Living with haemophilia and von Willebrand's: A descriptive qualitative study

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Abstract

Objective

The aim of this qualitative descriptive study was to describe the experience of living with bleeding disorders and to identify the associated salient issues from the perspectives of people living with haemophilia or von Willebrand's Disease (vWD).

Methods

Nine members of The Haemophilia Society took part in a semi-structured interview. The interviews were tape recorded and transcribed and the results subjected to thematic content analysis.

Results

Participants described the physical aspects of their condition, such as bleeding into joints, the implications of bleeds and development of co-morbid conditions (e.g. arthritis). Many felt that their bleeding disorder had impacted on their education, work, social activities and family life. Anxiety and depression were associated with daily management of symptoms, the unpredictable nature of bleeding disorders and concerns about the future. All participants had encountered a degree of stigmatisation related to their condition and felt that there was a public misconception about bleeding disorders. Many had experienced discrimination in educational and work settings. Some participants expressed a preference for seeking treatment from specialist services.

Conclusion

Living with haemophilia and vWD poses a number of daily and longer-term challenges not only for individuals, but also for their families. Further investigation of ways to support such individuals is warranted.

Practice implications

Greater awareness and understanding of the issues faced by people living with haemophilia and vWD is needed for the general public, employers, and healthcare staff, particularly those working in Accident and Emergency Departments.

Introduction

The term bleeding disorder refers to a condition of the blood clotting system, in which bleeding is prolonged and excessive. There are different types of bleeding disorders including haemophilia A and B and von Willebrand's Disease (vWD). The number of people with haemophilia worldwide is estimated to increase from approximately 350,000 in 1990 to 440,000 by 2020 [1]. In the UK, there are approximately 7000 people living with haemophilia. There are several types of haemophilia. Haemophilia A is the most common and results from a deficiency of clotting factor VIII. People with haemophilia can experience severe spontaneous bleeding into the joints, which can cause acute pain and severe joint damage (where recurrent bleeding occurs), leading to disability [2].

VWD is believed to affect 1% of the population [3], [4], although this is highly inconsistent with the numbers diagnosed as approximately 7000 people with this disorder have been identified in the UK. VWD is a blood clotting condition, which is usually inherited and can affect both sexes [3]. Those mildly affected may have regular nosebleeds and bruising while those with the more severe type have symptoms similar to those of haemophilia. Replacement therapy is the mainstay of treatment for both conditions and involves the intravenous administration of blood plasma or recombinant concentrates to replace the missing or defective clotting factor. The impact of HIV and Hepatitis C infection have been profound and since the 1980s, association with bloodborne viruses has increased the reluctance of individuals and their families to be open about haemophilia [5].

Several studies have examined health-related quality of life (QoL) in patients with haemophilia and vWD, using generic, standardised questionnaires such as the SF-36 (e.g. Refs. [5], [6], [7]). Compared to haemophilia patients with mild or moderate disease and UK norms, three key studies have reported that those with severe haemophilia had lower levels of HR-QoL in terms of mobility and pain [6], [7], [8]. These findings were independent of age. In accord with these findings, Solovieva et al. [9] found that HR-QoL was lower in most domains compared to healthy controls (n = 164). The areas of life most affected by their condition were financial security, their own health and relationships with family/relations. Similarly, Talaulikar et al. [10] found that a small sample (n = 20) of patients with haemophilia and vWD had more physical role limitations, less vitality and lower perceptions of general health compared with Australian norms for the SF-36.

In a study covering six European countries, Bullinger and Von Mackensen [11] focused on HR-QoL among children with haemophila (n = 339) and their parents (n = 330) using a generic QoL measure (KINDL) [12] and a specific, Haemo-Qol measure [13]. Results showed that children with haemophilia (n = 190) had higher QoL scores on all dimensions (e.g. self-esteem, friends, family) compared with children with asthma, dermatitis and obesity, although the level of scores suggested relatively low impairment. Regression analysis showed that HR-QoL was associated with life satisfaction, social support, locus of control and number of bleeds.

Molleman and Van Knippenberg [14] investigated the origins and consequences of psychosocial problems among 43 patients with haemophilia in the Netherlands. Not surprisingly, severe haemophilia was associated with increased physical and psychological problems such as worry and depressive feelings. Stigmatisation was found to have a negative effect on psychological well-being. An Italian study [15] compared the psychological well-being of 60 patients with haemophilia with that of a matched control group of healthy participants. There were no significant differences on anxiety or depression although participants with haemophilia had comparatively lower levels of self-esteem.

The lack of qualitative studies regarding patients’ perspectives of life with haemophilia and vWD has been noted [10], despite the fact that such information provides useful insight into the daily challenges faced by patients and local service provision. In Australia, Talaukilar et al. [10] interviewed 30 patients with bleeding disorders as part of an exploration of HR-QoL. Qualitative data were used to develop a proforma designed to collect data on issues perceived to be important by patients such as schooling, employment, and relationships. These data were subsequently reported quantitatively in terms of frequencies: for example, 22 (73.3%) participants had major fears related to complications arising from bleeds, disability, viral transmissible diseases and emotional issues. Similar findings emerged from a further Australian study by Petersen [16], which aimed to gain insight into the experiences of people living with a genetic condition. In-depth interviews were conducted with 21 members of genetic support groups four of whom had haemophilia; other participants had either cystic fibrosis or thalassaemia. Experiences of social exclusion, managing the uncertainty of these conditions and the negative impact on physical functioning were common amongst participants.

In a UK exploration of quality of life, Beeton et al. [17] conducted two focus groups and eleven interviews with haemophilia and vWD patients. Participants spoke of the benefits of factor replacements and recalled their negative experiences of life before this treatment was available. They commented on the advancements and improvements in haemophilia treatment over the last 20 years and held positive perceptions of their HR-QoL. They regarded planning around their health problems as an inconvenience rather than a major problem and viewed their condition as integral to their perception of ‘self’. A degree of disease acceptance was evident.

The aim of this qualitative study was to describe the experience of living with bleeding disorders in the UK and to identify the associated salient issues from the perspectives of people living with haemophilia or vWD.

Section snippets

Participants

A purposive sample of nine members of The Haemophilia Society were identified and invited to take part in the study by The Haemophilia Society as representing a cross section of their members; the research team were excluded from this selection. All participants gave consent for their contact details to be given to the research team, who contacted them to arrange an interview. The sample comprised eight males with haemophilia and one female with vWD; the age range was 28–84 years. This number

Results

Findings highlighted issues that are common to many individuals with long-term medical conditions and some that were specific to individuals with bleeding disorders. The first theme illustrates a critical area of impact for individuals with bleeding disorders—the physical problems related to the condition, associated viruses and treatment.

Discussion

This study explored the experiences of living with bleeding disorders from the individual's perspective. Some experiences identified accord with those noted in previous literature, e.g. Refs. [10], [17], however, a number of additional issues and experiences that were salient to interviewees emerged, such as the awareness of the impact on their own parents’ quality of life, disruption to family roles, and the side effects of medication.

In accord with previous literature [16], [20], participants

Acknowledgements

The research team would like to extend their thanks to the members of The Haemophilia Society (UK) who participated in this research and also to The Haemophilia Society (UK) for their help and support.

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