“It's a way of life”: Results from the Perceptions of Pain in Haemophilia study

Perceptions of Pain in Haemophilia is a cross-sectional, non-interventional study using mixed qualitative research methods (an anonymous paper-based questionnaire and focus group interviews) to explore pain in males with haemophilia in the UK. The questionnaire, designed to address the prevalence and perceptions of pain, was developed by a panel of experts and distributed by post to 2,433 men with haemophilia, regardless of severity, age or treatment, by 11 participating haemophilia centres. A total of 599 (24.6%) responses were received; this data will be presented elsewhere.

At the end of the questionnaire, participants were asked to provide their name and email address if they wanted to be contacted by the research team to participate in an online focus group. Using this contact list and through advertisements on UK social media sites, UK males with haemophilia aged >18 years were invited to participate in a focus group. We were pragmatic about recruiting participants but aimed to ensure, as far as possible, that a diverse range of experience was presented. Each focus group included participants across age ranges, with haemophilia A or B and a variety of current treatments. The discussion was moderated by a researcher with experience in clinical haemophilia care and structured using an interview guide (see Appendix) to elicit the nature of acute and chronic pain and to discuss the currently used pain assessment tools for acceptability (length, questions asked) and validity (asking the right questions, whether or not they adequately reflect day-to-day pain experiences).

Each focus group discussion was digitally recorded and transcribed verbatim (with participants anonymised to a study number) and manually coded after each focus group took place. The themes identified were then raised in subsequent focus groups (e.g. by saying ‘’Others have said … do you agree with this?”). Finally, all transcripts were re-analysed using NVivo 12 by a second member of the study team. After the initial coding of each transcript, all codes were refined and organised into a common thematic framework which was agreed with all researchers. The data from the focus groups are presented in this paper.

Ethical approval was granted for the questionnaire via the HRA, study number 19/NW/0608. The focus groups were deemed to not need full ethical approval using the MRC research tool (http://www.hra-decisiontools.org.uk/research/) as there was no randomisation or change to treatment and findings would not be generalisable. Consent was assumed for those completing the questionnaire and taken verbally before recording the interviews.

Thirty-two PWH expressed an interest in participating in the focus groups; five were ineligible (non-UK n=3, not male n=1, not haemophilia n=1). Eighteen participants, age range 18–58 years (median 32.5 years) joined one of three focus groups lasting an average of 65 minutes (range 58–76 minutes), with five to seven participants per group. Nine who had expressed interest declined focus group invitations.

Thirteen focus group participants had haemophilia A (12 severe, 1 moderate), five had severe haemophilia B. There were no participants with inhibitors. The majority (95%) were currently treated with prophylaxis (only two participants had received primary prophylaxis) and reported few recent bleeding episodes. Further participant demographics are presented in Table 1.

Three main themes emerged: factors influencing the experience of pain (including 13 sub-themes mentioned 108 times), ways to manage pain (including eight sub-themes mentioned 86 times), and the impact of pain (including six sub-themes mentioned 32 times). These are reported in more detail below with direct quotes attributed to participants by study number (P1–P18) and reflect the views expressed by the participants. Table 2 summarises the themes and sub-themes discussed.

Pain is a well-recognised side effect of bleeding in PWH. Acute pain following bleeding and chronic pain is associated with haemophilic arthropathy and joint disease due to recurrent bleeds. There are many publications that address pain in haemophilia, with prevalence quoted at between 17–84% for acute pain and 46% for chronic pain ^[13]. Whilst many studies have assessed pain using validated assessment tools ^[7,14,15], few have interviewed PWH to ask about their pain and pain experience. In this study, we aimed to identify the impact of pain on PWH in the UK and to describe their perceptions about current pain management by haemophilia HCPs. We identified three key overarching themes: factors influencing the experience of pain, ways to manage pain, and the impact of pain. Participants reported longstanding pain memories, remembering pain being present from childhood following bleeds. This has been shown in other studies ^[10,16] and is known to affect long-term physical and mental health by impacting on daily routine and hobbies. PWH reporting higher pain levels have also been shown to report worse health using patient reported outcome measures compared to peers with haemophilia and no pain ^[17]. In our study even young PWH treated with intensive prophylaxis and apparently ‘good’ joint health reported pain. This is contrary to previous findings reporting that treatment adherence improved chronic pain ^[18].

Living with the memory of pain since childhood led many participants to state that they accepted their pain and just got on with living a life in pain. Participants were able to differentiate between the pain of acute bleeds, for which they would use factor infusions and analgesia, and that of arthritic damage, where they reported a reluctance to take analgesia but often did because the pain was unrelenting. This differs from the findings of Witkop et al., who showed that factor replacement was used 38% of the time for pain management ^[19]. Study participants who reported using opioid analgesia expressed concern that HCPs prescribed these drugs in a seemingly ‘free’ manner, with little if any discussion of other means of symptom relief, advanced pain assessment and/or management. They reported a dislike of pain assessment using pain scales, particularly those using faces to represent pain severity, saying that these felt meaningless as individual scores would mean different things to each individual with haemophilia. Di Minno et al. describe ‘limited validated guidelines’ for the management of pain in haemophilia and ask whether pain specialists should be part of the haemophilia team ^[20]. A recent study involving HCPs has described how haemophilia and pain management are discordant, with limited pain management options for PWH ^[21]. Whilst guidelines may help to improve pain assessment, they may not be the appropriate manner in which to address the physiological and psychosocial aspects of pain experienced by PWH ^[22].

Although there were some negative comments about HCPs in our study with regard to pain management in haemophilia care, conversely, participants shared positive comments about physiotherapists. There is currently limited evidence for the role of physiotherapy in addressing pain in PWH ^[15]. However, in our study, physiotherapists were stated as having been helpful in supporting PWH to be active (even with pain) and encouraging individualised rehabilitation and mobility.

Talking about pain was seen as important – sharing experiences with others with haemophilia was described as supportive for those affected but also educational for PWH in the future. Despite a call to action to prioritise pain management in haemophilia care, including development of haemophilia specific assessment tools ^[23], there remains a paucity of published in-depth data exploring PWH's views of living with current and past experiences of pain ^[24]. Addressing this is a role that patient support organisations should embrace along with patient education and support ^[25].

Acute pain in haemophilia related to bleeding is well recognised. Recurrent joint bleeding leads to chronic pain which becomes ‘normal’ for PWH who “become used to this kind of pain” (P7) and adopt it into part of their everyday life experience. HCPs are ideally placed to impact on this experience but according to study participants they seem to lack insight into the extent of pain and how to manage it beyond prescribing stronger analgesia, which PWH find unacceptable due to side effects. The social and psychological implications of chronic pain should be better addressed by the haemophilia multidisciplinary team. This includes increased awareness of pain in PWH and mechanisms for treatment beyond analgesia, such as physiotherapy and psychological support. This is an area ripe for future research and should include children and those treated with primary prophylaxis as well as those with obvious joint disease.

The participants in this study reported few recent bleeds; their chronic pain is related to joint damage which occurred in their pasts, pre- prophylaxis as standard of care. It is important to recognise this as we move into an era of even better treatment of haemophilia – the majority in this study were treated with ‘new therapies’ using extended half-life (EHL) clotting factors and bi-specific antibody treatments. We need to be cognisant that new therapies will not resolve old pain.

This is a small qualitative study of PWH in the UK. We have not tried to compare our results with other conditions/general populations as study groups would not be comparable.