Teens and Cystic Fibrosis:  The Quest For Healthy Independence By Siri Vaeth-Dunn 

As anyone who has survived adolescence knows, it can be fraught with many challenges. Dramatic physical, intellectual and emotional changes occur simultaneously at a time when one’s peer group may supersede parental influence. Teenagers seek increased independence from their parents, and experiment with how this independence will be manifested. While some of the choices made during this period can be detrimental to teens’ health, for those with cystic fibrosis (CF), the consequences can be far more serious. 

Developmentally, teenagers often feel invincible, and have a more difficult time connecting the impact of their current activities with their future. Adolescent experimentation can include many things: from new hair colors, body piercings and styles of dress, to drug and alcohol use, and sexual activity. Because the peer group often takes on greater importance and influence, many teens seek to fit in, and do not want to stand out as “different.” It is “normal” for adolescents to challenge authority. These developmental issues face all teens, but their impact can be accentuated for those with CF. 

Many teens with CF are consistent with their treatments, and in studies, these “adherent” teens said that doing so gives them more energy and enables them to take part in activities, and that when they had skipped treatments, they experienced negative impacts. Nonetheless, adolescence has been identified by numerous studies – and countless parents – as a challenging time for adherence to the CF medical regimen due to the developmental issues discussed above. In studies, teens cited numerous reasons for skipping treatments, including a desire to fit in and not lose time with their peer group, being frustrated with being told what to do, and the sense that treatments are not having an impact. 

Cystic fibrosis is a progressive disease, impacting numerous organs and systems in the body. Teenagers have spent much of their lives in the CF clinic, where the care team has communicated primarily with the parents. As teens transition to young adulthood, it is important that information is clearly communicated to them as well. There are often medical issues that teens may be hesitant to ask about in front of their parents, and it is important that they have the opportunity to speak alone with their team. 

Members of the care team should understand that teenagers are often embarrassed to ask questions, and should present information proactively. Many teenage girls with CF suffer in embarrassed silence with uncomfortable yeast infections caused by the antibiotics they are taking. The nurse or physician should ask girls directly about this, to provide them the opportunity to allay their fear and shame, and to provide them with treatment. Additionally, many teens with CF - primarily girls - experience stress incontinence due to increased pressure on the bladder from chronic coughing. There are exercises that can reduce the symptoms, but this is another issue they may be too shy to bring up on their own. 

For many teens with CF, puberty is delayed, which can impact their self-esteem and accentuate their sense of being different. Reproductive health may be hard for teens to talk about, yet must be addressed. CF can impact birth control options for girls - for example, certain antibiotics may interfere with the effectiveness of oral contraceptives. And while male teens with CF are likely infertile, a slight percentage are not. 

It is estimated that over 95% of men with CF have Congenital Bilateral Absence of the Vas Deferens (CBAVD). This means that the vas deferens tubes are blocked or absent, and while sperm is produced, it is not ejaculated with the semen. Male teens with CF should not assume that they are infertile, because without testing there is no way to know if they have CBAVD. Teens with CBAVD should know that they are not sterile (unable to produce children) and that advances in medical technology have made it possible for men with CF to father biological children. 

Teens need to be reminded about the importance of respiratory therapy. While scare tactics are seldom successful, teens who spend more time on their own need to feel comfortable communicating any increased respiratory symptoms. The CF medical team should make teens aware of complications such as hemoptysis (coughing up blood), which impacts over 60% of people with CF, so that they know what to do if this occurs. Nutrition is key for teens with CF, who due to malabsorption need extra calories, and often, extra calcium. For those teens with CF related diabetes (CFRD), the care team must help teenagers understand the direct correlation between blood sugar levels and lung function. 

While healthy adolescents need over 9 hours of sleep each night, few teenagers achieve this, with one recent study finding that only 15% of surveyed teens slept over 8 hours per night during the school week. For teens with CF, the need for sleep is even more vital for optimal health, yet studies have found that they consistently report sleep disruptions. A chronic cough, and the side effects of medication can play a large role. In addition to causing a greater vulnerability to infection, the lack of sleep can also impact students’ ability to concentrate, and can lead to dramatic mood swings. Because teens with CF are more prone to anxiety and depression, lack of sleep can worsen the situation. 

Teens with CF report being depressed due to feeling physically unwell, the challenges of endless daily treatments, the side effects of medications, missing major life events (sports, parties, concerts, prom) due to frequent hospitalizations, and feeling different from their peers. As one teen put it, “Living with CF is like having a clock ticking above my head. My friends don’t have to deal with that.” In addition to issues specific to their disease, these teens also cope with the multitude of stressors that teenagers commonly face. 

Among these stressors is peer pressure. Just because teens with cystic fibrosis have multiple health challenges does not mean that they are immune to the pressure to experiment with cigarettes, drugs, alcohol and sexual behavior. While studies find lower rates of “risky” behavior with CF adolescents, a study of teens at the five major CF centers in North Carolina found that 1 out of 5 teens had smoked cigarettes, and nearly 30% had had intercourse. Another study found that youth with chronic health conditions had similar rates of sexual activity as their healthy peers. 

Teens with CF may use alcohol or drugs to self medicate - due to depression and anxiety - seeking a temporary relief from the challenges of CF. Depression also contributes to self harm, namely “cutting,” as well as eating disorders. Because self-care and adherence to one’s medical regimen is negatively correlated to levels of depression and anxiety, parents and the CF care team must help teenagers to find support and develop coping mechanisms. These can include therapy, sports, artistic expression, spirituality and participation in CF-related or other social support networks.   

In many CF Centers, the teen years mark the transition from the pediatric to the adult CF Center. This can be a challenging time, as youth are often nervous about seeing a new care team. At the same time, many teenagers are thinking about goals beyond high school. The process of applying for colleges can be both exciting and daunting. For teens with CF, the prospect of moving away from all that is familiar, without the daily health care support provided by their parents, can create simultaneous excitement and anxiety. Teenagers should be encouraged to take on increased responsibility with their regimen to help them feel secure in their ability to manage on their own. 

Teenagers with CF cope with extraordinary challenges. By acknowledging the unique pressures they face, and helping them to access the resources and support they need, teens will be better able to navigate their adolescent years. They must be recognized for their resilience, and supported in their quest for healthy independence. 

Links For Teens

http://www.cfvoice.com/index.jsp

http://www.hopkinscf.org/teens/living/taking.html

http://www.cysticfibrosis.com/

http://kidshealth.org/teen/

http://www.cfri.org/scholarship.shtml

http://www.cysticfibrosis.ca/wp-content/uploads/2013/09/TEEN_SEXUALITY_WEB_Compressed.pdf

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Author Siri Vaeth-Dunn, MSW, has a teenager with cystic fibrosis. A social worker and nonprofit administrator for many years, she is now a grant writer, parent liaison at her daughter’s CF center, and a member of CFRI’s Board of Directors.  

Originally published in the Summer 2011 Edition of CFRI news. Reprinted with permission.