How Are Teens and Young Adults Affected by Cystic Fibrosis?

Teens with cystic fibrosis (CF) are transitioning from childhood to adulthood but with extra challenges to remain as healthy as possible. They go on first dates, learn to drive, try alcohol or smoking, go to college, and get their first jobs. However, these rites of passage must take place within a framework of daily breathing treatments, drug adherence, regular exercise, doctor’s appointments, and learning to care for oneself.

Independence and rebellion

Wanting to fit in with peers and be independent from family are a natural part of adolescence and one that teens with CF experience like anyone else. However, normal teenage rebellion holds particular dangers for teens with CF.

Non-compliance with treatment becomes common in adolescence and young adulthood. Sometimes this comes from a wish to rebel against the disease or a desire to be like others. Other times it comes from the person underestimating the severity of their disease, or a poor doctor-patient relationship.¹

Regardless of the cause, good nutrition, exercise, and adherence to the daily treatment plan are all vital to maintain a good quality of life and prevent or delay the serious side effects of CF, such as hospitalizations for declines in lung function, exacerbations, osteoporosis and CF-related diabetes.²

Delayed puberty

Delayed puberty is common in young people with CF, leaving them physically smaller and younger-looking than their schoolmates. This delay averages two to four years, depending on when that individual’s sex hormones reach peak levels and their overall health. While this is a temporary condition, it can be hard on a teen’s self-image and cause anxiety or depression.³

Anxiety and depression

Worries about feeling different or alone, worries about early death, and body image issues can lead to higher rates of anxiety and depression in teens and young adults with CF, especially if their disease gets worse. However, if the teen with CF is in good overall health, rates of depression and anxiety are similar to the general population.¹

Transition to adult care

Transitioning from a pediatric care clinic to an adult clinic is a gradual process that begins around 8 to 10 years of age. By 12 to 14 years old, children with CF should be handling most of their own care, from taking enzymes and medicines to sticking to daily breathing treatments. They should also begin talking with their CF care team alone for at least part of their visit, so they feel free to talk about personal issues. Throughout adolescence, teens should take on increasing self-care and responsibilities so that between 18 and 21, they are ready to move to an adult CF clinic.^1,2

Sexuality and reproduction

While most men with CF are infertile, and many women with CF have trouble with fertility, their sexual desires and performance are normal. That is why teens with CF should receive the same education about sex and contraception as their peers. Doctor’s appointments without parents in the room help make teens more comfortable asking questions about sexuality.

They should also be encouraged to protect themselves from sexually transmitted diseases (STDs) by using condoms. Other forms of contraception helps prevent pregnancy without protecting against STDs.²