Pumping iron: compliance with chelation ... - Wiley Online Library

Sociology of Health & Illness Vol. 22 No. 4 2000 ISSN 0141±9889, pp. 500±524

Pumping iron: compliance with chelation therapy among young people who have thalassaemia major Karl Atkin and Waqar I.U. Ahmad Centre for Research in Primary Care, University of Leeds

Abstract

Compliance with medical regimens is not simply a technical or practical task but part of the everyday experience of living with a chronic illness. Any discussion of compliance must, therefore, begin from the individual's personal and social context. This paper explores how young people who have thalassaemia major respond to their daily chelation therapy. It suggests that compliance dominates the young person's narratives and represents the most disruptive aspect of their illness. More specifically, compliance evokes both practical and emotional difficulties as the young person tries to make sense of the relationship between body, self and illness. Within this dynamic process the young person has to reconcile the consequences of non-compliance within the broader experience and responsibilities of `growing-up' and maintaining a positive selfidentity.

Keywords: young people; chronic illness; compliance; thalassaemia; ethnicity; genetics. Introduction Compliance with treatment is a major problem in many childhood chronic illnesses, even when medical regimens are essential to the child's wellbeing (see Varni and Wallander 1984, Stark et al. 1987, Hayford and Ross 1988, Lemanek 1990, Geiss et al. 1992). The reasons for non-compliance transcend straightforward medical concerns. Compliance is not simply a technical or practical task but is bound up both with the imagery and connotations associated with the illness and personal biographies (Bury 1991). Treatment regimens are experienced in the context of everyday experience of living with a chronic illness (Marteau and Johnston 1986) and any discussion of compliance must, therefore, begin from the individual's # Blackwell Publishers Ltd/Editorial Board 2000. Published by Blackwell Publishers, 108 Cowley Road, Oxford OX4 1JF, UK and 350 Main Street, Malden MA 02148, USA.

Chelation therapy for thalassaemia major and young people's compliance

501

personal and social context (Atkin et al. 1998a). This paper explores how young people who have thalassaemia major view and respond to their treatment regimens. We examine the wider context within which chelation therapy ± nightly infusion of an agent, through a slow operating pump, to break down excess iron ± assumes meaning and significance. We begin with a brief description of thalassaemia1. Thalassaemia major is a recessive genetic condition in which individuals who inherit the thalassaemia gene from both parents (i.e. carriers) develop the disease. In the UK, the thalassaemia gene is more commonly found among Cypriot, South Asian and Chinese people, but also among AfricanCaribbean and indigenous `white' British people. There are estimated to be around 600 people with thalassaemia major. A child born with thalassaemia major is unable to make sufficient haemoglobin and needs blood transfusions every four to six weeks, for life. Blood transfusions, result in a potentially dangerous excess of iron in the bloodstream. Excretion of excess iron gained from transfusions requires injections of a drug such as desferrioxamine (desferal), using a battery operated infusion pump, eight to 12 hours a day, five to seven nights a week. Many of the complications associated with thalassaemia major result from non-compliance with these infusions (Modell and Anionwu 1996). Non-compliance causes ìron overload' and can affect various organs. Specific complications include diabetes, delay or failure to enter puberty and heart problems. Non-use of the infusion pump is the most common cause of death among those with thalassaemia and many young people die in their 20s and 30s. The effects are cumulative and not immediately evident. The first signs of ìron overload' are often apparent only after irreversible organ damage has occurred and this may be several years after non-compliance took place. Other problems associated with thalassaemia major include infections, such as hepatitis C, acquired through blood transfusions. Chelation therapy and thalassaemia major Not surprisingly, young people find the regular use of the infusion pump the most difficult and disruptive aspect of their illness (Ratip et al. 1995). Continuous use of the pump is seen to be restrictive, imposing limitations on the young person's day-to-day existence (Ahmad and Atkin 1996). In this way, treatment regimens can become central to young people's illness narrative and are part of how they understand, experience and cope with their chronic illness (Strunk et al. 1985, Prout et al. 1999). Various interrelated themes inform the young person's response to chelation therapy and symbolically these go beyond the actual task of self-injection, the physical discomfort associated with the procedure or knowledge of the importance of chelation to their survival (Darr 1990). Compliance needs to be seen as part of the wider process by which young people make sense of the relationship # Blackwell Publishers Ltd/Editorial Board 2000

502 Karl Atkin and Waqar I.U. Ahmad

between their body, self and illness as they struggle to lead valued lives and maintain and construct a positive self-image (Bury 1991, Locker 1997). Exploring this `struggle' is an important starting point in understanding young people's response to medical regimens, particularly since it occurs against the background of `growing up' (Ebata and Moss 1991). Coping with the new experiences and responsibilities of growing up, for example, becomes difficult as previous certainties are questioned (Dornbusch et al. 1991). This occurs irrespective of whether a young person has a chronic illness or not and the emerging emotional difficulties associated with negotiating freedoms and responsibilities with parents, establishing peer relationships, coping with school, and making decisions about future ambitions can impinge on the compliance process. A young person's experience of chronic illness does not occur within a social or psychological vacuum. Social networks ± such as relationships with peers ± provide young people with a framework within which to make sense of their illness (see James 1996). Peers can offer support as well as provide an ongoing commentary on the young person's experiences (Alderson et al. 1996). Young people, by comparing themselves to their peers, also construct a sense of `normalcy' (Frydenberg 1997), in which they come to understand what is `normal' behaviour for those of the same age and gender. Chelation therapy is accommodated within this understanding as the young person attempts to limit the consequences of having a chronic illness. This perhaps explains why non-use of the infusion pump, for example, is especially common among àdolescents' as they strive to be `normal', adopt lifestyles which are consistent with their own wishes and identities and are valued by their peers, and take greater personal control of their own care. The issue of non-compliance among àdolescents' is also noted in other childhood chronic illnesses such as cystic fibrosis (Mador and Smith 1989, Geiss et al. 1992), asthma (Lamenek 1990) and diabetes (Hoare and Mann 1994)2. The family is another important feature of the child's illness narrative (Thompson 1994). The care of a young person is negotiated within the context of family relationships, obligations and reciprocities (Finch and Mason 1993) and young people take decisions about their health within the family context (Brannen et al. 1994). As important mediators between the child and health professionals, parents assume a central role in negotiating compliance with their children (Midence et al. 1996, Prout et al. 1999). The relationship between child and parent is fundamental to this and compliance can itself become a site around which relationships are developed and challenged (Ahmad and Atkin 1996). Compliance, for instance, can be a specific source of disagreement especially as the child attempts to assert his or her growing sense of independence (Ahmad and Atkin 1996) and this is typical of other chronic conditions (Geiss et al. 1992). Parents never fully trust their child to comply with medical regimens (Atkin et al. 1998a). However, young people, for their part, may feel their parents over-react to their illness (Midence et al. 1996). These feelings can lead to tensions in the # Blackwell Publishers Ltd/Editorial Board 2000


503

parent-child relationship, with young people accusing parents of being overprotective: a common theme in the literature on childhood disability (Eiser 1990, Davis and Wasserman 1992) as well as the more general literature on `growing up' (Brannen et al. 1994). Yet at the same time, parents often have legitimate reasons for distrusting children's claims about compliance. In thalassaemia, for example, routine blood checks easily identify raised feretin levels, indicative of non-compliance. Following an account of the methods, we explore how young people manage chelation therapy and the impact it has on daily living; how they cope with non-compliance; the way in which responsibility for chelation therapy is negotiated with parents; and young people's views on the value of such therapy. The study The aim of the study was to provide a detailed understanding of young people's experience of living with a genetic condition, within the broader context of `growing up'. We conducted in-depth interviews with 25 young people with thalassaemia (age range 10±19 years). (The study also included 26 young people with SCD. This paper, however, does not include their experiences.) Each young person was interviewed twice over a six-month period. This enabled the young person's narrative to reflect the variability of the condition and its possible influence on their experience. The interviews illustrated the value of this approach as we were able to explore how young people's coping strategies changed over time as well as what might have been responsible for this change. This, as we shall see, proved especially valuable when making sense of a young person's response to chelation therapy. The sample was drawn from the records of health professionals, such as paediatricians and specialist haemoglobinopathy (disorders of the haemoglobin) workers, in six localities in the Midlands and Northern England. In all areas but one, the sample represented all known cases of thalassaemia. In the one remaining area, respondents were randomly selected in order to reach the target sample of 25. The eventual sample included 12 males and 13 females. Their average age was 13.9 years and the mode was 11 years. We interviewed two 10-year-old children; 12 young people were aged between 11 and 14 years; seven were aged between 16 and 17 years and four were aged between 18 and 19 years. In terms of ethnicity, 22 described themselves as Pakistani Muslim; one as Bangladeshi Muslim; one as Iranian Muslim and one Indian Hindu. Fifteen young people were still at school; seven were at college; two were seeking work and one was at university. In terms of family structure, 21 young people lived with their two `natural' parents. Two lived with widowed mothers and one lived with his divorced mother, but still had regular contact with his father. Another young person lived with his # Blackwell Publishers Ltd/Editorial Board 2000


brother and brother's wife, who were his legal guardians. The sample also included two young people who were married, but who were not living with their spouses. Three sample members lived in three-generation households and all but one lived with at least one sibling. The mean family size was 6.8 (including the person with thalassaemia). Young people were offered an interviewer of their own sex. All interviews were in English and were tape-recorded. Before the interview began, written informed consent was obtained from the young person. The health professionals who provided the sample explained the study to the young person, gave them a brief information sheet and, with their consent, passed us their names. We followed up with further information on the nature of the study and the interview and asked if they were willing to be interviewed. Nobody refused. At this stage, we also explained our work to their parents and none objected3. All respondents were interviewed in their home at a time of their choosing. The project used qualitative methods and analysis, based on semi-structured interviews. Such methods allow an examination of complex and contingent situations, behaviours and interactions. A topic guide identified a number of key themes developed from a review of the relevant literature, discussions with key informants, advice from an èxpert' advisory committee and our own previous work (see, for example, Atkin et al. 1998a and 1998b). The purpose of the interview was to get the child to discuss their illness within the broader experience of `growing-up'. Rather than assume that the illness would dominate their narratives, we created an environment in which they could reflect on their experience and tell us to what extent the illness did affect their life. The topic guides reflected this by asking general questions about family relationship and social networks. All young people saw themselves as ill, but to focus exclusively on their illness failed to recognise how they have to negotiate other aspects of their identity. This emerges as an important analytical point, particularly since previous work on thalassaemia has tended to attribute the problems experienced by children to their illness, rather than discuss their experience within this broader context (see Tsiantis et al. 1996). Many of the concerns and difficulties articulated by those with thalassaemia, however, are similar to those of their peers (Zani et al. 1995). Transcribed interviews were organised according to analytical headings. Following accepted conventions of qualitative analysis (Gubrium and Silverman 1989), information was taken from the transcripts and transferred onto a map or framework, allowing comparison by theme and case. The respondents' accounts were organised by categories and sub-categories, suggested by the topic guides as well as new categories that emerged from analysis of transcripts. The material included under each heading reflected both the range and the frequency of respondents' views on particular issues and formed the basis of generalising their experience. This enabled a comparative analysis of different aspects and variations in experience, as well as the significance of the individual's background in making sense of this experience. Analysis could then begin to define concepts, account for # Blackwell Publishers Ltd/Editorial Board 2000


505

patterns and ranges, establish linkages and give explanations. This paper explores one specific subject, compliance with chelation therapy. In the accounts below, pseudonyms are used to protect respondents' identities. The findings Managing chelation therapy and its impact on the young person's life Nearly all young people, irrespective of gender, cited the regular use of the infusion pump as the most difficult and disruptive aspect of thalassaemia major. All those over 13 years had failed to comply with chelation therapy at some stage and for those aged 13±16, non-compliance was more common than compliance. Seventeen-year-old Ashiq Javed remarked: I get stressed especially when I'm putting the pump on at night. I think, `The hell, why?' I mean the pump is, I mean I don't like, I don't mind the transfusion at all, it's the pump, you know. I think every person with thalassaemia major will say, it's the pump. The pump's the problem. Only those under 12 years of age were likely to play down the difficulties associated with chelation therapy. For them, chelation remained a parental responsibility and the young person did not yet dwell on the potential consequences of medical regimens for their sense of difference. Nonetheless, for older children, managing chelation therapy emerged as central to their illness narrative. They are physically and symbolically tied to the infusion pump and chelation therapy was seen to dominate their life. Not surprisingly, this caused resentment, with the use of the pump often becoming a focal point of the difficulties of living with thalassaemia major. Chelation therapy specifically evoked both practical and emotional difficulties and this explained why three-quarters of the sample specifically remarked that they hated using the pump. Nineteen-year-old Robina Begum described why she stopped using her pump: Because I started to hate it. I just didn't want it and, I don't know, things just got on top of me and I didn't want to wear my injections. This sense of hatred is fundamental to understanding their relationship to chelation therapy and, as we shall see, will regularly recur throughout their narratives as we unpack these emotional and practical difficulties. Their response to treatment, however, would appear no different from those with equally demanding medical regimens, such as severe asthma and diabetes (Crisp et al. 1996) and many of the difficulties leading to non-compliance seem common to other chronic illnesses (Lenny et al. 1994, Prout et al. 1999). In itself the use of the infusion pump was not a difficult practical task, especially as the respondent got older. Practical problems, however, do # Blackwell Publishers Ltd/Editorial Board 2000


preoccupy younger children. Nearly all those under 12 complained that the pump was uncomfortable and cumbersome. These younger children were also more likely to complain about the pain associated with inserting the needle into their body. For some, this pain often became associated with fear. These problems are also mentioned by parents, and explained their dislike of chelation therapy (Atkin et al. 1998a). Nonetheless, once the child had entered their teens they had usually accommodated these practical difficulties, or at least their relevance to the illness narrative had become less important. Chelation therapy still represented a practically demanding and disruptive chore, but this is not where its significance to their narrative lay. It was more what chelation therapy came to symbolise, and this introduced more emotional concerns as the young person responded to the broader social context. These older children's concerns had thus begun to transcend the practical task of chelation therapy. To a large extent, older children hated chelation therapy because it marked out their `difference'. The young people themselves constructed this sense of `difference' through their relationships with peers, and it exercised an important influence on their life and identity. As part of this process they began to compare themselves with their peers and to dislike anything that suggested divergence from acceptable youth culture (Brannen et al. 1994). This is often an overriding concern when a child enters their teens (Frydenberg 1997), irrespective of whether they have a chronic illness or not (see Corsaro 1997). Jamil Rahman's account was typical: I actually go round seeing my mates and all that and like they're just like living normal, and they're doing everything. Not surprisingly, the consequences of their illness become caught up in this and rejection of chelation therapy represented a symbolic attempt at securing `normalcy' within the broader context of `growing up' (see Lemanek 1990 in relation to asthma). This failure to escape from chelation therapy caused sadness as well as depression. Seventeen-year-old Gulab Maqbool described why he did not use the infusion pump regularly because of his wish to be `normal' like his peers: Ì see other people running around free, with nothing'. Sixteen-year-old Ashiq Javed explained why he did not use the pump during his early teens: To be honest I couldn't be arsed [sic] to use it. I just got fed up. I wanted to be like everybody else. To this extent, chelation therapy becomes a symbolic site of strategy and struggle as young people attempt to assert their claims to self-determination and emphasise their similarity with peers. There are various elements informing their response. # Blackwell Publishers Ltd/Editorial Board 2000


507

Making sense of chelation therapy As they grow older, young people are keen to assume what they regard as adult roles in the British context, in the form of asserting their independence and own identity (Brannen et al. 1994, Frydenberg 1997). Independence and autonomy represent social and cultural constructs which may not have the same meaning among different ethnic groups (Ahmad 1996). This is further complicated for young Asian people in the UK; they construct their identities in the context of both their ethnic and religious culture and the broader British culture. Leaving home and establishing an independent existence seemed less important to South Asian young people than the African-Caribbean people we talked to (see also Brannen et al. 1994). South Asian young people, therefore, were not concerned about the potential impact of chelation therapy on establishing a separate household, but they did worry about whether potential partners would be able to cope with their illness. They also worried about how the illness would affect their own abilities to fulfil family obligations. The narratives of African-Caribbean respondents (not reported here) as well as the more general literature on young people (Brannen et al. 1994), suggests such concerns may be more common among South Asian young people. South Asian boys, for instance, felt they might not be able to look after their parents when they got older. Girls, on the other hand, expressed more immediate concerns about discharging their domestic responsibilities. These concerns, although contributing to their sense of difference, were rarely implicated directly in compliance, except as a more general influence on their coping strategies. We return to this later. Nonetheless, South Asian young people do feel they have to develop and sustain an identity separate from their parents and exercise some control over their own lives. They expect to be less emotionally dependent on their parents, as they grow older. Young people articulated these concerns regardless of gender, although achieving independence was a particular issue for young men, who were worried about their possible future dependence on parents. Sixteen-year-old Amjad Javed's account was typical of other boys his age: I don't want my parents to look after me for all my life. I don't want them to worry. It's not right, is it? I mean, my friends don't have that problem. To this extent, the experience of South Asian young people seems similar to that of all young people (Corsaro 1997), although their ideas about what constitutes independence may be slightly different. Establishing an independent identity can contribute directly to non-compliance among those with thalassaemia, as they reject others' definitions of their problems; # Blackwell Publishers Ltd/Editorial Board 2000


something noted for young people with chronic illness in general (see Hayford and Ross 1988, Geiss et al. 1992, Midence 1994). More generally, young people had to face the dilemma of how to reconcile a growing wish for independence with the threat of increased illness-related dependence: a tension common among young people with a chronic illness (Sinnema 1992). This finds particular expression through the young person's relationship with their parents. Those over 13 especially felt dependent on their parents and unable to exercise the choices available to their peers. This was seen as a threat to their growing sense of assuming adult roles and explains why they disliked constant reminders about the value of medical regimens by their parents. Such feelings are common in other chronic illnesses such as cystic fibrosis and diabetes (Mador and Smith 1989, Geiss et al. 1992). As we have seen, younger children did not usually worry about this dependence on their parents, as they saw it as natural for children of their age. This is perhaps why non-compliance was especially high for those aged 13±17; an age when status transition is being negotiated (Frydenberg 1997) and when young people begin to take responsibility for their own treatment. Older respondents especially found the regular use of the infusion pump restrictive and disruptive preventing them from pursuing, what they regard as valued pursuits, which would help emphasise their `normality' and similarity with peers. The use of the pump prevented them from keeping up with peers and socialising with them. Not being able to spend nights at friends' houses as spontaneously as their peers, was noted by many as an indication of the restriction imposed by chelation therapy. Older boys, usually those over 13, saw chelation therapy and more generally the condition, as a threat to their masculinity. This threat related to concerns such as delayed puberty, potentially smaller stature and a compromised ability to engage in sport. This further emphasised their sense of difference and made it difficult to sustain a shared identity with their male peers. Girls, on the other hand, were concerned about the impact of chelation therapy on maintaining social relationships. For both boys and girls, the response of their peers could also emphasise their sense of difference. Several recounted upsetting comments from their peers on their use of chelation therapy. This made them feel ashamed, and undermined their claims to `normality'. Some also pointed out how the disruptions caused by chelation therapy could undermine their cultural identity and thus further contribute to their sense of difference. Many of the older boys, for instance, bemoaned their inability to fast during Ramadan. Others complained that they could not celebrate Eid with other family members because they had to come home and apply their infusion pump. Some of the older respondents were unable to have prolonged visits to Pakistan because of concerns over their ability to continue with chelation therapy or obtain satisfactory blood transfusions. Visits to parents' country of origin were regarded as an important symbol of their ethnic identity as well as a valued `treat' enjoyed by their ethnic group peers. Exclusion from this was resented. # Blackwell Publishers Ltd/Editorial Board 2000


509

Coping with non-compliance Despite their general ambivalence to chelation therapy, all ± regardless of age ± were clear about the importance of chelation therapy to their wellbeing. Balancing this tension is, as we have seen, a particular problem for young people when they reach their teenage years. Consequently, they were frustrated by the demands chelation therapy makes on them. Most were aware of the dilemma they faced in using the infusion pump to maintain their wellbeing while at the same attempting to limit the impact of its use on their lives. Achieving this balance is part of a dynamic process and explains why their responses to chelation therapy were constantly shifting and at times appeared contradictory. As part of this constant balancing process, the young person's response to thalassaemia is an important mediating factor in how they cope with chelation therapy. This is bound up with their general response to their illness and offers another reminder of the importance of discussing chelation therapy within the context of growing-up with a chronic illness. Young people's coping strategies are vulnerable and there are occasions when they are overwhelmed by the difficulties they face. These difficulties emerge from both the direct consequences of living with a chronic illness as well as the impact of the chronic illness in maintaining a sense of normalcy. The greater the sense of being overwhelmed the more likely they are to reject chelation therapy, as compliance becomes part of the symbolic struggle associated with living with thalassaemia. For most of the time, the vast majority successfully adapted to their illness and coped with the difficulties they faced. The importance of this is increasingly being recognised in literature on childhood chronic illness (Beresford et al. 1996, Prout et al. 1999). None of the respondents was constantly overwhelmed by their illness and few perceived illness as a destroyer on a regular basis (see Herzlich 1973). Most, although occasionally engulfed by the illness, attempted to ensure that thalassaemia did not become the defining feature of their lives. Young people, for instance, try not to dwell on what might have been and several of the older respondents said it was important not to become angry and resentful. As part of this, they tried to maintain a sense of optimism and played down the negative aspects of the illness. This allowed them to maintain their sense of normality and similarity with peers. Religion also emerged as an important coping resource, suggesting another possible ethnic difference in living with a chronic illness (see Currer 1986, Kelleher and Hillier 1996). Religion as a coping resource was more likely to be used by South Asian, irrespective of age and gender, than African-Caribbean respondents. The belief in Allah was seen as a source of strength, enabling them to come to terms with their illness. Chelation therapy was implicated in this. Several young people, for example, remarked that non-compliance was an insult to Allah. Eighteen-year-old Ashiq Javed explained: # Blackwell Publishers Ltd/Editorial Board 2000


The fact is I have to do anything to stay alive. If I don't put the pump on, you know I'm committing suicide. Committing suicide is the highest sin. You have an obligation to keep yourself alive. Several people were able to further sustain this sense of `normality' by separating their condition or treatment from other `normal' aspects of their life. Chelation therapy was often constructed as an activity with well-defined boundaries undertaken at specific times. Life was normal outside these boundaries; our previous work shows how parents often discounted the specific consequences of the condition in assessing the children's health and wellbeing (Atkin and Ahmad 2000). However, this is part of a constant tension because, as we have seen, young people complain that chelation therapy often contributes to their sense of difference, reminding them that they have a disruptive and intrusive chronic illness and do not have the same opportunities as their peers. Attempts to construct normality and reduce the impact of thalassaemia on their life can therefore only ever be partially successful. The severity and technology-dependent nature of the condition provides too many reminders of the dangers and limitations they face. Young people are constantly juggling the difficulties associated with their condition and the possibility of relief from the consequences of their illness. Consequently, there are times when the young person's sense of sadness at having thalassaemia is greater than at other times. This is when they feel at the mercy of the illness, feeling that the world is against them. This is when their sense of difference, anxiety, frustration and powerlessness is especially strong. Despondency, for instance, is often a feature of childhood responses to compliance (Lenny et al. 1994). At times young people can feel so overwhelmed by the difficulties associated with their illness that they do not see the point of complying with treatment (Strunk et al. 1985). This was a feature of the young people's accounts. As part of this, five young people explicitly mentioned death in explaining their previous non-use of the pump. In three cases this reflected their belief that there was little point in complying with treatment because they were going to die young anyway. As 19-year-old Amir Jan said: Ì couldn't see the point, to be honest. I was going to die anyway so why bother'. Two young people saw the prospect of early death as offering release from the suffering and depression the illness caused them. This was not a deliberate attempt to end their life, but more an expression of the helplessness they felt. Nineteen-year-old Robina Begum explained: I just felt a bit, you know, `So what, big deal if I die', I die you know. That's what it was, that's why I stopped wearing my injections. More generally, chelation therapy not only informs a young person's sense of estrangement, representing a symbolic reminder of the difficulties of # Blackwell Publishers Ltd/Editorial Board 2000


511

living with a chronic illness and their sense of `difference', these feelings of estrangement can also directly lead to non-compliance. Negotiating responsibility: The relationship between the young person and their parents As we have seen, young people, as they seek to develop their own identities and establish their `normality', may reject their parents' or professionals' definitions of what is in their interests (see also Hill 1994, Mador and Smith 1989, Frydenberg 1997). Chelation therapy is often a focal point of these disagreements (Ratip et al. 1995) and can become a particular site of strategy and struggle as a child gets older (Ahmad and Atkin 1996). Parents, for example, often say that the most frequent cause of arguments with their teenage offspring concerned the use of the infusion pump (Atkin et al. 1998a). Respondents, in this study, would agree with this. Ashiq Javed, for example, described how his parents constantly reminded him about the importance of chelation therapy. This annoyed him: That really, really, really makes me angry and I can't stand it. I don't want to be treated differently. I mean, at the end of the day they should realise it's up to me what I choose to do. The parent-child relationship becomes fundamental in negotiating responsibility for compliance with chelation therapy and gives specific expression to many of the issues discussed above. Exploring this relationship also helps to unpack further the young person's reasons for non-compliance. As we have seen, those under 12 were largely dependent on parents for chelation therapy. As they got older, parents and professionals progressively encouraged them to take an active part in chelation therapy, such as mixing of the medication. Young people usually enjoyed this involvement as a sign of being `grown up'. Most began to assume greater responsibility for their own care between the ages of 13 and 16 years. Managing treatment in other childhood chronic illnesses, such as severe asthma, follows a similar pattern (Hayford and Ross 1988, Lemanek 1990). These older children said they had looked forward to assuming responsibility for their treatment. This recognition of their growing maturity enhanced their self worth. For many, however, the pump quickly became a chore as well as a symbol of their difference. This, as we have seen, led to a sense of estrangement and consequently, young people soon began not to use the pump as regularly as recommended. Ismat Javed, for instance, remarked how she used her newfound freedom to disregard the use of the pump. Young people hid non-compliance from their parents to avoid conflict (see also Drury 1991, Brannen et al. 1994). This was also apparent in the responses of AfricanCaribbean young people and suggests that ethnicity made little difference in hiding compliance from parents. South Asian young people concealed their non-compliance because they knew how angry their parents would be if they # Blackwell Publishers Ltd/Editorial Board 2000


knew. Parents and children used various strategies in this regard. Ismat Javed, for example, said that her parents used to count her needles to ensure she was using the infusion pump. She systematically threw the needles away to create the impression that she was using the pump. Others described how they disconnected the needle at night, but still left the pump on. When their parents checked on them it appeared that the pump was connected. Gulab Maqbool explained: Sometimes they [I] just, when my parents are asleep or something, take it out quickly, put it on the side and then in the morning attach it. Such a strategy further illustrates the young person's complex relationship to chelation therapy. Disconnecting the pump seemed a futile form of resistance since the discomfort of connecting it had already been experienced, and reconnection would result in double the discomfort. However, as we have seen, the young person's response to chelation therapy is more than a reflection of the practical difficulties associated with using the infusion pump. It has an emotional dimension as well. No respondent, however, was able to disguise this for long: non-compliance became evident during routine blood checks. Young people were right in assuming that conflict with parents would follow. Rashid Mushtaq, for instance, described how his older brother always found out when he had not used the infusion pump regularly because he checked the feritin levels with the hospital constantly. His brother then `goes up the wall', giving him a `hard time'.4 Seventeen-year-old Farzana Azam had also attempted to disguise non-compliance from her parents and described her mother's anger when she found out: My mum, she would say `why you not having it, are you trying to die or what?' you know, `what you trying to do to yourself? Have the injection, otherwise I'm not going to talk to you', and all that. I goes `what's it got to do with you, why you asking me, I could take it if I wanted, don't have to take it if I don't want to'. So we did used to have arguments. More generally, young people resented these arguments believing that parental objections undermined their ability to choose for themselves, although their non-compliance could be seen to provide parents with a legitimate reason for intervention. Many, however, did not necessarily recognise this contradiction. They were annoyed that parents compromised their sense of personal autonomy, found parental responses patronising and felt they were not treated as à grown up'. As we have seen, Rashid Mushtaq had gone through periods of non-compliance. Yet, he still resented his brother's intervention and wished he was treated more like a `grown up'. This annoyance at their parents or guardians is perhaps another reflection of the young persons' complex relationship with chelation therapy. They # Blackwell Publishers Ltd/Editorial Board 2000


513

want recognition of their growing independence by taking responsibility for chelation therapy, while at the same time using this sense of autonomy to make a choice not to use the pump. Negotiating òver-protectiveness' Arguments about chelation therapy reflected another, more general concern of young people: òver-protectiveness', a common theme in the literature on childhood disability (Eiser 1990, Davis and Wasserman 1992). For their part, many parents are aware of this problem but are not sure how to deal with it (Atkin et al. 1998a). Most young people, of all ages and both sexes, felt that their mothers and fathers were over-protective and prevented them pursuing activities they regarded as `normal' among their peers. They provided many examples of the things they were prevented from doing. Several boys, for instance, said that their parents had stopped them playing sports because of their illness. Some mentioned not being able to stay overnight at a friend's house. Even when a young person was given permission to stay overnight, their parents still checked to ensure compliance. Sixteen-year-old Kaneez Mirza was glad her parents had eventually agreed to let her stay overnight with friends, but was embarrassed by their regular telephone calls to check that her infusion pump was working. Ashiq Javed related a similar experience and described his response: That really, really, really makes me angry and I can't stand it. I don't want to be treated differently. In nearly all the families, parental òver-protectiveness' had caused arguments. Young people felt that by over-emphasising chelation therapy, parents only saw the illness and not them. Consequently, many felt that the condition needlessly dominated their life. These concerns emphasised their difference, made it difficult to maintain a valued self-identity and sometimes resulted in conflict with parents. To some extent, this is an inevitable aspect of making sense of a chronic illness within the broader context of `growing-up'. Respondents, as they attempted to develop and sustain a positive selfidentity, described tensions in their relationships with their parents similar to those of young people in general. However, they seemed less desirous of living separately from parents. They also seemed less likely to challenge their parents' religious and cultural values than has been reported for white young people (Brannen et al. 1994). Discussion about independence and the more general process of `growing-up' among different ethnic groups, therefore, is not entirely straightforward. Young Asian people are attempting to express the cultural assumptions in which they have become socialised, while at the same time needing to live within a more `Western' frame of reference. Their peer networks reflect this; none of the respondents had # Blackwell Publishers Ltd/Editorial Board 2000


friends confined only to their own ethnic group. Establishing autonomy and independence seems as important to Asian young people living in the UK as their `white' counterparts, albeit independence may have different connotations among Asian and white young people. The feeling that chelation therapy was imposed rather than negotiated made compliance more difficult to accept, although most attempted gradually to `push back the boundaries' and demonstrate their sense of responsibility to their parents. Parental trust had to be earned. Despite their frustration about òver-protection', they usually understood their parents' concerns and acknowledged that parents had a right to be concerned about non-compliance. This understanding, however, is caught up with the young person's constantly shifting response to their illness noted above. Consequently, there are times when young people are more understanding than others. Our previous work also suggests there are times when parents are more conscious of non-compliance than at other times (Ahmad and Atkin 1996). Parental concerns become particularly heightened when they hear of a death of another child with thalassaemia major or when they are reminded of its importance by professionals or national organisations, such as the UK Thalassaemia Society.5 Following this, 17-year-old Gulab Maqbool said: It's like been with your child for 18 years. We can't understand what they've been through because we haven't been through it ourselves. They do try to look after me but I understand because I know where they are coming from. Nineteen-year-old Ismat Javed, who went through periods of noncompliance which she now regretted, attempted to make sense of her parents' worries: You expect families to be like that. I mean I always tell my mum, `You're being too over-protective' but you know, what can you do? I'd probably do the same. I'm sure a lot of parents of children without thalassaemia are very over protective of their children. But they [her parents] do worry too much. It's impossible. I mean obviously they don't worry as much now as they did before, I mean they still do in some ways but sometimes they just, get a bit scared that something might happen. Recognition of parental concerns did not, however, necessarily mean that it made their parents' approach any easier to deal with or that young people necessarily changed their behaviour. Acknowledgement of their parents' concerns and need to establish their own sense of autonomy added to confusion and made them feel guilty about arguing with their parents, even when they thought they had a just cause: # Blackwell Publishers Ltd/Editorial Board 2000


515

Oh, when they're telling you to do things, you know, oh, you have to wear injections, that got to me sometimes, thinking, `God man, this is me, I have to wear them, will you just leave me alone'. You know, `let me do what I want to do'. But I know, I mean they do have your best interests at heart (19-year-old Robina Begum). Guilt, in fact, becomes an important feature of the young person's illness narrative, finding specific expression in the relationship with their parents. Their conscious non-compliance with chelation therapy and the distress this caused to their parents also fed this guilt. Older respondents mentioned this and emphasised how much their parents had had to come to terms with. Guilt could also be manifest in other ways. Unlike other reports (e.g. Nash 1990), respondents rarely blamed parents for having passed on the condition. On the other hand, parents of thalassaemic children fear being blamed by children because of professionals' assertions that their illness resulted from parental consanguinity (Atkin et al. 1998b, see Ahmad 1995 for a critique of the consanguinity debate). The few who did blame their parents were then in their early teens and struggling to make sense of the illness. Not surprisingly, the need for regular chelation therapy was often the reason for the young people having such feelings. Seventeen-year-old, Farzana Azam, remarked: And I used to think, why me? Because my brothers were all right. They did not need blood transfusions. They did not need to use the pump. They've got no problems. And why is me that is going through medications and injections which I don't like at all. So I think I used to get cross and that I used to blame it on my mum. More generally, however, most children admitted there were times when they took out the frustrations associated with chelation therapy on their parents (see Geiss et al. 1992). Respondents were, at times, àwful' to their parents, being `grumpy' and `moody', and using parents as scapegoats for the problems associated with chelation therapy or the condition. As Jamil Rehman pointed out, `who else is there' to be the target of these frustrations? Regrets over their behaviour did not stop them repeating it. Such frustrations caused guilt and sometimes undermined respondents' coping abilities and acceptance of treatment. Despite the difficulties raised by chelation therapy, young people still valued the support of their parents in using the pump. This offers another reminder of the seemingly contradictory relationship between children and their parents. The tension was particularly evident among those aged between 13 and 16 who, although describing occasional conflicts, still valued the practical, emotional and social support they received from their parents. Though more common among younger children, those over 13 still often relied on parents to help with preparation of chelation therapy. Parents were # Blackwell Publishers Ltd/Editorial Board 2000


also a key resource when the young person became depressed with the use of the pump. Respondents also valued the social and emotional support they received from their parents. Many described how their parents encouraged them to keep their spirits high. Amjad Javed explained: When you're down and you do get emotional and you're crying, mum, my mum will know how to get out of that situation and makes you feel less worse than probably you did when you were on your own. And 13-year-old Rashida Rana said of her mother: `She says to be strong. It'll soon be over.' Understanding the value of chelation therapy The relationship between knowledge and non-compliance emerged as an interesting feature of the young person's behaviour. All, regardless of age, were clear about the importance of chelation therapy to their wellbeing. The value of the pump is strongly emphasised at patient group meetings, including the annual conference of the UK Thalassaemia Society, and by professionals. Deaths through non-compliance act as an unpleasant but strong reminder about the dangers of non-compliance. As noted, resistance to chelation therapy was related to practical, emotional and symbolic aspects of the therapy. Young people, however, still believed information was important in encouraging compliance and to some extent this reflects a belief in medical definitions of their problems. Many of the older respondents, for example, felt they should have been told more about the consequences of non-compliance when they were younger. As we have seen, Ismat Javed, now 19, stopped using the pump during her early teens. This, she said, was partly explained by not understanding the value of the infusion pump: You see, I mean, I've only realised the importance of the pump recently. I didn't know how important desferal was and what it [does], you know. You think you're just going to grow like everyone else. Her brother Ashiq, felt that young people should be made more aware of the consequences of non-compliance rather than simply being told that chelation therapy is important: I think it's really important because you know they don't tell us before, you know. It's too late when your feritin levels are too high. They should tell you these things, because they're sort of things that will make you put the pump on. You know if people say use your pump, no one's going to wear it. It's like somebody told you to do something, you're not going to do it unless there is a reason behind. People don't explain the reasons for using it, that's the problem. # Blackwell Publishers Ltd/Editorial Board 2000


517

Several young people were especially angry at health professionals for not telling them sooner about the problems of high feritin levels. Young people believe that service practitioners do not tailor information to their needs and fail to emphasise the value of compliance in a way the child would find useful. As we have seen, however, practical information is only one part of encouraging young people to use the pump. Service practitioners, for example, are especially concerned with getting over the important of compliance and say it is often a defining feature of their relationship with young people (Atkin et al. 1998a). Young people's response to compliance and, as we have seen, consequences of chelation therapy for the struggle to maintain a valued self-identity, often meant that the advice given by health professionals is accorded little legitimacy. To this extent, health professionals and parents face a similar problem, as they encourage the child to comply with chelation therapy. In some cases, respondents directed their anger at health professionals, blaming them for the need to have regular chelation therapy. And as both parents and professionals emphasised the importance of chelation therapy, professionals were sometimes thought to be colluding with parents. For example, the high regard for haemoglobinopathy workers expressed by parents was not always shared by young people who saw them as parents' rather than their own allies (Atkin et al. 1998a). Thirteen-year-old Rashida Rana used to hate her consultant because of the treatment associated with thalassaemia. She now realises she was wrong to blame him for her problem. Nineteen-year-old Ismat Javed described how she used to dislike the way the specialist haemoglobinopathy nurse forced her to use the infusion pump. She now accepts that the worker's action was well intentioned, and wished other practitioners adopted a similar approach: You know she practically forced us, sit us down and use the pump. You feel like saying `You're not my mum, you can't tell me what to do' but you know it was for the best, you know. The young people's response also illustrates that information in the abstract is of little value in making sense of chelation therapy: something shared by their parents (Atkin et al. 1998a). Those over 16, despite going through long periods of non-compliance when they were younger, were more likely to comply with chelation therapy than those aged 13±16 years. Moreover, those over 16 were not only more likely to comply but had also become passionate advocates of the infusion pump. Several reasons explain this return to compliance. Many, for instance, attributed it to a greater understanding of the problems of non-compliance, often gained from `lived experience'. The consequences of non-compliance are not immediately evident and a child can go several years before being aware of the irreversible damage caused by iron overload. In many cases it was the emergence of problems that made them appreciate the value of # Blackwell Publishers Ltd/Editorial Board 2000


chelation and using the infusion pump again. Sixteen-year-old Rashid Mustaq described how he began using his infusion pump again, after being told that he had an ènlarged' heart. The fear of death encouraged Robina Begum to begin using her pump again, despite her initial non-compliance being associated with a wish to escape the suffering through death. All the older respondents regretted earlier poor compliance with treatment; many were coming to terms with the consequences. Robina Begum described her earlier `stupidity': Yeah and I stopped for a bit but I've started up again now. Thank God. . .I mean, it's important to use the injections. And that's very important, because there are times you will get really depressed and you don't want to do it, you know. Especially when you are younger, you just think òh forget it, I don't want it, I don't want it. I don't want to know, I don't want to hear it'. But it's important that you keep wearing it because if you don't it's, you're just going to get more illner and illner [sic] and eventually, you know, you can just die. In general, these young people hoped that others would not repeat their mistakes. Sixteen-year-old Gulab Maqbool's advice to others was: `Don't be like me'. And Ismat Javed would advise them: . . .if I met somebody who was nine, 10, 11, I'd say there is something you can do about it now, you know. Make sure, you know, look after yourself and do your treatment. It is impossible to turn the clocks back, isn't it? Nonetheless, the response of these young people does not simply reflect their direct experience of the consequences of non-compliance. These older respondents also seemed better equipped to come to terms with living with a chronic illness. The sense of struggle associated with chelation therapy tended to reduce with age for both genders. They began to recognise the importance of working with the illness rather than against it. This is not to say that all older respondents had learnt to overcome the difficulties associated with thalassaemia. Sadness and frustration still occurred and became implicated in the compliance process throughout the person's life. Nonetheless, those over 16 seemed better adjusted to the difficulties associated with their illness; a range of coping strategies enabled them to cope with the illness. Compliance benefited from this and usually became more regular, although it rarely became absolute. As part of this process, young people as they became older, were also aware of the other priorities in their life as they faced new-found responsibilities. This might mean becoming more aware of their obligations to other family members, especially their parents. They might also be contemplating # Blackwell Publishers Ltd/Editorial Board 2000


519

marriage, starting a family, getting a job or pursuing further or higher education. The commitment required for these social activities seemed to make the young person more aware of the ìrresponsibility' of non-compliance. Such feelings informed the dynamic tension outlined above, in which the young person attempted to maintain a positive identity while at the same time limiting the impact of the illness on his or her life. Conclusion Exploring young people's response to compliance with chelation therapy demonstrates the complexity of their illness narrative and provides an important insight into how they live with a chronic illness. Young people's response to treatment is informed by various social and emotional concerns and is part of the broader process in which they attempt to make sense of the illness within the context of personal circumstances, identities, lifestyles and coping strategies. To this extent they share similarities with adults with a chronic illness, although the specific demands of `growing up' give the experience of young people a particular focus. This broader context becomes important in making sense of their response to chelation therapy and assumes a more general analytical significance since the voice of young people is rarely heard in discussion about chronic illness (Lewis 1995). Given this, young people's response to chelation therapy is both complex and dynamic. It represents a specific disruption to their biographies and is part of the wider process in which they make sense of the relationship between body, self and illness, and maintain relationships. Consequently the everyday experience of living with a chronic illness, the pleasures and tensions of growing-up, family relationships and social networks are all implicated in the compliance process. Thus the rejection of compliance therapy was rarely associated with practical difficulties alone. It more closely related to concerns around a valued self-identity, peer relations and being `normal'. Their response to chelation therapy has similarities to young people's response to perceived threats to their identity, more generally (Frydenberg 1997). Chelation therapy, for most at some time, signified this threat by marking their difference and compromising their desire for `normalcy'. And although many often constructed a notion of `normalcy' which discounted transfusions or chelation therapy, the frequency and visibility of these treatments made such constructions difficult to sustain. As noted, this was a particular issue for those aged 13±16 years. Young people constantly attempted to balance the value of regular therapy to their well-being, while coming to terms with the emotional difficulties caused by its use. Young people with other chronic illnesses seem to face similar dilemmas (Johnson 1988). This balancing process is dynamic and explains why the response to chelation therapy is constantly shifting, and varying between individuals and across time. This balance is further influ# Blackwell Publishers Ltd/Editorial Board 2000


enced by the young person's coping strategies: an increasingly important aspect of understanding the experience of those with a chronic illness (Beresford et al. 1996). Most coping strategies remain vulnerable and there are occasions when young people are overwhelmed by the difficulties they face. Not surprisingly, this is reflected in their response to chelation therapy. The greater the sense of being overwhelmed, the more likely is the rejection of chelation therapy. A positive identity and strong social relationships enhance the sufferer's coping abilities and improve the chance of complying with treatment, as also noted by others. South Asian young people's response to their treatment would seem no different from that of young people with other chronic illnesses, as diverse as severe asthma (Lemanek 1990), cystic fibrosis (Geiss et al. 1992), sickle cell disorders (Atkin and Ahmad 1996), rheumatoid arthritis (Anderson et al. 1985) and diabetes (Johnson 1988). This similarity would also suggest that èthnicity' or cultural difference did not greatly inform non-compliance. Discussions about independence and the more general process of `growingup' among different ethnic groups, therefore, are not entirely straightforward. Young Asian people were attempting to express the cultural assumptions in which they have become socialised while at the same time needing to live within a `Western' frame of reference. Establishing autonomy and independence would seem as important to Asian young people living in the UK as to their `white' counterparts (see Brannen et al. 1994 for a comparison), although, as noted, their notions of àutonomy' may differ. Gender was not strongly implicated in the compliance process despite reports that girls may cope with the emotional demands of medical regimens better than boys (see Frydenberg 1997). In this study, girls were as likely as boys not to comply and their coping strategies were as vulnerable. Nonetheless, some differences were evident in how respondents made sense of chelation therapy and this, in turn, may have influenced their response to compliance. Gender, for instance, influenced how respondents articulated their sense of autonomy and independence. Boys, for instance, were concerned about potential dependence on their parents as they grew older. They were also worried about the implications of chelation therapy for their masculinity. Girls, on the other hand, were more concerned about the limitations imposed by therapy and other treatment on maintaining social relationships and fulfilling obligations. Persuading users to follow treatment or advice remains an issue within health services. In relationship to thalassaemia, following treatment can make the difference between life and death. As this paper demonstrates, to understand young people's reasons for non-use of life-saving chelation therapy, one needs to move beyond a concern with practical difficulties. Instead the symbolic meaning of chelation therapy as a threat to their `normalcy', and the importance of lived experience, are vital to recognise if more effective partnerships are to be built between young sufferers, their families and health professionals. # Blackwell Publishers Ltd/Editorial Board 2000


521

Address for correspondence: Karl Atkin, Centre for Research in Primary Care, Nuffield Institute for Health, 71±75 Clarendon Road, University of Leeds, Leeds LS2 9PL e-mail: [email protected] Acknowledgements Our thanks to the National Lottery Charities Board for financial support; our Professional Advisory Committee and Young People's Advisory Group for valuable guidance; Kanwal Mand for assistance with interviews; and three anonymous referees for helpful comments on earlier drafts of this paper. This research is taken from a joint project with Al-Falah, a voluntary organisation based in Bradford offering welfare advice and support.

Notes 1. Providing this description is not intended to support a medical model of thalassaemia. There is a long established critique of the medical model (see Oliver 1996) and we broadly endorse this. The need for chelation therapy, however, does complicate the debate and despite the potentially oppressive nature of `medical' accounts, those with thalassaemia major are dependent on medical interventions for survival. 2. Recent work on childhood development suggests àdolescence' is a derogatory term, connoting becoming rather than being (see Frydenberg 1997). While not wishing to marginalise young people's narratives, evidence does suggest that they face new responsibilities and challenges as they acquire a growing sense of autonomy and independence. For those with a chronic illness, compliance is caught up in this being. 3. Fortunately this avoided an awkward situation. We discussed how we would manage a situation when a young person had agreed to be interviewed and their parents then objected. We decided to judge each case on its individual merits. Advice from local medical ethical committees provided little guidance and our discussions reflected considerable ambiguities in both the legal and moral status of young people (see Alderson and Siddle 1994 and Greig and Taylor 1998). 4. Rashid Mustaq's brother was his legal guardian. Their parents lived in Pakistan. 5. The UK Thalassaemia Society's annual conference and quarterly newsletter constantly remind people about the importance of chelation therapy. Most parents have some contact with the UK Thalassaemia Society (Atkin et al. 1998a). During the five years of our work in this area, two young people with thalassaemia died in West Yorkshire as a consequence of iron overload.

References Ahmad, W.I.U. (1995) Reflection on consanguinity and birth outcome debate, Journal of Public Health Medicine, 16, 4, 423±8. # Blackwell Publishers Ltd/Editorial Board 2000

522 Karl Atkin and Waqar I.U. Ahmad Ahmad, W.I.U. (1996) Family obligation and social change among Asian communities. In Ahmad, W.I.U. and Atkin, K. (eds) `Race' and Community Care. Buckingham: Open University Press. Ahmad, W.I.U. and Atkin, K. (1996) Ethnicity and caring for a disabled child: the case of children with sickle cell or thalassaemia, British Journal of Social Work, 26, 755±75. Alderson, P. and Siddle, A. (1994) Guidelines for ethical conduct of medical research, Archives of Disease in Childhood, 71, 3, 282. Alderson, P., Brill, S., Chalmers, I., Fuller, R., Hinkley-Smith, P., MacDonald, G., Newman, T., Oakley, A., Roberts, H. and Ward, H. (1996) What Works? Effective Social Interventions in Child Welfare. Barkingside: Barnardo's. Anderson, O., Bradley, L.A., Young, L.D. and McDaniel, L.K. (1985) Rheumatoid arthritis: review of psychological factors related to etiology, effects and treatment, Review of Psychology, 98, 358±87. Atkin, K. Ahmad, W.I.U. and Anionwu, E. (1998a) Service support to families caring for a child with a sickle cell disorder or thalassaemia, Health, 2, 3, 305±27. Atkin, K., Ahmad, W.I.U. and Anionwu, E. (1998b) Screening and counselling for sickle cell disorders and thalassaemia: the experience of parents and health professionals, Social Science and Medicine, 47, 11, 1639±51. Atkin, K. and Ahmad, W.I.U. (2000) Coping with childhood illness: experiences of parents whose children have thalassaemia major or sickle cell disorder, Health and Social Care in the Community, 8, 1, 57±69. Beresford, B., Sloper, P., Baldwin, S. and Newman, T. (1996) What Works in Services for Families with a Disabled Child. Essex: Barnardo's. Brannen, J., Dodd, K., Oakley, A. and Storey, P. (1994) Young People, Health and Family Life. Buckingham: Open University Press. Bury, M. (1991) The sociology of chronic illness, Sociology of Health and Illness, 13, 4, 451±68. Corsaro, W.A. (1997) The Sociology of Childhood. Pine Forge: London. Crisp, J., Ungerer, J.A. and Goodnow, J.J. (1996) The impact of experience on children's understanding of illness, Journal of Paediatric Psychology, 2, 1, 57±72. Currer, C. (1986) Concepts of mental well- and ill-being: the case of Pathan mothers in Britain. In Currer, C. and Stacey, M. (eds) Concepts of Health, Illness and Disease. Leamington Spa: Berg. Darr, A. (1990) The Social Implications of Thalassaemia among Muslims of Pakistani Origin in England: Family Experience and Service Delivery. Unpublished PhD thesis, University of London. Davis, J.K. and Wasserman, E. (1992) Behavioural aspects of asthma in children, Clinical Paediatrics, November, 678±81. Dornbusch, S.M., Patersen, A.C. and Hetherington, E.M. (1991) Projecting the future of research on adolescence, Journal of Research on Adolescence, 1, 7±17. Drury, B. (1991) Sikh girls and the maintenance of an ethnic identity, New Community, 17, 3, 387±99. Ebata, A. and Moss, T. (1991) Coping and adjustment in distressed and healthy adolescents, Journal of Applied Developmental Psychology, 12, 33±54. Eiser, C. (1990) Chronic Childhood Disease: an Introduction to Psychological Theory and Research. Cambridge: Cambridge University Press. Finch, J. and Mason, J. (1993) Negotiating Family Responsibilities. London: Routledge. # Blackwell Publishers Ltd/Editorial Board 2000


523

Frydenberg, E. (1997) Adolescent Coping: Theoretical and Research Perspectives. London: Routledge. Geiss, S.K., Hobbs, S.A., Hammersley-Maercklein, G., Kramer, J.C. and Henley, M. (1992) Psychosocial factors related to perceived compliance with cystic fibrosis treatment, Journal of Clinical Psychology, 48, 1, 99±103. Grieg, A.D. and Taylor, J. (1998) Doing Research with Children. London: Sage. Gubrium, J.F. and Silverman, D. (1989) The Politics of Field Research: Sociology Beyond Enlightenment. London: Sage. Hayford, J.R. and Ross, C.K. (1988) Medical compliance with juvenile rheumatoid arthritis, Arthritis Care and Research, 1, 190±7. Herzlich, C. (1973) Health and Illness: a Social Psychological Analysis. London: Academic Press. Hill, S.A. (1994) Managing Sickle Cell Disease in Low-income Families. Philadelphia: Temple University Press. Hoare, P. and Mann, H. (1994) Self-esteem and behavioural adjustment in children with epilepsy and children with diabetes, Journal of Psychosomatic Research, 38, 8, 859±69. James, A. (1996) Learning to be friends: methodological lessons from participant observation among English school children, Childhood, 3, 3, 313±30. Johnson, S.B. (1988) Psychological aspects of childhood diabetes, Journal of Child Psychology and Psychiatry, 29, 729±38. Kelleher, D. and Hillier, S. (1996) Researching Cultural Differences in Health. London: Routledge. Lemanek, K.L. (1990) Adherence issues in the medical management of asthma, Journal of Paediatric Psychology, 15, 437±58. Lenney, W., Wells, N.E.J. and O'Neill, B.A. (1994) Burden of paediatric asthma, European Respiratory Review, 4, 18, 49±62. Lewis, A. (1995) Children's Understanding of Disability. London: Routledge. Locker, D. (1997) Living with a chronic illness. In Scambler, G. (ed) Sociology as Applied to Medicine. London: W.B.Saunders. Mador, J.A. and Smith, D.H. (1989) The psychological adaptation of adolescents with cystic-fibrosis: the review of the literature, Journal of Adolescent Health Care, 10, 2, 136±42. Marteau, T.M. and Johnston, M. (1986) Determinants of beliefs about illness: a study of parents of children with diabetes, asthma, epilepsy and no asthma, Journal of Psychosomatic Research, 30, 673±83. Midence, K. (1994) The effects of chronic illness on children, Genetic Social and General Psychology Monographs, 120, 3, 311±26. Midence, K., McManus, C., Fuggle, P. and Davies, S. (1996) Psychological adjustment and family functioning in a group of British children with sickle cell disease: preliminary empirical findings and a meta-analysis, British Journal of Clinical Psychology, 35, 439±50. Modell, B. and Anionwu, E. (1996) Guidelines for screening for haemoglobin disorders: service specification for low and high-prevalence district health authorities. In Ahmad, W.I.U., Sheldon, T. and Stuart, O. (eds) Reviews of Ethnicity and Health. York: NHS Centre for Reviews and Dissemination. Nash, K.B. (1990) Growing up with thalassaemia: a chronic disorder, Annals of the New York Academy of Sciences, 612, 442±50. Oliver, M. (1996) Understanding Disability: from Theory to Practice. Basingstoke: Macmillan. # Blackwell Publishers Ltd/Editorial Board 2000

524 Karl Atkin and Waqar I.U. Ahmad Prout, A., Hayes, L. and Gelder, L. (1999) Medication and the maintenance of ordinariness in household management of childhood asthma, Sociology of Health and Illness, 21, 2, 137±62. Ratip, S., Skuse, D., Porter, J., Wonke, B., Yardumian, A. and Modell, B. (1995) Psychosocial and clinical burden of thalassemia-intermedia and its implications for pre-natal diagnosis, Archives of Diseases in Childhood, 72, 5, 408±12. Sinnema, G. (1992) Youths with chronic illness and disability on their way to social and economic participation: a health care perspective, Journal of Adolescent Health, 13, 369±71. Stark, L.J., Dahlquist, L.M. and Collins, F.L. (1987) Improving children's compliance with diabetes management, Clinical Psychology Review, 7, 223±42. Strunk, R.C., Mrazek, D.A., Fuhrmann, G.S.W. and LaBrecque, J.F. (1985) Physiologic and psychological characteristics associated with deaths due to asthma in childhood: a case-controlled study, Journal of the American Medical Association, 254, 1193±8. Thompson, R.J. (1994) Stability and change in psychological adjustments of mothers of children with cystic fibrosis and sickle cell disease, Journal of Paediatric Psychology, 19, 2, 171±88. Tsiantis, J., Dragonas, T., Richardson, C., Anastasopoulous, D., Masera, G. and Spinetta, J. (1996) Psychosocial problems and adjustment of children with betathalassaemia and their families, European Child and Adolescent Psychiatry, 5, 193±203. Varni, J.W. and Wallander, J.L. (1984) Adherence to health-related regimens in paediatric chronic disorders, Clinical Psychology Review, 4, 585±96. Zani, B., Dipalma, A. and Vulli, C. (1995) Psychosocial aspects of chronic illness in adolescents with thalassaemia major, Journal of Adolelescence, 18, 4, 387±402.

# Blackwell Publishers Ltd/Editorial Board 2000