A recent study exploring physical activity among young men with haemophilia highlights their keenness to incorporate gym use into their personal fitness regimens and the importance of a tailored approach to support this
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Historically, physical activity was contraindicated in people with haemophilia due to limited access to treatment for bleeding episodes and the perception that harm would be caused[1]. Despite dramatic improvements in the availability of treatment, such as primary prophylaxis for those with severe haemophilia[2], together with a recognition that exercise is beneficial[3], the perception amongst some clinicians and people with haemophilia that physical activity can be harmful still persists. The fear of adverse consequences of injury result in risk aversion and a reluctance to engage in physical activity.
Today, for people with haemophilia, physiotherapy and physical activity are recommended where risks can be managed[4,5]. This change in approach is supported by a growing body of evidence that physical activity in people with haemophilia increases strength and flexibility, decreases body fat, and reduces the risk of metabolic and cardiovascular disease, osteoporosis, arthropathy and intramuscular or joint bleeds[3,6,7]. Physical activity has been shown to improve physical performance and health-related quality of life[8,9,10,11]. However, the optimal dose-response for exercise prescription for people with haemophilia has not been well addressed[12]. A recent Cochrane review concluded that most exercise interventions for people with haemophilia produced improvement in outcomes such as pain, range of motion, strength and walking tolerance but that the quality of the evidence was poor[13].
Pilot study data support the importance of resistance training in people with haemophilia for increasing muscle strength and decreasing the frequency and severity of bleeding episodes and associated pain[14]. However, in a subsequent observational study of young people with haemophilia aged 12–25, the same group found that those who exercised strenuously had a higher proportion of trauma-related bleeds[15].
London haemophilia services work with approximately one third of the UK's haemophilia population and includes a cohort of around 150 young men aged 18–25 years. This group consists of a large number of young men who, despite receiving intensive prophylaxis and home care programmes, have damaged ankles because of traumatic injury or recurrent spontaneous joint bleeds during childhood and/or adolescence. Paradoxically, the success of treatment has led to a lack of awareness in some young people of the long-term consequences of non-adherence to treatment as well as social isolation[16]. The need to address this is reinforced by the 2014 Public Health England policy, which stated that: “There is a lot of evidence that the social element behind physical activity aids enjoyment. It also encourages people to stick at it”[17].
For all the known benefits of physical activity, there is anecdotal evidence among the patient community to suggest that those living with haemophilia may be more averse to engaging in physical activity, due to fears of causing bleeding, joint pain and (further) joint damage.
METHODOLOGY
A series of qualitative interviews were conducted with young men with haemophilia of differing severity, to explore and identify reasons why young men show risk-averse behaviour towards exercise and physical activity. Participants, who all attended comprehensive care centres in London, were approached by the authors through their personal contact networks. After providing verbal consent, they were asked a series of open-ended questions during a telephone interview conducted by the authors, who together had formulated the interview schedule. These questions were designed to prompt discussion and capture opinions relating to participants’ physical activity and gym membership/use, and the degree to which their haemophilia impacts on both (see Table 1). The interviews were recorded, transcribed verbatim and thematically analysed to identify key themes relating to attitudes towards physical activity. Direct quotes are used with consent. To protect anonymity, participants are known only by study number (P1, etc.). Ethical approval was not necessary using the Health Research Authority (HRA) decision tool application[18] as the project did not include randomisation, change treatment/care or promote generalisable findings.
Table 1
Interview schedule
RESULTS
Ten participants aged 17–25 years with mild, moderate or severe haemophilia A or B participated in the study (see Table 2). Each participant was interviewed once, with an average interview recording time of 35 minutes (range 30–45 minutes). The themes identified from the interviews were: preferred activity, self-motivation, financial implications, sports injury, and the role of physiotherapy for musculoskeletal health.
Table 2
Patient demographics and summary responses from discussions
Preferred activity
The extent to which the participants engaged in daily activity and their range of preferred activities varied widely. Five of the participants reported being ‘very physically active’, three reported ‘moderate physical activity’, and two did ‘very little activity’. Four reported attending a gym and described themselves as having ‘fairly high’ to ‘high confidence’ in using a gym, whilst a further four reported being ‘not gym confident’.
Self-motivation
Seven participants described themselves as highly or moderately motivated to engage in physical activity and exercise. In the case of Participant 3, weight loss was the main motivation to become more physically active:
“I got weighed at the [haemophilia] centre and I was like, ‘OK, I’m not happy with that, let's go back to the gym.’”
[P3]
For others, social motivations were revealed, such as wanting to get fit ahead of a summer holiday with a group of friends:
Individuals with low levels of motivation described themselves as being ‘lazy’:
“When I was younger, I was doing martial arts, football, swimming… I was like any other ordinary kid. As I’ve got older, I get more lazy.”
[P5]
Despite a close relationship with his clinical team and physiotherapist, P5 said he “rarely did the exercises recommended to him by his physiotherapist”. P10, who described himself as highly motivated, said he was “trying to build muscle and strength but felt he was often too lazy”.
The participants also raised the issue of confidence in undertaking weight-bearing exercise, with one participant revealing that he was “more confident to try these types of exercises with [his] physio at the centre,” rather than on his own. There was some anxiety around exercising due to fear of pain or injury:
Financial aspects
All participants had exposure to personal trainers (PT) at their gyms and recognised the potential benefits of working with a PT. The importance of an introduction to training equipment was expressed by the participants:
“When you start doing some exercises, you always want to do them correctly and… you can only do them correctly when you’re under someone's supervision… who is a professional in that area.”
[P2]
“When you work out a lot, sometimes it's hard to recognise if you’re doing something a little wrong. With a personal trainer you can correct your activity.”
[P8]
Some participants recognised the potential benefits of working with a PT but had concerns about their own motivation:
All participants felt that working with a PT would be too expensive for them and four had not used a PT due to cost alone. One frequent gym user had previously used a trainer to devise his own programme but added:
Injury
The majority of participants reported that they had sustained sports-related injuries, which they acknowledged was likely worsened in severity and recovery due to their haemophilia. They appeared to be aware of their limitations, especially those with severe haemophilia who understood the need for prophylaxis prior to physical activity:
Physiotherapy
The participants reported that physiotherapists are often their first point of contact for advice and support regarding safe physical activity:
“You do hear repetitively from physios that the healthier you are or the more you do with your joints, the more protected they are from bleeding.”
[P6]
The participants reported a view that most physiotherapists provided only a ‘prescription’ of stretches and exercises, usually focused on specific musculoskeletal problems rather than on endpoints, such as being able to lift weights, run a 10K race and participate in sport without thinking about haemophilia likely in their everyday lives. All participants recognised the benefits of physical activity and expressed an interest in wanting to do more. Most said they had been encouraged to do so by their physiotherapists in order to augment the efficacy of their treatment. Participants acknowledged that improving fitness and building muscle would lead to better outcomes:
DISCUSSION
We have shown that this group of young men with haemophilia, mostly treated with regular prophylaxis, already participate in sporting activity, including some that were previously considered ‘risky’ (e.g. martial arts, cricket) despite target joint(s) and/or the risk of injury. Despite this, there is evidence to suggest that they are not reaching their full potential for physical activity, due to lack of knowledge and fears around potential bleeding, joint pain and exacerbating previous injuries. This suggests that there is a need to establish a more tailored approach to exercise, focusing on identifying and addressing an individual's underlying medical and psychological issues that may impact on their personal motivation.
Gue et al. describe a new model of haemophilia care that supports patient autonomy in decisions related to managing their haemophilia, by de-emphasising treatment adherence as the primary goal and focusing on a healthcare plan that is customised by the patient and aligned with their priorities[19]. To support physical activity, this could include personalised exercise plans developed between the person with haemophilia and his clinical team and include motivational interviewing and goal setting[20].
Our results indicated varied motivation to engage in physical activity, including weight loss and getting fit for the summer. Overweight and obesity in people with haemophilia is recognised as a double-edged sword with the impact of haemophilia (bleed-related risks) negatively influencing the ability and intensity of exercise[21]. Individualised education and “tailoring engagement in physical activity to avoid the risk of traumatic bleeding” is suggested by Wilding et al.[22]. This would involve both a physiotherapy assessment to examine musculoskeletal health and personalised training programmes developed with a PT. Participants saw the potential benefits of working with a PT to become more confident and competent in a gym setting, acknowledging the importance of feeling self-assured and being familiar with using equipment and practicing various exercises safely and effectively in order to reduce the risk of injury.
Participants commented that PTs were expensive, and for some this prevented them from using this expertise, which may lead to injury. Several participants recounted joint or muscle bleeds following sporting trauma (Table 2) despite pre-sport prophylaxis, some of which caused lasting impact. Participant 8 reported a target knee joint that started ten years previously following a football injury. Physiotherapists within the haemophilia team are used as the first point of contact after bleeds occur for rehabilitation but are also reported to encourage sport and activity, including gym use, to improve fitness and wellbeing.