Preparation of Individuals with Cystic Fibrosis and Their Families

Individuals with CF and their families need to be aware that transition to an adult care program takes place as a normal part of growing up just like graduating from one school to another. Before a child sets foot in high school or college, he or she goes through years of education to develop a base of knowledge and often a specific orientation to help him or her become familiar with the new environment. Learning about transition well before it happens can help prepare an individual. Transfer to an adult care center cannot be abruptly announced when someone turns 18 without creating confusion, reluctance, and a variety of other untoward emotions. Early discussion of transition, even in the first years of life, can be framed as a picture of optimism and expectation of an independent adult future.

Preparing individuals with CF and their caregivers for transition involves providing clear expectations, ongoing education, introducing them to adult care team members and facilities, and recognizing commonly encountered emotions. Expectations delivered are two-fold. First, individuals with CF should expect to live well into adulthood and should hear that message early and often from their pediatric care team. The communication of this expectation affords individuals and their families the opportunity to plan for the future instead of having tunnel vision for the present time. Adolescence is a hazardous time for individuals with chronic medical conditions. Several challenges to be expected are listed in Table 1 . Normal adult development involves maturing into a mindset that extends past the adolescent attitude of immediate self-gratification. Taking this into account, if hope for the future is nonexistent, an adolescent may engage in higher risk, pleasure-driven behaviors rather than more responsible health-sustaining actions. Thus, the introduction of transition to adult care should be rooted in the expectation that people with CF need to develop self-care skills to support them in their later years.

The second layer of expectations to be delivered in the process of transition are the specifics of when transfer of care will take place and what is required for successful navigation of the adult care program. Setting a goal age or time of transfer, commonly at age 18 or after high school, is akin to establishing another milestone that occurs with age, such as driving, voting, or graduation. A concrete timeframe can help normalize the change that will take place and highlight its importance and improve patient satisfaction. Along with establishing a set time for transfer, communication of basic skills that are expected for independence in an adult care program is helpful. These expectations and self-identified competencies have been presented and measured in several ways. Questionnaires and checklists have been implemented by some CF centers ( Fig. 4 ). Initiation of readiness assessments early in the process of transition can allow for targeted education and intervention in areas that need improvement while providing a framework of what is expected in an adult care program. Care teams may find that the more difficult parties to transition are the parents of an individual with CF because of the intense investment of time, energy, and emotion to maintain their child’s health. For that reason, assessment of parent readiness may be beneficial given a specific center’s needs ( Fig. 5 ). These checklists in themselves are intended to be tools for organizing the many facets of transition that need to be addressed before moving from one care program to the next. Sawicki developed and studied a Transition Readiness Assessment Questionnaire. In analysis it was found that 70% of youth and 67% of parents believed that they/their child could manage their care but less than half of them reported thinking about health care transition to adulthood and less than one-third had a plan to transition. These findings illustrate the sparse understanding of the process of transition and support the fact that more work needs to be done to prepare individuals with CF and their families for this major life change.

Patient readiness checklist.

Parent readiness checklist.

Self-management is the set of behaviors that help individuals to choose, monitor, and sustain daily treatment and to manage the effects of illness on their lives. Education about CF and self-management skills can build the foundation for successful transition. A variety of resources are available from the CF Patient Resource Center that can be used spanning all age ranges. Ensuring that individuals know how different medications and therapies work can help them understand their treatment regimen. Education alone, however, does not guarantee adherence to a treatment regimen, which is a challenge that spans all ages. Assessment of barriers to adherence is a major upcoming goal of the Success with Therapies Research Consortium of the CFF. Although not strong, a recent Cochrane Review found some evidence to suggest that self-management education may result in positively changing a small number of behaviors in patients and caregivers.

It is important for teenagers to meet the adult CF care team. An ideal concept that some larger centers may be able to use is that of a dedicated transition clinic. A transition clinic is one where both pediatric and adult providers see patients in the same visit along with the multidisciplinary team of therapists and ancillary staff to allow for maximum overlap of care. For most centers, logistics do not allow for this to be a practical consideration. It has been shown that some overlap in involvement of pediatric and adult providers that is achieved by a transition clinic or other means results in improved satisfaction among young adults with CF. Adult providers can often make a point to meet individuals and their families in the pediatric clinic setting before the first official adult clinic appointment. Hospital and clinic tours guided by either pediatric or adult care team members can help transitioning individuals gain familiarity in their new health care environment. Regular meetings with pediatric and adult care providers focused on discussing individuals in the process of transition can help foster a collaborative effort to attend to the most active issues in an adolescent’s health care.

Emotions surrounding transition vary from pride and excitement to anxiety and reluctance to leave a long and trusted relationship. Parents and patients may have divergent feelings about transition. Validation of these emotions and appreciation of the sensitivity of the time should be considered. At the same time, pediatric and adult providers should aim to foster self-efficacy and independence and convey confidence in the necessity and importance of transitioning. In other chronic disease populations, overprotection has been associated with decreased quality of life, whereas self-efficacy is associated with a sense of well-being. In general, when effort is made to create a formal transition process to a well-established program, patients and parents report satisfaction. Further discussion of mental health in this age population and depression and anxiety screening is found later in this article.

Preparation of the Adult Cystic Fibrosis Care Center

All CF centers with more than 40 adults are required to have an adult CF program. The requirements of an adult program as established by the CFF Care Center Accreditation Committee are as follows:

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  Physician leadership: appropriately trained physicians with CF care experience

  
  
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  A multidisciplinary team: ideally as many of the following ancillary care providers as possible: respiratory therapist, dietician, social worker, pharmacist, physical therapist, psychologist, case manager, patient advocate, chaplain

  
  
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  Participation in the CFF National Patient Registry (CFF-NPR)

  
  
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  Compliance with clinical and research requirements

Adult care providers face many challenges and this is one reason why transition can be difficult. Support received from adult institutions for care of individuals with CF and other chronic illnesses is often less than that of their pediatric counterparts in the outpatient and inpatient realms. As the adult population grows, it is essential to have access to resources necessary to maintain excellence in care for this increasingly complex and aging population.

Although there has been much center-based research on transition perceptions and practices, there is a paucity of data regarding the relationship of transition processes and health care outcomes. One small study did demonstrate that formal transition process avoided negative health outcomes during this time of change but larger scale studies are needed to identify which if any of these interventions lead to improved patient outcomes. There is no one-size-fits-all approach to achieving success in transition; however, the CF care team needs to be thoughtful in planning out how the process of transition will take place over time and ensure their population has an appropriately resourced center.