, information regarding sexual and reproductive health must be clearly communicated to people with CF. Two hundred twenty-six female patients with CF were pregnant in 2009; as late as the 1980s, women with CF were cautioned against having children (Cystic Fibrosis Foundation [CFF], 2011). To improve the lives of female patients with CF, more comprehensive and thorough sexual and reproductive health information is necessary to promote and sustain the health and quality of life of patients. This is especially true as the adult CF population continues to grow. Social workers are the experts in psychosocial issues on the CF multidisciplinary team, but there is a lack of social work literature on this topic. To inform social work practice with women who have CF and other chronic and life-shortening illnesses, a systematic review is needed. CF is a genetic disorder characterized by multiple body-system dysfunctions (Strausbaugh & Davis, 2007). Although advances in CF care have extended and improved the lives of CF patients over the last couple of decades, the median age for survival is still only 35.9 years of age (CFF, 2011). Rigorous daily treatments aim to thin and expel mucus, open airways, aid in nutrient absorption, and manage symptoms, and as a result, patients must adhere to regular medical care and treatment regimens to delay progression of the disease. Even so, progression cannot be stopped, and there is no cure for CF. To manage the progression of CF a multidisciplinary team usually collaborates to provide a comprehensive approach to care, an approach that has been associated with better health outcomes in patients with CF (Mahadeva et al., 1998). A social worker plays an integral role on the team by contributing psychosocial expertise for holistic care, and this pivotal role can influence the formation of care plans beyond medical model perspectives. In particular, social workers play an important role by providing psychosocial support and education during the adjustment accompanying medical treatment and advancement of the disease. According to the CFF's clinical practice guidelines, an MSW or clinical psychologist should perform functional psychosocial assessments of a variety of issues, including family planning, emotional development, family structure and coping, and understanding of the CF disease, at least annually (CFF, 1997). The progression of CF differs by gender, both psychosocially and physiologically. Female adolescents with CF were associated with lower quality-of-life scores than males with CF (Arrington-Sanders et al., 2006; Gee, Abbott, Conway, Etherington, & Webb, 2003), and female patients with CF have shorter life spans than men with CF (Rosenfeld, Davis, Fitz-Simmons, Pepe, & Ramsey, 1997). Compared with their male counterparts, female patients with CF tend to have more rapid reduction in lung function (Demko, Byard, & Davis, 1995). In addition, they more frequently contract Pseudomonas aeruginosa, a low antibiotic-susceptible bacteria (Demko et al., 1995), and have more acute pulmonary exacerbations (Block et al., 2006). Researchers have provided preliminary data supporting a hypothesis that women's increased estrogen levels during menstruation may cause additional thickening of mucus, which results in increased difficulty expelling mucus and susceptibility to infection and disease exacerbation (Coakley et al., 2008). Despite normal reproductive anatomy, the mucus in the cervix and fallopian tubes of women with CF is not as conducive to conception as that of women without CF (Oppenheimer & Esterly, 1970). Women with CF do have a better chance at natural reproduction than men with CF. …