A mixed-method systematic review evaluating interventions in paediatric rheumatology to address caregiver support and well-being

Abstract

Background

Paediatric rheumatology conditions are chronic autoimmune and auto-inflammatory conditions that affect predominantly the musculoskeletal systems of children and young people (CYP). About 6–7 million children are estimated to be affected worldwide. Having a rheumatological condition affects CYP as well as the wider family. Negative outcomes on psycho-social well-being, quality of life, family relationships and family functioning are commonly observed. The current review addresses interventions for caregivers of CYP with paediatric rheumatological conditions.

Methods

The Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) 2020 statement guided this review. Eligibility criteria were pre-defined and registered in the International Prospective Register of Systematic Reviews (PROSPERO). Articles were included if they (1) were targeted at caregivers of CYP with rheumatological conditions, and (2) included an intervention to improve well-being of caregivers.

Results

Of 1065 identified studies, 15 studies were included in the final review. A mixed-method systematic review was conducted, and included literature was assessed using the Mixed-Method Appraisal Tool (MMAT). Quantitative, qualitative and mixed methods studies, as well as review articles and abstracts investigating the effectiveness of caregiver interventions were evaluated. A third of the identified literature did not report on outcome measures. A narrative synthesis was employed to appraise interventions tailored at caregivers.

Conclusions

Despite evidence suggesting that a family approach is needed to support caregivers and CYP with rheumatological conditions as well as the wider family to improve health outcomes for the child, increase family functioning, reduce family conflict, and increase psycho-social well-being, only a small number of caregiver interventions have been carried out to date. The review highlights the need for caregiver interventions to be appraised to better understand what interventions yield results that lead to better quality of life for families who are caring for a child with a chronic rheumatological condition.

Trial registration

PROSPERO CRD42024524570.

Background

Paediatric Rheumatology conditions are chronic multi-systemic inflammatory conditions affecting predominantly the musculoskeletal system [1]. Six to seven million children are estimated to have a rheumatological condition worldwide [2]. Rheumatological conditions affect children and young people (CYP) in different ways due to a heterogenous presentation of symptoms with some children experiencing joint inflammation, pain, and/or fatigue [3], whilst others present with unexplained fevers or rashes [1] or even systemic involvement which impairs a child’s overall health especially when internal organs are affected [4]. Prevalence rates vary between conditions as some paediatric rheumatology conditions are common whilst others are rare. The most common condition is Juvenile Idiopathic Arthritis (JIA) which affects approximately one in 1000 children [4]. There are no known cures for paediatric rheumatological conditions, and available treatments aim to reduce as well as manage symptoms [5] with the goal to achieve inactive disease (remission) [6]. Chronic conditions (which include rheumatology conditions) have an impact on CYP’s physical and social life and are associated with significant stress as well as emotional and behavioural problems [7, 8].

Living with a rheumatological condition is often associated with frequent hospital appointments which has implications on education and schooling as well as peer relationships for CYP. Adapting to a new normal and learning to cope with a chronic health condition and treatment regimens causes challenges for CYP and their families. CYP with rheumatological conditions experience higher levels of pain, fatigue, physical disabilities and psychological problems [9, 10]. A retrospective cross-sectional survey conducted in the United States looking at data from 2008 through to 2013 as part of the Medical Expenditure Panel Survey (MEPS) found that CYP with a chronic physical condition were 62% more likely to have a diagnosis of a mental health disorder [11], while other research suggests that CYP with rheumatological conditions are 15% to 65% more likely of having a mental health disorder such as anxiety or depression [12]. The additional psychological burden on CYP in turn exacerbates adverse outcomes. Mental health disorders are associated with decreased quality of life [10, 13], increased pain and disease activity [9, 13], lower physical activity levels and reduced functional ability [14], greater functional disability [9], poor disease control [15], medication non-adherence [16] and transition challenges [17, 18].

Moreover, it is well established that the impact upon the wider family can be profound, as caring for a CYP with a rheumatological condition is challenging, especially for caregivers [19]. Research showed that 82% of caregivers reported that their child’s diagnosis had an impact on their own mental health [20]. Furthermore, caregivers feel that a diagnosis has major repercussions on their emotional well-being, social, economic, and work life [21]. Caring for a child with a rheumatological condition can affect a caregiver’s quality of life and their mood, and is associated with higher levels of worry and decreased family functioning [22].

Caregivers tend to experience a roller-coaster of feelings throughout their child’s diagnosis journey [23]. They often struggle to adjust to the ‘new normal’ after receiving a diagnosis [24, 25], and they experience feelings of sadness, denial [21], anxiety, and fear [26]. However, some caregivers have also expressed relief at receiving a diagnosis with caregivers having encountered disbelief relating to their child’s symptoms in the beginning of their journey which led to a loss of trust in health professionals [21, 25]. Feelings of anxiety, confusion and powerlessness continue throughout the disease journey as caregivers learn to accept their child’s life-long disease [23]. Additionally, caregivers often encounter negative feelings of frustration and fatigue relating to ongoing treatments, disease flares, pain and limitations to their child’s life but also admiration for their child’s resilience as well as feelings of hope when things are looking up [23].

One of the biggest challenges for caregivers is worrying about their child’s quality of life [21], due to a lack of understanding of the condition [24] which is exacerbated by a lack of information [26] and uncertainty about what the future holds [21, 25]. Some caregivers report that as the disease progresses, CYP can demonstrate challenging behaviours such as non-adherence to medication, with caregivers then struggling to find the balance with allowing their adolescents’ autonomy in relation to monitoring their medication adherence [24].

Another aspect impacting on parents’ caregiving abilities when caring for a child with a chronic condition is uncertainty. Parents have expressed uncertainty relating to their child’s condition and treatment management, however, the perceived uncertainty went beyond the medical aspects of their child’s condition. They also expressed uncertainty relating to their role as parents and their own coping abilities [27]. Parental uncertainty relating to their child’s disease is associated with parent and CYP distress [28, 29]. Furthermore, parental distress is associated with depressive symptoms in children which is further amplified if the child is experiencing illness intrusiveness which refers to a child’s belief that their illness is impacting on their everyday life and functioning [30]. Child reported pain and fatigue are also negatively associated with family functioning impacting upon family activities and communication [31].

Another problem that families face relates to the lack of awareness of rheumatological diseases, and this lack of social awareness is further magnified by the lack of information that is available to caregivers [27]. Caregivers have expressed a need for support with school advocacy relating to school absenteeism due to their child’s symptoms and hospital appointments [24]. Caregivers also encounter frustrations when dealing with their child’s ‘hidden disease’ during absences of symptoms and are faced with ongoing struggles surrounding teachers’ and peers’ as well as wider family members’ lack of understanding of the disease [23]. These school problems often lead to worries about the future transition to further education, for example, college, and work opportunities [24].

Making sure caregivers are able to cope with their child’s diagnosis and understand how to better support their CYP is important to mitigate some of the challenges that CYP with paediatric rheumatology conditions and their families face on a daily basis. However, the well-being of caregivers is not always considered or prioritised. Therefore, the objective was to systematically review and evaluate existing interventions tailored at providing support for caregivers of CYP with rheumatological conditions and assess the impact of these on improving well-being.

Methods

The present systematic review followed the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) 2020 statement [32].

Eligibility criteria

Eligibility criteria were pre-defined and registered in the International Prospective Register of Systematic Reviews (PROSPERO, PROSPERO ID: CRD42024524570). Articles were eligible for inclusion if they (1) included caregivers of CYP with any rheumatological condition, and (2) included interventions targeted at caregivers. Articles were excluded if the interventions were not targeted at caregivers. For example, some interventions were identified in the screening process that were targeted at CYP but also tested by caregivers. If the caregiver was not the target population for the intervention, the article was not included in this review. Only articles written in English or those that included an English translation were included. Review papers, abstracts as well as grey literature were reviewed and included in the systematic review if they met the eligibility criteria.

Search strategy

Eligible articles were identified in October and November 2023 by searching electronic databases which included Embase (Ovid), Scopus, Medline (Ovid), Web of Science Core Collection and CINAHL (EBSCOhost). A Google Scholar search as was conducted in April and May 2024 and internet resources from rheumatology charities and societies were reviewed but no additional articles were identified. When screening Google Scholar results, only the first 1000 identified results have been reviewed. Google Scholar was used as an additional search to look for any potentially missed literature. Google Scholar offered a vast amount of literature to review and on average different searches have shown that beyond 10–20 pages (with 10 results per page), search results were often not relevant anymore and there was a lot of overlap with the same articles being repeatedly shown across several searches. When the literature was not matching keywords from the searched terms for more than 2 consecutive pages, the search was abandoned, and new search terms have been applied. The complete search strategy can be found in Additional file 1.

Study selection

All references from the database searches were imported into Covidence [33]. Covidence is an online platform that helps facilitate the management of the literature that was obtained from the database searches. It allows reviewers to complete the study selection process systematically by having access to the literature on one platform. The whole study selection process from sifting through titles and abstracts as well as full texts and resolving conflict can be completed via this tool. Initially, the screening was based on titles and abstracts. Two independent reviewers screened all titles and abstracts based on the inclusion and exclusion criteria listed above (always KK, and either AN, JL or PL). On occasions where consensus was not reached, a team decision was sought to decide on whether to include the article. If a decision could not be made based on the title and abstract alone, the article was included for full text review to allow a more in-depth review of the article. The same rigour was applied to the full text review with two independent reviewers assessing each article (KK, AN, JL or PL), and a team decision was sought on occasions where consensus could not be reached.

Methodological quality assessment

The Mixed Methods Appraisal Tool (MMAT) [34] was used to assess risk of bias and quality [35]. Two independent reviewers (KK & AN) assessed the quality of the 15 articles that were included in the final review. In the MMAT, there are initial screening questions relating to the research question and data collection, and these helped inform whether a study was suitable for assessment using the MMAT. Consensus was reached between the reviewers using the MMAT, so no further actions were required.

Data extraction

Data extraction was based on predefined characteristics. Data extracted from the studies included first author, year of publication, study title, location (country and region of the intervention), setting (such as primary, secondary, tertiary care, community health or urban/rural/regional where applicable), population (including demographics such as age, gender, condition, socioeconomic status, ethnicity), study design, study aim(s), details of the intervention (type, aim of use, duration), outcome measures employed, and outcomes and main findings.

Data synthesis

Data were synthesised narratively due to the heterogeneity of study designs. Studies were grouped together based on type of intervention (e.g. online peer support, retreat, parent consultant programme, webpage), as well as study design (e.g. quantitative, qualitative, mixed method designs). Narrative synthesis is an approach that aims to summarise and explain results and findings from a range of different studies that employ diverse methods, populations and/or measures, and cannot be synthesised via standardised approaches such as a statistical meta-analysis or other comprehensive syntheses [35].

Results

Study selection

Fifteen studies were included in the final review. Interventions that were providing support to caregivers of CYP that went above providing solely education were deemed eligible. The search strategy identified 1054 studies for review once duplicate entries were removed (N = 11). The flow diagram for the whole search strategy can be found in Fig. 1.

Fig. 1

Flow diagram of article search strategy

Study characteristics

Table 1 provides a description of the final set of caregiver interventions that were included in the present review (N = 15). Studies were grouped together based on the type of intervention (online peer support, retreat, parent consultant programme, website). Eight of the interventions were published as an abstract [36,37,38,39,40,41,42,43]. Of those 8 abstracts, only 2 presented data [40, 43]. Eight studies have evaluated quantitative results [40, 42,43,44,45,46,47,48]. Of those eight studies, one did not collect any data [42], one was still collecting data [43], three reported on quantitative descriptive results [40, 44, 46], one was a non-randomised study [47], and four were randomised-controlled trials (RCT) [42, 43, 45, 48]. Two studies used mixed methods designs, analysing both quantitative and qualitative data [49, 50]. Four studies in this review focused on the same intervention (a website) that has been disseminated in different publications highlighting various milestones of the intervention [41,42,43, 48].

Table 1 Extracted information of included literature (N = 15)

Methodological quality

It is recommended to provide an overview of all criteria instead of providing an overall score when using the MMAT [34]. Table 2 shows the outcomes of the MMAT quality review of the 15 articles. The MMAT facilitates a review and appraisal of quantitative, qualitative and mixed method study designs, but is not suitable for reviews and theoretical papers [34], 5 abstracts could therefore not be reviewed past the initial screening [36,37,38,39, 41].

Table 2 MMAT assessment [36,37,38,39,40,41,42,43,44,45,46,47,48,49,50]

Synthesis of results

Narrative synthesis was used as outlined by Popay [51] to analyse the data due to heterogeneity of results and outcome measures that were employed. Five abstracts of the 15 studies that were included in this review could not be analysed as data collection did not take place [36,37,38,39, 41]. Three of the included studies that relied on quantitative measures used non-standardised questionnaires, and relied on surveys and feedback forms as well as predefined questions [40, 46, 49]. Six studies using quantitative measures relied on a range of standardised questionnaires [43,44,45, 47, 48, 50]. Different outcomes measures were employed across these studies. To assess self-reported psychological distress of caregivers the Symptom Checklist- 90-Revised (SCL- 90-R) [44] was used. To measure perceived mental health symptoms the Hospital Anxiety Depression Scale (HADS) [50], or the Psychiatric Symptom Index (PSI) [45] was used. To assess parenting stress the Pediatric Inventory for Parents (PIP) was employed [43, 48], and to assess perceived disability-related stress the Parents of Children with Disabilities Inventory (PCDI) [50] was chosen. Additionally, one study relied on translated and adapted measures to assess perceived support using the Iceland Family Perceived Support Questionnaire (ICE-FPSQ) [47], to assess illness beliefs using the Icelandic-Family Illness Beliefs Questionnaire (ICE-FIBQ) [47], to assess the impact on the family using the PedsQL Family Impact Module [47], and to assess healthcare satisfaction using the PedsQL Healthcare Satisfaction Generic Module [47]. None of the studies (N = 15) relied on qualitative data only, and of the two designs that used a mixed-methods approach, only one passed the quality assessment from the MMAT as can be seen in Table 2. Therefore, no further synthesis was feasible.

Discussion

This review highlights the need for interventions tailored at caregivers of CYP with rheumatological conditions. Our findings show that there is limited support offered to caregivers in the first instance, and the few interventions that were identified in this review have not always been suitably assessed. There are various means in which support was offered to caregivers which involved peer support (via Facebook groups, meet ups, residential workshops, or in clinics), websites and tailored information sessions, and on one occasion a Family Strengths Oriented Therapeutic Conversation (FAM-SOTC) intervention. However, a third of the studies that were identified in this review have not been formally assessed.

Understanding the implications of caregiver interventions is important to help mitigate some of the challenges that families face daily, but also to improve outcomes for CYP and their families. A mixed-method systematic review by Knafl et al. [52] that evaluated 29 articles showed a moderate positive association between caregivers’ and children’s psychological functioning highlighting the complementary relationship between caregivers’ and CYP’s well-being. They also found negative associations between family conflict and psycho-social well-being of children as well as parental depression and children’s physical functioning [52]. This suggests that by supporting the caregiver and improving family functioning, positive implications for both the caregiver as well as the CYP should be observed and may have further advantageous implications on the wider family. Moreover, even though some interventions that were evaluated in this review have shown to have a positive impact, these interventions are not available outside of the research context. For example, the WebParC website is no longer available to families. This is mostly due to the challenging steps required to have an intervention adopted into clinical care which often require further time, and funding being awarded. Having viable interventions that are not accessible to caregivers after the initial testing phase means that the outcomes are only short-lived and cannot be rolled out on a larger scale.

Caregiver roles and relationships change as a result of receiving a paediatric rheumatology diagnosis and having to care for a child with a chronic condition. Adjusting to the new normal and learning to adapt to new responsibilities are essential. Caregivers need to learn to facilitate CYP’s treatment management and exercise regimens which can take an emotional toll on caregivers and may lead to changes in relationships between caregivers and their child [53]. Moreover, relationships between caregivers and other family members are also affected which frequently stems from family members’ lack of understanding of the disease [23]. The literature also highlights the potential impact on workplace relationships, which can be significant as well [53]. Therefore, interventions aimed at caregivers are necessary to improve outcomes for caregivers so that they are able to better support their CYP.

Caring for a CYP with a rheumatological condition can be a very lonely and isolating experience. Peer support for caregivers such as informal support groups and family weekends were found to be a good way of providing reassurance and lessening the impact of a perceived sense of isolation [54], and loneliness [40]. While interventions aimed at providing caregiver peer support are available and have been included in this review, the impact of peer support needs to be analysed and evaluated to understand the positive effects of those interventions.

Interventions should also be tailored depending on caregiver demands, level of understanding of CYP and caregivers, and stage of child’s disease journey. For example, CYP and caregivers prefer interventions that are adapted to individual needs and different support should be provided depending on factors such as disease severity and developmental age [54]. Caregivers also want positive and easy to understand information without complex jargon and terminology when dealing with their child’s ‘unpredictable’ disease [55]. Support should be offered to the wider family and family-based education tailored to individual needs [56]. Newly diagnosed CYP and their families are often in shock, denial and disbelief when receiving a diagnosis and require different support at the beginning of their journey. By comparison, when CYP have been diagnosed for longer, these more experienced caregivers face challenges in relation to continued treatment management and unpredictability of flares, and report that they would benefit from monitoring checklists to be able to record symptoms over time and favour support relating to coping with flares by having access to relevant resources and guidance [55]. Additionally, caregivers often struggle in supporting their child in relation to pain management and procedural responsibilities [52], so this should be addressed in future interventions as well.

Addressing the need for more information and reliability of sources is also important. Research suggests that caregivers prefer to obtain information from physicians (98.8%), and websites (47.9%), as opposed to seminars (3.5%), and books (1.7%) [57]. However, even when caregivers felt that they have been well informed by their medical teams relating to their child’s disease and treatment, the need for more information prevailed [58]. Parents reported wanting more information on topics that they already have been given information on as well as other topics such as complementary and alternative therapies, psychological support as well as educational and vocational rehabilitation for their child [58].

Conclusions

The present review highlights the need for tailored interventions for caregivers of CYP with rheumatological conditions. The review has shown that caregivers value meeting peers and hearing other people’s stories as they provide empathic and relatable sources of support, and reduce feelings of loneliness and isolation. However, support offered to caregivers needs to be evaluated to better understand the mechanisms and benefits of currently available interventions, as well as to guide future interventions. There is limited evidence from this review that caregivers receive adequate support. Considering the full impact of a chronic paediatric rheumatological diagnosis upon the whole family, this review justifies the need for better interventions to improve the quality of life of families.