A growing body of evidence supports the recommendation of both physiotherapy and physical activity in people with haemophilia. Physical benefits include increasing strength and flexibility and reducing the risks of osteoporosis, arthropathy, and intramuscular and joint bleeds; social benefits have also been observed. However, anecdotal evidence suggests that people with haemophilia may still be averse to engaging with physical activity due to fears of causing bleeding, joint pain and joint damage.
Qualitative interviews were conducted with young men with haemophilia treated at comprehensive care centres in London, to explore and identify the reasons behind risk-averse behaviours towards exercise and physical activity. The interview 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.
Ten participants were interviewed. Preferred activities were variable, with five participants describing themselves as very physically active, three moderately active, and two reporting little physical activity; four described themselves as ‘not gym-confident’. Seven participants described themselves as highly or moderately motivated to undertake physical activity, with motivations including weight loss and getting fit for summer holidays. However, there was some anxiety around weight-bearing exercise due to the fear of pain or injury. All participants had been exposed to personal trainers (PTs) and recognised the importance of being properly introduced to training equipment but felt that PTs were too expensive for them. The majority of participants reported sports-related injuries and self-perceived limitations on activity due to their personal/individual experience of living with haemophlia. Physiotherapists were often the first point of contact for advice and support on safe physical activity. All participants recognised the benefits of physical activity and had been encouraged in this by their physiotherapists.
Young men with haemophilia are keen to use the gym as part of their personal fitness regimens. The ongoing safety concerns of health care professionals warrants further research.
People with haemophilia and other bleeding disorders, such as von Willebrand’s Disease (vWD), are frequently diagnosed in childhood. There is, therefore, a recognised need to prepare children and young people with these conditions for transfer to long-term care in the adult sector.
The Transforming Transition programme was designed to facilitate the sharing of knowledge and experience to enhance transition practice in the UK. The aim of the programme was to secure change in working practices to enable nurses to drive improved transition outcomes, enhance self-management by people with haemophilia and develop clinical leadership.
As part of the programme, we captured young people’s views of their transition experiences by means of a series of structured interviews conducted with young people identified through the patient association. Interviews revealed that transition tended to take place around the age of 16 but could be delayed in response to individual circumstances. The transition process did not always appear to be planned, with many participants reporting an abrupt or sudden change between paediatric and adult teams and/or centres.
Those with severe haemophilia tended to be well educated and prepared by their paediatric teams to be confident in managing their condition from an early age. They would learn to self-infuse between the ages of 5-11, and reported feeling confident in tailoring their treatment around their daily routine. Experience of transition to adult services varied, with about half describing it as fairly well planned. For those with bleeding disorders such as vWD, there was rarely a transition process. These individuals and people with mild haemophilia described having less exposure to opportunities to learn self-management and appeared to experience issues which coincided with significant lifestyle changes, such as leaving home and attending university. This highlights the importance of ensuring that patients across the spectrum of bleeding disorders are adequately supported in the lead-up to and transfer to adult services. The interviews also reinforce the need to continue to address the specific needs of women with bleeding disorders as they transfer to adult services. The learning from this phase of the Transforming Transition programme was submitted to NICE as part of the consultation on its guideline.
Transition is the term used to describe the process of approaching and crossing the chronological boundary between paediatric and adult care services. Transfer of care describes the administrative arrangements associated with moving from a paediatric to an adult service across this boundary.
Transforming Transition was a nurse-led initiative designed to facilitate the sharing of knowledge and experience to enhance transition practice in the UK. The aims of the programme were to secure changes in working practices that enabled nurses to drive improved transition outcomes, enhance self-management by people with haemophilia and develop clinical leadership. From the outset of the programme it was clear that there were differences in practice and that sharing practice would be one route to addressing some of these variations.
As a result of the knowledge and experience shared through the programme, resources described in this paper are available to all haemophilia centres. Sharing between people with haemophilia, their families and carers, and the haemophilia clinical team have resulted in the development of practical resources that enhance practice within the haemophilia centre. Sharing of practice between nurses and young people with haemophilia at the programme workshops provided a focus for development of local and regional action plans, a framework for reviewing progress, the introduction of transition clinics and improved transition documentation.