Preparing for Adult Care with cystic fibrosis
Although adulthood may seem far away, it comes quickly. The landscape of CF is looking better due to the medical and technological advances for most people with CF (pwCF). These advances have opened great possibilities for those living with CF. More than ever young children today all the way to young adults need to be the main agents in their CF care. In the past reaching adulthood was a monumental milestone, whereas today it is the norm. The day-to-day care of maintaining health for pwCF is indeed a challenge. The 24/7 challenges can lead to pwCF wanting to depend on others for care. CF teams recognize that parents want to help their kids and decrease their stress levels by “doing” tasks that their family member with CF should be doing. This, in fact, works against adolescents and young adults over time because it does not let them learn to do what they need to, by taking charge of their own health. PwCF must gradually take on their CF care responsibilities toward becoming independent adults, while parents, at the same time, must step back and encourage and support this process.
HOW CAN YOU PREPARE FOR ADULT CARE WITH CF?
Preparing to move to adult care is a national movement called health care transition (HCT). This is a three-pronged approach where parents, doctors/CF teams, as well as pwCF work together for their eventual launch into adulthood by acquiring the skills for managing their CF care. A website, Got Transition®, offers general comprehensive resources to prepare for adult health care. Across the country, health care providers, in general, have not shown great success in providing information about moving to adult care to the age group that needs it the most. However, CF occupies a unique niche because the adult CF care centers with their specialized CF teams mirror the pediatric care teams. This is a bonus for pwCF. There is an assumption that most CF Centers have a structured program to prepare young people to move to adult care. For some CF centers this may be true and for other centers the program may be less structured; inconsistencies across programs have been reported.
Brittany, young adult with CF, and her family
This HCT movement suggests that pwCF need to start early. In fact, young children with CF start earlier than most by learning to swallow a handful of their medicines at young ages. Mastering this task is the first of many. The life skills for pwCF to master include a lengthy checklist. Skills, such as cleaning a nebulizer and using airway clearance devices as well as gaining an understanding of the many facets of CF are critical to preparing for independent adult care.
The Cystic Fibrosis Foundation (CFF) supports the HCT movement. They provide programs such as R.I.S.E. (responsibility, independence, self-care, education) and Compass. R.I.S.E. is a program addressing age specific steps to prepare young pwCF to take charge of their CF health care and learn about the skills they need for independence. Compass helps with understanding insurance options, connecting to legal information and experts, and finding available financial resources, as well as tackling other life issues. In addition, a general pathway is promoted by national recommendations set forth by the Got Transition initiative, that defines the process for preparing for the move to adult care. These recommendations outline a path to follow starting at age 12, so when pwCF move to adult care they are ready. One key recommendation for pwCF is to meet 1:1 with your doctor starting at age 14. By doing so, a space is provided to practice talking to your doctor and gain a comfort level in talking about sensitive topics and issues that are important to you.
When pwCF approach the ages of 17-21, they often express that they don’t feel like an adolescent or like an adult. They are in a “tween” stage once again. Typically, special relationships developed with the CF care team from the age of diagnosis in infancy across the course of a lifetime. It is not a surprise, then, that the move to adult care is a bittersweet prospect. The time for graduating to adult care is usually at 21 years. A sentimental feeling of elation sprinkled with some apprehension of changing providers and CF team members may lead to a dreaded feeling of uncertainty. Of course, not everyone dreads the move to adult care, however having these feelings are very normal and understandable.
Most CF centers have access to checklists, booklets, and online resources to guide you for the eventual move to adult care. These resources outline activities, knowledge, and skills to be practiced and mastered at each stage of preparation. This is a step-by-step gradual process that happens over time. It is usually done with a slow shift from parent control to you taking control of your CF health care. Some steps are listed below that you can take to prepare yourself
Learn the age your pediatrician and pediatric CF team will no longer see you
Start learning about health insurance available to you
Decide what part of your CF care you will take on and create a plan with your parents. Review this plan periodically to make sure you are meeting your agreements
Make a chart or get a checklist from your CF center. Review what “you do,” “what you know” and “what you need to learn.” The items identified can become your goals to achieve with a target time. Ask your CF team about their HCT program to prepare you for adult care and ask about a handout or checklist.
Claire and her family are good examples of preparing for the eventual move to adult care. Claire took charge of her health in many ways. She directed her care by participating in her health care decisions from a young age, supported by her parents. Her involvement built her confidence as she grew in her independence and crystallized her certainty about decisions. Their journey was not always easy and smooth. However, her move to adult care was pretty smooth because the adult CF team, where she received care, overlapped with the pediatric CF team.
Being admitted to the adult unit in the hospital was a bit different. A visit with her when she was hospitalized on the adult care unit revealed the familiar decorated hospital room in the same Claire style, yet it had an “adult-like slant.” She had more books displayed and sat at her desk writing, in front of a sunny window with the familiar Clarie smile. She knew the routine exactly because she had been a real participant in her care. She did not seem to have doubts except for the care of her port, which she protected at all costs. She was a strong self-advocate, which she learned and expressed from a young age. Her journey was not easy. She worked hard as a self-advocate and thought deeply about her life choices. It takes a circle of support for pwCF to get to the adult world and take on the care that CF demands. Talk to your CF team about your HCT path so you are prepared, as best as you can be, for your adult care.
Here are a few great resources for more information:
Linda Tirabassi-Mathis PhD RN CPNP CNS has been a pediatric nurse for over 40 years. She started working with pwCF early in her nursing career and has seen the progress and hope for pwCF. As the Clinical Nurse Specialist with the CF team at Miller Children’s and Women’s Hospital, where Claire received much of her care, she focused on supporting patients in their self-care education. Her interests in self-care and health care transition to adult care for pwCF has spanned her nursing career. She spent much time with Claire during her hospitalizations, involved in coordinating her care, educating and supporting Claire through tough decisions related to her various challenges.