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Translated by Vincent Chartier
According to the Cystic Fibrosis Canada (n.d.), around one in every 3,600 children are affected by cystic fibrosis (CF). CF is a fatal genetic disease that causes an autoimmune response focusing essentially the lungs and the digestive system. The symptoms generally include difficulty breathing, chronic pulmonary infections, and digestive issues. Possible complications are cystic fibrosis-related diabetes, hepatic disease (i.e. liver), intestinals distal obstruction syndrome (accumulation of stool in the intestine), osteoporosis, and nasal polyps (Smyth et al., 2014). Because of these various health issues, people living with CF commonly experience pain and discomfort, which impairs their sleep, pulmonary function, and general quality of life (Lechtzin, 2016). The pain is generally less intense and of shorter duration during childhood and teenage years than in adulthood (Lechtzin, 2016).
Despite their illness, teenagers with CF (TCF) must face the same developmental challenges as their peers. Like them, they are building their identity. Like them, they have questions about their sexuality. Like them, they experience things that will affect their sexual trajectory. However, the disease represents a major obstacle to their sexual health. This article acts as an introduction to our present understanding of the sexual health of TCF by using the life course approach. It highlights some of the challenges faced by these boys and girls and offers solutions to the adults around them in order to give these children and teenagers the tools to achieve a fulfilling sexuality.
Towards a Comprehensive Approach to the Sexual Health of TCF
As defined by the life course approach, an individual’s development is a process linked to time and the social, cultural, and historical contexts in which it takes place (Gherghel, 2013). An individual’s life course is multidimensional. It is the combination of various life trajectories, such as familial, educational/professional, social, sexual, etc. Throughout all of these trajectories, the person experiences transitions, i.e. short periods of time that result in a status or role change. The importance of these transitions depends on the moment and context of their occurence (Macmillan and Copher, 2005).
So how does this apply to TCF? In the literature review titled “Sexual and reproductive health in cystic fibrosis: a life-course perspective,” Frayman and Sawyer (2015) note that, in the past, research about the relationship between sexual health and CF focused mainly on reproductive health. More recent research tends to study the sexuality of CF patients in a more global manner, taking into account the other life trajectories as well as major transitions during their teenage years. Using a table, Frayman and Sawyer show the health trajectory in parallel with the educational and sexual trajectories.
Table 1 : Developmental Stages (Frayman and Sawyer, 2015)
This table highlights the relation between the emergence of numerous complications caused by the disease and the period of puberty and first sexual experiences. This relationship aligns with the end of high school, which marks the transition between childhood and adulthood.
Similarly, Smyth and his colleagues (2014) reveal six significant transitions that CF patients go through:
i. Parent’s adjustment to diagnostic;
ii. Beginning of education (including preschool, elementary, and high school);
iii. Transition from parental treatment to self-directed treatment;
iv. Transition from pediatric care to adult care;
v. Entry onto the job market or beginning of higher education ;
vi. Loss of independence (i.e. retirement, loss of activities and functioning, dependance on more intrusive and intensive treatment, transplants, death).
The transitions mentioned above illustrate the passage from the ill child dependant on his parents to a self-reliant adult taking responsibility for his health. Meanwhile, the individual goes from childhood to adulthood, through puberty and their first romantic and sexual experiences. Thus, there is a synchronicity between the need to become mature on a sexual level and the need to become self-reliant with the treatment of the disease.
In other words, both trajectories cross and influence each other in a way that the functioning or malfunctioning of one can have serious consequences on the evolution of the other.
Cystic Fibrosis, Sexual Behaviour, and Risk-Taking
What do we know specifically about the sexual behaviour of TCF? Firstly, it does not differ greatly from other teenagers. Although studies do not agree on the median age at which the first sexual intercourse occurs, researchers have widely concluded that it is comparable to or slightly older than for the rest of the population, that is, around 17 years old (Frayman and Sawyer, 2015). In addition, studies on the sexuality of TCF tend to demonstrate that they are as sexually active as their peers (Frayman and Sawyer, 2015; Kazmerski et al., 2018).
As for risk-taking, Kazmerski and his colleagues (2018) studied 188 young American women with CF aged 15 to 24 and compared them to girls without CF of the same age, with the help of the National Survey of Family Growth (NSFG). The researchers report that sexually active girls with CF present more important gaps than on average when it comes to birth control and sexually transmitted and blood-borne infections (STBBI) screening. As a matter of fact, 35% of the girls with CF had not used any birth control methods in the last 12 months compared to 26% for the overall population. Similarly, only 24% had used a condom compared to 33% in the overall population. As for screening, 19% of the girls with CF had been tested in the last year compared to 34% of all American girls. Despite these results, Kazmerski and his colleagues (2018) have observed an inferior pregnancy rate to the rest of the population of the same age (2% against 24%).
Finding literature specifically about boys with CF has proven to be more difficult. It is possible that the results of the studies done on women also apply to them. For example, some scientific articles illustrate the lack of information for boys concerning STBBI and birth control. However, there is little data to quantify the risk-taking of TCF. Some authors have pointed to the taboo surrounding the sexuality of children with chronic illnesses as the cause.
An STBBI can have dire consequences for TCF of all genders. For example, the presence of certain chronic diseases like hepatitis B and C as well as HIV can be a rejection criterion for a lung transplant, a common surgery for people with CF (Smyth et al., 2014). Moreover, these chronic infections can exacerbate the symptoms of liver disease. It should also be noted that because girls with CF often need to take strong antibiotics, which can increase the likeliness of yeast infections, they are more vulnerable to them (Cystic Fibrosis Canada, n.d.; Malfroot et al., 2005). Nearly half of them will develop at least one yeast infection a year (Kazmerski, 2018).
CF is generally a disease associated with childhood. In the past, people affected by the illness rarely lived beyond the age of 12. Today, the median life expectancy for people with CF is estimated to be between 40 and 50 years old, with women having shorter life expectancy than men (Dodge et al., 2007; Mackenzie et al., 2014). Although the quality of care has significantly increased, it is difficult for TCF to project themselves into the future without knowing when the disease will end their lives prematurely.
Nevertheless, much like their peers without CF, this does not prevent them from looking for a sexual and/or romantic partner during their lifetime. The relational and health trajectories then meet, which poses several challenges. These challenges are rarely addressed by scientific literature, but CF associations raise these issues.
First challenge: finding a partner. This can prove to be difficult for those having to get accustomed to prolonged stays at the hospital or clinic (Living with Cystic Fibrosis, n.d.). Although the literature does not specifically cover this topic, it appears to be more difficult for TCF, especially boys, to find a partner (Besier et al., 2009). On top of that, because of the risks of lung infection, it is recommended that CF patients do not date one another (Cystic Fibrosis Trust, n.d.). This isolates them even more during their time in healthcare facilities.
Second challenge: the revelation. It takes a lot of courage to admit that you have a fatal chronic illness to someone that you like. It could mean having to face rejection. As such, only half of TCF reveal their condition firsthand to their partners, and only a third do it when they wish to develop a stable relationship with them (Kazmerski et al., 2018). Finding articles on this subject is very difficult when it comes to teenagers.
Third challenge: everyday necessities. Since CF affects respiratory capacity, activities that require physical effort, like sexual activities for exemple, are straining and require preparation (Living with Cystic Fibrosis, n.d.). To prepare, the person can use an inhaler (ventolin), do some breathing exercises, or lie down in a comfortable manner. In addition, there are appointments, complications, hospital stays, etc. However, the relational status of girls with CF does not present any significant differences from the rest of the population, whereas boys with CF are less likely to be in a relationship than their peers (Besier et al., 2009).
Studies generally agree that the vast majority of TCF want children (Fair et al., 2000). For a person with CF, having a child raises many issues. On the one hand, since the disease is hereditary, a parent with CF will surely transmit the disease gene. To develop the disease, the other parent would also have to be a carrier or ill. In Quebec, an estimated 1 out of every 25 people is a carrier of this gene (Cystic Fibrosis Canada, n.d.). Transmitting the disease gene can generate a great sense of guilt for many future parents (Withers, 2012). On the other hand, the implications of raising a child despite the parent’s short life expectancy need to be considered. For TCF and adults with CF, the possibility of death while the child is still young is a very real threat (Simcox et al., 2010; Withers, 2012), not to mention that the parent’s physical condition does not always allow them to care for a child in their daily activities. Therefore, the health trajectory can be an obstacle to the parental project. For these reasons, relatives can sometimes pressure older TCF into abandoning the idea of having children, which can spark tension with their loved ones (Cystic Fibrosis Trust, n.d.). The parental trajectory is therefore greatly influenced by the family and social context.
Physiological Factors and Body Image
However, parenthood is not always easy. For women with CF, the disease has little effect on fertility. Nonetheless, it can greatly interfere with pregnancy. In the literature review of pregnancy of mothers with CF, Lau and his colleagues (2010) report that weak pulmonary function can increase the risk of premature delivery and maternal mortality. Malnutrition, diabetes, certain infections, and having recently undergone a transplant are also risk factors. Nevertheless, the authors do not believe that the literature is substantial enough to evaluate the risk precisely.
For men, congenital defects make the vas deferens unable to carry sperm to the urethra (Claustres, 2005). This defect causes infertility in 98% of men with CF. This does not prevent the production of sperm. As such, CF men can generally have children using assisted reproduction methods (Frayman and Sawyer, 2015). Learning of their infertility can still raise negative emotions such as anger, the feeling of being different or having an inadequate body (Smyth et al., 2014).
Fertility is not the only issue that affects the perception of TCF have of their body. Since the disease interferes with the physiological development, CF patients tend to go through puberty later and keep certain juvenile traits. They tend to be smaller and underweight (Cystic Fibrosis Canada, n.d.; Simon et al., 2011). It is then vital for TCF to maintain a high-calorie diet. For boys, a frail body can cause a feeling of offset with male beauty standards, depicting the ideal man as tall and muscular (Withers, 2012). In fact, this points to boys with CF having worse body image than girls with CF (Tierney, 2012). For girls, weight loss is beneficial for their body image (Tierney, 2012). However, the contradiction between social standards and the necessity to gain weight can favour the non-adherence of teenage girls with CF to treatment goals (Simon et al., 2011). This puts them especially at risk of deteriorating their health condition (Simon et al., 2011).
Incidentally, deteriorating health is a significant aspect of body image. As their health deteriorates, the person requires more treatments. Since certain treatments leave visible traces (e.g.. post-transplant scars, gastrostomy, etc.), they can affect the person’s body image (Withers, 2012). Furthermore, TCF can start to perceive their body as a prison as it deteriorates (Tiemey, 2012).
Recommended Attitudes and Good Practices
Generally speaking, it has been observed that professional centers still lack sufficient and reliable information when it comes to TCF sexual health, especially in the pediatrics department (Kamerski et al., 2017). For instance, a quarter of adult men with CF are not aware of the cause of their infertility, and half of the parents of children with CF are not aware that men with CF are infertile (Sawyer et al., 2005). Almost all TCF agree that early teenage years are the ideal time to receive information about sexuality.
Yet, health services have very mixed opinions when it comes to the age at which to discuss sexual health with TCF (Sawyer et al., 2009) in addition to the subjects deemed important (Kazmerski et al., 2017).
Many professionals seem to feel uncomfortable discussing sexual health, especially when it does not involve reproductive health (Kazmerski et al., 2017; Withers, 2012).
However, the literature concludes that comprehensive positive sexual education based on partnership would address the needs expressed by the patients, their parents, and associations (Clancy and Jain, 2012; Kazmerski et al., 2017; Sawyer et al., 2009).
A positive approach is recognizing sexuality as an important part of an individual’s life and discussing it openly, without judgement. A comprehensive approach takes into account the context in which the sexuality of TCF takes place as well as the evolution of said context as the teenager goes through certain transitions (Frayman and Sawyer, 2015). A partnership approach connects with the patient-partner principle. This means involving TCF in their development by basing it on their knowledge and their life experiences and by giving them the tools they need to actively manage their sexual health (Clancy and Jain, 2012). Therefore, it does not simply come down to giving them a pamphlet or referring them to a website. TCF need to plan their sexual trajectory so that it exists in harmony alongside their health trajectory.
Some measures to put in place to favour an effective sexual education (Kazmerski et al., 2017):
1. Establish standards based on the scientific literature in order to standardize practices;
2. Ensure communication between pediatricians and physicians to make the transition efficient;
3. Get parents and main caregivers involved in the discussion about sexual health;
4. Plan what information to offer based on the development of the TCF.
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