Numerous individuals with chronic disease age into adulthood each year, necessitating transition from a pediatric to an adult medical care team. Transition should start early in adolescence and occur gradually over years, preparing the individual for the transfer to the adult team. Cystic fibrosis (CF) has a growing population of adults, as survival over the past several decades has increased. The CF Foundation has implemented guidelines for the transition process. The transition process for individuals with CF provides an example that could be adapted into other chronic disease populations, to provide a successful and meaningful transition into adult care.
Key points
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Transition from pediatric to adult medical care can be a difficult process, particularly combined with the challenges of adolescence.
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Cystic fibrosis (CF) is an example of a chronic disease that has a growing adult population and has initiated transition care guidelines.
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The transition process should start early in adolescence with the focus each year concentrating on age-appropriate goals. Therefore, the individual is independent and ready for transfer to adult medical care by age 18 to 19 years.
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Elements of a successful transition for patients with CF include initiation of a multidisciplinary team, early transition program, transition meeting between health care providers, and transition clinic for patients.
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The elements of a successful transition that the CF community has identified can be extrapolated to help individuals with other chronic diseases.
Introduction
More than 500,000 adolescents with special health care needs in the United States reach adulthood each year. These individuals have a wide variety of diseases, including cystic fibrosis (CF), sickle cell disease, congenital heart disease, diabetes, and neuromuscular disorders. The improvement in survival for children with chronic diseases that arise in childhood has necessitated the development of transition programs to facilitate transfer of care from pediatric to adult care providers. The importance of developing a strong transition plan that ensures that “high-quality, developmentally appropriate health care services are available in an uninterrupted manner as the person moves from adolescence to adulthood” has been emphasized by a joint consensus statement by the American Academy of Pediatrics, Academy of Family Physicians, and American College of Physicians/American Society of Internal Medicine.
Adolescence can be a challenging period, for both children and their families, and the addition of a chronic illness can affect normal development during this time. Similarly, the normal challenges of adolescence can adversely affect the health of an individual with a chronic illness. For example, medication adherence often decreases as teenagers take more responsibility for their own care. Patients can be concerned over how their peers view their disease and may hide their need to take medications. Pediatric care providers can help their patients by being aware of the issues that can affect adolescents and their impact on their health, and by planning effective interventions to prevent some of these complications from occurring. Therefore, the carefully planned transition of young adults with a chronic disease from a pediatric medical care team to an adult medical care team should be an important priority.
Transition has been defined as “the purposeful, planned movement of adolescents and young adults with chronic physical and medical conditions from child-centered to adult-oriented health care programs.” Transfer is the actual point in time when the patient moves from pediatric to adult care providers. Transition should be a planned process over time, because a rapid transfer to a new adult medical team could be ineffective or even disastrous. The danger of an abrupt transfer is the severing of important and solid relationships in the pediatric care team, with little to no assurance that appropriate adult care will be instituted.
The transition of patients from pediatric to adult care should begin years before the actual transfer. In fact, some would say that the process of transition begins at diagnosis when prognosis and expectations are discussed. The process of transition involves education of both patient and family, assuring that the patient has an understanding of his or her disease, including the rationale for therapies and prognosis, and an assessment that the patient can independently manage therapies and navigate the health care system. Work, relationships, reproductive health issues, and family planning emerge as important issues at the time of transfer. The transition of care is often made difficult because of the fear of losing long-standing relationships with pediatric caregivers as well as the fear of the new unknown adult caregiver. Other challenges include hesitance to be independent from parents, severity of illness, stability (recent exacerbations of illness that might delay transfer), emotional readiness of both the patient and the parent, longevity of care with the pediatric center, and emotional ties to providers. In addition, adult care practices may be different from previous experiences (eg, new treatments, new rules, new ways of treating infections, new faces [doctors, nurses, social worker, administrators, staff]), and new inpatient floors with all new nurses. Thus, assessing patient self-care readiness, adherence to medications, and psychosocial status is critical to ensuring a successful transfer.
Transition Issues in Cystic Fibrosis
CF is the most common, life-shortening, genetic disorder of Caucasians. Abnormal electrolyte transport in epithelial cells leads to a multisystem disorder characterized by significant sinus and pulmonary disease, pancreatic insufficiency, elevated sweat chloride, and male infertility. Individuals with CF develop progressive airways obstruction characterized by abnormal mucus, chronic endobronchial infection, and inflammation. Pulmonary exacerbations, characterized by increased cough and/or sputum production, are the hallmark of the disease and occur frequently in individuals with CF and are associated with loss of lung function, worsened quality of life, and decreased survival. Most CF individuals die from pulmonary complications, and thus, a major goal of CF treatment is slowing the progression of lung disease. Individuals with CF are prescribed a complex daily regimen of airway clearance and inhaled medications to modify the abnormal mucus and minimize infection. In addition, more intense therapies, including hospitalization, are often required to treat pulmonary exacerbations.
Fortunately, individuals with CF are living longer, because there has been significant improvement in survival over the past 40 years. In the 1950s, individuals survived to a median age of 5. The median predicted survival of only 16 years in 1970 is now up to 39.3 years ( Fig. 1 ). This increase in survival is largely due to improvements in therapies for CF, including inhaled medications, antibiotics, pancreatic enzymes, and airway clearance. In addition, the dramatic increase in median survival in CF has resulted in a significant growth in the number of individuals with CF more than 18 years old (adults), with 50% of individuals being 18 years or older ( Fig. 2 ).
The dramatic improvement in survival and the increasing number of individuals reaching adulthood have necessitated the development of adult CF care programs. In fact, by the end of 2000, CF centers that cared for 40 or more adults were required to have separate pediatric and adult programs staffed by care teams with age-appropriate training to maintain CF Foundation accreditation. This mandate also emphasized developing a process to transition to adult care and developing evidence-based clinical care guidelines.
The timing of transfer to adult CF care often coincides with changes associated with adolescence when lung function may be declining and treatment burden increasing. Adults with CF have more obstacles to hurdle, including worsening lung function, often requiring intravenous antibiotics and hospitalizations ( Fig. 3 ). Complications also become more prevalent in adulthood, specifically bone disease, CF-related diabetes, depression, and arthritis ( Fig. 4 ). Therefore, not only does the patient have to go through transition to a new medical team but also they also may have to face new diagnoses and additional medications and treatments requiring more effort, time, and dedication.
What We Can Learn from Cystic Fibrosis?
CF is an example of a chronic disease whereby much effort has been put into implementing models to improve transition from pediatric care to adult care. The CF Foundation, a nonprofit organization that supports CF research and clinical care, made the successful transition of pediatric patients to adult caregivers a critical part of its mission. The CF Foundation was established in 1955 and now accredits ∼120 CF centers. Beyond advocating transition, the CF Foundation developed Adult Care Consensus Guidelines to provide goals and help standardize care. In addition, when it became clear that there were an inadequate number of appropriately trained adult providers, the CF Foundation supported the training of additional providers. Perhaps the most important lesson from the CF experience is that developing a transition program from pediatric to adult care is critically important and does not develop without committed individuals. However, the success of the transition process also depends on making sure that there are an adequate number of appropriately trained adult care providers.
There are several principles that care providers of individuals with CF have adopted that can be applied generally to other chronic diseases that affect individuals in both young and older ages. There must be commitment by both the pediatric and the adult teams to ensure frequent communication and shared responsibility to make a transition program successful for their patients. Transition should be coordinated and gradual, addressing concerns of both the patient and his or her parents, promoting independence and education, and providing flexibility to address concerns of the individual being transitioned.
The model of a multidisciplinary team providing care for individuals with CF has been established in pediatric care centers and also has been incorporated into the care of adults with CF. Ideally, these care teams include physicians, nurses, dieticians, respiratory therapists, physical therapists, social workers, and psychologists. In an outpatient clinic visit, the patient has the opportunity to meet with several members of this team, depending on their current issues. These same team members are often also involved in the inpatient setting, providing continuity. As individuals age into adulthood, there is generally a higher incidence of more severe pulmonary disease, higher prevalence of CF-related diabetes, and more complex financial and psychosocial issues. Therefore, the expertise of this multidisciplinary team becomes necessary, and there is a consensus that a multidisciplinary team with training in adult CF should care for these patients. This model could be helpful in other chronic illnesses, such as sickle cell disease and congenital heart disease.
In 1999, Johns Hopkins Hospital developed an Adult CF Program and transitioned 80 adults from the pediatric center over 5 months. This allowed for an evaluation of the transition process as well as assessment of the concerns of patients transitioning from the pediatric center to the adult center. Sixty patients and their parents filled out a pretransition survey 3 months before transfer and an after-transition interview 8 to 12 months after transfer. Survey questions were used to identify concerns individuals had about transitioning as well as expectations individuals had for an adult CF program. Results showed that individuals with CF had significant concerns about potential exposure to infection, having to leave their previous physician, meeting a new care team, and a potential decrease in the quality of medical care. After the transition, there was a statistically significant decrease in all areas except for concerns about potential exposure to infection and being admitted to the adult hospital ( Fig. 5 ). In particular, concerns about leaving behind a previous physician and meeting a new team dropped to “no concern” levels. Of the 52 patients who completed the pretransition survey, 30 had met the adult team in the pediatric clinic before they completed the survey. In those patients who had met the adult team, their responses demonstrated statistically significant lower levels of concern (compared with those patients who had not met the adult team) in all areas, particularly in levels of concern about leaving previous physicians and a potential decline in quality of care ( Fig. 6 ). Overall, young adults with CF reported satisfaction in the transfer process when they were able to meet with both the pediatric and the adult teams together, before their first appointment with the adult team. Hence, most CF centers have worked to establish transition clinics where pediatric and adult care providers communicate about each patient transitioning, and members of the adult care team meet with the patient along with the pediatric team.
In 2008, more than 85% of individuals with CF were followed by CF Foundation accredited centers, indicating that research data from these centers would represent a comprehensive view of the transition process in the CF community. Therefore, McLaughlin and colleagues conducted a survey of the 195 CF care programs in the United States on transition practices. Transition activities were accessed in 7 functional domains: patient preparation, patient readiness assessment, coordination of services and benefits, information transfer (communication of medical summary), primary and preventative health care, patient follow-up and program evaluation, and transition program self-evaluation. Responses were received from 87% of CF Foundation–accredited centers. Results showed that the initial discussion of transition occurred at median age of 17 years (range “time from diagnosis” to 25 years). Transfer of care took place at a median age of 19 years (range 14–30). More than 80% of programs reported having a discussion involving transition of a patient at a pediatric care team meeting, yet only one-third of programs had the adult care provider present for those meetings. This study had several key findings including that the process of transition was beginning late (at 17 years), which left little time to adequately prepare the patient and their families for transfer. Strengths of the existent transition process at that time included high involvement with counseling on substance abuse, smoking, and dietary behavior. However, only half of the centers focused on the ability to list medications, taking medications without being reminded, and being able to contact caregiver or insurance independently. Almost half of the programs reported rarely preparing a medical summary before transfer. The recognition that the transition process in CF was not acceptable led to the recommendations mentioned in later discussion, and in particular, the development of a list of self-management skills per age group that the CF Foundation could endorse. Specifically, the goals were to introduce the transition at an earlier age, aim to help the individual with CF develop self-management skills, and establish nationally recognized age-specific goals for people transitioning to adult care.
How to Improve Transition: Addressing Barriers
Over the last 20 years, the CF Care Center Network has developed adult CF programs to augment pediatric programs to provide care to people with CF across the age spectrum. Research and quality improvement projects and the CF center have identified areas that can make transition a success. Although further work is still needed, there are several processes that have been shown to be beneficial in transition.
Make transition to adult care a gradual process
A successful transition to adult care should not begin at age 18 to 21. It can begin as early as age 13 or earlier. Transition is a process that occurs over years, and transfer is the actual point in which the transfer to adult care takes place. The initiation of age-appropriate responsibilities can be introduced each year, and the initial steps can be simple at first. For instance, an initial goal at age 12 could be patients knowing the names of their medications and why they are using them. At age 13, the child should be able to talk about the amount of medication they take and when they should be taking them each day. At age 14, the responsibility of talking directly with their pediatrician during clinic visits with occasional input from parents should be a reachable goal. The adolescent can gradually be given more responsibility for self-care and decision making and should be seen alone in clinic. Therefore, by the age of 18, the child should know enough about their chronic disease but also feel comfortable talking to their physician on their own. They should be able to contact their physicians with changes in health, schedule appointments, and refill prescriptions. A more intense preparation should take place the year before transfer is planned, which may include tours of the adult inpatient and outpatient centers and meeting members of the adult team. Taking these gradual steps over the years will lead to a successful transition when it becomes time to transition to an adult medical team. Table 1 lists specific CF age-specific recommendations.
