More than 90% of children born with congenitally malformed hearts now survive to adulthood, as a result of the spectacular achievements of pediatric cardiology and surgery over the past half-century. This has created a new and growing population of adults who often have complex congenital cardiac malformations that are unfamiliar to conventionally trained adult cardiologists. The need for an integrated strategy of care for such patients with congenitally malformed hearts, who usually require life-long medical care, has been recognized. Several panels of international experts have provided frameworks for provision of services. These share a number of common recommendations. First, all recognize the need for management in an adult environment, as the average age of the population of adults with congenital cardiac disease increases. Second, all recommend a hierarchical structure for care, with specialist centers providing a full range of diagnostic facilities and treatments interacting, and often sharing care, with other adult cardiac units. Third, all accept the need for training of a new subspecialty of medical and allied health practitioners with expertise in the management of congenital cardiac disease and the care of adults. Throughout childhood, patients with congenital cardiac disease are treated and followed up at pediatric cardiology centers. When they become adults, it is advocated that their medical surveillance and follow-up care is transferred to adult-focused facilities. This chapter defines transfer and transition, describes the key issues in transition, and explains the core components of effective transition programs.
Transfer From Pediatric to Adult Care
Transfer is defined as “An event or series of events through which adolescents and young adults with chronic physical and medical conditions move their care from pediatric to an adult care environment.” A seamless transfer between pediatric and adult settings warrant that age- and developmental-appropriate care is provided, while assuring that patients remain under follow-up. Research, however, has indicated that a substantial number of patients do have gaps in their care. In patients with congenital cardiac disease, care gap rates range from 7% to 76%, with a median proportion of 42%. The consequences of such care gaps may be far-reaching. Studies have reported that patients who presented for medical check-up after a care gap more often had a new diagnosis of hemodynamic significance and had a greater likelihood of needing an urgent surgical catheter-based intervention. Therefore the identification of patients at risk for care gaps and the implementation of interventions that prevent such care gaps are of utmost importance. In this respect, use of mobile phone messaging reminders to reduce missed appointments in outpatient clinics and the implementation of transition programs to make patients and families aware about the need for life-long care may facilitate the transfer from pediatrics to adult care and may avoid care gaps.
Transition
Transition can be seen both as a developmental process and as a healthcare intervention. As a developmental process, transitions are passages from one life phase, physical condition, or social role to another, resulting in a temporary disconnectedness of the normal way of living, which demands an adjustment of the patient and the environment. As a healthcare intervention, transition is defined as “a multifaceted, active process that attends to the medical, psychosocial, and educational/vocational needs of adolescents as they move from the child-focused to the adult-focused healthcare system.” In such an intervention, the adolescents are prepared to take charge of their lives and their health in adulthood. Transition takes time, and is not necessarily completed on entry to adult care. Its duration varies considerably from patient to patient, and is influenced by a number of factors, including the background, development, and intellect of the patient and the level of support provided by the family. The whole process takes several years, and is only successfully achieved when the patient is fully able to take responsibility for their own health and issues of lifestyle. It is important therefore that during the process, young adults with complex congenital cardiac malformations appreciate that although they have the potential to live healthy and productive lives, most will require life-long cardiac surveillance. They also need to understand the requirement to obtain appropriately skilled care. During transition, issues both generic and disease-based need to be addressed. Frequently, the coordinated transfer of care from the pediatric to the adult healthcare environment is the final step in a successful process of transition.
Pediatricians tend to consult predominantly with the parents, whereas adult practitioners seek to develop a partnership with the patient. This involves directing information and education toward the patient and, at the same time, encouraging responsibility and self-reliance. It is critical that during adolescence, this connection with the young patient is successfully achieved so that the patients begin to understand the need for an active role in their own health and cardiac management. If this fails, adolescents and young adults may disappear from medical follow-up and return only when potentially avoidable problems have developed.
Key Issues in Transition
When Should Patients Begin the Process of Transition and When to Transfer?
The timing of entry from a pediatric clinic into a service responsible for transition, and the subsequent transfer to adult clinics, will vary according to the provision of local services and the individual needs of the patient. It is important, however, that transition and transfer occur in a predictable and planned manner. Referral to the clinics or programs responsible for transition should ideally begin by 12 years of age. Compared to older ages, at the age of 12, individuals are more susceptible for suggestions from adult people and for building up a trusting relationship with new healthcare professionals. Attendance at such clinics involves a change in approach from the typical pediatric cardiac clinic. Parents should continue to attend, but there should be greater emphasis on communication with the adolescent, including opportunities for confidential one-on-one discussion with doctors, nurses, and other relevant counselors. The approach to communication needs to evolve as the teenager grows older, with provision for discussion appropriate to age and maturity.
Transfer to the adult clinic most commonly occurs at the age of 18 years or on completion of schooling. The timing, however, should be flexible so as to meet the needs of the patient. For example, if a patient has developmental delay, or multiple medical problems under active follow-up in the pediatric hospital, transfer may need to occur at an older age. Patients should understand that transfer to adult care is a natural process and part of growing up. In general, adolescents deem it appropriate to move their care to adult-focused facilities. However, parents are often more hesitant because they strongly rely on the trusting relationship that they have built up with the pediatric care providers. Early discussion, and agreement on an age of transfer, removes the frequent anxiety of families that expert care is being lost, and makes transfer to adult care a development to be viewed optimistically. In some medical systems, provision of adult services for grown-up patients with congenitally malformed hearts is not yet available at a level comparable to that provided during childhood. In these cases, delay of transfer is clearly advisable.
Where Should Care Be Undertaken?
Availability of clinical resources will determine the location of the transition clinic. Ideally, this should be within the pediatric hospital, as this makes the first move from the pediatric clinic less traumatic to the patients and their families. Those staffing the clinic have the opportunity, during several visits over years, to organize the subsequent transfer to adult care, which may need to be to a different hospital.
Facilities for in-patient care should be designed around the needs of young people, and ideally should be separate from pediatric and adult wards, with a different atmosphere and focus. A dedicated adolescent environment for patients with a range of medical conditions can work well for the needs of cardiac patients. This permits provision of facilities, including Internet access, a study area, television, a social area, and kitchen facilities. The adolescent ward should be organized by staff with special awareness and training in the issues of this age group.
Who Should Be Involved in Transitional Care?
The balance of staffing for the transition clinic depends on local resources. It is, however, essential that members of the team providing pediatric care and of the team looking after their future needs as adults with cardiac problems are both involved. This provides an opportunity for discussion between teams of the specific medical needs of the patient, as well as creating a visible connection for the patient and their families.
Specialist nurses play a crucial role in successful transition by taking up the role of transition coordinator. They should have experience in the needs of adolescents and young adults with congenitally malformed hearts, and should have received training in counseling. There should be facilities for the specialist nurses/transition coordinators to consult with the patient separately from the doctor, as this encourages early discussion of sensitive problems and anxieties. The specialist nurses/transition coordinators frequently are the main contact with the patient and his or her family, developing a unique relationship with them.
How Should Transitional Care Be Provided?
Transitional care comprises several interacting components. Therefore it is considered to be a complex intervention and ought to be developed from a programmatic point of view. This programmatic approach assures that transitional care is provided in a systematic and structured way. Transition programs for young persons with chronic conditions in general, and with congenital cardiac disease in particular, are developed and tested. Some transition programs are extensive and require a lot of time, whereas other transition programs can been seen as brief interventions.
Core Components of Transition Programs
To date, some transition programs have been described in the literature. These transition programs are common practice in the respective hospitals, or are under investigation as part of an effectiveness study. Core components of transition programs have been described.
Transition Coordinator
A transition coordinator is an indispensable component of successful transition programs. Transition coordinators is often a specialist nurse, advanced practice nurse, clinical nurse specialist, or nurse practitioner. The transition coordinator often is the main point of access for patients and their families when it comes to transition, but also coordinates the multiprofessional team pertaining to delivering transitional care. He/she assures continuity of care; supports the youth and their parents throughout the transition process; liaise the hospital/clinic with the community; serves as a patient advocate; safe-guards family- and person-centered care; and provides youth and parent education.
Transition Plan
For each individual patient who enters the transition process, a written transition plan should be developed. A transition plan documents the needs of the young person and his/her parents; addresses the health, psychosocial, and educational issues; details the objectives of the transition; and comprises a timing of the planned meetings and the foreseen moment of transfer. The transition coordinator, together with the adolescent, will be in charge of determining which topics are important to discuss during the transition meetings and which goals related to patient development and functioning are to be advanced. The transition plan is a working document that evolves over the course of the transition process.
Education and Counseling
It is crucial for the patient to understand his or her cardiac defect and its consequences. This is rarely the case when the patient enters the transition clinic. Effective communication requires repetition, especially when patients have learning disabilities, complex cardiac conditions, or low levels of education. In people with congenital cardiac disease, the effectiveness of providing patient education has been demonstrated. Aspects on which patients are to be educated are the heart defect, prior and current treatments and interventions, infective endocarditis and how to prevent it, contraception, pregnancy, the risk of recurrence, the importance of life-long follow-up, and the symptoms that require medical check-up if experienced.
Being knowledgeable does not necessarily mean that the patient is presenting with the corresponding behaviors. Therefore attention ought to be given to the acquisition of self-management skills. Important self-management behaviors for people with malformed heart pertain to sports and other physical activities, tobacco use, alcohol consumption, nutrition, and illicit drug use.
In addition, psychosocial aspects should be covered during transition. These aspects typically include career and vocational planning, employment, marriage and family planning, and insurability. Indeed, patients wonder how their cardiac disease will affect their ability to obtain a job, health or life insurance, or a mortgage. Provision of support and counseling on such issues during the transition to adulthood is essential. Many children and young people with congenitally malformed hearts will be able to have any career they may choose. The future of some patients, however, will include physical or other limitations. They may need to be advised to pursue appropriate education and career pathways. For example, a patient with the Fontan circulation should know not to become a bricklayer or a commercial trucker. These patients, and their parents, should have a chance to discuss such issues before the patient reaches high-school age, and before important decisions are made, or actions taken, such as choosing a curriculum or dropping out of school. As the long-term outcome of many complex cardiac conditions becomes clearer, evidence-based recommendations can be made in regard to future careers. These will carry weight with future employers, and represent an opportunity for the clinic staff to act as advocates for their patients, who are often otherwise disadvantaged by the stigma attached to chronic cardiac disease.
Many patients will have difficulty in obtaining both health and life insurance. They may be denied coverage, or offered a heavily loaded premium based on out-of-date information relating to the risk of death. Even favorable clinical factors, such as mild defect, normal functional status, and good left ventricular ejection fraction do not protect against constraints in insurance and mortgage applications. Support groups, such as the Grown Up Congenital Heart Disease Association in the United Kingdom (available at: http://www.guch.org.uk ), have excellent links with insurance companies. In the United States, the difficulties encountered by young patients with chronic conditions in obtaining health insurance are now recognized. Opportunities vary between states, and it is hoped that legislation to provide a more uniform level of support will be enacted in the next few years.
A frequently neglected psychosocial aspect is end-of-life. It may sound premature to talk about end-of-life issues with adolescents. However, irrespective the age or the complexity of the cardiac anomaly, patients with congenital cardiac disease have expressed the strong wish to talk about end-of-life decisions with their health care providers.
Before any educational or counseling intervention can be implemented, individual patients should be comprehensively assessed in terms of their knowledge, self-management, and psychosocial aspects. In order to undertake a broad and generic assessment of the adolescent’s health, the HEADS method has been developed. HEADS stands for Home; Education; Eating; Activities; Drugs including tobacco, alcohol and illegal drugs; Depression and suicide ideation; Sexuality, including contraception; Safety from injury and violence; and Self-harm. HEADS is to be administered during an interview, and it guides the health care professional in systematically addressing all the issues that are important in terms of adolescent health.
Availability by Telephone, Email, or Other Technologies
During the transition from being a dependent child to becoming an independent adult, the adolescent has to accomplish numerous developmental tasks. This may be associated with substantial uncertainties and a lot of questions. In between the scheduled outpatient visits, ad-hoc questions regarding the condition, therapy, transition process, appropriate health behaviors, or clinic appointments may emerge. The congenital cardiac team should be available for questions from the patients and families, to avoid growing uncertainties or adoption of detrimental behavior. The transition coordinator will play an essential role in this, by being available by telephone, email, or other contemporary communication technologies.
Information About and Contact With the Adult Congenital Heart Disease Clinic
When patients are transferred from pediatrics to adult care, they are leaving a familiar environment and health care team. In itself, this is seen as normal. However, adolescents and their families express their wish to be informed about the processes in and organization of the adult congenital cardiac disease clinic, and want to meet the cardiologists, nurses, and other team members beforehand. Information leaflets about the team, group sessions to meet the health care professionals, or guided tours through the outpatient clinic are useful tools to inform adolescents and their parents in the pretransfer phase.
Guidance of Parents
During the transition, there is an obvious shift in roles between the adolescent and the parents. Adolescents expect that health care workers direct the information to them. They want to be heard. At the same time, they still have the same expectations toward their parents, in that the parents keep them posted of all developments. The adolescents consider their parents as the first point of contact and most important source of information. This means that parents should not be excluded from the transition. Even if transition programs have dedicated time alone with the adolescents during transition visits, they still need to schedule time together with the parents to update and inform them. It should not be underestimated that parents are going through transition as well, and therefore need guidance.
Meeting With Peers
Adolescents with congenital cardiac disease explicitly indicate that they want to talk with peers who are in the same situation. Contact with peers can be confronting, but it also can be characterized by identification and feelings of fellowship. Transition programs ought to integrate meeting with peers in the care package that they provide. This could be done as part of an adolescent day or a camp.
Actual Transfer to the Adult Congenital Cardiac Clinic
The actual transfer from pediatric cardiology to the adult congenital cardiac clinic is a milestone in the transition. This transfer usually takes place around the age of 18 years. A proper handover has to be scheduled. There are different ways to organize this handover. It can be done by means of a referral letter including a summary of the medical history. However, a personal contact between the pediatric and the adult providers would be more optimal. In this respect, a joint outpatient visit with the pediatric cardiologist and the adult congenital cardiologist is sometimes advocated. Given the time consumption and practical difficulties to organize it, such joint outpatient visits are seldom sustainable. Therefore, transition coordinators are deemed to play a crucial role. For instance, at the first visit at the adult congenital cardiac outpatient clinic, the patient meets the transition coordinator, who is then joined by the adult congenital cardiologist. In addition to handing over the medical file, the transition coordinator explains the issues that have been discussed and worked on during the transition. Input from the patient and parents are facilitated. This kind of transfer visit has been found to lower the barrier toward the adult program, and has been shown to be effective.
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