Healthy Heart

Australian Teen's Experience Living with Cystic Fibrosis

Australian Teen's Experience Living with Cystic Fibrosis

Photo credit: The Canberra Times

Every day when Kate Daly, a teen from Canberra, joins her friends for lunch at Radford College, she has to open a small package that her mother, Ellen, tapes to her lunchbox.

Inside the package is Kate's daily dose of digestive enzymes. Kate has to take these enzymes with every single meal. Her mom tapes the enzymes to the outside of her breakfast container, her lunchbox, and the tea she drinks every day after school.

The enzyme ritual is just one of the many aspects of living with cystic fibrosis (CF), which is the most common life-threatening genetic condition in Australia. About 100 people from Canberra are affected by the disease, with an estimated 12,000 or more in the area thought to be carriers of the CF gene.

CF makes it difficult for the body's protein to manage the flow of salt into and out of cells. When there is too much salt in the body, a thick mucus builds up inside of organs, such as the lungs. CF causes the body to suffer more easily from infections and causes the pancreas to affect digestion, which is where the enzymes come into play.

When Kate was 3-weeks-old, she became critically ill and was hospitalized at John James Hospital, which is when she was diagnosed.

Kate's dad, Laurie, says Kate was unable to gain weight no matter how much he and her mother fed her. Laurie adds that Kate was eating really well but her little body just seemed to be fading away. Within 3 weeks, her weight was so severe that she looked like she was someone out of a "refugee camp."

Now, Kate is 13 and her daily schedule includes the digestive enzymes at every single meal, insulin injections, morning and evening sessions of an inhaled hypertonic saline solution, and a PEP mask to force her to breathe harder than normal in order to loosen up the mucus in her lungs. Kate takes more than 30 pills each day, which totals to around 12,000 pills each year.

In spite of all of the pills and medical treatments, Kate looks just like any other healthy teenager.

Kate says that a lot of her friends don't realize that she has anything wrong because the symptoms of CF are all on the inside.

Kate has always been an advocate for children and teens living with CF. She is an active ACT representative swimmer and was the face of CF in previous national marketing campaigns.

She says people don't understand CF very well and she wants them to know more about it. CF is a life-threatening disease that requires daily care and she wants the community to know more so that it can be a better source of support.

With no known cure for CF, Kate is only expected to live to the age of 31.

Teens and Cystic Fibrosis

There's no dispute that adolescence can be full of many challenges. Dramatic emotional, intellectual, and physical changes seem to occur simultaneously during a period of time when the peer pressure of friends seems to override the pressure and guidance of parents. When children become teenagers, they begin to seek a higher level of independence from their parents and experiment with how to manage the newfound independence. While some choices made during this crucial time in a teen's life seem trivial, for teens with CF, the consequences can be detrimental.

Teenagers often feel above their parents and nearly invincible. They also have a more difficult time predicting the consequences that may follow certain decisions. Adolescents begin to experiment with a number of things, including how to dress, getting piercings, changing hair colors, drug and alcohol experimentation, and participating in sexual activities. Teenagers don't partake in these behaviors simply to rebel, but are usually trying to fit in with their important peer groups, as peer pressure often becomes very important and influential during adolescence. These issues seem to affect all teenagers but have an extra crucial meaning for teens with CF.

Adolescence had been pointed out by many studies as an extra challenging time against the importance of adhering to a CF medical regimen. While many teenagers with CF are good about keeping their treatment consistent, several have cited reasons for skipping treatments as ranging from trying to fit in with normal, healthy friends, and not wanting to miss out on time with peers.

CF is a very progressive disease which affects numerous systems and organs in the body. Most teens with CF have already spent their lifetime in and out of CF clinics where the CF care team members shared most of the important information with their parents, instead of directly with them. As CF patients go from childhood to adolescence and eventually to young adulthood, it is important that the CF care team and parents begin to share some of the important information directly with the CF patient.

Many teens with CF would like to know more but are embarrassed to ask questions, which is something that the CF care team should keep in mind. For example, many of the adolescent girls with CF cited that they were too embarrassed to tell anyone that their antibiotics were causing uncomfortable yeast infections. The CF care team should remember to ask girls about this directly and offer them an ear and a proactive treatment. Another impact that CF often has, primarily on girls, is causing an increased pressure on the bladder from chronic coughing. This symptom is common among teen girls with CF, yet few feel comfortable enough to discuss this with their parents or CF team.

CF also causes a delay in puberty for many teens. This delay may have an adverse effect in their self-esteem and increase the distance that they already feel from being different from most of their peers. The CF care team should handle the topic of reproductive health and puberty delicately and encourage the teen to ask any questions and voice any concerns that they may have. As an added caution, the CF care team should be aware of the adverse effect that some CF antibiotics can have on birth control and offer alternatives.

The Transition to Adult CF Care

Along with all of the normal changes of adolescence, many teens with CF must also contend with the ordeal of transitioning from the pediatric CF center into the adult center. Switching to a brand new care team may be additionally stressful to teens as many have been with the same pediatric unit since their first moment of diagnosis.

Many teenagers also begin to think about their future beyond high school. The process of leaving home for college is usually both exciting and stressful for even teens without CF, but especially daunting for teens with CF. Parents and members of the CF care team should encourage their teenage patients to begin taking on more responsibility regarding proper treatments and the regularly scheduled aspects of their treatment regimen.

Teens who have CF must face extraordinary challenges. When parents, care team members, and the community can acknowledge the incredible pressures that teens with CF face, they can help their teens access the support, care, and resources that the need. With the proper support, teens with CF will have a better chance at navigating their teenage years with responsibility and optimal health, both physically and emotionally.

References

Vaeth-Dunn, S. (2011). Teens and Cystic Fibrosis: The Quest for Healthy Independence. [Web]. In Happy Heart Families. Retrieved from: http://www.happyheartfamilies.com/articles/article/8351689/162081.htm

Winchester, B. (2017, July 25). Canberra teen Kate Daly on living with cystic fibrosis. [Web]. Canberra Times. Retrieved from: http://www.canberratimes.com.au/act-news/canberra-life/you-cant-tell-from-the-outside-canberra-teen-kate-daly-on-living-with-cystic-fibrosis-20170724-gxhqxk.html