Literature search results
A total of 3787 studies were identified (Figure 1). These were imported into Endnote X8 and the full texts of 95 papers were retrieved and screened for inclusion. 32 studies fulfilled the inclusion criteria and were included in the review (Table 2)
[Figure 1. Study identification and selection process]
[Table 2 to be inserted here]
The 32 studies were published from 1983 to 2018 and conducted in 11 countries: United Kingdom (10), United States (8), Canada (4), Sweden (3), Australia (2), Finland (1), Germany (1), Ireland (1), Mexico (1), Norway (1), and The Netherlands (1). Interviews were conducted in 29 studies, with 9 studies also combining interviews with focus groups, observations, questionnaires, researcher journaling, visual depictions and diaries. One study collected data using only social media posts, one using blog posts and one using focus groups.
In total 536 young people with JIA or CIPS participated in the studies, of which 509 had a diagnosis of JIA, and 27 with CIPS. Although all included studies reported adolescent’s experience of pain, only 19 studies provide insight into the impact of fluctuating pain on an individual’s life and lived experience. 21 studies included data from parents, siblings, health professionals and/or individuals with other chronic illnesses, but efforts were made to only extract data referring to or expressed by adolescents with JIA or CIPS. Ages of patients ranged from 3 to 23 years, but all studies averaged within the adolescent range.
Comprehensiveness of reporting
The comprehensiveness of reporting was variable, with studies reporting between 10 and 28 of the 32 COREQ-checklist items (Table 3). 25 studies provided details on at least 50% of the criteria.
[Table 3 to be inserted here]
Synthesis of studies
Three themes emerged from the synthesis: symptom experience; disruption and loss; and regaining control. These themes can be seen to describe a journey through which the adolescent experiences fluctuating pain and associated symptoms, encounters the challenges to lifestyle that fluctuating pain presents, followed by employing coping strategies to regain a sense of control. This journey of experiencing pain is not a linear process and the themes are not mutually exclusive. Each stage is experienced differently depending on individual factors such as developmental status, pain condition, and duration of the pain experience (Figure 2). Quotations corresponding to the themes are shown in Table 4.
[Figure. Themes illustrating adolescents’ experiences of fluctuating pain]
1. Symptom experience
1.1. Fluctuating pain
Adolescents with JIA and CIPS identified pain as the defining characteristic of their disease (30–32). The intensity of pain was described as both constant and varying, such that, although pain was constantly present and experienced at a daily background level, there were periods of heightened perception of pain intensity which could occur regularly or with no logical cause (33,34).
In one study of CIPS, participants referred to these high levels of pain as ‘pain attacks’ or ‘flare-ups’, which could occur several times a day and last for hours (33). Similarly, the term ‘flare-up’ was used in seven JIA studies, though this time referring to periods of acute disease (30,35–40). Little differences were observed between the experiences of disease and pain flares, with both reported to negatively impact adolescents’ lives, including their level of confidence, identity and independence in daily living and social activities (36). They were unpredictable, with participants not knowing what activity might cause intensified disease or pain (33,36). Participants described rest, lying down, avoiding activities and using aids such as crutches to manage flares, but when suspected to be a disease flare in JIA, the hospital team were contacted for advice and for potential change in medication (33,37–39).
1.2. Causes of fluctuating pain
Adolescents reported feelings of uncertainty about why pain increases (32,34,41,42), but expressed interest in knowing what precipitated them and how to prevent them (30). In JIA, increased pain was sometimes indicative of disease activity (31,43,44), particularly among older adolescents who believed pain to be caused by their internal pathology (e.g. inflammation) (44). Younger children showed greater difficulties understanding and conceptualising their pain (43,44) which may be due to cognitive immaturity and not having learnt how to label their experience (36). In other cases, the cause of increased pain was not perceived by them to be the arthritis, but a result of their behaviour (e.g. overdoing activities), emotions (e.g. stress) and external factors (e.g. cold weather) (32,38,42). For others, particularly young people with CIPS, they were unable to relate the pain to any specific event (33), with pain suddenly occurring “for no reason” (32). This unpredictability and uncertainty of not knowing what might cause intensified pain appeared to be a source of concern for adolescents with both arthritis and CIPS (32–34).
Pain and fatigue were found to enter into a feedback loop in which an increase in pain at night disturbed the quantity and quality of adolescents’ sleep and led to fatigue in the morning (45,46). Daytime fatigue, in turn, was found to exacerbate pain which further disrupts sleep at night (46).
1.4. Psychological distress
Adolescents’ lives were controlled by the uncertainty related to pain (45). In periods of stability, adolescents saw themselves as normal (47), but getting new pain, or having changes in pain, could lead to feelings of sadness and anxiety especially when it struck with no logical cause (48,49). As a result, young people described emotionally oscillating between feelings of hope to feelings of despair that they had lost control of their pain (49). It was difficult for young people to be spontaneous which compromised their ability to engage in daily activities and set them apart from their peers. This had a considerable emotional and psychological impact on adolescents as it led to feelings of isolation, difference, and decreased competence (50).
2. Disruption and loss
2.1. Restricted body
All adolescents have to learn to live with a restrictive body which can be described as ‘feeling like a ticking fire bomb’, where suddenly ‘the worst case’ could happen (45). They have to put adjustments in place throughout the day to accommodate and reduce the event of an increase in pain (51), however, this increased feelings of being different from peers and set up barriers to what they want to do (51).
2.3. Loss of social life
During times of pain worsening, adolescents describe difficulties engaging in normal social experiences such as sports and going to friends’ houses (32,34,40,50,52,53), and report impacts on school attendance, concentration and their engagement in school activities (32–34,38,45,49). Being able to participate in education and leisure activities were seen as features of normal adolescence, and an inability to do so may lead to feelings of being different, social isolation, and difficulty building and maintaining an extensive social network (45,54–57).
2.4. Future uncertainty
Adolescents expressed concerns about how long the pain would last, when it would recur, how it would affect their plans and the long-term prospects of requiring medication (32,37,41,49,50,58). Concerns about the future appeared to be more signiﬁcant for older compared with younger adolescents (37,49–51,58) who may be more aware of the implications on their lives due to greater abstract thought in middle to late adolescence (44). Concerns were also expressed more in those who had experienced pain for a shorter period, suggesting that those with longer pain duration may normalise their experiences and learn to adapt their lives to their pain (50).
2.5. Disbelieved and stigmatised
Adolescents faced issues with legitimacy with peers (37,50,59), teachers (37,49), siblings (52) and professionals (33,41,50) when there were no visible signs of pain or disease. Particularly among adolescents with CIPS, when professionals were unable to find an organic cause to their condition and referred them to a psychologist, they felt that the pain was perceived to be imagined (33). This was further compounded by the unpredictable and fluctuating nature of pain, which makes it difficult for others to understand the nature of pain (37,45,60). Individuals took steps to control feelings of stigmatisation, such as choosing not to disclose their pain as they perceived that nobody would understand or believe them (39,54).
3. Regaining control
3.1. Medication and therapies
The fluctuating nature of pain was problematic because it could affect adolescents need for medication and other therapies (48). Pharmacological treatments were often the first treatment option for pain but could be painful if given by injection, last only a short duration, have unpleasant side effects, and interrupt daily routines (32,45,51). Among the non-pharmacological measures, physical activity was mentioned the most, with some deeming it to be effective at relieving pain and stiffness (49,53,59,60), while others believing it to be an aggravating factor of pain (38,43). Those that viewed physical activity as an aggravating factor of pain were more likely to be newly diagnosed with a pain disorder (150), who may not understand that physical activity does not provide immediate pain relief but it is more effective in the long term, as recognised by adolescents who have greater experience of the disease.
3.2. Social support
Positive social support appeared to have a buffering effect on coping with pain (45). Adolescents relied heavily on their parents for practical and emotional support when they experienced severe pain (33,61), and turned to friends for helping them maintain a sense of normality (45,47). Schools were important for providing practical assistance by means of excusing adolescents from physical education, allowing them to photocopy notes, stay inside during cold weather, and providing extra time to get to their next class (33,37,49,51).
3.3. Adapting behaviours
Older adolescents and those who had experienced pain for longer come to accept that pain is a part of their identity and adapt their lives to the condition (40,50,62). To deal with challenges related to unpredictability, they report employing behaviours to resist or accommodate pain including rest, pacing, planning activities and using carefully structured daily routines (32,39,43,45,50,53,54).
3.4. Cognitive strategies
Acquiring knowledge and understanding symptom manifestations were critical aspects in learning to effectively cope with the pain, and helped overcome feelings of helplessness and distress when increased pain was experienced (37,45,60). Engaging in positive thinking also helped minimise the signiﬁcance of pain (34,47,51,58,62), as well as mentally preparing themselves for pain when they ‘overdo’ activities (32,45), and ignoring the pain since ‘there is nothing you can do about it’ (47).
3.5 Avoiding pain
Concealing or minimising pain was common, where adolescents refrained from telling their parents about pain to prevent them from becoming anxious or from hassling them (33,50), and from their peers in order to maintain a positive social identity (34,40,41,45,51,58). Those who were more likely to conceal their pain were in the early to middle phases of adolescence (39,40), while older adolescents were more open about their condition and avoided activities they perceive as potentially pain triggering (51).
[Table 4 to be inserted here]