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Growing up with hemophilia
Health related quality of life and psychosocial functioning
Limperg, P.F.
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2017
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Limperg, P. F. (2017). Growing up with hemophilia: Health related quality of life and
psychosocial functioning.
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Chapter 4
Reliability, validity and evaluation of the Haemophilia
Coping and Perception Test
P.F. Limperg, M. Peters, V.T. Colland, C.H. van Ommen, M. Beijlevelt, M. A. Grootenhuis, L. Haverman
Haemophilia is an X-linked bleeding disorder, caused by an inherited defi ciency of clotting factor VIII (FVIII; haemophilia A) or FIX (haemophilia B) and leads to spontaneous and post-traumatic 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 may still endure diffi culties 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 infl uence not only physical outcomes, but also psychosocial well-being, such as improvement of health related quality of life (HRQOL) [3].
Coping is defi ned 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 defi ned 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 Haemophilia Comprehensive Care Treatment Centre 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 fi nd diffi cult and to provide psychosocial support needed 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.
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Therefore, all boys aged 8-12 years with haemophilia under treatment in one of six participating Dutch Haemophilia Treatment Centres 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 minutes 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 haemophilia?’). 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 the coping scale, coping skills were assessed with the 28-item Coping with a Disease (CODI) questionnaire [9]. The CODI asks children to indicate on a 5-point scale how often they apply a certain
coping strategy, across six subscales. A higher score indicates a more frequent use of a coping strategy. The Cronbach’s alphas of the CODI scales [9] in our Dutch sample range from 0.57 to 0.86. Disease-related coping skills were also assessed with the 26-item Questionnaire Op Koers for Children (QOK-c) [10]. The QOK-c asks children whether they agree with given statements in six subscales on a 5-point scale. A higher score indicates the use of more disease-related coping skills. Cronbach’s alphas of the QOK-c [10] in our sample range from 0.41 to 0.72.
The 20-item ‘trait’ scale of the State-Trait Anxiety Inventory for Children (STAI-C) [11] was used to assess construct validity of the HCPT perception scale. Higher scores represent higher levels of anxiety. Cronbach’s alpha of the STAI-C [11] in our sample is 0.84. A self-composed evaluation questionnaire was completed by children and parents after fi nishing the HCPT.
The sample was compared to non-respondents with Mann-Whitney and chi-squared tests. Descriptive analyses were performed to describe the sample Fig. 1. Haemophilia Coping and Perception Test boards: treasure seeking (left)
and sports (right). 1. Question cards (blue/red/yellow) 2. Scoring form
3. Scoring manual 4. Indication bar
5. Dice (regular/colored) 6. Pieces to walk around the board 7. Treasure/prices (rewards)
4
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Boys (N=32) Mean (SD)
Age (years) 9.8 (1.6)
Median (range) Number of bleedings past 3 months 0 (0-4) Number of visits to hospital due to acute bleedings past 3 months 0 (0-7)
N % Type of haemophilia Haemophilia A 25 78.1 Haemophilia B 7 21.9 Severity of haemophilia Severe 18 56.3 Moderate 2 6.3 Mild 12 37.5 On prophylaxis (yes) 18 56.3 Parents (N=30*) Mean (SD) Age (years) 41.6 (4.3) N % Gender (female) 28 93.3 Married/living together 23 76.7
Country of birth (Netherlands) 26 86.7 More children with haemophilia in family (yes) 10 33.3 Educational level¹
Low 1 3.3
Intermediate 18 60.0
High 11 36.7
Employment (paid job) 26 86.7
Table 1. Socio-demographic characteristics and health status.
* Two sets of brothers participated, therefore only 30 parents were included. Participating Haemophilia Treatment Centers: Emma Children’s Hospital Amsterdam, University Medical Center Groningen, Catharina Hospital Eindhoven, Leiden University Medical Center, Radboud University Medical Center Nijmegen and Sophia Children’s Hospital Rotterdam.
1 Highest level completed. Low: primary education, lower or middle general secondary education; Intermediate: middle
vocational education, higher secondary education, pre-university education; High: higher vocational education, university
and the answers on the HCPT items. To determine internal consistency of the HCPT scales, Cronbach’s alpha coeffi cients were calculated based on average inter item correlation [12]. Item 19 from the perception scale was deleted, due to zero variance. To assess construct validity, correlations between the HCPT scales and the CODI, QOK-c and STAI-C questionnaires were determined with Spearman’s rank correlation. The evaluation questionnaire was examined explorative.
In total, of 63 eligible boys, 32 boys participated (response rate = 51%, mean age = 9.8 years, SD = 1.6). Table 1 presents the socio-demographics and
health status of the sample. Respondents had severe haemophilia more often (p<0.01; not shown in table).
The two versions of the HCPT coping scale showed satisfactory reliability (α 0.77 and α 0.81). The perception scale showed low reliability (α 0.55). The correlations between the HCPT coping scales and the scales of the CODI and QOK-c were not signifi cant (data not shown). The correlation between the HCPT perception scale and the STAI-C was signifi cant and medium-size (rs=.38, p<0.05).
Table 2 depicts the literal HCPT items and the median scores of the items. Items with lower medians (1.0) in the coping scales were items related to social competences or recognizing and explaining joint bleeds. With regard to the perception scale, items concerning anxiety received higher scores.
On the general evaluation question, boys rated the HCPT on a 10-point scale with a median of 10.0 (range = 6.0-10.0) and parents with a median of 8.0 (range = 7.0-10.0; data not shown). Children reported to appreciate the playful character of the HCPT. Parents reported to be surprised about their son being less reluctant to talk about haemophilia while playing.
In this study, we described the reliability and construct validity of the HCPT. With regard to reliability, the HCPT coping scales showed satisfactory reliability. The perception scale showed relatively low reliability, which may be caused by the items tackling diverging aspects of this concept.
With regard to construct validity, the HCPT coping scale did not correlate with the coping questionnaires. Based on the insights gained during this study, we hypothesize that the HCPT coping scale assesses the more practical competencies of coping, such as self-management, rather than the psychological construct of coping. In earlier literature, self-management was incorporated in the concept of coping [4]. Nowadays, the term ‘self-management’ is seen as a separate concept referring to activities people with chronic illness undertake to create order, discipline and control their illness [13]. From the evaluation, we conclude that boys and parents had a positive attitude towards the HCPT.
91 Table 2. Literal representation of the items in the Haemophilia Coping and Perception Test
(HCPT) scales and median scores per item.
N Median Range Coping scalea
1. A friend asks you: what is hemophilia? Please explain. 32 2.0 1.0-2.0 2. What are you good at in school (4 subjects minimum)? Eliminated from the HCPT
3. When you have a bleed, what are you supposed to do? 32 2.0 1.0-2.0 4. What sports can children with hemophilia do? (Name >4) 32 2.0 1.0-2.0 5. Sometimes children will behave different towards you because they know you have
hemophilia. What could you say to them? Name two things. 32 1.0 0.0-2.0 6. What do you do when you have a bleed at school? Give 3 examples. 32 2.0 1.0-2.0 7. Some children have severe hemophilia. Do you think children with severe hemophilia
should infuse at fixed days or do you think they can sometimes change the day they infuse? Give two reasons to change the day of infusion.*
18 1.0 0.0-2.0 9. During the break, when you’re playing with the other children at school, you get a joint
bleed. What could you do and say? Give at least two examples. 32 2.0 0.0-2.0 10. How can you recognize a joint bleed? Give at least two examples. 32 2.0 0.0-2.0 11. When the nurse tells you to keep a diary of your bleeds, what should you write down?
Give two examples.* 18 1.5 0.0-2.0
13. What can you do yourself to decrease the chance of getting bleeds? 32 2.0 0.0-2.0 14. What if: children in your class or in the neighborhood tease you because of your
hemophilia. What could you say or do? 32 1.0 0.0-2.0 15. When you’re going to give a presentation to your class about hemophilia, what will
you tell about hemophilia? What is important to know for the children in your class? 32 2.0 0.0-2.0 17. Do you ever try to keep your hemophilia a secret from other children? How much do
you try? Please indicate on the indication bars. 32 2.0 0.0-2.0 19. Are there sports, hobbies or other activities that you would like to do, but are not
allowed to do because of your hemophilia? Please name them. Eliminated from the HCPT due to zero variance 20. If you’re going to school camp, what should your teacher know about your
hemophilia? Name as many things as possible. 32 1.0 0.0-2.0 22. What if you can already infuse yourself and you are going to a sleepover at your
friend’s house, what should you bring for your hemophilia?* 18 2.0 1.0-2.0 23. If your PE teacher tells you: ‘You cannot join, otherwise you may get a bleed.’ What
could you say? 32 2.0 0.0-2.0
24. Please name what can happen if you don’t tell anyone you are having a joint bleed. 32 2.0 0.0-2.0 26. Can you explain to your doctor what you feel when you think you are having a joint
bleed? Please give examples of what you would say. 32 1.0 0.0-2.0 28. When you use a pain killer, for example when you bleed or when you are at the
dentist, what pain killer can you use? 32 2.0 0.0-2.0 29. What can you do yourself to suffer less from a joint bleed? Please give at least three
examples.* 18 1.0 0.0-2.0
33. What if you are 18 years old and from now on you have to take care of everything
yourself regarding your hemophilia. What do you still have to learn? 32 2.0 0.0-2.0 Perception scaleb
8. Do you ever feel different from your friends because you have hemophilia? Please
indicate how much you feel that way. 32 1.0 1.0-4.0 12. Do you mind when other people know that you have hemophilia? Please indicate on
the indication bars how much you do mind this. 32 1.0 1.0-2.0 16. Some children are afraid to be alone when they have a joint or muscle bleed. How
17. Do you ever try to keep your hemophilia a secret from other children? How much do
you try? Please indicate on the indication bars. Moved to the Coping scale 18. Did you ever have to stay home for a few days because of your hemophilia? If so, how
much did you mind? 32 1.0 1.0-3.0
21. Some children are sad or angry when they have a bleed. How is that for you? Please
indicate on the indication bars how strong that feeling is. 32 2.0 1.0-4.0 25. Some children are scared when they have a bleed. How scared are you when you
have a bleed? Please indicate on the indication bars. 32 2.0 1.0-4.0 27. Some children are afraid to die if they fall on their head hard or have a bleed in their
head. How afraid are you for this? Please indicate on the indication bars. 32 2.0 1.0-4.0 30. Please indicate on the indication bars how severe you think your hemophilia is? 32 2.0 1.0-4.0 31. Please indicate on the indication bars how good you think you are at recognizing joint
or muscle bleeds. 31 2.0 1.0-4.0
32. Please indicate on the indication bars how much your parents/mother mind(s) that
you have hemophilia. What makes you feel this way? 32 2.0 1.0-4.0
a A higher score indicates more adequate coping and knowledge
* These items (7, 11, 22 and 29) are suitable for severe patients only and constitute the coping-S scale b A lower score indicates more adequate perception
Table 2. Literal representation of the items in the Haemophilia Coping and Perception Test (HCPT) scales and median scores per item. (Continued)
One of the limitations of this study is the small sample size, due to the narrow age range of the HCPT. However, the age range should not be widened, since the HCPT would be too diffi cult for boys younger than 8 and too childish for boys older than 12. Also, the perception scale showed relatively low reliability.
The HCPT can be played in a one-on-one situation or in group settings with a haemophilia nurse and peers with haemophilia. In clinical practice, it is essential for paediatric haemophilia care providers to address gaps in knowledge to encourage the development of self-management skills in a manner that is accessible and appealing to boys with haemophilia [14]. To do so, the HCPT can be used by nurses or psychologists as a tool to get insight into the knowledge, self-management and perception of boys with haemophilia and to provide tailored psycho-education. Because the HCPT is a game, it encourages boys to talk about their diffi culties regarding knowledge, self-management and perception of their disease in a way they appreciate.
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Acknowledgements
We would like to thank all participating children and parents in this study. Also, we would like to thank all contributing paediatric haematologists and nurses, especially Corinne Liem, Minke Douma and Janske Lock, in assisting the data collection. We would also like to thank Pfi zer Pharmaceuticals BV for providing the unrestricted research grant to execute this study.
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