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Growing up with hemophilia
Health related quality of life and psychosocial functioning
Limperg, P.F.
Publication date
2017
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Citation for published version (APA):
Limperg, P. F. (2017). Growing up with hemophilia: Health related quality of life and
psychosocial functioning.
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Themes in daily life of adolescents and young adults
with congenital bleeding disorders: a qualitative study
P.F. Limperg, M. Peters, E. Gibbons, M. Coppens, M.C.E. Valk, M.A. Grootenhuis, L. Haverman
Transition into adulthood is a critical phase of life. Adolescents have to pass developmental stages such as achieving personal identities, gaining independence from their parents, initiating employment and building signifi cant relationships outside of their families [1]. Children and adolescents with chronic illnesses are expected to pass similar developmental stages as healthy peers, but often have a delay in their developmental trajectory [2]. This does not only affect patients during adolescence, the consequences often continue into (young) adulthood and result in having more trouble fi nding employment, leaving the parental home, marrying, becoming parents and having lower 2 income levels than their healthy peers [3, 4].
Research has shown that adolescents and young adults (AYA) with haemophilia are struggling to achieve self-management, to cope with the impact of their bleeding disorder on lifestyle and to adhere to therapy [1]. Compliance with prophylactic treatment frequently declines during teenage years [5]. To fully understand what it means to grow up with haemophilia, it is valuable to include AYA themselves.
The aim of this study is to capture the perspectives of AYA with congenital bleeding disorders on their condition and to identify which themes are relevant in daily life, based on qualitative data. A focus group approach was chosen in order to obtain insight in experiences of AYA with bleeding disorders. The study design is shown in Fig. 1.
Patients affected by a severe congenital bleeding disorder (haemophilia A/B, von Willebrand Disease (VWD) type 2 or 3, or other severe congenital bleeding disorder) were recruited from the Dutch Haemophilia Patient Society and from the Academic Medical Centre in Amsterdam (16-30 years). The focus groups consisted of a moderated discussion (90 min). Using thematic analysis methodology, focus group audio recordings were transcribed verbatim and three psychologists (PL, FW and AS) independently read and open-coded the transcripts using NVivo version 10 (Doncaster, Victoria, Australia). General initial codes were discussed in team meetings and grouped into themes. The themes were built through axial coding and team consensus was reached on the major themes (those who had the most codes). Finally, data were re-checked and
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In total, 12 AYA (mean age 24.0 years, SD 4.32) participated in one of three focus groups. Nine males and 3 females were included. Seven AYA (59%) had severe Haemophilia A and 5 AYA (41%) had other severe congenital clotting factor defi ciencies (VWD type 3, Glanzmann thrombasthenia, severe Factor VII defi ciency, severe Factor XIII defi ciency, afi brinogenemia).
Major themes were: relationship with parents (gaining independence, taking responsibility and feelings of guilt in parents), capabilities in sports (importance of playing sports, barriers in terms of joint damage or pain, guidance from hospital), capabilities in outings and travelling (barriers such as the trouble of preparing trips, no adequate treatment on location), self-management (importance of being in
control over bleeding disorder and having enough knowledge), illness perception and acceptance (negative experiences leading to more trouble accepting the condition), and capabilities in education and employment (being able to pursue ambitions as much as healthy peers). Table 1 depicts the most important themes (in the order of magnitude) and corresponding examples of statements expressed by AYA in the focus groups.
Fig. 1. Study design.
Open-ended research question What are the experiences and issues of young adults with bleeding disorders?
Approval ethics committee
Focus group 2: 3 YAs with bleeding disorders Focus group 1: 5 YAs with
bleeding disorders
Focus group 3: 4 YAs with bleeding disorders Purposive sampling
Data analysis: general initial coding and discussions with co-investigators and authors Continuous discussions between moderator and co-investigators about focus groups,
emerging themes, exhaustiveness of data etc.
Discussion and mapping of themes, refining concepts
Data saturation
Themes and concepts finalized, reporting
Selection of relevant questionnaires and testing in upcoming cross-sectional study Selection of patient reported outcomes measurements (PROMs) in daily clinical care for
Themes Examples
1. Relation with parents ‘My father feels very guilty towards me, for what I’m going through.’
‘Sometimes I don’t tell my parents I have a bleed, because I don’t want to burden them.' ‘I grew up very protected.’
‘It’s very annoying when my mom wants to interfere when I have a bleed.’ ‘It’s nice when your mom is there to support you when you have a bleed.’ 2. Capabilities in sports ‘Playing sports is very important for people with hemophilia.’
‘It is important to play sports with adequate supervision.’
‘I feel limited in participating in sports, because I have to hold back. I can’t give a 100%.’ ‘Unfortunately, I had to stop playing soccer because I can’t run anymore.’
3. Capabilities in outings and traveling ‘My parents felt it was too much trouble to arrange everything for a holiday, so we didn’t go.’ ‘I never went to a theme park, because I know I can’t go on the rides.’
‘This made me aware of the fact that I can’t just go anywhere I want.’ ‘I have never had any trouble while traveling.’
‘I have been to rural Africa, not a HTC in sight.’
‘I bring a letter for customs and put my medication in my carry-on luggage, no problem.’ 4. Self-management ‘I decide for myself when I infuse.’
‘It’s very important, as a patient, to know exactly what you have.’ ‘I arrange everything myself.’
‘I infuse when I think it’s necessary, I can feel it coming.’ 5. Illness perception and acceptance ‘Hemophilia isn’t bad for me.’
‘It doesn’t bother me, it doesn’t hold me back.’ ‘It has never impeded me.’
‘Aversion; don’t want to see that I have it.’
‘I don’t want people to see me as someone with an illness.’ ‘The confrontation with hemophilia bothers me sometimes.’ 6. Capabilities in education and
employment ‘Teachers are afraid that something will happen, so they won’t let me participate in physical education.’ ‘I had to drop out of my dance training program.’
‘They kicked me out of my internship because they thought I was physically inapt.’ ‘I don’t pay attention to hemophilia when it comes to work.’
‘I did not choose this profession because of my bleeding disorder; I chose it because this is what I wanted to do.’
Table 1. Themes emerging from focus groups.
This is the fi rst qualitative study to explore the experiences and issues AYA with bleeding disorders encounter in daily life, obtained in an interactive focus group setting. This study shows that AYA, despite growing up in a developed country with adequate treatments available, still experience obstacles and issues in daily life.
At fi rst, AYA shared mostly positive experiences. However, when probing, most AYA did experience barriers and negative consequences from their condition, such as having to quit a hobby or school. These problems were not exposed easily. Moreover, with having the responsibility over their own treatment, AYA may feel like the consequences, such as joint damage or pain, are somewhat in their own hands. This responsibility creates a complex
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‘normal’. On the other hand, as soon as patients deviate, confrontation with the consequences is immediate. This paradox has been identifi ed by Khair et al. [6] as well, who described that in order to keep wellness in the foreground, disease management must also be foremost. A second paradox was that the AYA are often being told to be grateful for living in a country and era where treatment is available, unlike many developing countries and previous generations, while they are still suffering from pain, arthropathy and limitations.
Our study shows the importance of the relationship with parents. Given the nature of the condition, with a large and long-term responsibility for parents with regard to treatment, this theme is not completely surprising. Support from family, friends, and psychosocial professionals is crucial [7]. Nonetheless, the process of gaining independence from parents is a complicated process. The transition from depending on parents to self-responsibility seems straightforward, but AYA nor their parents are able to accept a change in responsibility and loss of control [3]. This underlines the importance of paying attention to this parent-child relationship in clinical practice.
AYA voiced the importance of playing sports and the wish for adequate guidance from the hospital. This fi nding emphasizes that decisions regarding choosing a suitable sport should be made in dialogue between the patient, the doctor and the physiotherapist. It is important to take into account the patient’s physical status, interests and social needs [8].
Some AYA did not feel limited in participating in outings or to travel, but most AYA did experience certain limitations in the type, the destination and the length of trips. Ringwald et al. [9] studied travel behaviour in German patients with haemophilia and found that parents of patients under 18 years old were less likely to travel outside of Europe if their children were on prophylactic treatment and they could not self-administer medication, which is a fi nding we recognize in our study. Thus, it is important for AYA to have suffi cient knowledge and guidance from the hospital.
With regard to illness perception and acceptance, it seemed like aversion towards the bleeding disorder could be traced back to negative experiences.
Similarly, research in AYA with chronic illnesses has shown that negative illness perception or lack of acceptance of the condition is associated with low quality of life and feelings of anxiety and depression. Early recognition of psychological distress and negative perceptions may be helpful in identifying YA at risk for long-term problems. Also, identifi cation of negative illness perceptions enables early psychosocial intervention (e.g. a psychologist), to optimize well-being [10].
Furthermore, the AYA reported diffi culties in fi nding a suitable job or education. A social worker can help prepare AYA for a job or a psychologist can help individuals explore different career paths [7]. Also, Emma at Work, a job mediation agency especially for AYA with somatic diseases at the Academic Medical Centre in Amsterdam, is an example of a useful service [10].
This study has several limitations that need to be taken into account. First, we are aware of the fact that we studied AYA growing up in a developed country. Therefore, some of the responses could be different if we had conducted the study in a developing country. Moreover, recruitment was challenging and the response rate was low, probably because of the time consuming aspect of the study and the age range of the patients. Despite the low response rate, data saturation was achieved.
In conclusion, this study identifi es themes that play a role in daily life with bleeding disorders voiced by AYA in the Netherlands. Despite growing up in a developed country with adequate treatment, AYA with severe congenital bleeding disorders still experience obstacles in daily life. Our fi ndings provide additional insights into AYA’s experiences, especially regarding the transition period to adulthood. When discussing and monitoring psychosocial consequences in clinical practice, these themes should be included.
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We thank all participating young adults in this study. Also, we thank the Dutch Hemophilia Patient Society for assisting the patient recruitment, especially Manuel Baarslag. We are also very thankful to our colleagues who assisted in transcribing the focus groups and assisting in data analyses: Florrie Walraven (FW), Anouck Splinter (AS) and Hedy van Oers (HvO).
References
1. Breakey VR, Blanchette VS, Bolton-Maggs PH. Towards comprehensive care in transition for young people with haemophilia. Haemophilia 2010; 16: 848-9.
2. Stam H, Hartman EE, Deurloo JA, Groothoff J, Grootenhuis MA. Young Adult Patients with a History of Pediatric Disease: Impact on Course of Life and Transition into Adulthood. J Adolesc Health 2006; 39: 4-13.
3. Young G. Transitioning issues in adolescent to young adult hemophilia patients with inhibitors: an approach for a growing population. Blood Coagul Fibrinolysis 2010; 21: 848-57.
4. Pinquart M. Achievement of developmental milestones in emerging and young adults with and without pediatric chronic illness--a meta-analysis. J Pediatr Psychol 2014; 39: 577-87. 5. Petrini P, Seuser A. Haemophilia care in adolescents – compliance and lifestyle issues.
Haemophilia 2009; 15: 15-9.
6. Khair K, Meerabeau L, Gibson F. Self-management and skills acquisition in boys with haemophilia. Health Expectations 2013; 18: 1105-9.
7. Cassis FRMY. Psychosocial Care for People with Hemophilia. Treatment of Hemophilia, 2007. 8. von Mackensen S. Quality of life and sports activities in patients with haemophilia.
Haemophilia 2007; 13: 38-43.
9. Ringwald J, Rudolph P, Biner M, Fiessler C, Mayr A, Lohmann M, et al. Travel behaviour of patients with haemophilia. Travel medicine and infectious disease 2013; 11: 159-65. 10. Verhoof E, Maurice-Stam H, Heymans H, Evers A, Grootenhuis M. Psychosocial well-being
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