Perspectives and experiences of parents of children with juvenile dermatomyositis: A semi-structured interview study.

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Amy Kelly, Ayano Kelly, Davinder Singh Grewal, Jeffrey Chaitow, and 1 more This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-4858416/v1 This work is licensed under a CC BY 4.0 License Status: Published Journal Publication published 28 Mar, 2025 Read the published version in Pediatric Rheumatology → Version 1 posted 5 You are reading this latest preprint version Abstract Background Juvenile Dermatomyositis (JDM) is a rare, childhood inflammatory disease and its management can be challenging and confronting for both clinicians and caregivers. Little is known about the perspectives of parental caregivers of children with JDM. This study aimed to describe the experiences of parents of children with JDM to inform person-centred care. Methods Semi-structured interviews (face-to-face, telephone) were conducted with parents of children with JDM from three centres in Australia. Transcripts were analysed thematically. Results Nineteen parents (15 mothers) of 18 children aged 8 to 21 with JDM participated. Six themes were identified. Rapid crescendo of fear and desperation (alarming deterioration, sudden realisation of seriousness, desperate for a diagnosis ), lost and unsupported in the health system (at the mercy of the medical team, frustrated at the lack of services, neglected priorities, protracted and painful search for answers), disrupting family routines (sibling neglect and loss, overloaded with a medicalised schedule, always on standby, burdened by financial strains), grieving what has been lost (missing the sunlight, struggling with the loss of physical function, disrupted schooling, changes in their child from steroid side effects), managing an uncertain future (bound to chronicity, fearing relapse, insecurity with transition to adult care), gaining confidence and motivation (strengthening partnerships with clinicians, growing maturity and independence, gaining hope from shared experiences). Conclusions The diagnosis of JDM is often delayed and caregivers of children with JDM report distress, disruption and uncertainty throughout their treatment journey with their child. Addressing these fears and establishing support mechanisms that help parents navigate their way through the medical system and support changing family dynamics are vital to optimise health outcomes for children diagnosed with JDM. Juvenile Dermatomyositis qualitative methods interviews patient-centred care caregivers Figures Figure 1 INTRODUCTION Juvenile Dermatomyositis (JDM) is a rare, chronic, childhood autoimmune disease, characterised by inflammation of small blood vessels of the tissues and organs, leading to a characteristic rash, muscle weakness, elevated muscle enzymes and sometimes involvement of vital organs. Better outcomes are achieved with early diagnosis and early aggressive treatment, often with significant side effects( 1 ). Due to the heterogeneity in presentation and the rarity of the disease, the diagnosis of JDM is often delayed ( 2 ), enhancing anxiety, confusion and a sense of isolation for caregivers of children with JDM. Caregivers of children with JDM have reported increased levels of stress, higher levels of anxiety and poorer quality of life( 2 ) and parenting stress may adversely affect child health-related outcomes as it could potentially interfere with the management of the child’s chronic illness ( 3 ). The challenges for parents in caring for a child diagnosed with JDM may be amplified by the limited knowledge of JDM among non-paediatric rheumatology clinicians, difficulty accessing appropriate services, prolonged treatment, complexities in managing the condition, the burden of side effects of treatment and impacts on the family unit. There are few studies examining the perspectives of parents or caregivers of children with JDM. This study aimed to describe the experiences of caregivers of children with JDM to inform strategies and interventions to address their needs. METHODS Participant selection Participants were eligible if they were a parent or caregiver of a child (aged 0 to 18 years) with clinician diagnosed JDM and were English speaking. Participants were recruited through the Sydney Children’s Hospital Network, including the Children’s Hospital Westmead, The Children’s Hospital at Randwick and John Hunter Hospitals. Purposive sampling was used to capture a broad range of perspectives based on socioeconomic status, geographic location, ethnicity, their child’s disease course, sex and age. The project was approved by the Sydney Childrens’ Hospital Network Ethics Committee under project number 2021/ETH00053. Data collection A preliminary interview guide was developed based on the literature of the experiences and perspectives of caregivers of children with other paediatric rheumatic diseases( 2 , 4 , 5 ). AHK conducted a semi-structured interview either face to face at a hospital clinic or by telephone, depending on participants preference or COVID- 19 requirements. Recruitment ceased once data saturation was reached (when no new themes or new concepts were emerging in the data). Interviews were recorded and then transcribed verbatim. Data analysis Transcripts were entered into HyperRESEARCH version 4.0 to assist with the coding, storage and searching of the data. Using thematic analysis, the first author (AHK) coded the transcripts, line by line, conceptualising and categorizing the data and assigning codes to inductively identified concepts. Relationships between common concepts were explored in the data to develop analytical themes. A thematic schema was mapped to demonstrate the connection between themes. A second and third investigator AJ and AK, read and reviewed the preliminary themes to ensure that all experiences and perspectives of participants were included. RESULTS The characteristics of the participants are shown in Table 1 . Nineteen of 27 (70%) caregivers who were contacted agreed to participate. The majority of participants were female (15, 79%) and all participants identified as their child’s biological parent. Table 2 details the characteristics of the children of participants, including their initial presentation, the treatments they received and the number of specialists they had seen prior to a diagnosis being made. Table 1 Characteristics of participants Characteristic No. (%) Biological Mother 15(79) Biological Father 4(21) Age (years) 40s 14(74) 50s 4(21) 60 and over 1(5) Education Secondary 1(5) Certificate/ Diploma 2(11) Bachelors/Higher 16(84) Marital Status Married/defacto 19(100) Employment Casual 1(5) Full time 9(47) Part time 5(26) none 4(21) Geographical Location Metropolitan 14(74) Rural 5(26) Religion None 9(47) Catholic 4 (21) Protestant 2(11) Christian 2(11) Ethnicity Caucasian 15(79) Greek 1(5) Fijian Indian 1(5) Japanese 2(11) Table 2 Characteristics of the children of participants Characteristics No. (%) n = 18 Females Males 9 (50) 9 (50) Country of birth Australia Other 17 (95) 1 (5) Religion Nil Catholic Other Christian 9 (50) 5 (28) 2 (11) Ethnicity Caucasian/Australian Tongan Indian/ Fijian Greek Australian Japanese 14 (78) 1 (5) 1 (5) 1 (5) 1 (5) Clinical presentation of illness Muscle weakness Skin rash Lethargy Pain Falls 8 (44) 11 (61) 1 (5) 4 (22) 2 (11) Age at diagnosis 1–5 years 6–11 years 12–15 years 15–20 years 8 (44) 5 (28) 5 (28) 0 (0) Current treatment Prednisone Methotrexate IVIG Other DMARD Tofacitinib None 6 (33) 11 (61) 3 (17) 1 (5) 2 (11) 4 (22) Previous treatment None Methylprednisone Prednisone IVIG Methotrexate Other DMARD 3 (17) 4 (22) 10 (56) 1 (5) 7 (39) 1 (5) Comorbidities None Perthes disease Asthma Coeliac disease ADHD Osteoporosis Skin striae 11 (61) 1 (5) 1 (5) 1 (5) 1 (5) 1 (5) 2 (11) No. of doctors initially referred to 1 2 3 4 4 (22) 11(61) 2(11) 1(6) We identified six themes: Rapid crescendo of fear and desperation, lost and unsupported in the health system, disrupting family routines, grieving what has been lost, managing an uncertain future, gaining confidence and motivation. The respective subthemes are described below and selected quotations are illustrated in Table 3 . Figure 1 illustrates the relationship between these themes in a thematic schema. Table 3 Selected quotations supporting each theme Themes and Subthemes Quotations Rapid crescendo of fear and desperation Alarming deterioration She never goes to the doctor because she’s never sick, and then one day she said to me ‘can you take me to the doctor, it’s really hurting’, and that’s when I thought oh, God, this must be not growing pains. (Mother, age 40s). Sudden realisation of seriousness …she needed help wiping herself and things like that, and we were like oh God, what’s going to happen, are we going to have to become carers, are we going to have to, one of us resign our jobs, things like that. (Mother, age 40s). Desperate for a diagnosis …then we couldn’t get him to see the neurologist for I think two months. We had this terrible time where we didn’t know what it was and we didn’t know what to do. (mother, age 40s). Lost and unsupported in the health system At the mercy of the medical team I do feel a little in the dark….sometimes I wonder if there are things we are just not being told (Father, aged 50s). Frustrated at lack of services Because JDM is so specialised, you didn’t really fit anywhere (mother, aged 40s). Neglected priorities Having a teenager is pretty stressful as you may know …but the combination of a teenager and then someone who’s unwell, the compounding can impact mental health (mother, aged 50s). Protracted and painful search for answers We probably saw six or seven doctors. Only one had heard of it, but didn’t know much about it (father, aged 50s) Disrupting family routines Sibling neglect and loss …during that time, she basically lost a little friend…That was emotionally quite difficult for her and obviously for him, because he just wasn’t able to do anything (mother, aged 40s). Overloaded with a medicalised schedule Generally, you find that when you come into the hospital and you see the rheumatologist, it’s busy. It’s chaos…I’d like to be able to ask a question and have a bit more time (mother, aged 40s). I guess the medication is probably the biggest responsibility, just ensuring that that’s all okay and making sure his scripts, monitoring that (mother, aged 50s) Always on standby If he needs to take a day off school because he’s just not up to it, you know, I make sure I work from home that day …(father, aged 50s). Burdened by financial strains Our family was based on my single income and (my wife) wasn’t working at the time. I just had to find a way to somehow keep it together (father, aged 40s). Grieving what has been lost Missing the sunlight I know she shouldn’t be out in the sun, and I know that that’s not great, but I don’t know to what extent…I was like, does that mean no lunch time at school, no sport? (mother, aged 40s). Struggling with the loss of physical function …he was walking with someone who had a broken arm, and he was feeling just as sick as this kid that had a broken arm, and people were saying to him ‘well, why aren’t you helping that boy with the broken arm, because you’re fine?’ and he was like I’m not fine, I’m really struggling to walk up and down the stairs. I think because you can’t see it, people are not as empathetic (mother, aged 50s). Disrupted schooling … there are days when he’s got a headache or a cold now... I’m a bit more lenient. If he needs a day off here or there, I’m all right (mother, aged 60s). Changes in their child from steroid related side effects Once he started the prednisone it completely interrupted his sleep, so there’s some days he’s completely exhausted from waking up continuously through the night (mother, aged 50s). Managing an uncertain future Bound to chronicity …what’s it going to do, will it go away, will it get worse? You know…it’s a very unpredictable disease, it can be chronic (mother, aged 40s). Fearing relapse I think it’s always there and present for him, because he knows it will never go away. So yes, I think maybe that’s the uncertainty around the disease coming back (mother, aged 40s). Insecurity with transition to adult care I think the transition to adult and the adult rheumatology area, that’s probably where we’re a bit nervous at the moment because we know next year when he finishes school, he has to move into the adult world (mother, age 40s). Gaining confidence and motivation Strengthening partnership with clinicians They relied on our observation to report anything that’s happening on a day to day basis and how the treatment is going. We felt quite involved in the treatment in that way, which was good (father, aged 40s) Growing maturity and independence He is conscious of eating properly now. Whether this is due to the experiences that he had with this supposed dietary intervention or whether he’s doing it because he just doesn’t want to appear overweight, he’s conscious about what he eats. I think it’s in a positive space at the moment (father, aged 50s) Gaining hope from shared experiences But she’s scoring goals, she’s getting awards, she’s loving it. Socially, she’s loving it, and the teams are fantastic. That’s one of the joys now that we’re out the other end (father, aged 50s). Rapid crescendo of fear and desperation Alarming deterioration Parents felt frightened and panicked at the rapid decline they saw in their child’s physical abilities. They were very concerned to witness their child being unable to do up their seat belt, falling over at the supermarket, or being unable to get out of bed, “… we went away bushwalking with some friends, and I had to carry her in the backpack” (mother, 60s). Sudden realisation of seriousness Parents initially were dismissive of child’s symptoms as a minor illness, part of normal development, or even growing pains, “I just thought it might be a cold. I just thought it was growing pains.” (mother, age 40s). However, they gradually began to realise the seriousness of their condition as they could see their child was not recovering and in many cases deteriorating. Desperate for a diagnosis Parents felt an increasing desperation when numerous clinicians were unable to find a diagnosis for their child’s symptoms – “The hardest part was the long wait to diagnosis” (mother, 40s). They felt disappointed and let down because of the delayed diagnosis, particularly when they felt general practitioners did not know about the condition – “ our GP had not even heard of it” (mother, aged 40s). They found it difficult to understand that there was no clear answer as to why their child developed JDM and described feeling stupid when they repeatedly asked the clinicians why this happened to their child. They felt a sense of relief once the diagnosis of JDM was made. Lost and unsupported in the health system At the mercy of the medical team Parents felt intimidated and disempowered by the use of medical terms that were difficult to understand. Protocols for administration of medications, including weekly dosing and the regular blood monitoring for medications also contributed to a sense of being overwhelmed with a rigid medical schedule. Parents relied on the medical team to educate them about JDM and its treatments. However, many parents described feeling lost, “ I do feel a little in the dark…. sometimes I wonder if there are things we are just not being told. (father, aged 50s)”. Parents from culturally and linguistically diverse backgrounds (CALD), who were not familiar with the health system found it even more difficult to understand. Parents felt overwhelmed by the complexity of the disease including the varying way the disease could initially present and its response to treatment. They understood that treatments were being determined by an individual clinician’s experience, leading them to feel like there was no standard treatment protocol being followed, ‘’ There seems to be so much happening with the disease… it’s all very subjective, I feel like we are 100% reliant on the (Doctor’s) gut feel” (mother, aged 40s). Frustrated at lack of services Parents were shocked by the limited services once their child was diagnosed with JDM. They described feeling forgotten and alone when they discovered there was no single point of contact to coordinate their child’s care. Parents in rural or remote areas felt vulnerable as it was difficult to travel long distances to medical appointments in a large metropolitan city, “it was scary being 5 hours away from the specialist , it’s hard when we are the only ones in a rural town (with the disease)’’ (mother, aged 40s). Neglected priorities Parents reported being disillusioned and upset when they perceived that clinicians were focussed on the treatment of muscle weakness and did not address other symptoms, including pain, and mental health issues, “I can’t localise it, it’s pretty broad. It’s either muscle pain, back pain, stomach pain, it comes in different forms. I don’t think that has really been addressed or understood in a detailed manner….it’s something that should’ve been teased out a little bit more” (father, aged 50s). Protracted and painful search for answers Parents were desperate for the treatments for their children to work, they were prepared to try anything, and some sought non-pharmacological ways to manage the condition including seeking out alternative therapies “ We did try a few elimination diets” (mother aged 40s) and, “ We were prepared to try anything” (mother aged 40s) , knowing that these were not recommended therapies for children with JDM. Parents described spending many hours researching online and struggled with the lack of specific information relevant to the Australian health care system. Parents discovered that treatment practices varied internationally depending on the availability of treatments and the local health care system. Variation in treatment practices internationally coupled with discovering worst-case scenarios online, including finding examples of children with lifelong disabilities, was both alarming and frightening. Disrupting family routines Sibling neglect and loss In focussing their attention on their child with JDM, some parents felt that their relationship with their other children, or among siblings, suffered. For example, previously siblings were able to play together, this was not possible because of disease symptoms or medication side effects, ‘’ she basically lost a little friend’ (mother, 40s). Participants were frustrated that their other children were missing out on school and important life events because of the time taken caring for their child with JDM. Parents were also concerned for future potential mental health problems for siblings, “we [the whole family, including siblings] were semi-suicidal for a lot of last year…” (mother, aged 40s). Overloaded with a medicalised schedule Attending multiple medical appointments was time consuming and parents described the changes they had made to their family’s routine ‘’ our family has made Saturday a rest day; that is the day she takes medications’’ (mother, aged 40s). Parents spoke of having to accommodate the burden of responsibility for managing their child’s medications, including minimising family activities because of side effects from medications, monitoring for their child’s response to treatment as well as being the central coordinators of their child’s care, made them feel that they were different to other families; “we are not a normal family anymore’’ (mother, aged 50s). Always on standby Some participants described having to give up all other parts of their lives, including their job, travel, social events and career aspirations to constantly be there for their child, ‘’I sat outside the school for hours, knowing that he might need me to come and get him at any point” (mother, aged 50s). This was described as frustrating, emotionally demanding and time consuming, particularly for mothers who felt they carried the majority of the caring load for their child. Burdened by financial strains Parents were stressed juggling their work or business, for example, time taken attending multiple medical appointments was time they had to take off work, which was particularly burdensome when they ran their own businesses. Families carried the burden of costs of travelling to multiple appointments (particularly for regional families), including self-funding allied health therapies, because there were no publicly available services. Finances were a source of tension in many households, “ the only thing we fight about every month is the credit card bill” (mother, aged 50s). Grieving what has been lost Missing the sunlight Parents understood it was important to avoid the sun to prevent worsening their child’s skin disease. It saddened them to have to reconsider family outings and avoid the hottest time of the day, however it also provided an opportunity to proactively manage part of their child’s disease. Sun exposure did cause anxiety in parents, and they were confused and worried about managing their child’s exposure to the sun particularly when they were not with them. They felt sorry for their child when they missed out on activities to avoid the sun; “ You’ve got a little boy who just wants to be like every other kid and doesn’t want to be out of the sun ”, (father, aged 50s). Struggling with the loss of physical function Parents were upset at the manifestations of the disease in their child and felt compelled to protect their children from the negative comments of others, knowing that their child’s symptoms were only visible to those that knew them well. They felt vulnerable and defensive towards those that did not appreciate the suffering of their child, ‘’ He was walking with someone who had a broken arm, and he was feeling just as sick as the kid with the broken arm and people were saying, Why aren’t you helping that boy with the broken arm?” ( mother, aged 50s). Parents wanted their child to do more, hoping that exercise would improve their symptoms and they felt guilty when they saw their child struggling or in pain. Disrupted schooling Parents were despondent to see their child struggle to attend school because of their illness and suffer from side effects of treatment; “ my child is a primary school drop out’’ (mother, aged 50s). The majority of parents felt supported by the child’s school, however some reported having to justify and negotiate more support services from their school, “ school says he will catch up….but this disease isn’t going anywhere” (mother, aged 40s). Paradoxically, parents reported that COVID remote learning helped their child focus on improving their health whilst maintaining connection with their schoolwork, in a safe environment, ‘’ We had a COVID safe bubble ”(mother, aged 40s). Parents described a sense of relief and satisfaction that their child was not missing out on school. Changes in their child from steroid related side effects Participants described feeling very despondent by the changes they saw in their child from the side effects of treatments, particularly steroid side effects. They saw their child gain weight and become self-conscious ‘’every couple of months we go up a size, he is starting to feel different to other kids” (mother aged 50s). Parents were upset by memories of their child’s struggles and were sensitive to their self-image, which was amplified when their child moved into their teenage years, “’She won’t even go back and look at photos of herself when she was like that (mother, aged 40s). Other steroid side effects, including emotional lability was very alarming; “ I give my son toxins every day that make him feel like he wants to die, and then have to spend the rest of the day telling him that it’s worth living and there is hope” (mother, aged 40s). Managing an uncertain future Bound to chronicity Parents did not expect JDM to take so long to achieve remission, nor did they initially appreciate the need for ongoing treatment, “ we didn’t realise that it’s not fixed overnight’’ (mother, aged 50s). Parents became aware that their child may cycle in and out of remission and many parents were shocked and confused by this, they had not expected the disease to behave in this way. Fearing relapse Participants described their greatest fear was a relapse of their child’s illness, requiring further treatment and the potential side effects that may follow. They felt anxious about what the future might hold for their family; to the extent that some “ tried not to think about the future” (mother aged, 40s). Parents were unsure as to how they or their child would cope if they experienced a relapse of the disease. Insecurity with transition to adult care Parents were concerned about how their child would transition from paediatric care to adult medical services, including worrying about how their child would navigate the complexity of their disease such as sun avoidance and know if their disease had relapsed,… ‘’ we know next year when he finishes school, he has to move into the adult world. I don’t think the experience will be as one-on-one or personable as what it is under the paediatric rheumatology environment (mother, aged 50s)”. Gaining confidence and motivation Strengthening partnerships with clinicians Parents appreciated feeling involved in their child’s treatment. This was aided by clinicians sharing pathology results with them, such as creatinine kinase (CK) to monitor response to treatment. At regular follow up appointments, they waited anxiously for their child’s results and celebrated when they saw improvements in objective markers, such as CK. Growing maturity and independence With the intense focus on their child’s physical health and managing side effects, such as weight gain from steroids or worsening skin disease from sun exposure, parents saw their child become more aware of healthy habits and avoiding sunlight. Parents felt a sense of pride when they observed how their child matured and learned to navigate their condition with their support, “ I know that [he has an] awareness that he doesn’t want in be in the sun” (mother aged 60s). Gaining hope from shared experiences Parents found it helpful to talk to other parents affected by JDM, they wanted to understand other’s experiences. This enabled them to visualise what disease remission looked like. It was their way “…. of looking for the light at the end of the tunnel’’ (father, aged 50 s) and it gave them hope for the future. Discussion Parents of children with JDM experienced a sudden fear when they realised that their child was seriously unwell and become desperate for a diagnosis. They felt frustrated at the lack of knowledge by many in the medical community about JDM. Once the diagnosis had been made and their child was receiving treatment for JDM, parents described feeling overwhelmed by the rigid medical schedule that took over their family life. They felt isolated because of the lack of services to support their child, finding it challenging to access allied health services with adequate knowledge of the disease. Parents found it difficult to process the diagnosis because their doctors were unable to determine the cause of JDM. Parents also reported experiencing increased financial strain. Complicated treatment regimes, including high doses of steroids, sun avoidance measures and the requirement for regular blood monitoring led parents to describe a sense of feeling overwhelmed by the “medicalisation’’ of their lives and they grieved the disruption in their family’s routine. Parents were saddened to see their child have their schooling disrupted and feared relapse, often because they had seen online worse case scenarios of children with severe disease, yet they gained hope and confidence when they witnessed positive progress in their child. Parents were pleased when they saw the development of independence and confidence in their child’s self-management. Parents of children with chronic illness experience greater levels of stress and lower quality of life( 2 ). A study examining the experiences of caregivers of children with JDM in the United States, reported that parents’ quality of life was reduced and their mood adversely affected ( 2 ). Our study identified similar stress amongst parents. Another childhood rheumatic disease, Juvenile idiopathic arthritis has been described as a family disease( 5 ), similarly our study also identifies direct impacts on parents and their families. Parents described their sadness at the disruption to their normal family routine, changes in relationships between siblings, demands of attending medical appointments and the need to drop everything to support their child. These burdens were especially carried by mothers in our cohort and existing evidence in the literature confirms the major caregiving role to the mother( 6 ) in childhood chronic diseases. The demands of caregiving and effects on financial resources can have a negative impact on women’s professional and social lives( 6 ). Our study provides broad insights into the perspectives of parents of children with JDM. Purposive sampling was used to include a diverse group of participants including men, women, culturally and linguistically diverse participants and those from regional and metropolitan areas. However, there are some potential limitations. Most of the participants had a high level of educational attainment, and approximately 20% of participants were from culturally and linguistically diverse backgrounds (CALD). The transferability of our findings to non-English speaking populations is thus uncertain. We identified specific areas of concern for parents with implications for clinical practice, including delayed diagnosis, lack of JDM specific services, monitoring treatment response, sun avoidance strategies and disruption to schooling. Limited awareness of JDM, its heterogenous presentation( 4 ) and limited paediatric rheumatology services across Australia all contribute to the likelihood of a delayed diagnosis. Previous studies have indicated that the mean time to diagnosis is 8.5 months( 2 ). In our cohort, there was a high likelihood of delayed diagnosis, with 78% of participants reported seeing at least 2 specialists (after being referred by their General Practitioner) prior to the diagnosis of JDM being made (Table 2 ). In other chronic diseases, delayed access to specialist care results in deteriorating health, more frequent hospital admissions and poorer health outcomes( 7 ). This is particularly true for rural and regional patients, who may have to travel long distances with the associated time and the financial demands that entails( 7 ). It may not be possible to educate every health practitioner specifically about JDM, however understanding abnormal musculoskeletal and/or dermatological presentations in children is integral to reducing the time to diagnosis, in addition to the urgent need to expand access to paediatric rheumatology services across Australia. We identified further implications for clinical practice, including treating teams recognising the importance of involving parents and children in their disease’s management. Parents felt a sense of success ( 8 ) when “celebrating” a normal creatinine kinase level or seeing improvement in their child’s CMAS (Childhood Myositis Assessment Score). Many reported that closely following sun avoidance recommendations disrupted family routines, however managing their child’s sun exposure also gave parents a sense of control over an aspect of their child’s disease. Clinicians should focus on effectively communicating the goals and measurements of treatment, recognising the importance of communicating when those goals have been met and providing a clear framework for sun avoidance measures, to avoid confusion and empower parents. Further, parents understand the importance of minimising disruption to their child’s schooling ( 9 ), with current research suggesting that school disruptions during the COVID-19 era had adverse effects on child health and wellbeing ( 10 ). Our study was conducted during the COVID-19 pandemic and participants in our study reported a sense of relief that their child was not missing out on educational opportunities when compared to their peers. Multidisciplinary management of families with JDM should include working collaboratively with educational partners to ensure minimal disruption to schooling. Pandemic home-schooling models may provide a template for JDM patients to better engage in schoolwork. Our study identified rationalising the use of steroids as an important area for future research. In our cohort, parental stress was impacted by steroid related side effects. Rheumatic diseases are often complex in their management and often require long term management( 11 ). Similarly JDM may also require complex medication regimes, often lacking evidence base and usually relying on the experience of the treating paediatric rheumatologist ( 12 ). It may be difficult to predict an individual patient’s response to treatment( 13 ) and high dose steroids may be required( 1 ), with the potential for significant side effects including; weight gain, stunted growth, bone loss ( 14 ), mood disorders and psychosis( 15 ). The distress and morbidity caused by side effects of high dose steroids (which can in part fuel the fear of relapse for parents) highlights the urgency to find new, less toxic treatments. Conclusion Our study provides a unique insight into the experience of caregivers of children with juvenile dermatomyositis as they navigated their child’s initial presentation, diagnosis and response to treatment. Key areas of concern include delays in diagnosis, lack of access to services, response to treatment, fear of relapse, toxic side effects from medications and the unknown causes of the disease. This study provides an important platform for understanding how to better support families with JDM, providing clinicians and policy makers the evidence they need to improve the comprehensive management of this rare disease, to enhance treatment strategies and better inform future research directions. Abbreviations JDM: Juvenile Dermatomyositis AHK: Dr Amy H Kelly AJ: Professor Allison Jaure AK: Dr Ayano Kelly CK: Creatinine Kinase CMAS: Childhood Myositis Assessment Score CALD: Culturally and linguistically diverse Declarations Ethics: the project was approved by the Sydney Childrens’ Hospital Network Ethics Committee under project number 2021/ETH00053 Consent for publication: not applicable Availability of data and materials: The datasets used and/or analysed during the current study are available from the corresponding author on reasonable request. Competing interests: The authors declare that they have no competing interests Funding: Dr A.H Kelly received a University of Sydney postgraduate research scholarship that contributed to funding this project. Authors' contributions:AHK devised the project, collected data, analysed data and was the major contributor to writing the final manuscript. AK contributed to analysing the data and contributed to writing the final manuscript. DSG contributed to the collection of data and to the writing of the final manuscript. JC contributed to the collection of data. AJ contributed to analysing the data and writing the final manuscript. Acknowledgements: not applicable References Wu Q, Wedderburn LR, McCann LJ. Juvenile dermatomyositis: Latest advances. Best Pract Res Clin Rheumatol 2017;31:535-57. Kountz-Edwards S, Aoki C, Gannon C, Gomez R, Cordova M, Packman W. The family impact of caring for a child with juvenile dermatomyositis. Chronic Illn 2017;13:262-74. Cousino MK, Hazen RA. Parenting stress among caregivers of children with chronic illness: A systematic review. J Pediatr Psychol 2013;38:809-28. Kountz-Edwards S. The psychosocial impact of juvenile dermatomyositis on pediatric patients and parents. Dissertation Abstracts International: Section B: The Sciences and Engineering 2016;77:No Pagination Specified. Grazziotin LR, Currie G, Twilt M, MJ IJ, Kip MMA, Koffijberg H, et al. 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Impact of lockdown and school closure on children's health and well-being during the first wave of covid-19: A narrative review. BMJ Paediatr Open 2021;5:e001043. Bakker MM, Putrik P, Dikovec C, Rademakers J, Vonkeman HE, Kok MR, et al. Exploring discordance between health literacy questionnaire scores of people with rmds and assessment by treating health professionals. Rheumatology (Oxford) 2022;62:52-64. Bellutti Enders F, Bader-Meunier B, Baildam E, Constantin T, Dolezalova P, Feldman BM, et al. Consensus-based recommendations for the management of juvenile dermatomyositis. Ann Rheum Dis 2017;76:329-40. Wu JQ, Lu MP, Reed AM. Juvenile dermatomyositis: Advances in clinical presentation, myositis-specific antibodies and treatment. World J Pediatr 2020;16:31-43. Ferrara M, Borrelli B, Greco N, Coppola L, Coppola A, Simeone G, et al. Side effects of corticosteroid therapy in children with chronic idiopathic thrombocytopenic purpura. Hematology 2005;10:401-3. Buchman AL. Side effects of corticosteroid therapy. J Clin Gastroenterol 2001;33:289-94. Cite Share Download PDF Status: Published Journal Publication published 28 Mar, 2025 Read the published version in Pediatric Rheumatology → Version 1 posted Editorial decision: Major revision 27 Dec, 2024 Reviewers agreed at journal 03 Sep, 2024 Reviewers invited by journal 03 Sep, 2024 Editor assigned by journal 03 Sep, 2024 First submitted to journal 28 Aug, 2024 You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. As a division of Research Square Company, we’re committed to making research communication faster, fairer, and more useful. We do this by developing innovative software and high quality services for the global research community. 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Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-4858416","acceptedTermsAndConditions":true,"allowDirectSubmit":false,"archivedVersions":[],"articleType":"Research Article","associatedPublications":[],"authors":[{"id":349085161,"identity":"9a5f7746-354f-4c67-8004-cfd07824e163","order_by":0,"name":"Amy Kelly","email":"data:image/png;base64,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","orcid":"https://orcid.org/0000-0002-7963-8159","institution":"Campbelltown Hospital","correspondingAuthor":true,"prefix":"","firstName":"Amy","middleName":"","lastName":"Kelly","suffix":""},{"id":349085162,"identity":"fc4a61d2-109e-4af7-8944-7390d8419717","order_by":1,"name":"Ayano Kelly","email":"","orcid":"","institution":"University of New South Wales","correspondingAuthor":false,"prefix":"","firstName":"Ayano","middleName":"","lastName":"Kelly","suffix":""},{"id":349085163,"identity":"18d0c1ab-03f8-403d-b291-d916d70bd28a","order_by":2,"name":"Davinder Singh Grewal","email":"","orcid":"","institution":"Sydney Children's Hospitals Network","correspondingAuthor":false,"prefix":"","firstName":"Davinder","middleName":"Singh","lastName":"Grewal","suffix":""},{"id":349085164,"identity":"d26d0762-9569-4d4a-9f54-b7889f7acacc","order_by":3,"name":"Jeffrey Chaitow","email":"","orcid":"","institution":"Sydney Children's Hospitals Network","correspondingAuthor":false,"prefix":"","firstName":"Jeffrey","middleName":"","lastName":"Chaitow","suffix":""},{"id":349085165,"identity":"036acd11-8bd7-40fe-8316-cdb4d3cb73f4","order_by":4,"name":"Allison Jaure","email":"","orcid":"","institution":"The University of Sydney","correspondingAuthor":false,"prefix":"","firstName":"Allison","middleName":"","lastName":"Jaure","suffix":""}],"badges":[],"createdAt":"2024-08-04 23:38:31","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-4858416/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-4858416/v1","draftVersion":[],"editorialEvents":[{"content":"https://doi.org/10.1186/s12969-025-01079-2","type":"published","date":"2025-03-28T15:57:48+00:00"}],"editorialNote":"","failedWorkflow":false,"files":[{"id":67117854,"identity":"f8aae020-4b66-4643-820f-120f65b167ce","added_by":"auto","created_at":"2024-10-21 10:47:24","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":84121,"visible":true,"origin":"","legend":"\u003cp\u003eSee image above for figure legend.\u003c/p\u003e","description":"","filename":"1.png","url":"https://assets-eu.researchsquare.com/files/rs-4858416/v1/11cb8f26d0669a186eeeb2a3.png"},{"id":79604897,"identity":"1667f4e0-2269-4367-a2ef-08a935dfe0de","added_by":"auto","created_at":"2025-03-31 16:08:34","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":1220635,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-4858416/v1/cc776054-fb96-4272-9f07-1985842f73d5.pdf"}],"financialInterests":"","formattedTitle":"Perspectives and experiences of parents of children with juvenile dermatomyositis: A semi-structured interview study.","fulltext":[{"header":"INTRODUCTION","content":"\u003cp\u003eJuvenile Dermatomyositis (JDM) is a rare, chronic, childhood autoimmune disease, characterised by inflammation of small blood vessels of the tissues and organs, leading to a characteristic rash, muscle weakness, elevated muscle enzymes and sometimes involvement of vital organs. Better outcomes are achieved with early diagnosis and early aggressive treatment, often with significant side effects(\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e). Due to the heterogeneity in presentation and the rarity of the disease, the diagnosis of JDM is often delayed (\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e), enhancing anxiety, confusion and a sense of isolation for caregivers of children with JDM.\u003c/p\u003e \u003cp\u003eCaregivers of children with JDM have reported increased levels of stress, higher levels of anxiety and poorer quality of life(\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e) and parenting stress may adversely affect child health-related outcomes as it could potentially interfere with the management of the child\u0026rsquo;s chronic illness (\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e). The challenges for parents in caring for a child diagnosed with JDM may be amplified by the limited knowledge of JDM among non-paediatric rheumatology clinicians, difficulty accessing appropriate services, prolonged treatment, complexities in managing the condition, the burden of side effects of treatment and impacts on the family unit.\u003c/p\u003e \u003cp\u003e There are few studies examining the perspectives of parents or caregivers of children with JDM. This study aimed to describe the experiences of caregivers of children with JDM to inform strategies and interventions to address their needs.\u003c/p\u003e"},{"header":"METHODS","content":"\u003cdiv id=\"Sec3\" class=\"Section2\"\u003e \u003ch2\u003eParticipant selection\u003c/h2\u003e \u003cp\u003eParticipants were eligible if they were a parent or caregiver of a child (aged 0 to 18 years) with clinician diagnosed JDM and were English speaking. Participants were recruited through the Sydney Children\u0026rsquo;s Hospital Network, including the Children\u0026rsquo;s Hospital Westmead, The Children\u0026rsquo;s Hospital at Randwick and John Hunter Hospitals. Purposive sampling was used to capture a broad range of perspectives based on socioeconomic status, geographic location, ethnicity, their child\u0026rsquo;s disease course, sex and age. The project was approved by the Sydney Childrens\u0026rsquo; Hospital Network Ethics Committee under project number 2021/ETH00053.\u003c/p\u003e \u003c/div\u003e \u003cdiv id=\"Sec4\" class=\"Section2\"\u003e \u003ch2\u003eData collection\u003c/h2\u003e \u003cp\u003eA preliminary interview guide was developed based on the literature of the experiences and perspectives of caregivers of children with other paediatric rheumatic diseases(\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e, \u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e, \u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e). AHK conducted a semi-structured interview either face to face at a hospital clinic or by telephone, depending on participants preference or COVID- 19 requirements. Recruitment ceased once data saturation was reached (when no new themes or new concepts were emerging in the data). Interviews were recorded and then transcribed verbatim.\u003c/p\u003e \u003c/div\u003e \u003cdiv id=\"Sec5\" class=\"Section2\"\u003e \u003ch2\u003eData analysis\u003c/h2\u003e \u003cp\u003eTranscripts were entered into HyperRESEARCH version 4.0 to assist with the coding, storage and searching of the data. Using thematic analysis, the first author (AHK) coded the transcripts, line by line, conceptualising and categorizing the data and assigning codes to inductively identified concepts. Relationships between common concepts were explored in the data to develop analytical themes. A thematic schema was mapped to demonstrate the connection between themes. A second and third investigator AJ and AK, read and reviewed the preliminary themes to ensure that all experiences and perspectives of participants were included.\u003c/p\u003e \u003c/div\u003e"},{"header":"RESULTS","content":"\u003cp\u003eThe characteristics of the participants are shown in Table \u003cspan refid=\"Tab1\" class=\"InternalRef\"\u003e1\u003c/span\u003e. Nineteen of 27 (70%) caregivers who were contacted agreed to participate. The majority of participants were female (15, 79%) and all participants identified as their child\u0026rsquo;s biological parent. Table\u0026nbsp;\u003cspan refid=\"Tab2\" class=\"InternalRef\"\u003e2\u003c/span\u003e details the characteristics of the children of participants, including their initial presentation, the treatments they received and the number of specialists they had seen prior to a diagnosis being made.\u003c/p\u003e \u003cp\u003e \u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"Yes\" id=\"Tab1\" border=\"1\"\u003e \u003ccaption language=\"En\"\u003e \u003cdiv class=\"CaptionNumber\"\u003eTable 1\u003c/div\u003e \u003cdiv class=\"CaptionContent\"\u003e \u003cp\u003eCharacteristics of participants\u003c/p\u003e \u003c/div\u003e \u003c/caption\u003e \u003ccolgroup cols=\"3\"\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c1\" colnum=\"1\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c2\" colnum=\"2\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c3\" colnum=\"3\"\u003e\u003c/div\u003e \u003cthead\u003e \u003ctr\u003e \u003cth align=\"left\" colspan=\"2\" nameend=\"c2\" namest=\"c1\"\u003e \u003cp\u003eCharacteristic\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c3\"\u003e \u003cp\u003eNo. (%)\u003c/p\u003e \u003c/th\u003e \u003c/tr\u003e \u003c/thead\u003e \u003ctbody\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eBiological Mother\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c3\" namest=\"c2\"\u003e \u003cp\u003e15(79)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eBiological Father\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c3\" namest=\"c2\"\u003e \u003cp\u003e4(21)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cb\u003eAge (years)\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c3\" namest=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e40s\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c3\" namest=\"c2\"\u003e \u003cp\u003e14(74)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e50s\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c3\" namest=\"c2\"\u003e \u003cp\u003e4(21)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e60 and over\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c3\" namest=\"c2\"\u003e \u003cp\u003e1(5)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cb\u003eEducation\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c3\" namest=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSecondary\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c3\" namest=\"c2\"\u003e \u003cp\u003e1(5)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eCertificate/ Diploma\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c3\" namest=\"c2\"\u003e \u003cp\u003e2(11)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eBachelors/Higher\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c3\" namest=\"c2\"\u003e \u003cp\u003e16(84)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cb\u003eMarital Status\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c3\" namest=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eMarried/defacto\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c3\" namest=\"c2\"\u003e \u003cp\u003e19(100)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cb\u003eEmployment\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c3\" namest=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eCasual\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c3\" namest=\"c2\"\u003e \u003cp\u003e1(5)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eFull time\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c3\" namest=\"c2\"\u003e \u003cp\u003e9(47)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003ePart time\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c3\" namest=\"c2\"\u003e \u003cp\u003e5(26)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003enone\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c3\" namest=\"c2\"\u003e \u003cp\u003e4(21)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cb\u003eGeographical Location\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c3\" namest=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eMetropolitan\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c3\" namest=\"c2\"\u003e \u003cp\u003e14(74)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eRural\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c3\" namest=\"c2\"\u003e \u003cp\u003e5(26)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cb\u003eReligion\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c3\" namest=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eNone\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c3\" namest=\"c2\"\u003e \u003cp\u003e9(47)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eCatholic\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c3\" namest=\"c2\"\u003e \u003cp\u003e4 (21)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eProtestant\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c3\" namest=\"c2\"\u003e \u003cp\u003e2(11)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eChristian\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c3\" namest=\"c2\"\u003e \u003cp\u003e2(11)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cb\u003eEthnicity\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c3\" namest=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eCaucasian\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c3\" namest=\"c2\"\u003e \u003cp\u003e15(79)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eGreek\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c3\" namest=\"c2\"\u003e \u003cp\u003e1(5)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eFijian Indian\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c3\" namest=\"c2\"\u003e \u003cp\u003e1(5)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eJapanese\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c3\" namest=\"c2\"\u003e \u003cp\u003e2(11)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003c/tbody\u003e \u003c/colgroup\u003e \u003c/table\u003e\u003c/div\u003e \u003c/p\u003e \u003cp\u003e \u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"Yes\" id=\"Tab2\" border=\"1\"\u003e \u003ccaption language=\"En\"\u003e \u003cdiv class=\"CaptionNumber\"\u003eTable 2\u003c/div\u003e \u003cdiv class=\"CaptionContent\"\u003e \u003cp\u003eCharacteristics of the children of participants\u003c/p\u003e \u003c/div\u003e \u003c/caption\u003e \u003ccolgroup cols=\"2\"\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c1\" colnum=\"1\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c2\" colnum=\"2\"\u003e\u003c/div\u003e \u003cthead\u003e \u003ctr\u003e \u003cth align=\"left\" colname=\"c1\"\u003e \u003cp\u003eCharacteristics\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c2\"\u003e \u003cp\u003eNo. (%) n\u0026thinsp;=\u0026thinsp;18\u003c/p\u003e \u003c/th\u003e \u003c/tr\u003e \u003c/thead\u003e \u003ctbody\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eFemales\u003c/p\u003e \u003cp\u003eMales\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e9 (50)\u003c/p\u003e \u003cp\u003e9 (50)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eCountry of birth\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eAustralia\u003c/p\u003e \u003cp\u003eOther\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e17 (95)\u003c/p\u003e \u003cp\u003e1 (5)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eReligion\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eNil\u003c/p\u003e \u003cp\u003eCatholic\u003c/p\u003e \u003cp\u003eOther Christian\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e9 (50)\u003c/p\u003e \u003cp\u003e5 (28)\u003c/p\u003e \u003cp\u003e2 (11)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eEthnicity\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eCaucasian/Australian\u003c/p\u003e \u003cp\u003eTongan\u003c/p\u003e \u003cp\u003eIndian/ Fijian\u003c/p\u003e \u003cp\u003eGreek Australian\u003c/p\u003e \u003cp\u003eJapanese\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e14 (78)\u003c/p\u003e \u003cp\u003e1 (5)\u003c/p\u003e \u003cp\u003e1 (5)\u003c/p\u003e \u003cp\u003e1 (5)\u003c/p\u003e \u003cp\u003e1 (5)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eClinical presentation of illness\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eMuscle weakness\u003c/p\u003e \u003cp\u003eSkin rash\u003c/p\u003e \u003cp\u003eLethargy\u003c/p\u003e \u003cp\u003ePain\u003c/p\u003e \u003cp\u003eFalls\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e8 (44)\u003c/p\u003e \u003cp\u003e11 (61)\u003c/p\u003e \u003cp\u003e1 (5)\u003c/p\u003e \u003cp\u003e4 (22)\u003c/p\u003e \u003cp\u003e2 (11)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eAge at diagnosis\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e1\u0026ndash;5 years\u003c/p\u003e \u003cp\u003e6\u0026ndash;11 years\u003c/p\u003e \u003cp\u003e12\u0026ndash;15 years\u003c/p\u003e \u003cp\u003e15\u0026ndash;20 years\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e8 (44)\u003c/p\u003e \u003cp\u003e5 (28)\u003c/p\u003e \u003cp\u003e5 (28)\u003c/p\u003e \u003cp\u003e0 (0)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eCurrent treatment\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003ePrednisone\u003c/p\u003e \u003cp\u003eMethotrexate\u003c/p\u003e \u003cp\u003eIVIG\u003c/p\u003e \u003cp\u003eOther DMARD\u003c/p\u003e \u003cp\u003eTofacitinib\u003c/p\u003e \u003cp\u003eNone\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e6 (33)\u003c/p\u003e \u003cp\u003e11 (61)\u003c/p\u003e \u003cp\u003e3 (17)\u003c/p\u003e \u003cp\u003e1 (5)\u003c/p\u003e \u003cp\u003e2 (11)\u003c/p\u003e \u003cp\u003e4 (22)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003ePrevious treatment\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eNone\u003c/p\u003e \u003cp\u003eMethylprednisone\u003c/p\u003e \u003cp\u003ePrednisone\u003c/p\u003e \u003cp\u003eIVIG\u003c/p\u003e \u003cp\u003eMethotrexate\u003c/p\u003e \u003cp\u003eOther DMARD\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e3 (17)\u003c/p\u003e \u003cp\u003e4 (22)\u003c/p\u003e \u003cp\u003e10 (56)\u003c/p\u003e \u003cp\u003e1 (5)\u003c/p\u003e \u003cp\u003e7 (39)\u003c/p\u003e \u003cp\u003e1 (5)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eComorbidities\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eNone\u003c/p\u003e \u003cp\u003ePerthes disease\u003c/p\u003e \u003cp\u003eAsthma\u003c/p\u003e \u003cp\u003eCoeliac disease\u003c/p\u003e \u003cp\u003eADHD\u003c/p\u003e \u003cp\u003eOsteoporosis\u003c/p\u003e \u003cp\u003eSkin striae\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e11 (61)\u003c/p\u003e \u003cp\u003e1 (5)\u003c/p\u003e \u003cp\u003e1 (5)\u003c/p\u003e \u003cp\u003e1 (5)\u003c/p\u003e \u003cp\u003e1 (5)\u003c/p\u003e \u003cp\u003e1 (5)\u003c/p\u003e \u003cp\u003e2 (11)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eNo. of doctors initially referred to\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e1\u003c/p\u003e \u003cp\u003e2\u003c/p\u003e \u003cp\u003e3\u003c/p\u003e \u003cp\u003e4\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e4 (22)\u003c/p\u003e \u003cp\u003e11(61)\u003c/p\u003e \u003cp\u003e2(11)\u003c/p\u003e \u003cp\u003e1(6)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003c/tbody\u003e \u003c/colgroup\u003e \u003c/table\u003e\u003c/div\u003e \u003c/p\u003e \u003cp\u003eWe identified six themes: Rapid crescendo of fear and desperation, lost and unsupported in the health system, disrupting family routines, grieving what has been lost, managing an uncertain future, gaining confidence and motivation. The respective subthemes are described below and selected quotations are illustrated in Table \u003cspan refid=\"Tab3\" class=\"InternalRef\"\u003e3\u003c/span\u003e. Figure\u0026nbsp;1 illustrates the relationship between these themes in a thematic schema.\u003c/p\u003e \u003cp\u003e \u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"Yes\" id=\"Tab3\" border=\"1\"\u003e \u003ccaption language=\"En\"\u003e \u003cdiv class=\"CaptionNumber\"\u003eTable 3\u003c/div\u003e \u003cdiv class=\"CaptionContent\"\u003e \u003cp\u003eSelected quotations supporting each theme\u003c/p\u003e \u003c/div\u003e \u003c/caption\u003e \u003ccolgroup cols=\"2\"\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c1\" colnum=\"1\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c2\" colnum=\"2\"\u003e\u003c/div\u003e \u003cthead\u003e \u003ctr\u003e \u003cth align=\"left\" colname=\"c1\"\u003e \u003cp\u003eThemes and Subthemes\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c2\"\u003e \u003cp\u003eQuotations\u003c/p\u003e \u003c/th\u003e \u003c/tr\u003e \u003ctr\u003e \u003cth align=\"left\" colname=\"c1\"\u003e \u003cp\u003eRapid crescendo of fear and desperation\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/th\u003e \u003c/tr\u003e \u003c/thead\u003e \u003ctbody\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eAlarming deterioration\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e\u003cem\u003eShe never goes to the doctor because she\u0026rsquo;s never sick, and then one day she said to me \u0026lsquo;can you take me to the doctor, it\u0026rsquo;s really hurting\u0026rsquo;, and that\u0026rsquo;s when I thought oh, God, this must be not growing pains. (Mother, age 40s).\u003c/em\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSudden realisation of seriousness\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e\u003cem\u003e\u0026hellip;she needed help wiping herself and things like that, and we were like oh God, what\u0026rsquo;s going to happen, are we going to have to become carers, are we going to have to, one of us resign our jobs, things like that. (Mother, age 40s).\u003c/em\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eDesperate for a diagnosis\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e\u003cem\u003e\u0026hellip;then we couldn\u0026rsquo;t get him to see the neurologist for I think two months. We had this terrible time where we didn\u0026rsquo;t know what it was and we didn\u0026rsquo;t know what to do. (mother, age 40s).\u003c/em\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cb\u003eLost and unsupported in the health system\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eAt the mercy of the medical team\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e\u003cem\u003eI do feel a little in the dark\u0026hellip;.sometimes I wonder if there are things we are just not being told (Father, aged 50s).\u003c/em\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eFrustrated at lack of services\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e\u003cem\u003eBecause JDM is so specialised, you didn\u0026rsquo;t really fit anywhere (mother, aged 40s).\u003c/em\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eNeglected priorities\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e\u003cem\u003eHaving a teenager is pretty stressful as you may know \u0026hellip;but the combination of a teenager and then someone who\u0026rsquo;s unwell, the compounding can impact mental health (mother, aged 50s).\u003c/em\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eProtracted and painful search for answers\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e\u003cem\u003eWe probably saw six or seven doctors. Only one had heard of it, but didn\u0026rsquo;t know much about it (father, aged 50s)\u003c/em\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cb\u003eDisrupting family routines\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSibling neglect and loss\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e\u003cem\u003e\u0026hellip;during that time, she basically lost a little friend\u0026hellip;That was emotionally quite difficult for her and obviously for him, because he just wasn\u0026rsquo;t able to do anything (mother, aged 40s).\u003c/em\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eOverloaded with a medicalised schedule\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e\u003cem\u003eGenerally, you find that when you come into the hospital and you see the rheumatologist, it\u0026rsquo;s busy. It\u0026rsquo;s chaos\u0026hellip;I\u0026rsquo;d like to be able to ask a question and have a bit more time (mother, aged 40s).\u003c/em\u003e\u003c/p\u003e \u003cp\u003e\u003cem\u003eI guess the medication is probably the biggest responsibility, just ensuring that that\u0026rsquo;s all okay and making sure his scripts, monitoring that (mother, aged 50s)\u003c/em\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eAlways on standby\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e\u003cem\u003eIf he needs to take a day off school because he\u0026rsquo;s just not up to it, you know, I make sure I work from home that day \u0026hellip;(father, aged 50s).\u003c/em\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eBurdened by financial strains\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e\u003cem\u003eOur family was based on my single income and (my wife) wasn\u0026rsquo;t working at the time. I just had to find a way to somehow keep it together (father, aged 40s).\u003c/em\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cb\u003eGrieving what has been lost\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eMissing the sunlight\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e\u003cem\u003eI know she shouldn\u0026rsquo;t be out in the sun, and I know that that\u0026rsquo;s not great, but I don\u0026rsquo;t know to what extent\u0026hellip;I was like, does that mean no lunch time at school, no sport? (mother, aged 40s).\u003c/em\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eStruggling with the loss of physical function\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e\u003cem\u003e\u0026hellip;he was walking with someone who had a broken arm, and he was feeling just as sick as this kid that had a broken arm, and people were saying to him \u0026lsquo;well, why aren\u0026rsquo;t you helping that boy with the broken arm, because you\u0026rsquo;re fine?\u0026rsquo; and he was like I\u0026rsquo;m not fine, I\u0026rsquo;m really struggling to walk up and down the stairs. I think because you can\u0026rsquo;t see it, people are not as empathetic (mother, aged 50s).\u003c/em\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eDisrupted schooling\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e\u003cem\u003e\u0026hellip; there are days when he\u0026rsquo;s got a headache or a cold now... I\u0026rsquo;m a bit more lenient. If he needs a day off here or there, I\u0026rsquo;m all right (mother, aged 60s).\u003c/em\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eChanges in their child from steroid related side effects\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e\u003cem\u003eOnce he started the prednisone it completely interrupted his sleep, so there\u0026rsquo;s some days he\u0026rsquo;s completely exhausted from waking up continuously through the night (mother, aged 50s).\u003c/em\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cb\u003eManaging an uncertain future\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eBound to chronicity\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e\u003cem\u003e\u0026hellip;what\u0026rsquo;s it going to do, will it go away, will it get worse? You know\u0026hellip;it\u0026rsquo;s a very unpredictable disease, it can be chronic (mother, aged 40s).\u003c/em\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eFearing relapse\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e\u003cem\u003eI think it\u0026rsquo;s always there and present for him, because he knows it will never go away. So yes, I think maybe that\u0026rsquo;s the uncertainty around the disease coming back (mother, aged 40s).\u003c/em\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eInsecurity with transition to adult care\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e\u003cem\u003eI think the transition to adult and the adult rheumatology area, that\u0026rsquo;s probably where we\u0026rsquo;re a bit nervous at the moment because we know next year when he finishes school, he has to move into the adult world (mother, age 40s).\u003c/em\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cb\u003eGaining confidence and motivation\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eStrengthening partnership with clinicians\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e\u003cem\u003eThey relied on our observation to report anything that\u0026rsquo;s happening on a day to day basis and how the treatment is going. We felt quite involved in the treatment in that way, which was good (father, aged 40s)\u003c/em\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eGrowing maturity and independence\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e\u003cem\u003eHe is conscious of eating properly now. Whether this is due to the experiences that he had with this supposed dietary intervention or whether he\u0026rsquo;s doing it because he just doesn\u0026rsquo;t want to appear overweight, he\u0026rsquo;s conscious about what he eats. I think it\u0026rsquo;s in a positive space at the moment (father, aged 50s)\u003c/em\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eGaining hope from shared experiences\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e\u003cem\u003eBut she\u0026rsquo;s scoring goals, she\u0026rsquo;s getting awards, she\u0026rsquo;s loving it. Socially, she\u0026rsquo;s loving it, and the teams are fantastic. That\u0026rsquo;s one of the joys now that we\u0026rsquo;re out the other end (father, aged 50s).\u003c/em\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003c/tbody\u003e \u003c/colgroup\u003e \u003c/table\u003e\u003c/div\u003e \u003c/p\u003e \u003cdiv id=\"Sec7\" class=\"Section2\"\u003e \u003ch2\u003eRapid crescendo of fear and desperation\u003c/h2\u003e \u003cdiv id=\"Sec8\" class=\"Section3\"\u003e \u003ch2\u003eAlarming deterioration\u003c/h2\u003e \u003cp\u003eParents felt frightened and panicked at the rapid decline they saw in their child\u0026rsquo;s physical abilities. They were very concerned to witness their child being unable to do up their seat belt, falling over at the supermarket, or being unable to get out of bed, \u003cem\u003e\u0026ldquo;\u0026hellip; we went away bushwalking with some friends, and I had to carry her in the backpack\u0026rdquo;\u003c/em\u003e (mother, 60s).\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv id=\"Sec9\" class=\"Section2\"\u003e \u003ch2\u003eSudden realisation of seriousness\u003c/h2\u003e \u003cp\u003eParents initially were dismissive of child\u0026rsquo;s symptoms as a minor illness, part of normal development, or even growing pains, \u003cem\u003e\u0026ldquo;I just thought it might be a cold. I just thought it was growing pains.\u0026rdquo;\u003c/em\u003e (mother, age 40s). However, they gradually began to realise the seriousness of their condition as they could see their child was not recovering and in many cases deteriorating.\u003c/p\u003e \u003cdiv id=\"Sec10\" class=\"Section3\"\u003e \u003ch2\u003eDesperate for a diagnosis\u003c/h2\u003e \u003cp\u003eParents felt an increasing desperation when numerous clinicians were unable to find a diagnosis for their child\u0026rsquo;s symptoms \u003cem\u003e\u0026ndash; \u0026ldquo;The hardest part was the long wait to diagnosis\u0026rdquo;\u003c/em\u003e (mother, 40s). They felt disappointed and let down because of the delayed diagnosis, particularly when they felt general practitioners did not know about the condition \u0026ndash; \u0026ldquo;\u003cem\u003eour GP had not even heard of it\u0026rdquo;\u003c/em\u003e (mother, aged 40s). They found it difficult to understand that there was no clear answer as to why their child developed JDM and described feeling stupid when they repeatedly asked the clinicians why this happened to their child. They felt a sense of relief once the diagnosis of JDM was made.\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv id=\"Sec11\" class=\"Section2\"\u003e \u003ch2\u003eLost and unsupported in the health system\u003c/h2\u003e \u003cdiv id=\"Sec12\" class=\"Section3\"\u003e \u003ch2\u003eAt the mercy of the medical team\u003c/h2\u003e \u003cp\u003eParents felt intimidated and disempowered by the use of medical terms that were difficult to understand. Protocols for administration of medications, including weekly dosing and the regular blood monitoring for medications also contributed to a sense of being overwhelmed with a rigid medical schedule. Parents relied on the medical team to educate them about JDM and its treatments. However, many parents described feeling lost, \u0026ldquo;\u003cem\u003eI do feel a little in the dark\u0026hellip;. sometimes I wonder if there are things we are just not being told.\u003c/em\u003e (father, aged 50s)\u0026rdquo;. Parents from culturally and linguistically diverse backgrounds (CALD), who were not familiar with the health system found it even more difficult to understand. Parents felt overwhelmed by the complexity of the disease including the varying way the disease could initially present and its response to treatment. They understood that treatments were being determined by an individual clinician\u0026rsquo;s experience, leading them to feel like there was no standard treatment protocol being followed, \u0026lsquo;\u0026rsquo;\u003cem\u003eThere seems to be so much happening with the disease\u0026hellip; it\u0026rsquo;s all very subjective, I feel like we are 100% reliant on the\u003c/em\u003e (Doctor\u0026rsquo;s) \u003cem\u003egut feel\u0026rdquo;\u003c/em\u003e (mother, aged 40s).\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv id=\"Sec13\" class=\"Section2\"\u003e \u003ch2\u003eFrustrated at lack of services\u003c/h2\u003e \u003cp\u003e Parents were shocked by the limited services once their child was diagnosed with JDM. They described feeling forgotten and alone when they discovered there was no single point of contact to coordinate their child\u0026rsquo;s care. Parents in rural or remote areas felt vulnerable as it was difficult to travel long distances to medical appointments in a large metropolitan city, \u003cem\u003e\u0026ldquo;it was scary being 5 hours away from the specialist\u003c/em\u003e, \u003cem\u003eit\u0026rsquo;s hard when we are the only ones in a rural town\u003c/em\u003e (with the disease)\u0026rsquo;\u0026rsquo; (mother, aged 40s).\u003c/p\u003e \u003c/div\u003e \u003cdiv id=\"Sec14\" class=\"Section2\"\u003e \u003ch2\u003eNeglected priorities\u003c/h2\u003e \u003cp\u003eParents reported being disillusioned and upset when they perceived that clinicians were focussed on the treatment of muscle weakness and did not address other symptoms, including pain, and mental health issues, \u003cem\u003e\u0026ldquo;I can\u0026rsquo;t localise it, it\u0026rsquo;s pretty broad. It\u0026rsquo;s either muscle pain, back pain, stomach pain, it comes in different forms. I don\u0026rsquo;t think that has really been addressed or understood in a detailed manner\u0026hellip;.it\u0026rsquo;s something that should\u0026rsquo;ve been teased out a little bit more\u0026rdquo; (father, aged 50s).\u003c/em\u003e\u003c/p\u003e \u003c/div\u003e \u003cdiv id=\"Sec15\" class=\"Section2\"\u003e \u003ch2\u003eProtracted and painful search for answers\u003c/h2\u003e \u003cp\u003eParents were desperate for the treatments for their children to work, they were prepared to try anything, and some sought non-pharmacological ways to manage the condition including seeking out alternative therapies \u0026ldquo;\u003cem\u003eWe did try a few elimination diets\u0026rdquo;\u003c/em\u003e (mother aged 40s) and, \u0026ldquo;\u003cem\u003eWe were prepared to try anything\u0026rdquo; (mother aged 40s)\u003c/em\u003e, knowing that these were not recommended therapies for children with JDM. Parents described spending many hours researching online and struggled with the lack of specific information relevant to the Australian health care system. Parents discovered that treatment practices varied internationally depending on the availability of treatments and the local health care system. Variation in treatment practices internationally coupled with discovering worst-case scenarios online, including finding examples of children with lifelong disabilities, was both alarming and frightening.\u003c/p\u003e \u003c/div\u003e \u003cdiv id=\"Sec16\" class=\"Section2\"\u003e \u003ch2\u003eDisrupting family routines\u003c/h2\u003e \u003cdiv id=\"Sec17\" class=\"Section3\"\u003e \u003ch2\u003eSibling neglect and loss\u003c/h2\u003e \u003cp\u003e In focussing their attention on their child with JDM, some parents felt that their relationship with their other children, or among siblings, suffered. For example, previously siblings were able to play together, this was not possible because of disease symptoms or medication side effects, \u0026lsquo;\u0026rsquo;\u003cem\u003eshe basically lost a little friend\u0026rsquo;\u003c/em\u003e (mother, 40s). Participants were frustrated that their other children were missing out on school and important life events because of the time taken caring for their child with JDM. Parents were also concerned for future potential mental health problems for siblings, \u003cem\u003e\u0026ldquo;we\u003c/em\u003e [the whole family, including siblings] \u003cem\u003ewere semi-suicidal for a lot of last year\u0026hellip;\u0026rdquo;\u003c/em\u003e (mother, aged 40s).\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv id=\"Sec18\" class=\"Section2\"\u003e \u003ch2\u003eOverloaded with a medicalised schedule\u003c/h2\u003e \u003cp\u003eAttending multiple medical appointments was time consuming and parents described the changes they had made to their family\u0026rsquo;s routine \u0026lsquo;\u0026rsquo;\u003cem\u003eour family has made Saturday a rest day; that is the day she takes medications\u0026rsquo;\u0026rsquo;\u003c/em\u003e (mother, aged 40s). Parents spoke of having to accommodate the burden of responsibility for managing their child\u0026rsquo;s medications, including minimising family activities because of side effects from medications, monitoring for their child\u0026rsquo;s response to treatment as well as being the central coordinators of their child\u0026rsquo;s care, made them feel that they were different to other families; \u003cem\u003e\u0026ldquo;we are not a normal family anymore\u0026rsquo;\u0026rsquo;\u003c/em\u003e (mother, aged 50s).\u003c/p\u003e \u003c/div\u003e \u003cdiv id=\"Sec19\" class=\"Section2\"\u003e \u003ch2\u003eAlways on standby\u003c/h2\u003e \u003cp\u003eSome participants described having to give up all other parts of their lives, including their job, travel, social events and career aspirations to constantly be there for their child, \u003cem\u003e\u0026lsquo;\u0026rsquo;I sat outside the school for hours, knowing that he might need me to come and get him at any point\u0026rdquo;\u003c/em\u003e (mother, aged 50s). This was described as frustrating, emotionally demanding and time consuming, particularly for mothers who felt they carried the majority of the caring load for their child.\u003c/p\u003e \u003c/div\u003e \u003cdiv id=\"Sec20\" class=\"Section2\"\u003e \u003ch2\u003eBurdened by financial strains\u003c/h2\u003e \u003cp\u003e Parents were stressed juggling their work or business, for example, time taken attending multiple medical appointments was time they had to take off work, which was particularly burdensome when they ran their own businesses. Families carried the burden of costs of travelling to multiple appointments (particularly for regional families), including self-funding allied health therapies, because there were no publicly available services. Finances were a source of tension in many households, \u0026ldquo;\u003cem\u003ethe only thing we fight about every month is the credit card bill\u0026rdquo;\u003c/em\u003e (mother, aged 50s).\u003c/p\u003e \u003c/div\u003e \u003cdiv id=\"Sec21\" class=\"Section2\"\u003e \u003ch2\u003eGrieving what has been lost\u003c/h2\u003e \u003cdiv id=\"Sec22\" class=\"Section3\"\u003e \u003ch2\u003eMissing the sunlight\u003c/h2\u003e \u003cp\u003eParents understood it was important to avoid the sun to prevent worsening their child\u0026rsquo;s skin disease. It saddened them to have to reconsider family outings and avoid the hottest time of the day, however it also provided an opportunity to proactively manage part of their child\u0026rsquo;s disease. Sun exposure did cause anxiety in parents, and they were confused and worried about managing their child\u0026rsquo;s exposure to the sun particularly when they were not with them. They felt sorry for their child when they missed out on activities to avoid the sun; \u0026ldquo;\u003cem\u003eYou\u0026rsquo;ve got a little boy who just wants to be like every other kid and doesn\u0026rsquo;t want to be out of the sun\u003c/em\u003e\u0026rdquo;, (father, aged 50s).\u003c/p\u003e \u003c/div\u003e \u003cdiv id=\"Sec23\" class=\"Section3\"\u003e \u003ch2\u003eStruggling with the loss of physical function\u003c/h2\u003e \u003cp\u003eParents were upset at the manifestations of the disease in their child and felt compelled to protect their children from the negative comments of others, knowing that their child\u0026rsquo;s symptoms were only visible to those that knew them well. They felt vulnerable and defensive towards those that did not appreciate the suffering of their child, \u0026lsquo;\u0026rsquo;\u003cem\u003eHe was walking with someone who had a broken arm, and he was feeling just as sick as the kid with the broken arm and people were saying, Why aren\u0026rsquo;t you helping that boy with the broken arm?\u0026rdquo; (\u003c/em\u003emother, aged 50s). Parents wanted their child to do more, hoping that exercise would improve their symptoms and they felt guilty when they saw their child struggling or in pain.\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv id=\"Sec24\" class=\"Section2\"\u003e \u003ch2\u003eDisrupted schooling\u003c/h2\u003e \u003cp\u003eParents were despondent to see their child struggle to attend school because of their illness and suffer from side effects of treatment; \u0026ldquo;\u003cem\u003emy child is a primary school drop out\u0026rsquo;\u0026rsquo;\u003c/em\u003e (mother, aged 50s). The majority of parents felt supported by the child\u0026rsquo;s school, however some reported having to justify and negotiate more support services from their school, \u0026ldquo;\u003cem\u003eschool says he will catch up\u0026hellip;.but this disease isn\u0026rsquo;t going anywhere\u0026rdquo;\u003c/em\u003e (mother, aged 40s). Paradoxically, parents reported that COVID remote learning helped their child focus on improving their health whilst maintaining connection with their schoolwork, in a safe environment, \u0026lsquo;\u0026rsquo;\u003cem\u003eWe had a COVID safe bubble\u003c/em\u003e\u0026rdquo;(mother, aged 40s). Parents described a sense of relief and satisfaction that their child was not missing out on school.\u003c/p\u003e \u003cdiv id=\"Sec25\" class=\"Section3\"\u003e \u003ch2\u003eChanges in their child from steroid related side effects\u003c/h2\u003e \u003cp\u003eParticipants described feeling very despondent by the changes they saw in their child from the side effects of treatments, particularly steroid side effects. They saw their child gain weight and become self-conscious \u003cem\u003e\u0026lsquo;\u0026rsquo;every couple of months we go up a size, he is starting to feel different to other kids\u0026rdquo;\u003c/em\u003e (mother aged 50s). Parents were upset by memories of their child\u0026rsquo;s struggles and were sensitive to their self-image, which was amplified when their child moved into their teenage years, \u003cem\u003e\u0026ldquo;\u0026rsquo;She won\u0026rsquo;t even go back and look at photos of herself when she was like that\u003c/em\u003e (mother, aged 40s). Other steroid side effects, including emotional lability was very alarming; \u0026ldquo;\u003cem\u003eI give my son toxins every day that make him feel like he wants to die, and then have to spend the rest of the day telling him that it\u0026rsquo;s worth living and there is hope\u0026rdquo;\u003c/em\u003e (mother, aged 40s).\u003c/p\u003e \u003c/div\u003e \u003cdiv id=\"Sec26\" class=\"Section3\"\u003e \u003ch2\u003eManaging an uncertain future\u003c/h2\u003e \u003cdiv id=\"Sec27\" class=\"Section4\"\u003e \u003ch2\u003eBound to chronicity\u003c/h2\u003e \u003cp\u003eParents did not expect JDM to take so long to achieve remission, nor did they initially appreciate the need for ongoing treatment, \u0026ldquo;\u003cem\u003ewe didn\u0026rsquo;t realise that it\u0026rsquo;s not fixed overnight\u0026rsquo;\u0026rsquo;\u003c/em\u003e (mother, aged 50s). Parents became aware that their child may cycle in and out of remission and many parents were shocked and confused by this, they had not expected the disease to behave in this way.\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv id=\"Sec28\" class=\"Section2\"\u003e \u003ch2\u003eFearing relapse\u003c/h2\u003e \u003cp\u003eParticipants described their greatest fear was a relapse of their child\u0026rsquo;s illness, requiring further treatment and the potential side effects that may follow. They felt anxious about what the future might hold for their family; to the extent that some \u0026ldquo;\u003cem\u003etried not to think about the future\u0026rdquo;\u003c/em\u003e (mother aged, 40s). Parents were unsure as to how they or their child would cope if they experienced a relapse of the disease.\u003c/p\u003e \u003c/div\u003e \u003cdiv id=\"Sec29\" class=\"Section2\"\u003e \u003ch2\u003eInsecurity with transition to adult care\u003c/h2\u003e \u003cp\u003eParents were concerned about how their child would transition from paediatric care to adult medical services, including worrying about how their child would navigate the complexity of their disease such as sun avoidance and know if their disease had relapsed,\u0026hellip; \u0026lsquo;\u0026rsquo;\u003cem\u003ewe know next year when he finishes school, he has to move into the adult world. I don\u0026rsquo;t think the experience will be as one-on-one or personable as what it is under the paediatric rheumatology environment\u003c/em\u003e (mother, aged 50s)\u0026rdquo;.\u003c/p\u003e \u003c/div\u003e\n\u003ch3\u003eGaining confidence and motivation\u003c/h3\u003e\n\u003cdiv id=\"Sec31\" class=\"Section2\"\u003e \u003ch2\u003eStrengthening partnerships with clinicians\u003c/h2\u003e \u003cp\u003eParents appreciated feeling involved in their child\u0026rsquo;s treatment. This was aided by clinicians sharing pathology results with them, such as creatinine kinase (CK) to monitor response to treatment. At regular follow up appointments, they waited anxiously for their child\u0026rsquo;s results and celebrated when they saw improvements in objective markers, such as CK.\u003c/p\u003e \u003c/div\u003e \u003cdiv id=\"Sec32\" class=\"Section2\"\u003e \u003ch2\u003eGrowing maturity and independence\u003c/h2\u003e \u003cp\u003eWith the intense focus on their child\u0026rsquo;s physical health and managing side effects, such as weight gain from steroids or worsening skin disease from sun exposure, parents saw their child become more aware of healthy habits and avoiding sunlight. Parents felt a sense of pride when they observed how their child matured and learned to navigate their condition with their support, \u0026ldquo;\u003cem\u003eI know that [he has an] awareness that he doesn\u0026rsquo;t want in be in the sun\u0026rdquo;\u003c/em\u003e (mother aged 60s).\u003c/p\u003e \u003cdiv id=\"Sec33\" class=\"Section3\"\u003e \u003ch2\u003eGaining hope from shared experiences\u003c/h2\u003e \u003cp\u003eParents found it helpful to talk to other parents affected by JDM, they wanted to understand other\u0026rsquo;s experiences. This enabled them to visualise what disease remission looked like. It was their way \u0026ldquo;\u0026hellip;. of \u003cem\u003elooking for the light at the end of the tunnel\u0026rsquo;\u0026rsquo;\u003c/em\u003e (father, aged 50\u003c/p\u003e \u003cp\u003es) and it gave them hope for the future.\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e"},{"header":"Discussion","content":"\u003cp\u003eParents of children with JDM experienced a sudden fear when they realised that their child was seriously unwell and become desperate for a diagnosis. They felt frustrated at the lack of knowledge by many in the medical community about JDM. Once the diagnosis had been made and their child was receiving treatment for JDM, parents described feeling overwhelmed by the rigid medical schedule that took over their family life. They felt isolated because of the lack of services to support their child, finding it challenging to access allied health services with adequate knowledge of the disease. Parents found it difficult to process the diagnosis because their doctors were unable to determine the cause of JDM. Parents also reported experiencing increased financial strain. Complicated treatment regimes, including high doses of steroids, sun avoidance measures and the requirement for regular blood monitoring led parents to describe a sense of feeling overwhelmed by the \u0026ldquo;medicalisation\u0026rsquo;\u0026rsquo; of their lives and they grieved the disruption in their family\u0026rsquo;s routine. Parents were saddened to see their child have their schooling disrupted and feared relapse, often because they had seen online worse case scenarios of children with severe disease, yet they gained hope and confidence when they witnessed positive progress in their child. Parents were pleased when they saw the development of independence and confidence in their child\u0026rsquo;s self-management.\u003c/p\u003e \u003cp\u003eParents of children with chronic illness experience greater levels of stress and lower quality of life(\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e). A study examining the experiences of caregivers of children with JDM in the United States, reported that parents\u0026rsquo; quality of life was reduced and their mood adversely affected (\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e). Our study identified similar stress amongst parents. Another childhood rheumatic disease, Juvenile idiopathic arthritis has been described as a family disease(\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e), similarly our study also identifies direct impacts on parents and their families. Parents described their sadness at the disruption to their normal family routine, changes in relationships between siblings, demands of attending medical appointments and the need to drop everything to support their child. These burdens were especially carried by mothers in our cohort and existing evidence in the literature confirms the major caregiving role to the mother(\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e) in childhood chronic diseases. The demands of caregiving and effects on financial resources can have a negative impact on women\u0026rsquo;s professional and social lives(\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e).\u003c/p\u003e \u003cp\u003e Our study provides broad insights into the perspectives of parents of children with JDM. Purposive sampling was used to include a diverse group of participants including men, women, culturally and linguistically diverse participants and those from regional and metropolitan areas. However, there are some potential limitations. Most of the participants had a high level of educational attainment, and approximately 20% of participants were from culturally and linguistically diverse backgrounds (CALD). The transferability of our findings to non-English speaking populations is thus uncertain.\u003c/p\u003e \u003cp\u003eWe identified specific areas of concern for parents with implications for clinical practice, including delayed diagnosis, lack of JDM specific services, monitoring treatment response, sun avoidance strategies and disruption to schooling. Limited awareness of JDM, its heterogenous presentation(\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e) and limited paediatric rheumatology services across Australia all contribute to the likelihood of a delayed diagnosis. Previous studies have indicated that the mean time to diagnosis is 8.5 months(\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e). In our cohort, there was a high likelihood of delayed diagnosis, with 78% of participants reported seeing at least 2 specialists (after being referred by their General Practitioner) prior to the diagnosis of JDM being made (Table\u0026nbsp;\u003cspan refid=\"Tab2\" class=\"InternalRef\"\u003e2\u003c/span\u003e). In other chronic diseases, delayed access to specialist care results in deteriorating health, more frequent hospital admissions and poorer health outcomes(\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e). This is particularly true for rural and regional patients, who may have to travel long distances with the associated time and the financial demands that entails(\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e). It may not be possible to educate every health practitioner specifically about JDM, however understanding abnormal musculoskeletal and/or dermatological presentations in children is integral to reducing the time to diagnosis, in addition to the urgent need to expand access to paediatric rheumatology services across Australia.\u003c/p\u003e \u003cp\u003eWe identified further implications for clinical practice, including treating teams recognising the importance of involving parents and children in their disease\u0026rsquo;s management. Parents felt a sense of success (\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e) when \u0026ldquo;celebrating\u0026rdquo; a normal creatinine kinase level or seeing improvement in their child\u0026rsquo;s CMAS (Childhood Myositis Assessment Score). Many reported that closely following sun avoidance recommendations disrupted family routines, however managing their child\u0026rsquo;s sun exposure also gave parents a sense of control over an aspect of their child\u0026rsquo;s disease. Clinicians should focus on effectively communicating the goals and measurements of treatment, recognising the importance of communicating when those goals have been met and providing a clear framework for sun avoidance measures, to avoid confusion and empower parents. Further, parents understand the importance of minimising disruption to their child\u0026rsquo;s schooling (\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e), with current research suggesting that school disruptions during the COVID-19 era had adverse effects on child health and wellbeing (\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e). Our study was conducted during the COVID-19 pandemic and participants in our study reported a sense of relief that their child was not missing out on educational opportunities when compared to their peers. Multidisciplinary management of families with JDM should include working collaboratively with educational partners to ensure minimal disruption to schooling. Pandemic home-schooling models may provide a template for JDM patients to better engage in schoolwork.\u003c/p\u003e \u003cp\u003eOur study identified rationalising the use of steroids as an important area for future research. In our cohort, parental stress was impacted by steroid related side effects. Rheumatic diseases are often complex in their management and often require long term management(\u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e). Similarly JDM may also require complex medication regimes, often lacking evidence base and usually relying on the experience of the treating paediatric rheumatologist (\u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e). It may be difficult to predict an individual patient\u0026rsquo;s response to treatment(\u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e) and high dose steroids may be required(\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e), with the potential for significant side effects including; weight gain, stunted growth, bone loss (\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e), mood disorders and psychosis(\u003cspan citationid=\"CR15\" class=\"CitationRef\"\u003e15\u003c/span\u003e). The distress and morbidity caused by side effects of high dose steroids (which can in part fuel the fear of relapse for parents) highlights the urgency to find new, less toxic treatments.\u003c/p\u003e"},{"header":"Conclusion","content":"\u003cp\u003eOur study provides a unique insight into the experience of caregivers of children with juvenile dermatomyositis as they navigated their child\u0026rsquo;s initial presentation, diagnosis and response to treatment. Key areas of concern include delays in diagnosis, lack of access to services, response to treatment, fear of relapse, toxic side effects from medications and the unknown causes of the disease. This study provides an important platform for understanding how to better support families with JDM, providing clinicians and policy makers the evidence they need to improve the comprehensive management of this rare disease, to enhance treatment strategies and better inform future research directions.\u0026nbsp;\u003c/p\u003e"},{"header":"Abbreviations","content":"\u003cp\u003eJDM: Juvenile Dermatomyositis\u003c/p\u003e\n\u003cp\u003eAHK: Dr Amy H Kelly\u003c/p\u003e\n\u003cp\u003eAJ: Professor Allison Jaure\u003c/p\u003e\n\u003cp\u003eAK: Dr Ayano Kelly\u003c/p\u003e\n\u003cp\u003eCK: Creatinine Kinase\u003c/p\u003e\n\u003cp\u003eCMAS: Childhood Myositis Assessment Score\u003c/p\u003e\n\u003cp\u003eCALD: Culturally and linguistically diverse\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003eEthics: the project\u0026nbsp;was approved by the Sydney Childrens’ Hospital Network Ethics Committee under project number\u0026nbsp;2021/ETH00053\u003c/p\u003e\n\u003cp\u003eConsent for publication: not applicable\u003c/p\u003e\n\u003cp\u003eAvailability of data and materials: The datasets used and/or analysed during the current study are available from the corresponding author on reasonable request.\u003c/p\u003e\n\u003cp\u003eCompeting interests:\u0026nbsp;The authors declare that they have no competing interests\u003c/p\u003e\n\u003cp\u003eFunding: Dr A.H Kelly received a University of Sydney postgraduate research scholarship that contributed to funding this project.\u003c/p\u003e\n\u003cp\u003eAuthors' contributions:AHK devised the project, collected data, analysed data and was the major contributor to writing the final manuscript. AK contributed to analysing the data and contributed to writing the final manuscript. DSG contributed to the collection of data and to the writing of the final manuscript. JC contributed to the collection of data. AJ contributed to analysing the data and writing the final manuscript.\u003c/p\u003e\n\u003cp\u003eAcknowledgements: not applicable\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\n \u003cli\u003eWu Q, Wedderburn LR, McCann LJ. Juvenile dermatomyositis: Latest advances. Best Pract Res Clin Rheumatol 2017;31:535-57.\u003c/li\u003e\n \u003cli\u003eKountz-Edwards S, Aoki C, Gannon C, Gomez R, Cordova M, Packman W. The family impact of caring for a child with juvenile dermatomyositis. Chronic Illn 2017;13:262-74.\u003c/li\u003e\n \u003cli\u003eCousino MK, Hazen RA. Parenting stress among caregivers of children with chronic illness: A systematic review. J Pediatr Psychol 2013;38:809-28.\u003c/li\u003e\n \u003cli\u003eKountz-Edwards S. The psychosocial impact of juvenile dermatomyositis on pediatric patients and parents. Dissertation Abstracts International: Section B: The Sciences and Engineering 2016;77:No Pagination Specified.\u003c/li\u003e\n \u003cli\u003eGrazziotin LR, Currie G, Twilt M, MJ IJ, Kip MMA, Koffijberg H, et al. Factors associated with care- and health-related quality of life of caregivers of children with juvenile idiopathic arthritis. Pediatric rheumatology online journal 2022;20:51.\u003c/li\u003e\n \u003cli\u003eSimşek IE, Erel S, Simşek TT, Atasavun Uysal S, Yakut H, Yakut Y, et al. Factors related to the impact of chronically disabled children on their families. Pediatr Neurol 2014;50:255-61.\u003c/li\u003e\n \u003cli\u003eJob J, Nicholson C, Calleja Z, Jackson C, Donald M. Implementing a general practitioner-to-general physician econsult service (econsultant) in australia. BMC Health Serv Res 2022;22:1278.\u003c/li\u003e\n \u003cli\u003eSmith J, Cheater F, Bekker H. Parents\u0026apos; experiences of living with a child with a long-term condition: A rapid structured review of the literature. Health Expect 2015;18:452-74.\u003c/li\u003e\n \u003cli\u003eThornton CP, Ruble K, Jacobson LA. Education for children with chronic illness: Moving forward in online and virtual learning. JAMA Pediatr 2022;176:341-2.\u003c/li\u003e\n \u003cli\u003eRajmil L, Hjern A, Boran P, Gunnlaugsson G, Kraus de Camargo O, Raman S. Impact of lockdown and school closure on children\u0026apos;s health and well-being during the first wave of covid-19: A narrative review. BMJ Paediatr Open 2021;5:e001043.\u003c/li\u003e\n \u003cli\u003eBakker MM, Putrik P, Dikovec C, Rademakers J, Vonkeman HE, Kok MR, et al. Exploring discordance between health literacy questionnaire scores of people with rmds and assessment by treating health professionals. Rheumatology (Oxford) 2022;62:52-64.\u003c/li\u003e\n \u003cli\u003eBellutti Enders F, Bader-Meunier B, Baildam E, Constantin T, Dolezalova P, Feldman BM, et al. Consensus-based recommendations for the management of juvenile dermatomyositis. Ann Rheum Dis 2017;76:329-40.\u003c/li\u003e\n \u003cli\u003eWu JQ, Lu MP, Reed AM. Juvenile dermatomyositis: Advances in clinical presentation, myositis-specific antibodies and treatment. World J Pediatr 2020;16:31-43.\u003c/li\u003e\n \u003cli\u003eFerrara M, Borrelli B, Greco N, Coppola L, Coppola A, Simeone G, et al. Side effects of corticosteroid therapy in children with chronic idiopathic thrombocytopenic purpura. Hematology 2005;10:401-3.\u003c/li\u003e\n \u003cli\u003eBuchman AL. Side effects of corticosteroid therapy. J Clin Gastroenterol 2001;33:289-94.\u003c/li\u003e\n\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":false,"highlight":"","institution":"","isAcceptedByJournal":true,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"pediatric-rheumatology","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"proj","sideBox":"Learn more about [Pediatric Rheumatology](http://ped-rheum.biomedcentral.com)","snPcode":"","submissionUrl":"https://www.editorialmanager.com/proj/default.aspx","title":"Pediatric Rheumatology","twitterHandle":"@BioMedCentral","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"em","reportingPortfolio":"BMC/SO AJ","inReviewEnabled":true,"inReviewRevisionsEnabled":true},"keywords":"Juvenile Dermatomyositis, qualitative methods, interviews, patient-centred care, caregivers","lastPublishedDoi":"10.21203/rs.3.rs-4858416/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-4858416/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003ch2\u003eBackground\u003c/h2\u003e \u003cp\u003eJuvenile Dermatomyositis (JDM) is a rare, childhood inflammatory disease and its management can be challenging and confronting for both clinicians and caregivers. Little is known about the perspectives of parental caregivers of children with JDM. This study aimed to describe the experiences of parents of children with JDM to inform person-centred care.\u003c/p\u003e\u003ch2\u003eMethods\u003c/h2\u003e \u003cp\u003eSemi-structured interviews (face-to-face, telephone) were conducted with parents of children with JDM from three centres in Australia. Transcripts were analysed thematically.\u003c/p\u003e\u003ch2\u003eResults\u003c/h2\u003e \u003cp\u003eNineteen parents (15 mothers) of 18 children aged 8 to 21 with JDM participated. Six themes were identified. Rapid crescendo of fear and desperation (alarming deterioration, sudden realisation of seriousness, desperate for a diagnosis ), lost and unsupported in the health system (at the mercy of the medical team, frustrated at the lack of services, neglected priorities, protracted and painful search for answers), disrupting family routines (sibling neglect and loss, overloaded with a medicalised schedule, always on standby, burdened by financial strains), grieving what has been lost (missing the sunlight, struggling with the loss of physical function, disrupted schooling, changes in their child from steroid side effects), managing an uncertain future (bound to chronicity, fearing relapse, insecurity with transition to adult care), gaining confidence and motivation (strengthening partnerships with clinicians, growing maturity and independence, gaining hope from shared experiences).\u003c/p\u003e\u003ch2\u003eConclusions\u003c/h2\u003e \u003cp\u003eThe diagnosis of JDM is often delayed and caregivers of children with JDM report distress, disruption and uncertainty throughout their treatment journey with their child. Addressing these fears and establishing support mechanisms that help parents navigate their way through the medical system and support changing family dynamics are vital to optimise health outcomes for children diagnosed with JDM.\u003c/p\u003e","manuscriptTitle":"Perspectives and experiences of parents of children with juvenile dermatomyositis: A semi-structured interview study.","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2024-10-21 10:47:20","doi":"10.21203/rs.3.rs-4858416/v1","editorialEvents":[{"type":"communityComments","content":0},{"type":"decision","content":"Major revision","date":"2024-12-27T11:21:07+00:00","index":"","fulltext":""},{"type":"reviewerAgreed","content":"","date":"2024-09-03T22:05:41+00:00","index":0,"fulltext":""},{"type":"reviewersInvited","content":"","date":"2024-09-03T16:57:07+00:00","index":"","fulltext":""},{"type":"editorAssigned","content":"","date":"2024-09-03T09:20:30+00:00","index":"","fulltext":""},{"type":"submitted","content":"Pediatric Rheumatology","date":"2024-08-28T22:09:11+00:00","index":"","fulltext":""}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"pediatric-rheumatology","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"proj","sideBox":"Learn more about [Pediatric Rheumatology](http://ped-rheum.biomedcentral.com)","snPcode":"","submissionUrl":"https://www.editorialmanager.com/proj/default.aspx","title":"Pediatric Rheumatology","twitterHandle":"@BioMedCentral","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"em","reportingPortfolio":"BMC/SO AJ","inReviewEnabled":true,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"18755ad8-457f-4e14-ae35-b4fc3d4c5e3d","owner":[],"postedDate":"October 21st, 2024","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"published-in-journal","subjectAreas":[],"tags":[],"updatedAt":"2025-03-31T16:02:50+00:00","versionOfRecord":{"articleIdentity":"rs-4858416","link":"https://doi.org/10.1186/s12969-025-01079-2","journal":{"identity":"pediatric-rheumatology","isVorOnly":false,"title":"Pediatric Rheumatology"},"publishedOn":"2025-03-28 15:57:48","publishedOnDateReadable":"March 28th, 2025"},"versionCreatedAt":"2024-10-21 10:47:20","video":"","vorDoi":"10.1186/s12969-025-01079-2","vorDoiUrl":"https://doi.org/10.1186/s12969-025-01079-2","workflowStages":[]},"version":"v1","identity":"rs-4858416","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-4858416","identity":"rs-4858416","version":["v1"]},"buildId":"qtupq5eGEP_6zYnWcrvyt","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}