TR-05245; No of Pages 4 Thrombosis Research xxx (2014) xxx–xxx

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Review Article

Relationship between haemophilia and social status Katharina Holstein ⁎, Barbara Eifrig, Florian Langer II. Medizinische Klinik und Poliklinik, Gerinnungsambulanz und Hämophiliezentrum, Universitätsklinikum Hamburg-Eppendorf, Hamburg, Germany

a r t i c l e Available online xxxx Keywords: Haemophilia Social status Quality of life Adolescence Childhood Elderly

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a b s t r a c t The impact of haemophilia and its treatment on social status has not been well studied, although research into the quality of life of patients with haemophilia has shed some light on aspects of social and role functioning. Studies conducted before the advent of safe and effective coagulation factor replacement therapy suggest that the haemophilia population was predominantly of low socioeconomic status with many social disadvantages, including high rates of disability and unemployment and low rates of marriage. Since the availability of purified factor VIII concentrates that could be used in a home-care setting and as prophylaxis, most research suggests that social status and well-being amongst children, adolescents, and adults with haemophilia is not compromised, and is comparable to that of the general population. Children and adolescents with haemophilia do not generally feel disadvantaged, although haemophilia-related issues at school and amongst peer groups do arise. Recent studies in adults show higher than average rates of marriage and cohabitation and the attainment of a generally good educational status, but, as in the past, employment rates remain comparatively lower. Social status amongst the elderly with haemophilia who may have developed severe disability as a result of their condition is poorly defined and has never been formally studied. Additional research is recommended. © 2013 Published by Elsevier Ltd.

Contents Introduction . . . . . . . . . . . . . . . . . . . Impact of haemophilia on childhood social status . . Impact of haemophilia on social status in adolescents Impact of haemophilia on social status in adulthood . Impact of haemophilia on social status in the elderly . Conclusions . . . . . . . . . . . . . . . . . . . Conflict of interest statement . . . . . . . . . . . Acknowledgements . . . . . . . . . . . . . . . . References . . . . . . . . . . . . . . . . . . . .

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Introduction The haemophilias are a group of life-long disorders associated with a chronic burden of morbidity that is worsened by episodes of acute bleeds and their consequences [1]. Before modern substitution treatment was introduced at the end of the 1960s, haemophilia often resulted in severe disability at a young age and premature death. The possibility of someone with haemophilia enjoying a full social life

Abbreviations: AIDS, acquired immunodeficiency syndrome; HIV, human immunodeficiency virus; SF-36, Short Form 36. ⁎ Corresponding author. Tel.: +49 40 7410 5 2453; fax: +49 40 7410 55193. E-mail address: [email protected] (K. Holstein).

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was non-existent or limited [2]. The availability of clotting factor concentrates and the introduction of prophylaxis and home treatment greatly improved quality of life for people with haemophilia and their families [2], and now, many individuals with haemophilia, especially younger patients, do not consider their condition to be a major burden [3]. The management of haemophilia needs to consider not only the patient’s medical requirements but also their emotional and social needs, including those relating to social status – an aspect that is often overlooked in haemophilia studies. In its simplest form, “social status” refers to the position an individual occupies in the social structure, such as being a teacher or office worker. Social status is often considered in combination with “social role”, which refers to the role an individual plays within their social group and in society as a whole.

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Please cite this article as: Holstein K, et al, Relationship between haemophilia and social status, Thromb Res (2014), http://dx.doi.org/10.1016/ j.thromres.2013.10.012

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Several factors help determine an individual’s social status. In adults, income, education, and occupation have traditionally been considered core components of social status, with studies focusing primarily on objective measures, including social class identification [4]. In children and adolescents, social status is determined more by subjective perceptions of the individual’s placement within the social hierarchy in terms of peer respect, academic or sporting success, and social “standing” [4]. In the elderly, social status is more likely to be determined by an individual’s mobility, residential situation, and social support network; however, this has not been well defined in the literature. Several different approaches to evaluating “social status” in haemophilia have been taken over the years. Quality-of-life studies, which utilize both generic and disease-specific questionnaires, have assessed certain components of social and role functioning, but do not specifically evaluate social status per se [5]. The Short Form 36 (SF-36), for example, which is one of the most commonly used generic instruments for assessing quality of life, quantifies physical and social functioning, and the ability of individuals to fulfil their physical and emotional “roles” [6]. Studies assessing social status in adults have evaluated marital, educational, and occupational status [3]; those in children and adolescents have focused on academic achievement [7] and perceived placement within the school community [4]. Impact of haemophilia on childhood social status School attendance and academic performance can be adversely affected by haemophilia and its treatment [7], although studies in adults suggest that most individuals with haemophilia ultimately attain a normal, if not elevated, educational status [3]. Children with haemophilia under prophylactic therapy can benefit significantly from participation in physical and sporting activities, both in terms of their health and quality of life [8–11]. Prior to the introduction of prophylactic factor replacement therapy, children were discouraged from physical activity due to the risk of bleeds, resulting in some children having lower levels of fitness and strength than their peers [12]. Although, today, additional protection may be required when engaging in sporting activities, the benefits to children far outweigh the risks, including normalizing strength and fitness [11], enhancing self-esteem, increasing social contact, and encouraging a more “normal” way of life [10]. Interest in quality-of-life research in children with haemophilia is growing [13]. One of the most comprehensive assessments in children evaluated academic performance and certain aspects of quality of life, and found that the number of bleeding episodes was positively correlated with school absenteeism, which, in turn, correlated with lower achievement scores in mathematics, reading, and total achievement [7]. More recently, Gringeri et al. evaluated the health status and health-related quality of life of 318 children, aged 4–16 years, from six European countries and found that, although quality of life was broadly satisfactory, the younger children (aged 4–7 years) were negatively affected in the areas of “family” and “treatment”, whereas older children had impairments in the social areas of “perceived support” and “friends” [14]. Impact of haemophilia on social status in adolescents Adolescence is a critical time in a child’s life in which he or she transitions between the social status of childhood, determined primarily by family status, and adult social status, which is largely self-determined [4]. Chronic illness during adolescence can have a profound effect on an individual’s long-term health and well-being. Several studies are suggesting that chronic conditions and the demands of treatment regimens during adolescence can adversely affect growth and development, identity, mental health, relationships with peers, and engagement with education and employment [15]. In a study from Iran, 49% of 100 haemophilia patients aged 16–67 (mean

age 28±9) reported that haemophilia had a negative impact on education [16]. Severity of disease and number of affected joints led to a negative attitude towards continuing education. Despite this period of heightened social changes, the effects of haemophilia on social status in adolescents remains poorly studied. Most teenagers with haemophilia participating in a Scandinavian study conducted between 2003 and 2004 reported that they had the same opportunities as their peers in terms of engaging in normal teenage activities (such as playing sport, having a girlfriend, having sex, drinking alcohol, and partying); just over half (52%) had contact with other haemophilia patients, and most (94%) had told their friends about their condition, even though this was considered to be one of the most difficult aspects of having haemophilia, along with the demands of treatment and restrictions in physical activities [17]. Adolescence is also a period fraught with sexuality issues, and having haemophilia can intensify most of the normally encountered concerns [18]. Dating and sexual intimacy can be daunting prospects for teenagers with haemophilia, and issues associated with sexual desirability, sexual performance, and transmission of sexual diseases and genetics are common [18]. Many young men struggle with how to disclose their diagnosis to a potential partner, and they often need assistance with handling disclosure and with confronting their own doubts about becoming intimately involved with another person [18]. The choice of education and careers, which are considered key components of social status, becomes especially important during adolescence and early adulthood because of the need to avoid or minimize the possibility of bleeding. Individuals with haemophilia are generally advised to avoid careers that require strenuous physical labour or work in industries where injuries can occur. Bleeding episodes and related absences may put employment at risk if employers favour a “healthy” employee over someone with a “disability” or with a higher possibility of frequent absences from work. Thus, it is unsurprising, that some adolescents with haemophilia believe they have fewer work and career opportunities than others [17]. Young adults with haemophilia who acquired human immunodeficiency virus (HIV) have additional psychosocial and status challenges to contend with, often confronting social stigma and having to cope with increased financial burden, disruption of normal activities, and poor social relations [19,20]. The combination of haemophilia and HIV can be especially difficult in adolescence, with parents reporting high levels of pessimism about their children’s futures, low levels of family integration, limited family opportunities [19], and intimacy concerns. Such individual vulnerabilities may be further compounded by the fact that adolescence is often the time patients transition from paediatric to adult haemophilia care services; a process that is known to increase the risk of deteriorating health and quality of life in other chronic conditions [21]. Thus, a close cooperation between child and adult comprehensive care centres is paramount. Impact of haemophilia on social status in adulthood Adults with haemophilia contend with a range of complex psychological and social issues as a result of their condition. Despite the availability of factor replacement therapy, most adults still experience occasional bleeds, and those who did not have access to effective treatment early in life may still suffer disability in adulthood as a result of joint dysfunction and chronic pain [3,16,22,23]. There are a significant number of adults with haemophilia living with comorbid hepatitis and/or HIV/acquired immunodeficiency syndrome (AIDS), and these individuals have a higher burden of morbidity than other haemophilia patients in terms of impaired mobility, poor ambulation, and pain [1]. Studies suggest that individuals with both haemophilia and HIV/AIDS experience significant interference with work, education, and community responsibilities, and frequently

Please cite this article as: Holstein K, et al, Relationship between haemophilia and social status, Thromb Res (2014), http://dx.doi.org/10.1016/ j.thromres.2013.10.012

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encounter negative interactions, prejudice, and avoidance by friends and health-care professionals [20]. One of the few studies that specifically evaluated the impact of haemophilia on social status was undertaken in Austria and involved 53 adult patients (aged 19–62 years) with severe or moderately severe haemophilia [3]. In that study, marital status, offspring status, and educational and occupational status were all evaluated as surrogates of social status. Compared with age- and sex-matched controls, a significantly higher number of haemophilia patients were married (57% vs 38%; p = 0.023); more patients than controls lived with a female partner or with a female partner and children (68% vs 57%; not significant) and patients had significantly fewer children than controls (average 1.5 vs 2.1 children; p = 0.007). Interestingly, despite similar educational backgrounds, more patients than controls were unemployed (34% vs 9%; p b 0.001) and more were retired (23% vs 4%; p b 0.001). The quality-of-life instrument used in this study (SF-36) also identified significant reductions in social functioning in the haemophilia group compared with controls. The fact that more patients than controls were married and living with their partners may suggest that individuals with haemophilia seek protection or stability in a family environment. This contrasts with findings from a study published 18 years earlier in which very low rates of marriage were observed amongst a Dutch haemophilia population [24]. At this point in time, when haemophilia treatment was less advanced, almost half of the study participants considered their disease an impediment to finding a partner, and almost a third reported that haemophilia had a negative effect on a current relationship. Similarly, a lower rate of marriage was observed in a group of Iranian haemophilia patients compared with the general population (40% vs. 54%) [16]. In this group, 73% wanted to have a child. This willingness was dependent on income and joint involvement [16]. The finding that, despite achieving a good educational status, unemployment rates amongst the haemophilia community are relatively high is disappointing, especially since having a job is generally considered an important correlate of successful functioning in society. Several other studies have reported a similar pattern of unemployment and workplace disability amongst adults with haemophilia, especially among those with a severe condition and consequent joint damage [25,26]. Studies in adults have shown that treatment modalities can have an impact on social outcome, for example prophylactic treatment improves quality of life across a range of dimensions [27], reduces hospitalization rates, results in fewer joint bleeds, and reduces the time spent off school or work [14]. Preliminary evidence also suggests that prophylaxis can improve work productivity [28] and reduce the need for early retirement [29]. A study comparing the Swedish high-dose with the Dutch intermediate-dose prophylactic regimen showed no significant differences in educational achievement, employment rates, or social participation; however, joint status was significantly better in the Swedish group [30]. Compared with the general population, fewer haemophilia patients from both countries achieved a university degree, while unemployment rates were similar (Netherlands) or lower (Sweden). The authors concluded that a more intensive and higherdosed regimen might provide slightly improved outcome at a significant cost increase. As these studies attest, the outlook for adults with haemophilia has changed beyond recognition in the past 20 years because of the availability of effective treatment. Many never expected to live as long as they have, and have had to adjust to the idea of living into old age and having a future [22]. Nevertheless, when interviewed, most adults report very positive perceptions of their quality of life, with many living life to the full, seizing opportunities, being fully involved in social activities, and enjoying success at work [22]. However, these individuals will be faced with comorbidities that are comparable to the normal elderly population (see Zimmermann and Huth-Kühne this supplement).

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Impact of haemophilia on social status in the elderly Individuals with haemophilia and other congenital bleeding disorders now have a near-normal life expectancy and, thanks to the availability of safe and effective treatments, will reach an old age without the often devastating (crippling) joint and muscle damage observed in previous generations [31-33]. In some countries, almost 10% of haemophilia patients are now over the age of 65 years; a trend that seems likely to continue into the future [34]. Despite effective treatments, however, haemophilia continues to impose an additional burden over normal ageing, since joint damage secondary to haemophilia affects the majority of today’s elderly survivors – some very severely [35]. Arthropathy in elderly individuals with haemophilia can markedly limit range of motion, putting the individual at risk of falls, and restricting their ability to undertake normal everyday activities [8,32,33,35]. A tendency towards early retirement as a result of disability was reported in Hartl et al.’s Austrian study [3]. Quality-of-life studies demonstrate that advancing age is associated with a reduction in the quality of life of haemophilia patients [36,37], and that elderly individuals with haemophilia have a generally poorer quality of life than age-matched controls [35]. The impact of haemophilia on social status in the elderly is difficult to determine, since social status in the twilight years is hard to define. Siboni et al. reported that elderly individuals with severe haemophilia suffer a loss of autonomy, with problems relating to transport and shopping being especially pronounced [35]. In that study, physical activity levels were notably reduced compared with non-haemophilia age-matched controls, leading to individuals staying at home for longer periods of time. Problems with mobility, depression, and moderate pain and/or discomfort were also encountered more frequently in elderly patients than elderly controls. Conclusions There seems little doubt that, theoretically at least, haemophilia could have a significant impact on social status. The condition has in the past been associated with isolation and social stigma, and the often intensive treatment regimens required might be expected to impact on daily routines, social interactions, and occupational success. There is certainly evidence from studies conducted before the introduction of safe and effective factor replacement therapy that haemophilia affected social status in terms of employment and marital status, but more recent evidence points to far better outcomes. In the studies reviewed here, it would seem that today’s children and adolescents with haemophilia are coping well with their condition and its treatment and do not let it affect their relationships with their peers or their enjoyment of life. Many adults with haemophilia secure good jobs, marry, and have children and, in doing so, achieve a good social status in classical terms. It is worrying, however, that career and employment opportunities do not appear to have improved greatly for the haemophilia community as a whole, despite effective treatment, and the impact of this on social status and well-being deserves fuller attention. The impact of haemophilia on social status in the elderly has not been adequately quantified, and could perhaps be a subject for future study. Conflict of interest statement The authors take part in a research project financially supported by CSL Behring. Acknowledgements The support of Dr. Mathias Jürs from CSL Behring and editorial assistance of Swiss Medical Press is highly appreciated.

Please cite this article as: Holstein K, et al, Relationship between haemophilia and social status, Thromb Res (2014), http://dx.doi.org/10.1016/ j.thromres.2013.10.012

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Please cite this article as: Holstein K, et al, Relationship between haemophilia and social status, Thromb Res (2014), http://dx.doi.org/10.1016/ j.thromres.2013.10.012

Relationship between haemophilia and social status.

The impact of haemophilia and its treatment on social status has not been well studied, although research into the quality of life of patients with ha...
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