What deficits in sexual and reproductive health knowledge exist among women with cystic fibrosis? A systematic review.
Abstract: The life span of patients with cystic fibrosis (CF) continues to extend due to advances in treatments and care. The rate of pregnancy for female patients with CF has also continued to rise. The purpose of this study was to systematically review the available literature on female patients with CF and their knowledge of sexual and reproductive health. A priori, the research question was posed: What deficits in sexual and reproductive health knowledge exist among women with cystic fibrosis? Three broad areas of knowledge lacking in female patients with CF were identified: physiological, genetic, and psychosocial. The results imply female patients with CF are not equipped with adequate information for the decision-making process involved in sexual and reproductive choices. Social workers play an important and dynamic role on the multidisciplinary CF care team and can improve the quality of life for women with CF by addressing the psychosocial processes associated with the disease. Because of social work's important role in the holistic care approach to CF and other chronic and debilitating illnesses, social work literature should focus greater attention on these issues.

KEY WORDS: chronic disease; cystic fibrosis; reproductive health; systematic literature review
Article Type: Report
Subject: Cystic fibrosis (Social aspects)
Cystic fibrosis (Care and treatment)
Reproductive health (Social aspects)
Health literacy (Research)
Women (Health aspects)
Women (Social aspects)
Author: Gage, L. Ashley
Pub Date: 02/01/2012
Publication: Name: Health and Social Work Publisher: Oxford University Press Audience: Academic; Professional Format: Magazine/Journal Subject: Health; Sociology and social work Copyright: COPYRIGHT 2012 Oxford University Press ISSN: 0360-7283
Issue: Date: Feb, 2012 Source Volume: 37 Source Issue: 1
Topic: Event Code: 290 Public affairs; 310 Science & research
Geographic: Geographic Scope: United States Geographic Code: 1USA United States
Accession Number: 306241022
Full Text: 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. In the event of a pregnancy, female patients with CF must be concerned with the dual care of CF and pregnancy and the possible short- and long-term physiological impacts (Edenborough et al., 2008).

Many complications accompany pregnancy, several of which are of particular concern to women with CF. For women without CF, the changes during pregnancy tend to be manageable; for women with CF, the changes may result in varying and more severe complications. The usual adjustments in pulmonary function during pregnancy pose a risk for female patients with CF who already have suppressed respiratory systems (Whitty, 2010). Nutritional needs increase in pregnancy, and the malabsorption issue associated with CF can decrease the ability to reach those nutritional needs; in particular, CF-related diabetes (CFRD) can cause serious complications during pregnancy (Whitty, 2010). Complications arise with lung transplantation, which is a potential treatment for CF patients (Whitty, 2010). Female patients with CF who become pregnant have increased health care costs as well. From a Canadian and U.S. sample, researchers showed that female patients with CF attended more outpatient appointments before and during pregnancy than female patients with CF who did not become pregnant (McMullen et al., 2006). They had more frequent exacerbations and inpatient stays during pregnancy and required more pharmacological interventions, and CFRD treatment doubled compared with their baseline (McMullen et al., 2006).

AIM

The purpose of this systematic review was to explore the a priori research question: What deficits in sexual and reproductive health knowledge exist among women with cystic fibrosis? The answer has the potential to improve clinical practice (Counsell, 1997) and has significance for identifying essential information that CF care teams might omit or poorly communicate to female patients with CF.

METHOD

In this literature review, I systematically evaluated what reproductive and sexual health knowledge female patients with CF reported lacking. A priori, a focused research question was developed to guide the systematic review. From this question, the following search terms were identified: "women and communication and cystic fibrosis," "pregnancy and cystic fibrosis," "communication and fertility and cystic fibrosis," "sexual health knowledge and cystic fibrosis," and "reproductive health knowledge and cystic fibrosis." MEDLINE/PubMed, CINAHL, and PsyciNFO databases were used to locate articles based on predetermined criteria. Inclusion in the review was based on the following criteria: addresses information between care center staff and female patients or patients' deficits in knowledge; provides empirical evidence from qualitative or quantitative designs; and was published in English. Date of data collection or date of publication was not considered for exclusion due to the lack of available literature.

Contributing factors were evaluated according to evaluative design, subjects, and communication themes. The MEDLINE/PubMed search resulted in 2,966 results, and a database search of CINAHL and PsycINFO resulted in 1,035 additional results. The search was also expanded to Google Scholar and Google Web searches, although no additional articles were retrieved from these search engines. Eighteen articles were identified as potentially applicable to the research question. From those, only eight articles met inclusion criteria, and a hand search was completed to locate other possible articles. Five more articles were located through the hand search, but four of those did not differentiate data based on gender (Conway, Pond, Watson, & Hanmett, 1996; Hames, Beesley, & Nelson, 1991; Houser et al., 2008; Nolan, Desmond, Herlich, & Hardy, 1986). Those were excluded, and only nine articles remained; the content in these nine articles compose the basis for this systematic review (see Table 1).

The final nine articles were assessed for methodological rigor. A scoring system was used for both quantitative and qualitative studies. Due to the lack of available research, all nine studies were included despite low scores of methodological rigor. A general data extraction form was completed for each article and then transformed into a spreadsheet for ease of comparison. The findings were summarized, and thematic analysis was completed to categorize major trends. With respect to the knowledge regarding sexual and reproductive behavior, the findings in this review indicate that women with CF lack knowledge about physiological and psychological effects of sexual and reproductive behavior as well as the genetics of CF.

RESULTS

The literature herein is a substantive presentation of the available literature, but it likely does not represent every article available on the topic. The majority of articles originated from Europe (United Kingdom, n = 4; Sweden, n = 1; Poland, n = 1), with two articles from the United States and one from Australia. The nine articles included both quantitative (n = 6) and qualitative analyses (n = 3). Quantitative designs largely focused on testing participants' knowledge, and qualitative analyses reported patients' perceptions of what knowledge they lacked. The themes were similar in both quantitative and qualitative studies. For example, female patients with CF reported that health information is difficult to understand because service providers present it in difficult terms (Simcox, Hewison, Duff, Morton, & Conway, 2009), and patients demonstrated a poor understanding of genetics, which may be reported in mathematical terms instead of through colloquial conversation (Korzeniewska, Smejda, Skorupa, & Stelmach, 2005; Sawyer, Tully, & Colin, 2001).

Physiological Aspects

The reported findings show that female patients with CF would like more information on the physiological aspects of CF in relation to sexual and reproductive health issues. Women with CF perceive that they lack this information, and, moreover, they scored poorly when their knowledge was assessed. These findings were identified in all nine reviewed articles and highlight reports of misunderstood fertility information and the physiological impact of pregnancy.

In a qualitative study, the participants with CF who were mothers reported they were unaware of the possible short- and long-term physical impacts of pregnancy prior to their becoming pregnant (Simcox et al., 2009). Women specifically cited a need to understand the rigors of postpartum care, which usually involves intravenous antibiotic treatment, the possibility of long-term damage as a result of a suppressed immune system, and the overall physiological decline that occurs during pregnancy (Simcox et al., 2009). Likewise, Fair, Griffiths, and Osman (2000) reported that women with CF specifically want information on the longterm effects of pregnancy. In a quantitative survey testing patient knowledge, Sawyer et al. (2001) found that only 50 percent of Australian female patients with CF knew pregnancy would have an impact on respiratory function. The findings in a recent study in Poland indicated that approximately 67 percent of Polish women with CF were aware that pregnancy would affect health (Korzeniewska et al., 2009).

Female patients with CF also reported misunderstanding their fertility. Thirty-three percent of Polish women with CF reported understanding that female fertility is decreased in female patients with CF, yet the majority of those women incorrectly responded that fertility was normal in female patients with CF (Korzeniewska et al., 2009). These contradictory reports illustrate the lack of clarity patients may have regarding fertility. Sawyer, Phelan, and Bowes (1995), however, reported that 65 percent of the women knew they had reduced fertility, and this is consistent with findings in a more recent study that showed 68 percent of women with CF reported being aware of reduced fertility (Siklosi, Gallagher, & McKone, 2010). Although female patients with CF reported in these studies that they are aware of reduced fertility, they may not be clear on the degree of reduction. In fact, in two samples, female patients with CF actually underestimated their fertility (Hull & Kass, 2000; Johannesson, Carlson, Brucefors, & Hjelte, 1998).

Women with CF reported that they lacked sufficient information about contraception (Gatiss, Mansour, Doe, & Bourke, 2009). Siklosi et al. (2010) found that 97.4 percent of female patients with CF correctly reported they must use contraception to avoid pregnancy. However, only 9.5 percent of women in another sample reported that they received contraception information from their CF care team (Gatiss et al., 2009). Specifically, Gatiss and colleagues found that 38 percent of women with CF reported that they were ever given advice on the interaction of CF malabsorption and contraception and 57 percent reported that they never received any information on the possible interaction between antibiotics and contraception.

Genetic Aspects

Sixty-five percent of female patients with CF reported it was important to know the risk of having a child with CF prior to having children (Sawyer et al., 1995). Women with CF tend to overestimate the likelihood of having children with CF (Korzeniewska et al., 2009; Sawyer et al., 1995), and only 15 percent of female patients accurately identified the risk of having a child with CF if the other parent is not a carrier (Sawyer et al., 1995). In two of the nine articles, female patients with CF were unable to accurately report their risk for having a child with CF or genetic transmission of the CF gene. Although female patients with CF demonstrated a deficit in genetic knowledge, they also reported that passage of the CF gene was an important factor in making decisions about pregnancy (Sawyer et al., 1995; Simcox et al., 2009).

Psychosocial Aspects

Three articles mentioned aspects of the psychosocial domain. Of those, only one article included an objective psychosocial measurement. The remaining studies collected qualitative data in which female patients with CF mentioned a need for more psychosocial information.

Female patients with CF reported that the information they received from their CF care providers was largely clinical in nature and did not focus on other information the women needed (Simcox et al., 2009). Some women reported that the clinical information provided was actually a source of anxiety due to the realization of CF-related health complications (Simcox et al., 2009). Korzeniwska et al. (2009) reported anxiety associated with pregnancy decision making, particularly the fear of having a child with CF.

Simcox et al. (2009) identified a distinct preparation process during the reproductive decision-making process. Women with CF reported that care providers should provide a more social view of pregnancy and sexual health information to better inform this process. Female patients with CF requested more experiential information, explaining that they would like to be informed of the full experiences of other women with CF who have been through the decision process and have a variety of health statuses that may affect the decisions they make (Simcox et al., 2009). This is congruent with information reported by Johannesson et al. (1998): Female CF patients reported a need for more psychoeducation and counseling during adolescence and young adulthood as they experience sexual maturation and begin to cope with decisions surrounding fertility and sexual health. Female CF patients reported that young women with CF would benefit from mentorship and support of patients older than them who can provide experiential information to normalize the experience of having CF and dealing with sexual and reproductive health issues (Johannesson et al., 1998).

DISCUSSION

On the basis of the findings of this review, female patients with CF report a lack of comprehensive sexual and reproductive health knowledge in several areas, including the physiological aspects of fertility and pregnancy, genetic transmission of the CF gene, and psychosocial implications of pregnancy and parenthood. Addressing these deficits allows patients to make more informed decisions and be better prepared for unexpected pregnancies or conception issues. When women understand the risks and limitations of pregnancy and motherhood, negative effects on the female patient with CF and her family can be avoided or prepared for.

The first piece of information women with CF need is that they are genetically predisposed to passing on the CF gene. Although the gene is not always passed on and the other parent must also carry the gene for expression of CF, understanding the transmission of the disease is important information for women making reproductive decisions. Given that genetic screening is available, further information could assist in the decision to conceive. If the potential biological father were tested and found to have a recessive CF gene, in which case it is possible to have a child with CF, the parents could make a more informed decision.

Women with CF need information about fertility to avoid risks associated with reproduction. A lack of clear information regarding the CF-specific physiology of reproduction places women with CF at risk of sexually transmitted infections and unplanned pregnancies. When risk perception of pregnancy is low, women are less likely to take precautions against pregnancy. If women underestimate their ability to conceive, they may put less importance on contraception, resulting in unplanned pregnancies. In contrast, if women overestimate their likelihood to conceive, they can experience unexpected distress as a result of a more difficult conception process than mothers without CF.

In the absence of information about reproduction, female patients with CF are challenged to make balanced decisions about whether to pursue pregnancy or to continue an unplanned pregnancy. There is a range of consequences for women when they misjudge the risks and long-term effects of pregnancy. The long- and short-term physiological effects of pregnancy can decrease an already-shortened life span.

Ultimately, the family members of women with CF are often burdened with the financial costs of the woman's health care and her work restrictions. The strain of a mother's CF, particularly the frequent appointments and intermittent hospitalizations that decrease the availability of a CF mother, affects children. The deterioration of health affects the parent's ability to interact and support children and the other parent. A shortened life span can leave children with one parent, encumbering that parent and extended family. The social strains of CF on the family unit bring into question the ethical considerations associated with the ever-decreasing physical functioning, shortened life spans, and genetic transmission of CF.

The limitations of this review include the key words selected and the journals indexed by the designated databases. The nine research studies discussed in this review of the literature show limited empiric research focused on the knowledge that women with CF have regarding sexual and reproductive behaviors. The majority of the evidence found was derived from quantitative studies with small samples that were descriptive rather than analytical, and the accompanying statistical methodology was not as powerful as more sophisticated analytical models. Also, data extraction and methodological testing did not involve multiple reviewers to test for reliability. The lack of rigorous literature makes it evident that multi-method designs are needed to understand the intersection of what deficits in knowledge exist and how and when knowledge is communicated. Finally, seven out of the nine articles originated outside of the United States.

Despite these limitations, this review demonstrates a clear lack of sexual and reproductive health knowledge among female patients with CF. We know that sexual and reproductive health is different for women with CF and should therefore be discussed with a CF team. Because of the sensitivity of the topics, we know patients are challenged to bring up topics related to sexual health. There is a need for greater communication between women with CF and their multidisciplinary care team members. Deficits in sexual and reproductive health knowledge can go unrecognized until women with CF have an unplanned pregnancy or decide they want to have a child.

The conclusions from this systematic review are clear. Female patients with CF report deficits in sexual and reproductive health knowledge. The need for specific sexual and reproductive health education is required to deter unplanned pregnancies and dissonance from ethical decision making. Knowledge is lacking in the physiological and genetic areas, but there is even less knowledge of psychosocial issues. As evidenced by these deficits, there is currently inadequate education regarding the aspects of sexual and reproductive health in female patients with CF.

Implications

The results of this review have several implications for practice and research. First, there is a need for research in the area of CF care that has methodological rigor and represents the U.S. CF population. Rigorous research is needed that investigates the extent to which a large sample of women with CF in the United States receive the sexual and reproductive knowledge they need to make informed decisions about their health. Similar studies are needed to understand the psychosocial needs of women with CF who have children throughout their life span and progression of their disease. Given that communication about sexual and reproductive health is lacking among women with CF, studies are needed that determine the extent to which enhanced communication affects knowledge and psychosocial outcomes. As the adult CF population grows and life spans lengthen, the quality of adult care must continue to be reviewed through empirical research.

Practice with CF patients can be improved through accessible, accurate, and understandable knowledge. Limited sexual and reproductive health literacy is costly for the family and female patients with CF and has the possibility for improvement. Sexual and reproductive health information must be introduced early in adolescence. The psychosocial challenges in regard to sexual maturation complicated by CF and the potential issues with future reproduction warrant an introduction of sexual and reproductive education and support during adolescence. Beyond physiological knowledge, women need psychosocial information and support related to reproductive issues. Multidisciplinary care teams have the expertise to provide the psychosocial resources, information, and support women need to deal with CF-specific reproductive issues. Discussions around sexual and reproductive health must extend beyond the somatic mechanics of health to prevent further psychosocial stress.

Implications for Social Work

The conclusions of this systematic review have numerous implications for social work practice with women who have CF and other chronic diseases. Social isolation, depressed quality of life, and treatment and symptom management are issues faced by other women with chronic diseases. These similarities are reason for social workers to provide the resources and formal support women with chronic disease require.

Psychosocial perspectives are especially sparse in the literature and, therefore, there is a dearth of evidence on which to inform social work practice. Social workers have a unique perspective and set of skills that are invaluable to the holistic care approach. By increasing awareness of these sexual and reproductive health issues encountered by women with disabilities, social workers can connect patients and families to the support and resources they require for coping. The health and well-being of women with disabilities can be improved by calling attention to the sexual and reproductive issues among women with disabilities. In particular, social workers must stay informed of the growing body of knowledge to perform interventions and promote advocacy for this population.

Original manuscript received May 23, 2011

Final revision received August 10, 2011

Accepted August 17, 2011

Advance Access Publication June 29, 2012

doi: 10.1093/hsw/hls003

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L. Ashley Gage, MSW, is a doctoral candidate, School of Social Work, University of Missouri, 718 Clark Hall, Columbia, MO 65211; e-mail: gagela@health.missouri.edu.
Table 1: Summary of Studies

                                  Sample
Study                 Country     Size        Design

Fair or al. (2000)    United      54          Quantitative
                      Kingdom

Simcox et al.         United      12          Qualitative
(2009)                Kingdom

Joharinesson et al.   Sweden      14          Qualitative
(1998)

Korzeniewska          Poland      Not         Quantitative
et al. (2009)                     available

Sawyer et al.         Australia   55          Quantitative
(1995)

Tuchman et al.        United      41          Quantitative
(2010)                States

Hull & Kass           United      Not         Qualitative
(2000)                States      available

Gatiss et al.         United      42          Quantitative
(2009)                Kingdom

Siklosi et al.        United      49          Quantitative
(2010)                Kingdom

Study                 Findings               Score

Fair or al. (2000)    Women were not           11
                      informed of the
                      long-and short-term
                      consequences on
                      their bodies from
                      pregnancy. Patients
                      suggested beginning
                      sexual and
                      reproductive health
                      education in
                      adolescence.

Simcox et al.         Pregnancy                11
(2009)                information is not
                      presented in an
                      easy-to-understand
                      format. This
                      includes the short-
                      and long-term
                      physiological
                      consequences,
                      psychosocial issues,
                      and genetics.
                      Patients reported
                      wanting ore
                      experiential reports
                      from other CF
                      mothers.

Joharinesson et al.   Cystic fibrosis-         11
(1998)                specific fertility
                      issues are not
                      clearly
                      communicated,
                      ethical discussions
                      surrounding
                      pregnancy are not
                      discussed, and women
                      tend to delay
                      relationships due to
                      their fears
                      surrounding CF.

Korzeniewska          Of participants,         10
et al. (2009)         32.4% correctly
                      understood problems
                      with fertility and
                      76.5% reported
                      anxiety associated
                      with the possibility
                      of having a child
                      with CF. Patients
                      tended to
                      overestimate the
                      risk of having a
                      child with CF.

Sawyer et al.         Only 49% knew            10
(1995)                pregnancy can reduce
                      lung function, and
                      85% were not able to
                      correctly report the
                      risk of having a
                      child with CF.

Tuchman et al.        Women reported they      10
(2010)                did not receive
                      enough CF-related
                      reproduction
                      information or
                      enough information
                      about the
                      psychosocial risks
                      associated with
                      pregnancy and their
                      fertility.

Hull & Kass           Women tended to          10
(2000)                underestimate their
                      fertility.

Gatiss et al.         Women reported           8
(2009)                deficits in
                      information
                      regarding
                      contraceptives and
                      CF-related sexual
                      and reproductive
                      health. Only 9.5%
                      reported that
                      contraceptive
                      information came
                      from CF staff.

Siklosi et al.        Of participants, 68%     11
(2010)                reported
                      understanding that
                      their fertility was
                      reduced.

Note: CF=cystic fibrosis.
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