What’s in a name? Pain is real if you live with it

Chronic pain is defined as pain that lasts for more than three months – something that many people with a bleeding disorder can identify with. But they are not alone. Introducing its new guideline, Chronic pain (primary and secondary) in over 16s: assessment of all chronic pain and management of chronic primary pain, NICE estimates that as many as one-third to one-half of the UK population is affected, though not all want help or treatment. The task facing the bleeding disorders community is to recognise the need in those who do. Identifying people whose quality of life is negatively affected by chronic pain and collaboratively engaging with them to develop a way to best manage their pain is the preliminary step to implementing this guideline.

Chronic primary pain is defined as pain for which there is ‘no clear underlying condition or the pain (or its impact) appears to be out of proportion to any observable injury or disease’. The recently updated eleventh revision of the International Classification of Diseases (ICD-11) identifies this type of chronic pain as a disease in itself. This includes chronic primary musculoskeletal pain (such as chronic low back pain). Secondary chronic pain is caused by an underlying condition or disease such as osteoarthritis, rheumatoid arthritis, ulcerative colitis, endometriosis. When this pain affects the musculoskeletal system it is referred to as chronic secondary musculoskeletal pain.

This should not imply that secondary chronic pain can always be resolved by treatment of the underlying disorder: pain persists in the face of disease-modifying treatment for rheumatoid arthritis and the symptomatic treatment of osteoarthritis. This is observed also in people with haemophilia, whereby pain from haemophilic arthropathy exists without bleeding being present. Furthermore, primary and secondary chronic pain can coexist. As yet little is understood about the pain mechanisms in people with haemophilia living with chronic joint damage, and further work is urgently needed to better define what influences their overall pain experience.


Assessment of all types of chronic pain

One the most notable take-home messages from this guideline is that of the assessment process for those with chronic pain. Recommendations on assessment are intended to ‘inform a care and support plan by setting out a comprehensive person‑centred assessment of the causes and effects of pain and agreeing possible management strategies, including self‑management’. The person should be treated as an individual and supported so they can contribute to a collaborative partnership, recognising that a diagnosis of chronic primary pain may change over time.

It is important to understand how a person’s pain affects their life and how their life affects their pain. NICE provides several examples of what issues to address when talking to people about pain (Table 1). These conversations should be sensitive to the person’s socioeconomic, cultural and ethnic background and faith group, and how these factors might influence their symptoms, understanding and choice of management. Living with pain can be distressing and this should be acknowledged to the person with pain. For those healthcare professionals working with haemophilia or other IBDs, they should be mindful of the lived experiences of that person with their bleeding disorder, the access to and type of treatment over their lifetime as well as their experiences of previous healthcare interactions.


Table 1. Talking to people about their chronic pain

Ask the person to describe how chronic pain affects their life, and that of their family, carers and significant others, and how aspects of their life may affect their chronic pain ·      lifestyle and day-to-day activities, including work and sleep disturbance

·      physical and psychological wellbeing

·      stressful life events, including previous or current physical or emotional trauma

·      current or past history of substance misuse

·      social interaction and relationships

·      difficulties with employment, housing, income and other social concerns

Explore a person’s strengths as well as the impact of pain on their life. ·      their views on living well

·      the skills they have for managing their pain

·      what helps when their pain is difficult to control

Ask the person about their understanding of their condition, and that of their family, carers and significant others. ·      their understanding of the causes of their pain

·      their expectations of what might happen in the future in relation to their pain

·      their understanding of the outcome of possible treatments

When assessing chronic pain in people aged 16 to 25 years, take these factors into account ·      any age-related differences in presentation of symptoms

·      the impact of the pain on family interactions and dynamics

·      the impact of the pain on education and social and emotional development


The key to empowerment is understanding. People should be given the information and advice they need to interpret what they are told and make decisions about management. They, and their families, should know that symptoms fluctuate over time and they may have flare-ups for unknown reasons. They need to know that the pain may not improve or could get worse but that quality of life can be better even if there is no change.

Individuals should understand the balance of benefit, risk and uncertainty in their treatment options, including the management of coexisting chronic primary and secondary pain. The care and support plan, agreed with the individual and their family/carers, should address their priorities, abilities and goals; identify the useful things they already do; and adopt their preferred approach to treatment and the balance of treatments for multiple conditions. Young adults (age 16 – 25) may need support for education and training. A flare-up should prompt a review of the care and support plan to identify contributory factors and assess new symptoms.


Management of chronic primary pain

The NICE guideline focuses on management of chronic primary pain and as yet it is unclear how much of a clinical and individual issue this type of pain may be for people with haemophilia/IBDs. The current burden of pain observed in people in haemophilia appears to be predominantly chronic secondary musculoskeletal pain. As such it remains unclear if the recommendations made in this guideline for primary chronic pain can be taken at face value.

However, the basic premise of being supported to be able to move well and be more physically active in meaningful activities as well as providing psychological support should underpin any intervention. Evidence for effective pain management interventions, including pharmacological strategies, remains limited in PWH and other IBDs and there is much yet to do.

So, in summary, the NICE guideline recognises the importance of thinking of people with chronic pain as individuals for whom pain is a major part of their life. It emphasises their role in deciding which interventions to use, for which a collaborative and supportive relationship is essential. The guideline’s distinction between primary and secondary chronic pain remains a clinically vague notion and for some is arguably more academic than real, given that the two may coexist and that pain persists despite optimal treatment of an underlying disorder. Those working with people with bleeding disorders may find the guidance towards better pain assessment most useful to try and integrate into their practice.

Listen to Paul McLaughlin talking about pain on our podcast