Living a ‘normal’ life: young people coping with thalassaemia major or sickle cell disorder

Abstract

This qualitative paper explores the strategies and resources young people use to cope with sickle cell disorder or thalassaemia major, two haemoglobin disorders with serious implications for health and survival. By focusing on coping strategies, we explore how young people attempt to take control over their lives. The respondents, largely of South Asian and African Caribbean origin, aged between 10 and 19 years, valued maintaining a ‘normal’ life and struggled to achieve this normalcy. Strategies were employed to minimise difference from peers but these strategies remained vulnerable. Coping occurred in a dynamic space, involving negotiation and engagement with both personal and structural factors. Threats to normalcy did not always reside in the condition; life transitions, changes in social relationships and racist, disablist or sexist marginalisation also threatened coping strategies.

Introduction

This paper explores how young people live and cope with a sickle cell disorder (SCD) or thalassaemia major, two serious recessive haemoglobin disorders, with life-limiting and potentially debilitating consequences. We address the worries and difficulties young people face, the coping resources and strategies they employ to live a ‘normal’ life, and the wider social factors which can support or undermine their coping strategies. By focusing on coping strategies we emphasise how young people can take control of their life and underline the importance of not seeing them as tragic victims at the mercy of their illness (see Morris, 1993). At the same time, by locating our discussion within the broader social context, we also suggest that many of the difficulties they faced are a consequence of the wider society's inability to accommodate ‘difference’ (see Oliver, 1996; Barnes, Mercer, & Shakespeare, 1999).

Such a broad approach is important because much previous work on haemoglobinopathies, largely located within the ‘medical model’, neglects the social and political aspects of these conditions. Discussions about the social construction of disability and chronic illness, and the relevance of racism to the experience of impairments have only recently begun to penetrate this literature (see Priestley, 1999; Ahmad, 2000). These debates, in addition to providing invaluable insights, offer a more general reminder that living and coping with a chronic illness occurs within a dynamic space, involving negotiation and engagement with structural and personal factors, as well as historical and present-day social relationships (see Bourdieu, 1977). With this as our starting point, we offer an account of young people's lives in which individual responses to their illness are discussed within a broader social context.¹

We begin our account, however, by providing a necessary summary of the two conditions. This provides an important background for locating the young people's narratives but raises, as we shall see, a constant — but perhaps necessary — tension between the ‘medical’ and ‘social’ models of disability. SCDs and thalassaemia major are recessive blood disorders where individuals who inherit a deleterious gene from both parents develop the disease. In the UK the groups most at risk of SCDs are of African Caribbean or West African origin. The thalassaemia gene is more commonly found among Cypriots, South Asians and Chinese, and less commonly among African Caribbean and white British people. SCDs include sickle cell anaemia, haemoglobin SC disease and sickle beta-thalassaemia. All have similar consequences; they are variable, unpredictable and, at times, life threatening. Those with SCDs are prone to ‘sickling’ of the red blood cells, causing blockages in smaller blood vessels and resulting in ‘the painful crisis’, as well as anaemia, leg ulcers, stroke and damage to various parts of the body including the spleen, kidneys, hips, eyes and lungs. Affected children are also vulnerable to conditions such as pneumonia and meningitis. Treatment and care include the prevention of life-threatening infections, pain management and the avoidance of circumstances that cause the red blood cells to ‘sickle’. A child born with thalassaemia major is unable to make a sufficient amount of haemoglobin and needs blood transfusions every 4–6 weeks, for life. Excretion of excess iron acquired through transfusions requires injections of a drug such as desferrioxamine (desferal), using a battery-operated infusion pump for 8–12 h a day, 5–7 nights a week. Many of the complications associated with thalassaemia major result from non-compliance with the daily infusion pump. This causes ‘iron overload’ and can affect various organs. Specific complications include diabetes, delay or failure to enter puberty and heart problems. Death through non-use of the infusion pump is not uncommon. Other problems can include infections, such as hepatitis C, acquired through blood transfusions.