Pediatric Annals

Special Issue Article 

Current Concepts of Transition of Care in Cystic Fibrosis

Ajanta Patel, MD, MPH; Maria Dowell, MD; B. Louise Giles, MD

Abstract

Over the past 6 decades, advances in cystic fibrosis (CF) diagnosis and management have extended the life expectancy of patients far beyond childhood; therefore, all pediatric CF patients must prepare for transition to adult care. Readiness assessment, knowledge and skill education, and support structures are all elements of ideal transition. Transition should begin early in life with teaching skills and knowledge for disease care, and in adolescence the readiness to transition should be addressed. Transition is a gradual process of increasing responsibilities in self-care and disease management, an improvement in the understanding of CF, and an iterative process of self-assessment with knowledge acquisition. Communication and collaboration between pediatric and adult providers is necessary to ensure a smooth and successful transition with minimum effect on outcomes. Although there is increased knowledge of successful transition practices, this area presents many opportunities for advancement of care for the patient with CF. [Pediatr Ann. 2017;46(5):e188–e192.]

Abstract

Over the past 6 decades, advances in cystic fibrosis (CF) diagnosis and management have extended the life expectancy of patients far beyond childhood; therefore, all pediatric CF patients must prepare for transition to adult care. Readiness assessment, knowledge and skill education, and support structures are all elements of ideal transition. Transition should begin early in life with teaching skills and knowledge for disease care, and in adolescence the readiness to transition should be addressed. Transition is a gradual process of increasing responsibilities in self-care and disease management, an improvement in the understanding of CF, and an iterative process of self-assessment with knowledge acquisition. Communication and collaboration between pediatric and adult providers is necessary to ensure a smooth and successful transition with minimum effect on outcomes. Although there is increased knowledge of successful transition practices, this area presents many opportunities for advancement of care for the patient with CF. [Pediatr Ann. 2017;46(5):e188–e192.]

Historically, cystic fibrosis (CF) was a pediatric disease, one in which children were not expected to live beyond childhood.1–3 Yet, advances in diagnosis and treatment over the last 60 years have allowed patients to survive well into adulthood. The first report of an adult CF clinic was in 1965,3 and in the early era of adult CF care, pediatricians continued to care for their maturing patients. However, as life expectancy for people with CF continued to increase in the latter decades of the 20th century, adult patients presented with different medical and emotional needs than those seen in children. Development of adult CF Care Centers and transferring adolescent patients to them became a medical necessity. Beginning in 1981, the United States-based CF Foundation (CFF) began to address the need for adolescent patients to transfer to adult clinics.4

The transfer of medical care and the simultaneous maturation from adolescence to adulthood present both common and unique challenges for patients with CF. Like their same-age peers, adolescents with CF face social-emotional challenges as they enter into adulthood. In addition, patients with CF must develop heightened self-awareness, organizational skills, and a sense of responsibility to properly care for their chronic disease. To facilitate this progress, CF Centers follow a transition process to guide their patients from pediatrics to the adult world.5

Components of Transition

The predominant description of the CF transition process is the movement of an adolescent or young adult (AYA) patient from pediatric to adult-oriented health care.6,7 Because adolescence involves more than attention to chronic health care needs, the consensus is for transition programs to consider all the needs of the developing AYA patient.6 Fostering independence in medical care, such as self-awareness and self-care, must be coupled with support for independent decision-making, developing relationships, and seeking and managing employment.6

Although programs vary greatly in the design of their transition processes, ideally, transition to some extent involves the components of assessment, education, and programmatic support. Assessment of the patient's readiness to transfer is essential and involves an evaluation of the patient's medical knowledge of CF disease basics as well as knowledge of the treatment regimen and the patient's emotional readiness for the transition. A second component is the education required prior to transfer to adult care. In CF and other chronic diseases affecting youth, it is essential that patients have a comprehension of their disease to manage their own care. Therefore, education can refer to either lifelong education or education specific to the transition program. By the time transfer to adult care occurs, the patient must be able to independently care for his or her disease. An important feature of transition programming is that the education and assessment components are periodic and iterative over the lifecycle of the transition process. Patients should repeatedly be assessed for developmental progress, self-management skills, and knowledge acquisition.6 The third component, which may vary due to resources or design of care coordination, is support for the logistics of the transfer. Structured team handoff and transfer coordinator services are examples of programmatic elements to support transition.

Early interviews with adolescents with CF helped determine that age by itself was not a sufficient determinant of readiness to transfer to adult care.8 Following the example set by psycho-social interviewing of these adolescents, patient assessment questionnaires were then used to determine transition readiness.4 These studies helped determine that assessing patient knowledge about essential elements of CF care was better for predicting successful transfer.

Methods for assessing patients' transition readiness have evolved over the decades, particularly in the general AYA arena. The 2011 Transition Readiness Assessment Questionnaire (TRAQ)9 for assessing transition readiness in AYA patients with special health care needs has been useful in CF.5 TRAQ is designed to measure domains that providers deem essential for successful self-management, appointment keeping, tracking own health issues, managing medications, talking with providers, and managing daily activities.10

In CF, transition readiness assessments have not been standardized, and historically, such assessments have been underutilized.6 In a 2004 survey of US-based CF Centers, roughly 50% said they assess readiness; this was primarily in the areas of medication use/reasons for use and airway clearance, followed by the ability to function in an adult health care environment.11 In the same survey, fewer than 1 in 5 centers had a defined program in place to improve transition readiness.11 Therefore, generic transition tools like TRAQ and the Got Transition toolkits12 became helpful in this landscape. Given the pace of improvement in CF care, however, these data are likely not reflective of current center practices.

Successful transition planning includes education of multiple facets of CF and self-care. Patients should understand basics of CF disease physiology and the mechanisms of action of treatments and medications, such as purpose of airway clearance techniques and nebulized medications. They must also have self-awareness about their own well-being, and they should start taking on the responsibility of self-advocacy when medical attention is needed. This includes both physical care and mental health. They must be able to anticipate upcoming medical needs, organize care including appointments and medication refills, and address issues like financial barriers and insurance concerns.

The actual transfer to adult care can be aided by support structures from within the CF Center. For one, communication between the adult and pediatric teams is essential. This should go beyond notification of the patient to be transferred; regular or recurrent meetings can allow providers to discuss management options or unique care challenges.13 In addition, the 2004 Adult CF Care Consensus report recommended a transition coordinator to facilitate appointment scheduling in the pediatric and adult clinics, follow up during the transfer process, and troubleshoot financial or social stressors.6,14 This support may align with care coordination services already in place. Patients with CF may also face changes in insurance provision or loss of insurance simultaneously to the transfer of care; therefore, assistance with identifying insurance options or specialized assistance programs is helpful.6 CF Centers vary in the resources available for such services.

As summarized by Okumura and Kleinhenz5 and Tuchman et al,6 the challenges of transition have also been increasingly described. Patients with CF can face new health complications after transfer, or develop exacerbations and become hospitalized. The disruption in their care practices can lead to nonadherence, and the loss of insurance historically has led to inability to adhere to treatment plans. The timing of medical transfer can coincide with major life changes such as work or new relationships, further complicating patients' ability to plan and organize.5,6 However, a study by Tuchman and Schwartz15 suggests that transferred patients have outcomes that are better than nontransferred patients, namely better lung function over time and body mass index.

Current Practices

Today, of the more than 120 CF Centers in the US, over 100 have an adult CF clinic.16 The 2008 CFF requirements for transition included formalized care plans for patients and annual meetings of adult and pediatric care teams.5 Given these structural requirements, CF Centers have otherwise been responsible for designing their own transition processes.5 This has resulted in varying approaches to transition, such that some programs focus on providers (and how to engage their patients) and others focus on patients' medical knowledge and skills.6 Evaluation of these processes, using both research and quality improvement methods, has begun to elucidate preferred practices. A systematic approach to designing a transition program has been described that includes standardized interviews and assessments of patient and provider stakeholders, followed by qualitative data analysis to assess priority needs and effect.17

In the 2004 CFF Adult Care Consensus Guidelines, models for different transition practices included the direct transfer of care and overlapping pediatric and adult clinics over time to ensure a smooth transfer of care.14 With direct transfer, caution must be taken to avoid an abrupt change in providers, as this risks severing the existing relationships before new ones are built.14 Overlapping clinics have received positive results in satisfaction surveys of young adults, suggesting the joint clinic model is useful.14 Centers may choose to gradually introduce pediatric patients to the adult care center through joint clinics or guided tours.5 With either model of transfer, including care center meetings of both adult and pediatric, providers can help ensure successful handoffs.5

The consensus of the Society for Adolescent Health is that the transition process be a family centered approach.7,18 Within this model, providers should partner with families to gradually shift personal medical care from the parent to the patient. As AYA patients progress during the transition process, responsibilities for self-care should be increasingly given to them, rather than to the parents. The medical care team should involve the patient in the decision-making process more and more with each visit. When the patient is ready, the pediatric care team can assist in the process by seeing the adolescent alone in clinic to promote the eventual transfer to adult services.13

In 2015, a digital toolkit endorsed by the CFF was launched to support CF Care Centers in facilitating their patients' transition processes. CF R.I.S.E, which stands for Responsibility, Independence, Self-care, and Education, is an online resource for practitioners and patients. The toolset includes knowledge self-assessment questionnaires, checklists, and progress reports for patients.19 An analysis of 10 pilot centers showed positive outcomes with CF provider satisfaction; providers reported improved communication with families, particularly when it came to assessing patients' knowledge and skills.20 Almost all providers listed time as the primary barrier to broad implementation of the program.20 The program's effectiveness on patient outcomes has yet to be determined.

Historically, an area of weakness in CF transition care has been the use of patient benchmarks during the iterative assessment process.5 In the 2004 US CF Center survey, fewer than 10% of Centers had a clear list of desirable skills the patients needed to know before transferring.11 In late 2016, CF R.I.S.E. launched a “CF Milestones” timeline and toolkit, which provides concrete knowledge and skill benchmarks patients should achieve within certain age ranges. Benchmarks are organized in the categories of understanding CF, managing CF care, taking CF treatments and therapies, and living with CF.19 As this is a new toolset, its outcomes have not yet been evaluated.

Another important initiative in CF care is in the area of mental health. Patients with CF of all ages are highly affected by depression and anxiety, due largely to the complexity and gravity of their disease and the effort required to manage it. A multinational study found 10% of adolescents with CF had symptoms of depression and 22% had symptoms of anxiety.21 With this in mind, the CFF has recommended annual depression and anxiety screening for all patients age 12 years and older.22 Integrating mental health care into the transition process is an important area of future work.

Currently, there are no standardized guidelines by the CFF regarding the steps or pillars of transition of care. Specifically, there remain insufficient data on the rates, timing, outcomes, and barriers to transition, which inhibits the development of a fully functional health delivery system for this process.6 Hopefully, with advancements in programs and increasing awareness about transition, repeated and refined evaluations will provide this necessary data.

Timing of Transition and Transfer

Similar to the recommendations for transition in other AYA patients with chronic health care needs, the transition process should be gradual and evolve over several years. A prolonged, gradual transition allows for development of skills and knowledge and prevents the sudden change of care provision that could be detrimental to the patient.5,6 The CFF Adult Care Consensus Guidelines address both the prolonged nature of the process and the age of transition, suggesting the process begin early in life and evolve over time as the patient moves through adolescence.14 Realistically, a patient's age continues to be the most common consideration for readiness to transfer or for beginning a transition process.11,23,24 The 2004 survey of US CF Centers reported a median age of beginning transition of 17 years, with a range from “at the time of diagnosis” to age 25 years.11 However, because components such as initial education or readiness assessment may be appropriate for younger adolescents, several Centers begin the transition process or assessments as early as age 11 to 14 years.5,10

Unlike some chronic conditions, CF is frequently diagnosed in the first year of life and increasingly so in the era of newborn screening.25 This presents a unique opportunity for CF patients to begin self-care skills as early as developmentally possible. Although the official start of a “transition program” may vary among centers but typically occurs in adolescence, early education may focus on understanding the basics of CF disease or taking the responsibility to independently perform treatments. These features of self-care, knowledge acquisition, and personal ownership should begin as early as possible in life; some centers even begin this education as early as the time of initial diagnosis.11 With this background, the recently developed CF R.I.S.E. Milestones timeline begins at age 6 years, with future plans to expand the program to children as young as age 5 years.19 Between ages 6 and 9 years, self-care skills and knowledge are kept developmentally appropriate, such as being able to set up medication equipment.19

It is important to note that the process of “transition” is not the same as the event of “transfer” of care, although transfer is often the final step in transition. The CFF recommendations available to date have primarily focused on the medical care transfer,6 which refers to a discrete event of change of care provider. National CF Center surveys in the US and Canada reported that many pediatric patients are transferred to adult clinics by age 19 years,11,26 ranging from age 14 to 30 years.11 The 2008 CFF requirements recommend transfer between age 18 and 21 years and that >90% of patients complete transfer by age 21 years.5

Conclusion

Transfer to adult CF care is anticipated for almost all patients with CF today. Evolving processes and research over the last 3 decades have led to awareness of successful transition strategies. Advancements in general transition care, successful CF institutional methods, CFF recommendations and Adult Care Guidelines, and programs like CF R.I.S.E. have paved the way for best practices. Ideal transition programs start early and follow a structured process over time, while pediatric and adult providers communicate frequently.27 Guidance for patient self-care and management should start early in childhood, with gradual progression of skill building and knowledge.19 Progressive education should be tailored to individual patients. As CF providers develop greater understanding of the needs of adolescents and adults with CF, the process of transitioning care must also mature and evolve.

References

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Authors

Ajanta Patel, MD, MPH, is a Fellow. Maria Dowell, MD, is an Associate Professor. B. Louise Giles, MD, is an Assistant Professor. All contributors are affiliated with Pediatric Pulmonology, The University of Chicago.

Address correspondence to B. Louise Giles, MD, Pediatric Pulmonology, The University of Chicago, 5841 S. Maryland Avenue, MC 4064, Chicago, IL 60637; email: bgiles@peds.bsd.uchicago.edu.

Disclosure: The authors have no relevant financial relationships to disclose.

10.3928/19382359-20170425-02

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