P.F. Limperg

Paediatric health-related quality of life: what is it and why should we measure it?

As a paediatrician, you follow a number of children with chronic health conditions in your practice. You provide them with a variety of therapies and would like to know whether your treatments are having an impact, in particular whether there has been a change in the patients health-related quality of life (HRQOL). HRQOL measures have the potential to augment the information that clinicians have available, to enhance their clinical decisions and assess the impact of a chronic health condition on a childs life. How should you try to capture this information? ### What is health-related quality of life? The WHO defines quality of life (QOL) as ‘a childs perception of their position in life in the context of the culture and value systems in which they live and in relation to their goals, expectations, standards and concerns’1 and HRQOL) as ‘a childs goals, expectations, standards or concerns about their overall health and health-related domains’.1 ,2 That being said, many other definitions of HRQOL have been proposed over the years, and a variety of terms are currently used.3 ,4 Although the term QOL is sometimes used interchangeably with HRQOL, QOL is actually a broader construct that encompasses aspects of life that may not be amenable to healthcare services.5 Thus, spirituality and financial resources are, for example, often included in QOL, but are not necessarily included as part of HRQOL. In this paper, we regard QOL in children as a multidimensional subjective concept that includes social, emotional, cognitive and physical functioning as well as cultural aspects of the child and family, while HRQOL incorporates measures of physical symptoms, functional status and disease impact on psychological and social functioning.6 ,7 Children growing up with chronic health conditions (or suffered a severe acute illness and experience late effects) are at greater risk for …

Themes in daily life of adolescents and young adults with congenital bleeding disorders: a qualitative study.

P. LIMPERG,* M. PETERS,† E. GIBBONS,‡ M. COPPENS,§ C. VALK,§ M. GROOTENHUIS* and L. HAVERMAN* *Psychosocial Department, Emma Children’s Hospital, AMC, Amsterdam, the Netherlands; †Department of PediatricHematology, Emma Children’s Hospital and Hemophilia Comprehensive Care Treatment Center, AMC, Amsterdam, the Netherlands; ‡Health Services Research Unit, Nuffield Department of Population Health, University of Oxford, Oxford, England; and §Department of Vascular Medicine, Hemophilia Comprehensive Care Treatment Center, AMC, Amsterdam, the Netherlands

Health-related quality of life questionnaires in individuals with haemophilia: a systematic review of their measurement properties

The evaluation of health related quality of life (HRQOL) is essential for a full assessment of the influence of an illness on patients’ lives. The aim of this paper is to critically appraise and compare the measurement properties of HRQOL questionnaires studied in haemophilia.

Psychosocial functioning of mothers of boys with haemophilia

Haemophilia is an X-linked bleeding disorder, caused by an inherited deficiency of clotting factor VIII (haemophilia A) or factor IX (haemophilia B), and leads to spontaneous and posttraumatic bleeds [1]. In the Netherlands, boys with severe haemophilia receive adequate prophylactic treatment to reduce the risk of irreversible joint damage and to increase quality of life. However, boys with haemophilia (BWH) still endure difficulties and impairments, such as hospital visits, frequent injections and limited participation in (sport) activities [2]. BWH remain at risk for (spontaneous) bleeds. Parents must continuously take into consideration the risks and safety, for boys with severe as well as non-severe haemophilia. In the western world, much of the haemophilia care is thus home based. Parents play a significant role in the care and treatment of BWH. Despite the advantages of care at home (e.g. less hospital visits, more independence), parents report to experience the large responsibility for management of haemophilia, such as administration of treatment by intravenous infusion of clotting factor concentrate, burdensome [3]. In addition, parents of BWH may experience similar problems as parents of children with other chronic illnesses. Research has shown that parents of chronically ill children frequently report anxiety, depression, physical problems and cognitive problems and are more likely to experience greater levels of parental distress and lower health related quality of life (HRQOL) than parents of healthy children [4]. Also, previous research showed that parental psychosocial problems influence the well-being of the child. For example, maternal depression negatively influences children’s adherence to therapy [5]. Up to date, the psychosocial impact of haemophilia on parents has only been addressed in a handful of studies, and results are quite ambiguous. Wiedebusch et al. [6] compared parents of BWH to other paediatric illness groups, and found that parents of BWH experience less impact of the disease on their quality of life and lower psychosocial strains than parents of children with other chronic conditions, such as diabetes. Other studies showed that the parents of BWH are deeply affected by their child’s condition. For example, Beeton et al. [7] showed that, besides feelings of anxiety, helplessness, guilt and worry over their child’s condition, parents also report to be largely impacted by their child’s pain. Especially mothers of BWH are at risk for psychosocial problems compared to fathers because they are usually more involved in the daily care for their child [6]. More insight into psychosocial functioning of mothers is important in assisting professionals [3] and therefore the aims of this study were to: (i) determine the degree of anxiety and depression of mothers of BWH in comparison with a Dutch reference group, (ii) determine the degree of maternal distress and (iii) explore the relation of anxiety, depression and distress (psychosocial functioning) with the HRQOL of their sons. Data were collected within the scope of a larger study aiming to assess the validity and reliability of the haemophilia coping and perception test [8]. BWH aged 8–12 years under treatment in one of six participating Dutch Haemophilia Treatment Centres (Emma Children’s Hospital Amsterdam, University Medical Centre Groningen, Catharina Hospital Eindhoven, Leiden University Medical Centre, Radboud University Medical Centre Nijmegen and Sophia Children’s Hospital Rotterdam), and their mothers, were invited to participate in this study between July 2011 and January 2013, after approval by the ethics committee. All participating mothers gave written informed consent. Mothers were asked to complete three paper– pencil questionnaires at home; one questionnaire concerning socio-demographics of the parents and child, one concerning anxiety and depression, and one concerning parental distress. BWH completed a disease-specific HRQOL questionnaire. Anxiety and depression of mothers were assessed with the Dutch version of the Hospital Anxiety and Depression Scale (HADS) [9]. This 14-item self-report questionnaire includes a 7-item depression scale, a 7item anxiety scale and a total scale. Questions are answered on a four-point scale (0–3), resulting in scores on each scale from 0–21, where higher scores indicate more anxiety or depression. The cut-off score is ≥8 for the depression and anxiety scales, indicating Correspondence: Lotte Haverman, PhD, Psychosocial Department, Emma Children’s Hospital, Academic Medical Centre, G8-136, Post box 22660, 1100 DD Amsterdam, the Netherlands. Tel.: +31 20 5665674; fax: +31 20 6091242; e-mail: l.haverman@amc.nl

Psychosocial care for children with haemophilia and their parents in the Netherlands

Children growing up with haemophilia are at greater risk for psychosocial problems than their healthy peers. Providing psychosocial care to children with haemophilia and their families is indispensable, since psychosocial factors can have a significant impact on health and health‐related quality of life (HRQOL).

Reliability, validity and evaluation of the Haemophilia Coping and Perception Test

Haemophilia is an X-linked bleeding disorder, caused by an inherited deficiency of clotting factor VIII (FVIII; haemophilia A) or FIX (haemophilia B) and leads to spontaneous and posttraumatic bleeds [1]. In the Netherlands, boys with severe haemophilia receive adequate prophylactic treatment to reduce the risk of irreversible joint damage and to increase the quality of life. However, boys may still endure difficulties and impairments, such as hospital visits, frequent injections and limited participation in (sport) activities [2]. Little is known about the knowledge, competencies, coping skills and perception children with haemophilia have to manage and cope with their disease. More insight into these concepts is important, because then it is possible to support patients, improve psycho-education and disease management, which can positively influence not only physical outcomes, but also psychosocial well-being, such as improvement of healthrelated quality of life (HRQOL) [3]. Coping is defined as ‘an emotional response to external negative or stressful events’ [4] and is an important factor in the acceptance of the disease and HRQOL in children with haemophilia [5]. Perception of disease is defined as ‘emotional and cognitive representations of illness’, including feelings of anxiety and worries about the illness [6]. To detect children with inadequate coping skills or non-realistic attitudes and anxiety, Colland & Fournier [7] developed and validated the Asthma Coping Test (ACT) for children with asthma aged 8–12 years. The test is unique because it is presented in board game form [7]. Recently, the ACT has been adapted by the Hemophilia Comprehensive Care Treatment Center in Amsterdam for boys with haemophilia and called the Haemophilia Coping and Perception Test (HCPT). By providing a tool to assess coping and perception in clinical practice, a contribution can be made to recognize what boys find difficult and to provide psychosocial support required for children with haemophilia [3]. Also, we know from research that board games are useful in counselling children [8]. The aims of this study are to assess reliability and construct validity of the HCPT, describe the coping and perception of boys with haemophilia and evaluate the usefulness of the HCPT. Therefore, all boys aged 8–12 years with haemophilia under treatment in one of six participating Dutch Hemophilia Treatment Centers were invited to participate in this study by a letter between July 2011 and January 2013 after approval by the ethics committee. All parents gave written informed consent. The paediatric psychologist (P.L.) visited all respondents at home. The HCPT was played one-on-one; parents could be present but not allowed to participate or intervene. Evaluation questionnaires were completed after the HCPT. Parents completed a socio-demographic questionnaire about themselves and their child. The HCPT takes approximately 45 min and has two sides – sports and pirates/treasures (Fig. 1). The boy chooses which side he prefers. Players roll the dice, advance the piece the corresponding number of squares on the board and read a corresponding card. The blue cards contain the true test items (32 items), which can be answered by the child only and consist of two scales; coping (e.g. ‘When you have a bleed, what are you supposed to do’?) and perception (e.g. ‘Do you ever feel different from your friends because you have hemophilia’?). The yellow cards (34 items) contain questions or assignments to amuse the players (e.g. ‘walk around the room like an elephant’). The red cards (34 items) contain questions to facilitate and promote conversations about more personal topics (e.g. ‘what do you do when you cannot sleep at night’?). The scale score of the coping items is calculated as the sum of the items. The coping scale has two versions; one for boys with moderate or mild haemophilia (coping) and one with four additional questions regarding prophylaxis for boys with severe haemophilia (coping-S). The higher the score, the better the reported coping skills. The scale score of perception items is calculated as the sum of the items. A lower score indicates realistic perception, with low anxiety. Questionnaires were mailed to respondents 1 week before the home visit. To assess construct validity of Correspondence: Lotte Haverman, PhD, Psychosocial Department, Emma Children’s Hospital, Academic Medical Center, A3-241, PO Box 22660, 1100 DD Amsterdam, The Netherlands. Tel.: +31 20 5665674; fax: +31 20 6091242; e-mail: l.haverman@amc.nl

Male gender, school attendance and sports participation are positively associated with health-related quality of life in children and adolescents with congenital bleeding disorders.

This study assesses health‐related quality of life (HRQOL), and variables associated with HRQOL, in children and adolescents with haemophilia and congenital bleeding disorders (CBD) in the Netherlands.