The effects of traumatic pain memories on current pain experience in men with hemophilia

ABSTRACT

Hemophilia is a bleeding disorder characterized by recurrent bleeding into muscles and joints. Many people with hemophilia experience multiple traumatic painful bleeding episodes, meaning that pain is often a significant problem for people with hemophilia, with a potentially high prevalence of posttraumatic stress and posttraumatic stress disorder symptoms. Current pain treatments are often ineffective and do not consider pain memories, which are experienced by almost half of people with posttraumatic stress disorder and which has not been explored in people with hemophilia. To fill this gap, 14 semistructured interviews with men with hemophilia were completed between November 2022 and January 2023 to explore their lived experiences of pain relating to their hemophilia. Data were analyzed using reflexive thematic analysis. Three overarching themes were constructed from the data: “trauma histories,” “pain management,” and “impact on the present.” Findings show that pain experienced by people with hemophilia is complex and does include a memory element for many individuals. Pain memories are clear and vivid and include visual, somatic, and emotional elements in intricate detail. Pain can also be experienced in the present when experiencing a pain flashback, and pain in the present can be a trigger to recalling and re-experiencing pain memories from the past. Self-taught active dissociation from pain, imagery, and distraction were described as useful pain management strategies. Findings from this study have implications for clinicians and service providers because current pharmacological and physical pain management techniques may be ineffective when pain memory is involved. Future interventions should consider how pain management is advanced for people with hemophilia and how hemophilia services become trauma informed.

Introduction

Hemophilia

Hemophilia is a bleeding disorder that causes low or absent levels of a blood clotting factor, and as an X-linked inherited disorder it affects over 1 million men worldwide.¹ It is characterized by recurrent bleeding into muscles and joints, causing acute pain at the time of the bleed and then subsequent chronic pain from damage to the joint lining and destruction of the articular cartilage and underlying bone, termed “haemophilic arthropathy.”² Treatment for hemophilia is predominantly through intravenous injections of replacement clotting factor. This is given either on demand, when an injury or bleed occurs, or, in some countries, prophylactically; for example, every other day. Prophylactic treatment for hemophilia will reduce bleeding, with annualized bleed rates on current treatments available in the UK being reported as ranging from 0 to 21.^3,4 As such, it is effective in reducing acute pain related to bleeding, but it will not affect or reduce joint damage and chronic pain experienced through this mechanism.

Hemophilia and pain

Chronic pain is a dominating and challenging experience for people living with hemophilia (PWH), influencing their behavior choices and activities.⁵ Fifty percent of PWH report experiencing chronic musculoskeletal pain, and between 35% and 50% report that current pain treatments they have tried are not effective, with significant debilitating personal and health care resource impacts.⁶ Identifying effective treatments for chronic pain is one of the top ten research priorities for PWH.⁷ Currently, factors that drive and influence the chronic pain cycle in PWH are poorly understood.^5,8,9 Musculoskeletal bleeding into muscles and joints causes acute pain, and, as such, many PWH have an ingrained belief that pain equals bleeding, leading to a complex relationship between the symptom of pain and the possibility of that pain being caused by bleeding.^5,8,9 Pain is also a personal experience, viewed by some PWH as part of how they identify with their condition.⁸

Trauma in hemophilia

Understanding the chronic pain cycle in PWH is further complicated by the potential for their pain experiences to be impacted by previous experiences of trauma and stress. Prophylaxis with regular clotting factor for the treatment of hemophilia was introduced in the UK for children in the 1990s, and it was not recommended for adults until the late 2000s. This meant that adults over the age of 30 with severe hemophilia did not have adequate factor levels to prevent bleeding before this time and, as such, experienced multiple repeated painful bleeding episodes during childhood and into adulthood. These repeated painful events, particularly during childhood, would often involve emergency trips to hospital, in-patient admissions, and long waits for treatment, which would then need to be administered through insertion of a needle into a vein. As such, these events were potentially psychologically traumatic and stressful for individuals, leading some to suggest that PWH may experience posttraumatic stress disorder (PTSD). PTSD can cause intrusive reexperiencing of the traumatic event, persistent avoidance, and increased arousal, hypervigilance, irritability.¹⁰ Stahl et al.¹¹ used the PTSD checklist for the Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition, as a screening/diagnostic tool and collected information from 178 adult PWH regarding past hemophilia-related hospitalizations, stressful episodes, and features of participants’ hemophilia that had caused significant distress. Results demonstrated a high prevalence of PTSD and posttraumatic stress symptoms, which increased with hemophilia severity. Twelve percent met the criteria for a provisional diagnosis of PTSD, the majority of whom had not received this diagnosis through their routine clinical care.¹¹

Pain memories

Pain memories can include all aspects of an individual’s recollection of painful events experienced in the past, with pain flashbacks specifically indicating the reexperiencing of pain in the present through memory processes.^12,13 Our understanding of how pain may be experienced through memory processes without a physical cause is still limited and is best conceptualized within the dual representation theory.^12,14 Evidence that people can experience a sensation of pain in the absence of an external nociceptive stimulus has been presented as case studies,^15–17 qualitative interviews,¹⁸ and cohort studies.^13,19 Schreiber and Galai-Gat¹⁵ stated that re-experiencing pain from a previous traumatic event can have a significant impact on the pain symptoms experienced in later years. More recently, Macdonald et al.¹⁹ reported that almost 50% of people with PTSD experience pain flashbacks. When pain memories are encoded under conditions of extreme stress, patients with PTSD can experience pain through a mechanism likely to involve memory rather than nociceptive pathways, which may elicit the experience of pain in the absence of a physical cause.¹⁹ This raises questions for understanding trauma in hemophilia, in the context of previous traumatic experiences, and as an ongoing issue with potential for re-traumatization with ongoing interaction with services. Traumatic memories may trigger pain in the form of pain flashbacks, and pain from a current nociceptive stimulus may also trigger memories of trauma.¹⁸ Many PWH have multijoint arthropathy and will experience chronic pain from mechanical and inflammatory processes, but pain from different sources is not mutually exclusive and may co-exist with one another.²⁰ The presence of chronic pain from any source may act as a trigger for the reexperiencing of previous painful events, adding an extra dimension to the chronic pain cycle.

Pain management in hemophilia

To date, these factors around trauma, pain memory, and pain flashbacks have not been considered in terms of pain management for PWH. Instead, current pain management guidelines by the World Federation of Hemophilia recommend pharmacological interventions and orthopedic surgery,²¹ focusing purely on physical and anatomical factors. However, in other areas, there is a clear shift in how chronic pain is managed and assessed. For example, guidance recently published by the National Institute for Health and Care Excellence²⁰ highlights the importance of social factors (including deprivation, isolation, lack of access to services), emotional factors (including anxiety, distress, previous trauma), expectations and beliefs, mental health (including depression and post-traumatic stress disorder), and biological factors influencing the experience of pain. There is a clear need to consider the physical, social, psychological, and functional ramifications of a life lived with pain in PWH.⁶

Study aim

The aim of this study is to provide an understanding of how memories of pain affect how current pain is experienced, understood, and managed in PWH.

Materials and methods

This study used a qualitative approach to capture the lived experience of participants in relation to their pain memories.²² The design and implementation of this study was undertaken in collaboration with a patient and public involvement (PPI) group made up of three men with hemophilia and the lead researcher. For the purposes of the interviews with PWH, input from the PPI group was key in finalizing the wording of the recruitment information, the participant information sheet, and the interview questions, as well as recruitment strategies and criteria and confirming safeguarding procedures. A pilot interview was conducted with one member of the PPI group, and results and manuscript drafts were also reviewed by all members.

Recruitment

Recruitment was coordinated through The Hemophilia Society, the UK-wide charity for people with bleeding disorders. Hemophilia is an X-linked inherited disorder, meaning that it is predominantly men who are diagnosed with it. Men over 18 years of age with a diagnosis of hemophilia who had experienced a physically painful event associated with their hemophilia and who continued to experience current pain from their hemophilia were invited to participate in this study. Information about the study was posted on The Hemophilia Society website and social media platforms on November 15, 2022, with a link to the study information sheet and an online consent form. Participants completed the consent form online, which included self-identifying themselves as meeting the inclusion criteria:

1. Diagnosis of hemophilia

2. Male

3. Aged 18 years or older

4. Physically painful experience connected with their hemophilia

5. Current pain associated with their hemophilia

6. Willing and able to give informed consent for participation in the study

Participants

Twenty-one eligible participants completed the consent process between November 15, 2022 and January 5, 2023. Seven participants did not respond to further e-mail correspondence regarding arranging an interview. The remaining 14 participants were all offered and completed interviews. Participants were aged between 21 and 71 (mean 41.5). The information held within this sample relevant for the aim of the study was assessed by the authors as having adequate information power to develop new knowledge.²³

Data collection

Data were collected via semistructured interviews online via Microsoft Teams by A.W. between November 2022 and January 2023. Interviews lasted between 19 and 91 min (average 50 min) and were recorded and transcribed verbatim (A.W.). At the beginning of each interview, the study rationale was explained and consent was revisited and given verbally. Following an interview topic guide, participants were then asked about their experience of pain from their hemophilia, what they thought caused and affected their pain, and how they felt when they experienced pain. They were asked to describe any specific painful experiences that were particularly vivid, how they felt while talking about these experiences, how they thought previous experiences of pain influenced how they felt pain now, and whether they were reminded of painful memories when they experienced pain now. The full interview guide is shown in the Appendix.

Data analysis

Reflexive thematic analysis was used to analyze the data.^24–26 This is an approach to a qualitative analysis that emphasizes the researcher’s subjective interpretation and role in analyzing data. This then enabled the main researcher’s clinical background working in hemophilia to be utilized when engaging with the data and the research question to explore these lived experiences. The inductive reflexive thematic analysis approach utilized the six phases described by Braun and Clarke:^24,25 familiarization with the data, generating initial codes, searching for themes, reviewing themes, defining and naming themes, and writing up the analysis. Data from the interviews were transcribed using NVivo after the completion of all interviews. Coding was completed systematically, working through each transcript in turn. An Excel spreadsheet was used to collate codes and store extracts. From this data set, themes were conceptualized and revised over several months, which included regular meetings with the supervisory team and with the PPI group (see the Rigor subsection). Pseudonyms were generated for the purpose of dissemination.

Ethical considerations

Ethics approval was gained from the University of Winchester (RKEEC220407). The interviews involved exploring personal experiences of previously painful and possibly psychologically traumatic events. Becaause this was potentially distressing, sources of support for participants were detailed in the participant information sheet and were reiterated to participants at the beginning and end of the interviews. Participants were also encouraged to only share what they were comfortable to, and they were told that the interview could be stopped at any time. If a participant disclosed a safeguarding concern, the interview would be stopped, and this concern would be discussed within the wider research team to work out how this could be safely taken forwards. During the 14 interviews, this did not occur.

Rigor

Throughout the research process, there was frequent reflection and discussion on the rigor, quality, and usefulness of this research among the study team (A.W., D.G., M.H., D.S.). The four criteria as described by Yardley²⁷ were used as a framework: sensitivity to context (e.g., using the main researcher’s clinical background in hemophilia; review and discussion of initial codes and themes with the PPI group), commitment and rigor (e.g., a reflective diary was kept by the main researcher; regular meetings were held between the study team to discuss and refine themes), transparency and coherence (e.g., full process documented throughout each stage using electronic highlighting of codes in transcripts, Excel spreadsheet, and diary of theme development; continued review of theoretical process throughout), impact and importance (e.g., dissemination of background and initial themes through meetings with clinicians working in hemophilia services and patient meetings with PWH; feedback from PPI group throughout). To ensure confidentiality of participants, no personal identity details were provided to other members of the research team, and interview content was only shared after names were redacted.

Results

Three themes were constructed from the data: “trauma histories,” “pain management” and “impact on the present.” Each of these is described in more detail below.

Trauma histories

The trauma histories theme explores participants’ detailed histories of repeated potentially traumatic experiences in childhood, as well as how these are remembered and experienced in the present. PWH interviewed for this study described many events associated with their hemophilia and hemophilia treatment in childhood that fit the criteria for being potentially traumatic due to threatened death or threatened serious injury,¹⁰ with pain identified as a specific factor in these events. For most, this was associated with the pain of musculoskeletal bleeding episodes, which were often described using terms such as “excruciating,” “horrific,” “agony,” and “brutal,” and many remember screaming in pain as a child. Many participants had little relief from the intensity of the pain, which they described as “inescapable” and “all consuming.” This was a regular occurrence for many; painful bleeding experiences were common, and spells in hospital were frequent. The repetitive and continuous nature of these experiences was difficult for participants to cope with. For example:

It was just just horrible, horrible, horrible for years and years and your whole childhood, you know, from being sort of six, seven years old right through to leaving school, is just being in pain. That was it. There was very little respite, maybe it’s because of the number of target joints I had, but, yeah, there was no respite at all. (David)

Bleeding could start from an injury or often with no obvious cause. Many PWH interviewed for this study described being at home or school when a bleed started and then having to travel a long distance to get to hospital, without any pain management treatment during this period. The intensity of the pain during these journeys was remembered, and participants also recalled their feelings of frustration at the time due to the elongated process to receive treatment. After the journey to get to hospital, treatment was not always immediately given. Often staff in emergency departments would have little knowledge of hemophilia, how a bleed would present, and the treatment needed to help. Participants said it would often take a significant amount of time for the appropriate treatment to be administered, during which they would be in increasing pain as the bleed intensified. This would mean that participants would be in significant pain—the body’s natural warning signal to harm—in a chaotic environment with no control.

In addition to repeated musculoskeletal bleeding, people with hemophilia interviewed for this study experienced other forms of bleeding. Nosebleeds, excessive bleeding after dental extractions, and throat bleeding after tonsillectomy were all described in a way that could be interpreted as traumatic. Memories of hemorrhages were vivid and still carried emotion. For example:

I can go back to the day—I can literally probably give you verbatim day-by-day by minute-by-minute what happened. … I’d been not long out of hospital, running around, and things and felt unwell. Had to go back to the house, folks weren’t about. And I had a massive haemorrhage, I was basically—blood, it was everywhere. It looked like someone had been killed in our house. So it’s quite a traumatic thing to remember. … Yeah […] it was the total unaware of what was happening, because I was actually I was nearest to the point of choking because there was actually—I remember the lumps of blood clots the size of golf balls coming up through my throat. I redecorated the curtains in the living room, redecorated the hall, redecorated the kitchen with my blood. (Simon)

Treatment for any bleeding episode would usually require an intravenous infusion and therefore a needle inserted into a vein. Many participants remember specifically the aspect of needles, knowing that this had to happen and feeling like there was no choice for them in this. Some participants described vivid memories of being out of control of the situation, of being in pain and being held down to have the needle inserted. It was recognized by participants that these repeated experiences with needles were psychologically traumatic, and there was an understanding in the present of how horrific the situation must have been as a child. For example:

As much as I can say now, it’s not that traumatic, I think I look back at it when I was very young and I was, like, it’s pretty bloody traumatic. (Luke)

For many participants, “pain memories” could be recollected in detail. It did not matter that painful bleeding events may have happened many years ago; the memories of these events were still clear and vivid. People would be able to describe events as if they had happened yesterday, expressing that it would be “clear as day.” They were able to visualize where they were, who was there, and details of the pain they were experiencing. These pain memories would include this visual information as well as sensation and emotion in intricate detail. Participants’ descriptions of these events indicate an alteration in memory processes, to enable this level of detail to be so clear to them so many years later. For example:

But yeah, I can almost picture, like it’s a photograph, these painful bleeds, alternating ankles for a number of weeks, with this frame in the bed because I couldn’t bear the bed clothes touching them, and riding in the back of my mum’s car, because it was just a way of getting me out of the house. Yeah, it’s interesting how it kind of fixes in your memory. (Mark)

Importantly, previous pain experiences informed what an individual would expect from the experience of a bleed or pain moving forwards—they became “imprinted.” This meant that, at the start of a bleed, an individual would immediately think of previous traumatic bleeding experiences and that would therefore create anticipation or dread of what was to come. Likewise, pain from arthropathy or other causes could trigger memories of pain and of previous bleeding events and cause both the recollection and re-experiencing of previous pain. The pain itself that was experienced in the present differed, however, from the pain experienced in the past. It was not felt to be a “physical” pain but a more complex psychological and physiological pain. It was very much described as a memory of that pain, which was far more complex than just the physical pain. For example:

That’s a pain memory, because if I do something with my right arm, I remember instantly the pain—instantly. If I pick something up, there is an instant reaction in my in my brain that says, “Don’t, that’s going to hurt.” And the pain comes back, the pain memory comes back straight away. (David)

These experiences of pain linked to memory were sometimes described by participants as flashbacks. Flashbacks for some participants could also include vivid images of where the person was and who was with them, as well as how they felt, the emotions they were going through, and the sensations, including the pain, they were experiencing. Significantly, somatic bodily pain in the present could be felt during these flashbacks. For example:

And what that pain memory brings is flashbacks, weirdly. … And then there’s an image in your head that is of a bleed, not anything specific. Just it can be random ones or random times or random places. And I can remember exactly where I was. And it’s a weird thing that your brain does. But I can see and feel the pain. I can feel it. (David)

I could flashback to dozens and dozens and dozens of different memories, of a particular elbow bleed. I can visualize one—sat downstairs here early hours of the morning just screaming in pain. … I just think it’s a trigger in your brain, just shows how good the brain is and how clever it can be at times that you can see things. Like I said. I can see a classroom, vividly, a music classroom. and experience that pain. And I can see all the kids, it’s so, so bizarre. (David)

Pain management

Perhaps unsurprisingly, a central theme of the interviews was about pain management. Many spoke of “medical management,” which relates to the most frequent pain management technique they had been offered: painkillers. This was often seen as a negative and destructive technique that many people chose to avoid. Having another option other than painkillers in the management of pain was identified as something that was both wanted and needed by participants. It was suggested that nonpharmacological options should be offered and that hemophilia “comprehensive care” should incorporate specialist knowledge of chronic pain.

It was just a lot of painkillers. Especially when it was lots of bleeds or big bleeds, they’d just give me painkillers, and that was the main way of dealing with it. Now I’d probably try and distract myself more from it. … It would be nice to have something that isn’t, just, like a painkiller. (James)

In contrast to this, “self-management” was the main way people coped with pain and highlights learned methods of managing pain in the present. Because conventional pharmacological means were often found to be inadequate or were refused, many participants had to learn how to manage pain themselves. The strategies used for this were varied but included a learned alteration in pain tolerance, self-taught active dissociation from the pain, and clinician-taught imagery and distraction techniques.

A learned alteration in pain tolerance was a key focus for participants in this study and was most often discussed in relation to the terms “pain tolerance,” “pain threshold,” and “pain gauge.” Pain was often described as being individual rather than relative to that of someone else, and pain gauge was felt to be a more appropriate term to use than pain threshold. This pain gauge was described by participants as an internal mechanism that was built up or altered over time and experience. Many participants felt their pain gauge had been altered from experiencing high levels of pain, which then meant that they had a much higher capacity to manage their pain in the present. An alteration in an individual’s pain gauge was seen as both a body and brain reaction and a direct result of having experienced repeated episodes of significantly painful events from a young age.

I would speculate that if you had high levels of pain, then low levels of pain are considered much lower. So you could call it a pain threshold, but I don’t really like that because it infers that you’ve got a higher pain threshold than someone else, I don’t think that’s necessarily true. … But you also have conditioned your body to it, probably. So your body is used to it. And your brain is perhaps used to it, so your brain is used to still being able to function with a certain level of pain all the time. (Andrew)

Loss of memory was also a common feature of the interviews, as participants described how, on occasion, previous events were blocked out in their memory of the past. It was explained that this was a way of coping as a child and a method subconsciously used to manage the difficult and painful situation they were in. It was also rationalized in the sense that these memories were not something the individual wanted to hold on to. This could be large chunks of memory or certain parts of specific memories. Memory loss during what have been described as potentially traumatic experiences can be seen as another aspect of trauma response and consequence that was not always identified as such by participants.

I don’t remember any—in terms of pain and that sort of thing—I don’t remember any of that before the age of about 12. I must have—I must have been in pain, I suppose, because I had joint bleeds and things like that, but I honestly don’t remember it. (Andrew)

One participant described actively learning to dissociate from pain. This was felt to be something that had developed over time as a way to manage and cope with being in pain regularly. Initially, it was a technique that was more reactive. However, it was then actively developed to manage other sources of pain. It was very specific in how it worked for the individual but highly effective and was a longer-term pain management technique that they could use:

I actively will think about a sort of complex problem or a complex issue, something that will take a lot of focus and concentration. … You’ve got to think about it in a very engaged brain—frontal brain type way. That’s what I do. And all this stuff that I think about going into a happy place, like dreamy, like saying, imagine you’re lying on a beach. I find that doesn’t work because it’s not enough for me to focus on. It’s not a strong enough thought to overpower feelings of discomfort. It needs to be, for me, a really complex, brow-furrowing topic for it to successfully enable me to disassociate from whatever the discomfort is. (Mark)

Another participant had been taught a pain management technique by a clinician as a child. It used the concept of imagery and distraction, and he reported that this had continued to be useful to him throughout his life. For example:

She was as desperate as I was. She it was, like, we’ve run out of ideas. And she taught me this pain management thought process, which I still use. I still use it almost 50 years later. And it was very simple. It was just imagining another part of your body. And then that part of the body was in extreme heat or extreme cold. So I always chose the heat. But you imagine in my case, if you’ve got a hip that’s really painful, you imagine an arm that is sitting in really hot sun or something like that. And you can literally imagine—you can literally feel that heat after a little while. But what it’s actually doing I suppose it’s just focusing the mind on another part of your body. And every time the pain comes back, you have to refocus and do it again. (Andrew)

Living with pain every day was likened by one participant to living near a train track; that is, they were able to focus in or focus out of this “pain noise.” Whether this was a technique that had developed over time or whether it was something the participant had consciously learned to do was unclear. It was seen, however, as a positive way to manage pain on a day-to-day basis.

The kind of pain that I feel a lot of the time, I have a lot of the time, and it becomes a kind of—it’s almost like living with a train track at the bottom of the garden. You’re aware it’s there, but you don’t particularly notice it at the forefront of your mind every second of the day, but you’re aware that it’s always there. And you notice it when something unusual happens, like the train’s going slowly or it stops at the bottom of the garden and it’s more noisy, you would notice. So I sort of don’t particularly—well, I do notice the day-to-day pain, but it’s—what I do notice more are changes in my mood or changes in my sleep pattern or changes in my productivity at work in terms of how easily I am distracted or not is things like that. (Mark)

The interaction between the brain and the body was often discussed by participants during the interviews. Participants felt that this was something that should have focus and effort put into it to help with understanding these interactions more fully. It was felt that if people can actively dissociate from pain or focus their mind elsewhere using techniques they have either learned themselves or been taught, services should be looking to develop and to harness this.

Impact on the present

This final theme describes how previous pain experiences have shaped how PWH approach and manage aspects of their hemophilia in the present. This was separated out into “facilitators to good health,” “barriers to good health,” and a potential bridge between the two of the positive influence of “talking.”

Facilitators to good health

What was clear across the interviews is that the impact from previous painful experiences was not only negative. Indeed, many participants felt that their experiences gave them an enhanced positive reflected in that previous hardship. That is, rather than focusing on the negatives, participants would focus on the positives, with a sense that “there is always someone worse off than you.” Some participants felt that their family and upbringing had taught them how to be resilient in those situations. Others felt this was a perspective on life that they had learned throughout childhood, as a direct result of the events they had experienced. Painful and potentially traumatic experiences were also a motivation to succeed and achieve. Some participants describe channeling those memories and using them to motivate themselves to make their life better. It was important to participants to not feel that their hemophilia had held them back in what they wanted to do in life. They did not want to feel or to be limited by pain or by hemophilia in any way, prioritizing what was important to them as an individual with no restrictions in their mind that it could not be achieved. It was recognized that some of the choices made when younger may have meant the participant experienced more bleeds and as a result had more arthropathy later in life. This was not seen as a negative with any sense of regret but was felt as a richer, fuller, positive part of life. For example:

Would I go back and change anything? No, I wouldn’t, because there’s things I’ve done I’ve loved that—I’ve loved some of the experiences I’ve had, even if they have meant that there’s things I can’t do now, because I don’t know what I’m missing now. If that makes sense, I’m happy, I—but I enjoyed the things I did do. (Mark)

Barriers to good health

However, though these impacts were often described in positive terms, it is also the case that previous ways of coping with pain can become ingrained and habitual and have a negative effect on social interaction and mental well-being. Indeed, many participants would manage bleeds by treating with clotting factor and then trying to ignore it. Whether an active, conscious decision to ignore the bleed and the trauma associated with it or a subconscious instinct to disconnect from the situation as discussed above, the technique of “ignoring” was commonplace. Some participants would actively avoid acknowledging the emotions they may be experiencing and would choose to focus on things outside of that. They would also deliberately put a barrier up to keep others out. This meant that participants would automatically then disconnect from others when in pain, whether this pain was from a bleed or not. For example:

We kind of build this little—put four walls around us, and no one comes in, and certainly in my mind, no one comes in. And this was like, so up until a few years ago, how I dealt with pain. If I was in pain, I told myself, I dealt with it. I got myself better. And I got on with my life and nobody else really saw what you were going through and what pain, how you are managing the pain. … I’m fine. I mean, it’s the two words that we use more than anything in the world. “I’m fine.” Right. But reality is, you’re in a really, really shit place. (David)

The impact of previous painful experiences and of then living with chronic pain in the present would lead some participants to feel very isolated. It was very much felt that no one else could understand what they were going through. The experience of having a joint or muscle bleed or experiencing the level of pain that having a bleed entailed was something that only another person with hemophilia could understand. Often participants would not divulge their hemophilia to others. The reasons for this were varied and included the association of hemophilia treatment with infected blood products, which had occurred in the past and could still be portrayed in the media. Some participants felt that people would view them as a victim, and they did not want to be seen in this way. Mostly, however, it was due to the energy it took to explain to people what hemophilia was and why it affected someone with hemophilia in the way it did. When managing pain daily, the extra energy required to do this was often just not there. For example:

It’s just easier to sometimes live that lie than to actually explain why you’re limping, because your joints hurt. And that is something I’ve struggled with for a long, long time. (Mark)

The impact of the pain and likely associated trauma experienced as part of hemophilia on an individual’s mental health and well-being was identified by many participants. Posttraumatic stress was specifically named and linked to the experience of pain memories, as well as other traumatic experiences associated with bleeds as a child. For example:

Pain memory and what you were going through when you sat in an ambulance, they would call it anxiety, followed by posttraumatic stress disorder, I guess. (David)

Importantly, though this impact on mental health was recognized, it was almost never discussed with the participant’s clinical team as part of their hemophilia care. In part, this was because experiencing repeated bleeds and pain was very much seen as a normal childhood for someone with hemophilia, and this normalization of potentially traumatic events may well have caused participants to feel that posttraumatic stress was not relevant to them. Indeed, many participants had vivid memories relating to their hemophilia and hemophilia treatment that were readily retrievable and, as such, could be interpreted as traumatic, but these were never spoken about. However, this was also related to the ways in which participants would ignore pain and put walls up around them, actively isolating themselves from others.

Talking

The coping mechanisms of ignoring and putting up a barrier were used by some for many years, and it was only recently that some participants said they had started to open up. One participant said they had attended an event hosted by The Hemophilia Society where they had spoken to other people with hemophilia, and they had found this to be helpful. It was identified as a way people could connect with other people who understood what they had been through, and this led to being able to talk more openly and honestly with others as well. Being given an opportunity to talk about previous traumatic events was identified by some as a way to help take down their barriers a little bit. For example:

Every time we do a call like this, you push an extra couple of bricks out. And that helps; it helps clear my mind and focus on actually what, you know, ’cause I am—I’m extremely, extremely lucky. … And I wish this had been around 25 years ago. It would—I—my life would have been different. (David)

Looking to the future, there was concern from participants that this apparent culture within hemophilia to both normalize these potentially traumatic experiences from childhood and therefore not discuss the impact of previous pain and trauma on the individual in the present would negatively impact mental health. It was recognized that this sort of talking was hard to do, and it could be easier to keep previous painful experiences in the box where they had been stored for many years. For example:

I’ll close that door and get on with something else, because if I open that door again, then, like this, it brings back some memories that, some are good, bad, some are—pain ones are obviously bad ones. Am I past it, am I past? You know, am I at a point where I don’t need to do it? Maybe. Maybe I am. (David)

There was an unknown and maybe an unease, where experiencing previous pain and bleeding events was normalized and the impact and lasting effects of that on daily life may not have been fully identified by participants. There was concern from some participants that many PWH may live with posttraumatic stress symptoms both in the present and into the future. Trying to break the stigma of talking, particularly within hemophilia communities, was seen as a priority. Participants identified that many PWH would be isolated from others because it is a rare condition. It may therefore sit with both hemophilia services as well as hemophilia support groups to potentially identify ways in which to make this happen. Talking was identified by many participants as a preventative measure to help with future mental health and posttraumatic stress for PWH.

Discussion

This study provides an increased understanding of how memories of pain can affect how current pain is experienced, how it is understood, and how it is managed by PWH. Our results show that pain is linked to trauma, and there is a flashback element that has not been described in hemophilia before. This study adds a valuable contribution to a small area of literature around pain memories and significantly adds to our understanding of the unique role of memory processes in chronic pain.

Our findings demonstrate the multiple impacts of repeated and traumatic experiences of pain for PWH in both childhood and adulthood. Our participants describe how pain memories remain clear and vivid in the present, including visual, somatic, and emotional elements, in intricate detail. In addition, they describe how such memories can cause the re-experiencing of previous pain in the present when triggers are encountered. This provides support for the idea that pain itself can cause PTSD (see Schreiber and Galai-Gat¹⁵). Moreover, it provides support for the idea that when memories are encoded under conditions of extreme stress, PWH can experience pain through a mechanism likely to involve memory rather than nociceptive pathways (see Macdonald et al.¹⁹). That is, pain can be experienced in the present when experiencing a pain flashback, and pain in the present from a current nociceptive stimulus can be a trigger to recalling and re-experiencing memories of pain from the past. Overall, this highlights how pain is not just physical but includes perceptual, emotional, and wider aspects that have a significant impact on the current pain symptoms experienced by an individual. If pain that PWH experience in the present could link with previous traumatic painful events and add a memory element to drive the pain cycle, this complicates and potentially impedes management if purely physical components are considered.

The foundational study by Schreiber and Galai-Gat¹⁵ describes a case study where the uncontrolled pain experienced was found to be the traumatic element and the stressor that led to the development of PTSD. This example where pain itself has caused PTSD can be directly applied to PWH, and it has been shown in this study that participants describe experiencing excruciating, prolonged, and untreated pain for many hours when unable to access factor replacement treatment after the onset of a musculoskeletal bleed. Alongside the trauma of the pain itself, often medical interventions and interactions with their medical provider are also psychologically traumatic for individuals.^11,28 How children in particular remember painful medical procedures affects their experience of pain and distress during subsequent procedures^29,30 and should be considered within hemophilia as a lifelong condition, to prevent ongoing traumatization and retraumatization of PWH when accessing care. Furthermore, the experience of trauma and the potential for pain memory to impact an individual should be considered more widely in other chronic pain conditions.

The experience of pain flashbacks stems from trauma-related memory processes and is best conceptualized in memory theory through Brewin’s revised dual representation model.^12,14,31,32 This model describes two systems within memory, the “verbally accessible memory,” which is accessible to deliberate retrieval and manipulation, and the “situationally accessible memory” (SAM), which is how extremely stressful events can be stored. The SAM system can give rise to involuntary PTSD flashbacks involving reliving the event as if it were occurring in the present. Brewin and colleagues explained that in conditions of extreme stress, there is a loss of hippocampal function, alongside an increased functioning of the amygdala and associated areas, which causes traumatic memories to be stored in rich detail without a date stamp. These events, including the somatic sensations, emotions, and images, can be re-experienced in the presence of triggers. Although the dual representation model is mostly associated with PTSD, it is important to consider the possibility that these SAM memory processes may be active for PWH without a PTSD diagnosis when previously traumatic events have been experienced.

Our results additionally highlight some interesting techniques utilized by participants in their management of pain. Active dissociation and distraction techniques were described as learned techniques that services should be looking to develop and harness. Research in other fields has found that animals and humans are able to inhibit pain by activating their endogenous pain inhibition system.³³ One of the participants in our study described an active method of dissociation, by thinking of a complex problem to manage pain. This links to research presented by Romero et al.³⁴ that looked at perceptual load, the psychological theory of attention. Romero et al.³⁴ found that a high perceptual load specifically reduced the intensity ratings of high-intensity nociceptive stimuli. They concluded that pain rating can be reduced by increasing the load of attentional resources at the perceptual level of a non–pain-related task, which may well be what the participant in our study is achieving. Distraction was identified as another useful nonpharmacological pain management technique by participants in our study. This has been studied by Valet et al.,³⁵ who found that distraction was associated with a significant reduction of the visual analogue scale ratings for pain intensity and unpleasantness and a reduction of pain-related activation in multiple brain areas. Guidelines for the management of pain for people with hemophilia need to incorporate the wider pain research fields and look to integrate more nonpharmacological pain management techniques.

Many PWH have experienced significant pain and associated trauma, which they perceive as part of a normal childhood for someone living with hemophilia. Trauma and pain were rarely discussed with health care professionals or with friends and family, despite talking being identified as being helpful. Many participants would ignore symptoms of pain and would put traumatic pain experiences into a “box” and keep a barrier up around them. Shorer et al.³⁶ presented an interesting paper on loneliness in war veterans with posttraumatic stress symptoms and pain. They suggested that solitude and avoidance can provide a place of safety and are often beneficial in the short term for someone who has experienced pain and trauma. They discussed how it is difficult for people to leave this place of safety and to move from emotional loneliness to emotional sharing but that, if this can be achieved, it is beneficial to share experiences with peers. This links with the data from our study and highlights the importance of both acknowledging that this type of work can be difficult for people and that clinicians, patients, and support groups need to work together to find a way for this to work within the hemophilia community.

Study strengths and limitations

The involvement of the PPI group throughout this research study is a key strength. Reflexive thematic analysis was used for this study, which enabled the lead researcher to acknowledge their influence and position from also having a clinical role working within hemophilia services. Thorough methodological rigor was used and was detailed earlier. This study, advertised through The Hemophilia Society, was not accessible to potential participants who do not engage with this charity. The sample does, however, provide a depth of insight that we feel captures well the lived experiences of PWH. Additionally, data collection was conducted online, which enabled a wide geographical area for recruitment. The data for this study were collected at the same time as the UK Infected Blood Inquiry was taking place. The potential impact of this on the data collection is not known.

Recommendations for future work

Further work is needed to explore clinicians’ perspectives on trauma and pain in PWH and how this is situated within hemophilia services. In addition, considerations on how a safe space for PWH to discuss both pain and trauma can be provided, as well as wider peer-to-peer support opportunities, are still needed. Further research in this area is needed to provide evidence and recommendations that will improve treatment and health and well-being outcomes for PWH.

Clinical implications

This research gives a greater understanding of the effects of traumatic pain memories on the current pain experience of PWH and the interaction between both physical and psychological factors that influence chronic pain. These findings are of importance to clinicians when considering current practice. Traditional pharmacological and physical pain management techniques may be ineffective when more complex centralized pain pathways, including a memory element, may be involved. In these cases, psychological treatments that address posttraumatic memory symptoms are likely to be beneficial. The findings show that clinicians need to be confident to discuss pain science and hemophilia services need to become trauma informed. It will be beneficial if clinicians and PWH can recognize the signs and symptoms of trauma and work together to prevent retraumatization with ongoing interaction with hemophilia services.

Appendix. Full Interview Guide

• Tell me about your experience of pain as a result of your hemophilia? (What has helped/what has made it worse?)

• When you experience pain now what do you think causes your pain or affects your pain?

• How do you feel when you experience pain? (e.g., body/physical sensations, feelings/emotions, hypervigilance, irrational anger and fear, panic attacks, flashbacks, digestive issues, feeling numb, nightmares, and exhaustion). Possible prompts: Do you feel irritable/angry? Do you feel watchful/on guard? Do you have other physical reactions like sweating, trouble breathing, nausea, or a pounding heart? Do you feel nervous, anxious, on edge?

• Do you have any of these feelings at other times?

• Do you have pain at the moment today? Where? Do any of your pains increase with prolonged sitting (60 min)?

• I would like to ask a few questions about previous bleeding episodes. I understand that talking about painful/traumatic experiences may be uncomfortable, and we can stop at any time.

• Can you tell me about a painful experience that particularly sticks in your mind/is particularly vivid? (Encourage as much detail as comfortable to give.) Possible prompts: How did it start? What happened then? How did it end? What happened then? Do you still think of that time/are you reminded of it? Do you have feelings about it?

• Are there other painful memories that particularly stick in your mind?

• The experiences you describe sound like they were very painful at the time. Can you tell me how your pain is now as you talk about those experiences? Confirm if different to the start. Other sensations in the body: chest, breathing etc? Can you tell me how you experience this now? How do your pain memories bring you into your body right now?

• How do you think your previous experiences of pain influence how you feel pain now?

• Are you reminded of painful memories when you experience pain now?

• I have asked all the questions that I wanted to for today. Do you have anything you want to add or anything you want to ask? Is there anything you were expecting me to ask that I haven’t asked?

Funding Statement

This work was supported by a 2022 European Association for Haemophilia and Allied Disorders (EAHAD) research grant awarded to Anna Wells.

Disclosure statement

No potential conflicts of interest are reported by the author(s).

Declaration statement

All authors report that there are no competing interests to declare. Ethics approval was gained from the University of Winchester (RKEEC220407). The authors confirm that all participants gave informed consent for participation in this study.