Most children born with congenitally malformed hearts now survive to adult life, as a result of the spectacular achievements of paediatric 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 which 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 recognised. Several panels of international experts have provided frameworks for provision of services. 1–9 These share a number of common recommendations. First, all recognise 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 centres 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. Despite the numerous statements expressing consensus, many adolescent patients with congenital cardiac disease still do not have the opportunity to participate in a defined process of transition that aims to optimise clinical education and social outcomes. As a result, few of these patients or their families have learnt what they need to know about their conditions and their responsibilities, and many have been lost to appropriate follow-up. 10 In this chapter, we focus on the steps required for the processes of transition, and then transfer of care, for young adults who have chronic childhood illnesses in general, and complex congenital cardiac diseases ( Table 61-1 ) specifically. We will review important general aspects of transition, as well as the key elements for an orderly programme of transition, which include timing of transfer, location, and staffing. Attention to the provision of care at this important stage of the journey of the patient is essential to maintain the excellent standards of care that are usually provided in the paediatric age group, and to insure that patients become empowered to take over their own issues with health, becoming autonomous and competent young adults.
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THE PROCESS OF TRANSITION
Transition is the generic process that applies to all children and adolescents with chronic disease. 11–13 It can be defined as an educational and experiential process that prepares patients to take responsibility for their own health care. The process 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 may take 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. This remains a challenge in many medical systems. During the process, issues both generic and disease-based need to be addressed. This has an important impact for provision of services, both in terms of location and staffing of the clinic responsible for the transition. Frequently, the coordinated transfer of care from the paediatric to the adult health care environment is the final step in a successful process of transition. This depends importantly on the relationship between the staff of the clinic responsible for transition and the patient, which should have been built up over the years. During and after this time, the nature of the relationship between doctor and patient will change.
Paediatricians tend to consult predominantly with the parents, whereas adult practitioners seek to develop a partnership with the patient. This involves directing information and education towards the patient and, at the same time, encouraging responsibility and self-reliance. It is critical that, during adolescence, in the transition clinic, 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. In a recent review, lapse of medical care, for a median duration of 10 years, occurred during the process of transition in two-thirds of grown-up patients with congenitally malformed hearts. Those who lapsed in this way were three times more likely to require urgent cardiac intervention when next seen. It is the lack of understanding by physicians of the need for continued care into adulthood that may underlie failure of effective communication. 10
Key Issues for the Process of Transition
When Should Patients Begin the Process of Transition and Transfer?
The timing of entry from a paediatric 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. 14 Referral to the clinics responsible for transition should ideally begin by 12 years of age. Attendance at such clinics involves a change in approach from the typical paediatric 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 counsellors. The approach to communication needs to evolve as the teenager grows older, with provision for discussion appropriate to age and maturity. Transfer from the transition clinic to the adult clinic most commonly occurs at the age of 18 years, 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 paediatric 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. 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 paediatric hospital, as this makes the first move from the paediatric clinic less traumatic to the patients and their families. Those staffing the clinic have the opportunity, during several visits over years, to organise 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 paediatric 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 organised 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 paediatric 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. This, in our experience, is a key step to avoiding lapse of care and loss to follow-up when the patient is eventually transferred.
Specialist nurses play a crucial role in successful transition. They should have experience in the needs of adolescents and young adults with congenitally malformed hearts, and should have received training in counselling. 15 There should be facilities for the specialist nurses to consult with the patient separately from the doctor, as this encourages early discussion of sensitive problems and anxieties. The specialist nurses frequently are the main contact with the patient and his or her family, developing a unique relationship with them.
What Is Involved in an Ideal Transitional Programme?
The programme involves both continuation of high-level care for cardiac and non-cardiac problems as well as a specific transitional curriculum designed to prepare the adolescent and young adult for successful transfer. In many centres, a comprehensive transitional curriculum has been developed, which covers education about the cardiac condition, its impact on future life, and strategies to smooth transfer into adulthood. 16
All children with congenitally malformed hearts should have a plan of care established prior to transfer. Ideally, this is initiated in childhood, and developed during transition. It should be based on a multi-disciplinary process of consultation, and communicated effectively to the adolescent and his or her family. A clear route of transfer to an appropriate level of adult cardiac services is an essential part of this plan. A major potential barrier to successful transition is the belief of patients and families that cardiac surgery or catheter intervention has cured their condition. Patients and families need to have explained to them, in a sensitive manner, that although in many cases life expectancy can be normal or near-normal, the patient’s heart is not normal and the patient requires life-long follow-up. It is important that patients understand that, despite treatment, their cardiac condition remains fundamentally different from that of most adult patients, and that relatively few providers have been trained to monitor their care. This should be accompanied by reassurance that their care plan will involve attendance, at least once, at a specialist unit dealing with adults with congenitally malformed hearts. It is equally important at the end of transition to decide which patients do not require long-term cardiac follow-up. Our current policy is to keep patients with conditions deemed to carry low risk on the database, with very infrequent visits. The premised value of this approach is to allow support for issues of life such as employment. Regular cardiac checks of patients with a very low risk of further complications, however, may have adverse consequences. For example, repeated visits may lead to an unnecessarily high level of anxiety about future health. Indeed, there is evidence for this in other medical conditions.
The complexities of the process of transition can seem so daunting that, as a result, little or nothing is done to prepare the young patient. It is clearly important that some steps be taken and that the transition become an integral part of the journey through life of the patient with a congenitally malformed heart, rather than simply being ignored because a comprehensive process seems unachievable. One approach is to adopt a strategy of starting small and aiming to improve. For a disease-based model, some basic information should be put together about the patient and transmitted to him or her clearly and repeatedly. Such a minimalist message might resemble the following:
You have had surgical repair of tetralogy of Fallot. As a result, you have a leaky pulmonary valve. You will need to be followed annually by a cardiologist who knows about congenital cardiac defects. In order to stay healthy, you should see such a person once a year for the rest of your life. If you have any cardiac problems, you should contact this person. You should become knowledgeable about your health history. You should have a copy of your own health information. You should make sure you always have good quality health insurance, and know how to get it. You are at risk for infective endocarditis, and should be sure that your teeth and gums are always healthy to reduce this risk.
The agenda can then be broadened beyond this brief approach by regular consultations with the patient. A more complete curriculum for the transition can be communicated using different strategies, involving the cardiologist and specialist nurses. These strategies can include seminars for patients and families and information provided on paper or online. These methods are invaluable, in particular, when providing access to new information. In many ways, the educational aspects of the transition should continue indefinitely, as patients often feel a greater sense of control over their medical health and management if they are able to seek new knowledge. Close collaboration with support groups for children and adults with congenitally malformed hearts, which have been established in many countries, is invaluable for promoting education.
While the needs of some patients during adolescence are straightforward, others may require cardiac or non-cardiac medical or surgical care. Access to services, including obstetrics and gynaecology, psychiatry, nephrology, respiratory, neurology, and orthopaedics, is important, and this should be provided either within the specialist institution or within a network of linked local centres. A recent model of care developed by the Department of Health in the United Kingdom illustrates a model for primary, secondary, and tertiary provision of care for adults with congenitally malformed hearts ( Fig. 61-1 ). 17
