Transition of patients with thalassaemia and sickle cell disease from paediatric to adult medicine
Based on a presentation given at the 2nd Pan-European Conference on Haemoglobinopathies
By Dr Holger Cario, Department.of Paediatrics and Adolescent Medicine, University Hospital Ulm, Germany
More than 30 years ago, the majority of chronically ill children did not survive into adulthood. With the advent of innovative technologies and medical advances, many chronically ill adolescents – including patients with haemoglobinopathies – cross the threshold into adulthood every year. Successful transition of chronically ill adolescents from paediatric to adult medical care continues to be a challenging process. Often, the transitioning experience consists of an abrupt transfer, which leaves the youth unprepared for the movement to adult health care. Because adolescents and young adults with haemoglobinopathy are at risk of developing severe medical complications, obstacles that impede transition to adult care must be identified to promote an uninterrupted transfer.
The transition of patients with haemoglobinopathies from paediatric to adult health care is a complex and multisided process. The consideration of the following “sides”, or aspects, of this process is of particular importance for its successful management and finally, for an optimal continuous medical care for patients with thalassaemia major (TM) or sickle cell disease (SCD).
1.Medical Treatment
This comprises all aspects of intrinsic medical treatment, i.e. treatment with regular medical drugs (for instance analgesics and hydroxycarbamide in SCD, chelation therapy and drugs for secondary complications in TM), emergency treatment, and transfusion in TM or exchange transfusion/erythrocytapheresis in SCD. It is of particular importance for the patients’ adherence and compliance, and thus for treatment success, that medical care for a specific disorder in both paediatric and adult health care is based on common standards. Depending on national or regional conditions, the degree of implementation of such common standards is currently varying.
2. Education and Training of medical staff
This aspect is very closely related to the first. Apart from the central medical care provider, the paediatric or adult haematologist, many other persons involved in the medical care need adequate training to meet the needs of these complex disorders. This group comprises subspecialists such as cardiologists and endocrinologists, nurses, educators, psychologists, social workers and others. A very close collaboration of these caregivers is essential for a successful treatment.
In countries where haemoglobinopathies affect primarily patients with a background of migration, and where migration occurred rather recently, patients only started to enter adulthood, and to look for treatment by adult health care services, during the last ten or fifteen years. Thus, adult health care providers inevitably had limited experience in the management of these rare disorders. In order to pass over knowledge and experience, not only is it important to have a close collaboration between different subspecialists and health care professionals within a medical care unit, but also a very close collaboration between paediatric and adult haematologists.
3. Health Care Setting
The health care setting varies from country to country, and sometimes even between regions within a country. For many patients, the transition from paediatric to adult care will be associated with transition from a rather familiar ambience within an in-patient clinic to an out-patient clinic focussed on the care for self-managing individuals. Even patients treated in paediatric out-patient clinics will feel such fundamental change, since they got used to the ambience and to individual health care providers, particularly nurses and other supporting staff, knowing them for their whole life.Therefore, transition to adult health care must include the careful preparation of patients to these changes to avoid frustration and retraction.
4. Adolescence and Puberty
At the time of transition, patients usually are at the end of their puberty. Classic adolescent rebellion can be an added problem for patients with chronic illness. Actually, puberty certainly represents one of the most vulnerable periods in the life of each patient with haemoglobinopathy. Adolescents with thalassaemia begin to challenge the need to continue chelation treatment, even after the introduction of alternative medical drugs without the physical burden of deferoxamine. They stop adhering. Patients become aware of physical problems caused by their disease or its treatment. And puberty itself can be disturbed by hypogonadism caused by iron overload representing a major burden for affected patients. All these problems have to be considered within the transition process, which should follow a standardised plan but has to be realised in an individualised way.
In addition to hypogonadism, other complications of iron overload, such as cardiac disease or diabetes mellitus, may occur in the same period. In these cases it has to be weighed very carefully where (in paediatric or adult medicine) treatment of such complications should be initiated. The ideal way, of course, would be the collaborative approach.
5. Social Activities
At the end of adolescence, there are other issues besides the transition from paediatric to adult medical care that predominate the life of young adult patients with TM and SCD. These include professional training, jobhunting, love, family planning and others. In some countries, personal responsibilities for health insurance and the payment for medical care are associated with additional problems for the patient. These aspects should be included in a transition programme. Both paediatric and adult health care workers have to develop possibilities to provide continuous support concerning these questions before, during, and after the transition.
6. The Families
The role of the family in the medical care of patients with haemoglobinopathies, and the transition process, varies depending on national, regional, and ethnic conditions, but everywhere it is of great importance. During infancy and childhood, the families and the parents in particular guarantee adequate medical treatment of their children. In adult medicine, the individual patient with self-care responsibility is the immediate partner of the physician or subspecialist. With regard to this aspect, an abrupt change from paediatric to adult care would certainly have an adverse effect on further treatment, adherence and compliance.
The influence of the parents may decrease during puberty. In the positive case, patients develop self-efficacy and self-care responsibility. In the negative case, the lack of compliance and adherence result in a long period of inadequate treatment, which can have even fatal consequences. Thus, patients’ education concerning their diagnosis, treatment, and preventive measures, but also psychological and social support, are of central importance within this period. Parents need encouragement to handle this situation, to acknowledge the increasing personal responsibility of their children, and to settle into their new role as accompanying partner and supporter. The transition of patients with haemoglobinopathies and other chronic disorders is not a one-off event, but a long-lasting process. Transition programmes must be developed in collaboration between paediatric and adult health care providers. They should consider the above-mentioned aspects, and therefore include the availability of common treatment standards, the collaboration within a multidisciplinary team and between paediatric and adult haematologists, the training of health care professionals, the education of the patient, the integration of the patient into decision-making, and the help for the families to strengthen their ability to support the patient in and after the transition process. The administrative handling of the transfer from paediatric to adult medical care should be planned in advance, including the transfer of relevant records. The timing of transfer should be flexible, depending on the patient’s developmental and social background. Thus, based on a standardised programme, the transition process should be tailored for each individual patient.
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