Psychological considerations in the management of patients with thalassaemia major: (1) Reactions to chronic illness in childhood and adolescence: A report of a case

Abstract

The observations reported are of the emotional, cognitive and affective reactions of youngsters with thalassaemia major, and are based upon direct, long-term clinical experience with more than a hundred children and adolescent patients who have been treated for this fatal illness, in the Department of Paediatrics and Child Care, Hadassah University Hospital, Jerusalem, Israel. These psychological reactions, which may be unique to this illness, appear to be significantly influenced by predisposing specific and idiosyncratic cognitive and cultural approaches and conceptualisations, and by the combined effects of early diagnosis and subsequent availability of modern medical therapeutics and technology. Maintenance of a relatively high haemoglobin level (above 9 dl) allows affected children to attend normal school settings and to lead relatively unrestricted lives during the early childhood and adolescent years. However, denial, unrealistic expectations, linked and combined with wide ranging cognitive distortions of illness, profound clinical depression and a pathological passivity and easy regression, social withdrawal and isolation, unsolicited dependency and profound psychological decompensation often appear to be common dynamic elements, at least during the adolescent years, foreshadowing imminent death. Emotional reactivity of the affected adolescent may be quite bewildering, and a case presentation together with an analysis of human figure drawings produced by youngsters with this illness has been included in an attempt to document these special neuro-psychological and active disruptive management issues. When affected children grow up and emotionally mature, thalassaemic adolescents, in our experience, tend to exhibit an uncharacteristic unprotesting and uncritically acquiescent and pathologically-based passivity and, further, exhibit unwarranted and, hence, often unrealistically-maintained high levels of optimism, unconsciously linked to much denial in what is clearly and tragically an inescapable confrontation with the prospects of a fatal illness with a prognosticated early death. Survival beyond age twenty-five is rare. Organising a more comprehensively orchestrated concerned psychological approach to both developmental and maturational needs with the establishment of a trusting, mutually-respectful therapeutic alliance with the medical team may in part help to overcome some of the inevitable difficulties which may otherwise menacingly interfere with optimal medical management. Hundreds of medical and technician staff contacts with frequent hospitalisations and the prospect of surgical intervention contribute to the equation of anxiety. Parental reactions to the inescapable prospects of an early death at adolescence or later often parallel those of the affected children. Anxiety, guilt, body-image disturbances of a profound and malignant psychological order combine with chronic inclinations and an easy readiness towards depression which are transparently evident in both children and their respective parents at times of acute crises, surgery or later medical decompensation. Therapeutic guidelines and principles of behavioural management are laid down which will assist the active clinician in better recognising and understanding some often difficult problems which are too easily avoided lest special attention be drawn to them.