Unveiling the humanistic burden of C3 glomerulopathy in Italy: a narrative medicine multicentre project

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Evaluating the impact of C3 glomerulopathy (C3G) on quality of life (QoL) and its humanistic burden is critical to a deeper understanding of the condition in clinical practice. This Narrative Medicine (NM) project collected C3G narratives to investigate QoL and humanistic challenges associated with C3G and to gain insights for a more profound knowledge of this disease. Methods. The project was conducted in Italy from January to October 2023 and involved eight hospital-based centres and two C3G patient associations. Illness plots and parallel charts were collected through the project website, along with a survey to collect sociodemographic and QoL data. Narratives were analysed using MAXQDA software and interpretive coding. Results. Seventeen adult patients with C3G and 14 informal caregivers participated in the project, along with 12 treating nephrologists who wrote 15 parallel charts. On receiving the diagnosis, 36% of patients and 20% of caregivers reported feeling anxious, while 46% of patients and 50% of caregivers expressed disbelief. Nephrologists experienced anxiety in 20% and found the diagnosis a challenging moment in 26% of parallel charts. Concerns about C3G evolution were reported by 25% of patients and 40% of caregivers and nephrologists, while reassurance was expressed by 42% of patients and 60% of caregivers and nephrologists. Treatments were defined as burdensome by 33% of patients and hopeful by 17%. Patient and caregiver satisfaction with the care pathway was reported by 58% and 60%, respectively, while 45% of patients, 18% of caregivers and nephrologists in 40% of parallel charts called for more research funding and therapies. C3G significantly impacted work for 50% of patients, resulting in an average loss of 52.7% of working days. The impact on social activities and relationships varied, with 40% of patients reporting a low impact and 40% a moderate to high impact. Conclusions. This project marked the first Italian NM initiative on C3G. NM has shown to be a valuable tool for understanding the C3G illness experience and raising awareness about the living with this condition. C3 glomerulopathy quality of life humanistic burden narrative medicine illness experience Figures Figure 1 Figure 2 Introduction The term ‘C3 glomerulopathy’ (C3G) refers to a group of rare and chronic kidney diseases characterised by the accumulation of the third component (C3) of complement in renal tissue [ 1 – 3 ]; this C3 accumulation is the primary diagnostic criterion [ 4 ]. The electron microscopic examination of kidney biopsy distinguishes two subtypes of C3G, namely dense deposit disease (DDD) and C3 glomerulonephritis (C3GN), based on the location and density of deposits [ 5 , 6 ]. In Europe, the estimated number of C3G cases per 1,000,000 ranges from 0.2 to 1.0, with prevalence ranging from 14 to 140 cases per 1,000,000 [ 6 – 8 ]. C3G typically presents in children and young adults [ 9 ] and is characterised by micro- or macroscopic haematuria, light or heavy proteinuria, and a progressive reduction in kidney function [ 10 , 11 ]. Following the development of terminal kidney failure, patients require maintenance dialysis or kidney transplantation [ 12 ], with a significant risk of disease recurrence after transplantation [ 13 ]. Although C3G poses one of the highest risks for kidney failure among all primary glomerular diseases, there are currently no approved therapies that target the underlying cause of the disease [ 2 ]. Previous research has highlighted the lack of evidence on the humanistic burden of C3G in the literature [ 14 , 15 ]; additionally, studies on other kidney diseases have emphasised the critical importance of systematically evaluating the impact of these conditions on quality of life (QoL) [ 16 ], including the use of patient-reported outcomes (PROs), as healthcare professionals may face challenges in recognising and understanding symptoms that are not reported in the literature and patients’ coping mechanisms with the disease [ 17 ]. The World Health Organisation (WHO) has endorsed narrative research as a means of investigating the illness (subjective) experience related to a medical condition [ 18 ] within a real-world context [ 19 ]; the aim is to reveal personal and societal aspects that could be relevant to improving the care continuum. Specifically, Narrative Medicine (NM), which is based on illness narratives [ 20 ], pursues the integration between the biomedical domain, focusing on the disease , and the individual and social experiences of a condition – illness - and sickness -centred approaches, respectively [ 21 ]. NM considers the viewpoints of all actors involved in the care pathway and proposes practical interventions for a specific disorder [ 22 , 23 ] to improve the quality of care, as discussed in previous research on other rare diseases [ 24 , 25 ]. The ‘TIGRE – Finding the identity of C3 glomerulopathy through the experiences of patients, family members and healthcare professionals’ project collected and analysed narratives of patients with C3G, their informal caregivers, and their treating nephrologists to address their perception of QoL and humanistic challenges associated with C3G, and to gain a deeper understanding of C3G and patients’ unmet needs. To the best of our knowledge, this is the first research project to use NM to investigate the humanistic burden of C3G from all three viewpoints simultaneously. Methods The project was conducted from January to October 2023 and focused on adult patients at different stages of C3G, their informal caregivers, and their treating nephrologists. Enrolment occurred across eight hospital-based centres expert in managing C3G and two patient associations (PAs) (Supplement 1). Nephrologists from the involved centres attended a webinar organised by ISTUD (Istituto Studi Direzionali) researchers to receive training in NM and discuss the project’s purpose, design, and data collection tools; they were then invited to involve patients with C3G and their informal caregivers by directing them to the project’s webpage https://www.medicinanarrativa.eu/tigre . A diagnosis of C3G, at various stages and irrespective of follow-up time postdiagnosis, or the caregiving of a person diagnosed with C3G constituted the inclusion criteria for patients and informal caregivers, although the ability to write in Italian was crucial for the inclusion. Narrative and data collection Anonymously written narratives were collected through the Alchemer platform, which was accessible to participants via the project webpage. Patients and caregivers were asked to complete a sociodemographic and QoL survey and to write their narrative supported by an illness plot [ 26 ]. The plot was composed of evocative words encouraging personal expression and was chronologically structured, thus enabling the identification of changes experienced over time [ 27 ]. Researchers collected treating nephrologists’ narratives through the parallel chart [ 28 ], i.e., a personal block, parallel to the clinical chart, where considerations and emotions related to the care relationship are noted in plain language. The patients described in parallel charts could not coincide with patients participating in the project. The investigation tools (Supplement 2) addressed two common aspects: (a) the personal, emotional and care experience of C3G from the onset of symptoms onwards, and (b) the perception of QoL, current daily life, and care pathway with C3G. The data collection tools were designed by two ISTUD researchers with different academic backgrounds and reviewed by the project Steering Committee, including three experienced nephrologists in C3G, as well as one member from each of two Italian C3G PAs (Supplement 1), to reduce cognitive bias. Ethical considerations The project was conducted in accordance with the Declaration of Helsinki and received an ethical waiver by the Ethical Committee of the Mario Negri Research Institute (Bergamo, Italy). Participants provided their written informed consent before their involvement and after being briefed on the project purposes and personal data handling procedures, in accordance with the General Data Protection Regulation of the European Union 2016/679 [ 29 ] and the Italian Law 196/2003 [ 30 ]. The informed consent included the publication of anonymised responses and narratives. Narrative and data analysis The sociodemographic data were analysed using descriptive statistics. Answering survey questions or filling in fields in the illness plots and parallel charts was not mandatory; non-respondents were considered as a separate category. Narratives were entered into MAXQDA (VERBI Software) qualitative analysis software [ 31 ] for coding and content analysis [ 32 ]. To assess consistency across the research team, two narratives from each group were collectively coded; then, the narratives were separately coded and reviewed during weekly peer debriefings to limit any interpretation bias. Open interpretive coding was used to identify and analyse the emerging themes in all narratives. Additionally, narratives were retrospectively classified following Frank’s classification [ 33 ], which distinguishes between three types: (a) chaos narratives, which are anti-narratives revealing the narrator’s vulnerability, (b) restitution narratives, in which the narrator explores the experience and meaning of the care pathway, and (c) quest narratives, in which the disease becomes a motivation for change. The Steering Committee collectively discussed the results to address the emerging issues and data interpretation. The research team followed the COREQ reporting guidelines [ 34 ]. Results Seventeen adult patients diagnosed with C3G and 14 informal caregivers participated in the project; 12 treating nephrologists wrote 15 parallel charts. Table 1 shows their sociodemographic data, as well as those of the patients described in the parallel charts, including non-responders as a separate category. Table 1 – Sociodemographic data of participants Patients with C3 glomerulopathy (N = 17) Caregivers (N = 14) Treating nephrologists (N = 12) Patients in parallel charts (N = 15) Gender Female 11 (64%) 11 (79%) 6 (50%) 9 (60%) Male 3 (18%) 2 (14%) 6 (50%) 6 (40%) Non-responders 3 (18%) 1 (7%) - - Age 26 (19–35) 49 (22–59) 41 (27–66) 26 (11–60) Nationality Italian 12 (70%) 13 (93%) 12 (100%) 15 (100%) Non-responders 5 (30%) 1 (7%) - - Education Elementary school - - - 1 (7%) Middle school 1 (6%) 1 (7%) - 3(15%) High school 8 (46%) 3 (21%) - 8 (53%) Bachelor or higher degree 3 (18%) 9 (64%) - 3 (15%) Non-responders 5 (30%) 1 (7%) - - Employment status Student 3 (18%) 1 (7%) - 7 (47%) Working 8 (46%) 11 (79%) - 7 (47%) Not working 1 (6%) 1 (7%) - 1 (6%) Non-responders 5 (30%) 1 (7%) - - Marital state Single 7 (41%) 1 (7%) - - Married or cohabitant 4 (24%) 10 (71%) - - Separated - 2 (14%) - - Non-responders 6 (35%) 1 (7%) - - Professional activity - - 11.5 (2–41) - Workplace Research and Care Institute - - 8 (67%) - Hospital - - 1 (8%) - University Hospital - - 3 (25%) - Data are presented as N (%) or median (range). [Table 1 ] Results are presented by focusing on three main areas: (a) the C3G care pathway from early symptoms to current treatments; (b) patients’ and informal caregivers’ perception of QoL, including the impact of C3G on daily life and the associated emotional burden; and (c) the C3G illness experience as analysed through Frank’s classification and metaphors. Narratives informed (a) to (c), while both narratives and quantitative survey data were used to investigate (a) and (b). Figure 2 and Tables 2 – 3 provide supporting quotes from narratives; additionally, Supplementary file 3 contains three narratives in English. The majority of patients (92%), caregivers (63%), and nephrologists (86% of parallel charts) reported positive experiences with writing (– I’m happy when my testimony gives strength to another person , P006; – I hope it helps similar cases and research , C007). Conversely, 8% of patients, 37% of caregivers, and nephrologists in 14% of parallel charts considered it a challenge (– The memory of this journey gave me strong emotions, because the suffering of this patient due to an untreatable, relapsing disease, which led him to dialysis and the failure of the kidney transplant, is also a suffering of the doctor who takes care of the patient , PC011). The C3G care pathway from early symptoms to current treatments Seventy-three per cent of patients discovered the condition because of symptoms, e.g., swelling, fever, tiredness (– I remember one summer evening when my parents were very worried about my very swollen ankles and feet. It had been a few weeks since we had noticed widespread oedema, first around the eyes and then suddenly in my lower limbs , P008), while 27% discovered it from the results of analyses carried out in other circumstances; the same was reported by 45% and 55% respectively of caregivers (– We became aware of the disease purely by chance by doing routine tests required for sporting activity, no symptoms of the disease had ever appeared , C003). Nephrologists highlighted that some patients ‘denied’ C3G symptoms in 40% of parallel charts (– One could see how his will/need to deny change and therefore the presence of an ‘illness’ shone through. The feigned indifference was a sign of concern and fear of something he could not define but did not want to be confronted with. A desire to get rid of the problem so as not to be upset by his certainties and habits , PC004). The mean age of onset of C3G symptoms in patients was 18.8 years (±9.1); of the 17 patients, five reported consulting a nephrologist as their first specialist after symptom onset. The diagnosis was communicated to all patients by the nephrologist. When patients were asked to write about their biopsy experience, only 17% described it as traumatic (– I will never forget the first biopsy because of the pain I felt when the anaesthetic wore off , P001; – It was not too painful, but I felt some discomfort for the next few days , P002); patients were more likely to describe the procedure itself (50%) and express gratitude towards the healthcare professionals who carried it out (– They all always took care of me , P004). Thirty-six per cent of caregivers described the diagnosis as a traumatic experience (– The day of the kidney biopsy, the only way to diagnose the disease without any doubt, was perhaps one of the most difficult days of my life , C008), as also highlighted in parallel charts (– Luckily, each time we did it, it was an uncomplicated procedure. Nevertheless, I could not accept that a child could be so unfortunate as to be subjected to such invasive acts , PC011). On receiving the diagnosis, 36% of patients and 20% of caregivers reported feeling anxious, while 46% of patients and 50% of caregivers expressed disbelief (Table 2). Similarly, nephrologists experienced anxiety in 20% and found the diagnosis a challenging moment in 26% of parallel charts. Twenty-five percent of patients and 40% of caregivers and nephrologists reported ongoing concerns about the potential development of C3G. Meanwhile, 42% of patients and 60% of caregivers and nephrologists in 20% of parallel charts reported feeling reassured. In addition, 33% of patients expressed gratitude for their treatment, while nephrologists in 40% of parallel charts reported feeling frustrated. [Table 2] Table 2 – Emotions experienced by participants at diagnosis and today: quotes from narratives Emotions at diagnosis Emotions today Patients – At first I did not know what it was all about. I was very scared and had a hard time processing it, also because they told me that I would also have to start a special course on nutrition . (P011) – At the time of diagnosis, the name C3 glomerulopathy did not exist [...]. Despite the limited knowledge at the time, it was already known that the disease leads to terminal kidney failure in a good percentage of patients, so the news was obviously terrible for my parents. For me, a seemingly healthy child, the news was certainly frightening, but with faith in the progress of medicine, I saw the option of transplant/dialysis as a distant one . (P012) – I am grateful because what I have gone through has made me a strong and independent woman. I definitely feel happier and less angry at the world, also thanks to psychotherapy. There are days when the darkness returns and fears take over, but I try to manage the panic by concentrating on the present . (P002) – Today, after a kidney transplant, I feel reborn. I still have my fears and anxieties, but it takes little to know how to manage them and live your life harmoniously . (P004) Caregivers – I had read about this disease on the internet. When they explained the disease and the uncertainty of the treatment and its results, the world collapsed on us. It was just me and her in a room 24 hours a day, with no contact with the outside world. It was a very difficult and complex time, but we were always supported with great professionalism and empathy . (C001) – We didn’t know what it was all about. In a world where a cure is expected for every disease, I was particularly shocked when the doctors told us that there was currently no cure for C3 glomerulonephritis, and that the child would have to live with the disease for the rest of her life. We did not know (and still do not know) what that meant . (C005) – I have ups and downs as a parent. In truth, I am always a little afraid of the future and I still find it difficult to accept my child’s illness. Thanks to the medical staff, who are always attentive, present, prepared and humane, I always try to see the positive side and think that a definitive cure can be found . (C003) – I feel blessed and happy. We keep going back for check-ups, but I feel like a survivor of a shipwreck with my family. When I see my son so strong and beautiful and my family so ‘normal’, I am a happy woman . (C008) Nephrologists – In the last biopsy, the damage was so advanced that it was not worth doing anything more. It was difficult to tell her. Of course, she gave us one of her many life lessons: ‘I thought this is the way it’s supposed to be.... I’d better start dialysis’. As usual, she was more oriented than we were. She already knew everything . (PC005) – I talked to the parents for about an hour, keeping them busy with the excuse of a diagnostic test where she needed to be alone. Then, in agreement with the parents, we explained to her what had happened, without lying, but without giving up hope of remission . (PC012) – I have an underlying concern when I think about the uncertainty of the future course of her illness, but I am very happy about the strength and positivity this person shows, even though I am aware of the uncertainties of the future . (PC003) – I feel like a boxer in the last round. I must land a hook, just one, and hope I have the right aim and strength. I feel under pressure, but I don't want to give up. I consult the literature and ongoing trials to find the best drug. But as I do so, I feel the weight of time passing, taking away nephrons and limiting my options . (PC006) Six out of 17 patients underwent genetic testing, while 7/17 patients had follow-up visits within 0–3 months. Nephrologists reported that patients were living with the disease and had stable kidney function at this point in their care pathway in 60% of parallel charts, while reported that patients were experiencing or feared clinical deterioration in 40% of parallel charts. In the narratives, 33% of patients and 20% of caregivers mentioned experimental treatments. Another 33% of patients described treatments as burdensome, while 17% of patients and 20% of caregivers expressed that current treatments offered hope. Nephrologists stated that their goal was to stabilise or reverse C3G in 60%, mentioned the possibility of transplantation in 20%, and aimed to return patients to their previous lifestyle in another 20% of parallel charts. [Table 3] Table 3 – The experience of C3G current treatments according to participants: quotes from narratives Patients – Therapies are always present in our lives and cooperation with the doctor is essential. Dialysis removes most of the medications and restrictions of the terminal period, but it also introduces new ones. In my experience, having to feel attached to the dialysis session has a much stronger negative psychological impact than any therapy I might undergo . (P012) – There are so many treatments these days, especially after a kidney transplant... There are days when I feel tired and think about all the therapies I have to do, but I know it is for my own good and especially for the gift I have received . (P004) Caregivers – Over time, thanks to the new avenues opened up by scientific research, treatments are giving people more confidence, hope and the ability to live as normal a life as possible. Treatments are becoming less invasive and more manageable . (C003) – Cures are possible, difficult to detect and potentially very long, but they are improving . (C006) Nephrologists – I would like him to be able to perform a kidney transplant with real success. Unfortunately, there is no guarantee of this; on the contrary, he has a high risk of further transplant failure. Now, our knowledge does not provide us with a definitive solution or a stable approach to this pathological condition. Today, as yesterday, there are the same complaints and the same predictions of failure, albeit to a lesser extent . (PC011) – We have fought, but we have lost. For the future, I am already looking for the ‘best possible medicine’, but I am not sure I will find it. In the meantime, I pray that the dragon will not return . (PC005) Looking back on the care pathway, 58% of patients and 60% of caregivers reported satisfaction (– I wouldn’t change a thing, because to this day I am proud of who I am and the scars I carry, especially when you are an inspiration to your generation , P004); in 60% of parallel charts, nephrologists stated that they had done everything possible (– In this case, I have no regrets or remorse about what we did medically or what we communicated in terms of our relationship with the family and the patient. [...] The time available should be more than we can offer. Proper and thorough communication is also part of the process of caring for a patient. Not talking enough becomes a source of regret, especially in situations where the outcome is not positive , PC004); 33% of patients, 20% of caregivers and nephrologists in 60% of parallel charts expressed some regret about the care pathway (– I think that if there had been more cooperation between all the centres, given the rarity of the disease, things might have been different , P012; – I would have liked to have been diagnosed earlier and to have experienced less suffering and anxiety , P010; – Maybe I could have shared my experience with other parents of people with the same condition , C005). Finally, 8% of patients and 20% of caregivers reported that the care pathway was complex (– I hope everything goes well, but I am very afraid of another relapse , P008). Forty-five per cent of patients, 18% of caregivers and nephrologists in 40% of parallel charts expressed a desire for more research funding and more available therapies for C3G (– I hope that research will continue and that over the years we will learn more about the disease , P001; – I wish there was more money for research! Not just for this disease, but for all diseases , C009; – I hope that new drugs will be developed that will at least further reduce the risk of transplant failure, or at least delay the need for dialysis for as long as possible , PC011). Living with C3G and quality of life Figure 1 shows the self-assessment of the impact of C3G on work activities by the patients; ten out of 17 patients responded. Specifically, 50% of respondents reported that C3G impacts ‘quite a lot’ and 20% ‘a lot’ on what they do at work, while 40% reported being ‘quite a lot’ and 20% ‘a lot’ less focused than they would like at work. [Figure 1 ] Patients reported losing an average of 133 working days per year due to C3G, which accounts for 52.7% of the total working days; additionally, they spend an average of EUR 400 per year on medication and transport. Family members report losing an average of one working day per month to care for their loved one, with the main impact being on their social life. Forty per cent of patients reported a low impact on social activities and relationships due to C3G, while another 40% reported a moderate to high impact. In terms of the patient's relationships with others, 70% of caregivers and only 11% of patients reported improvement since the onset of C3G; conversely, 55% stated that they often did not disclose the disease to others (– Only a few of my friends know about my illness. I was always reserved; I did not like to tell people about my illness. I didn't want to change my image and I was afraid that not everyone would understand , P009), as also highlighted in 21% of parallel charts (– He is always in a good mood, although he may be hiding the anguish associated with his condition , PC001) and in 10% of caregiver narratives. Regarding their relationship with food, 55% of patients reported having a highly restricted diet, while 45% reported less restriction (– At home I try to limit the use of salt, outside I eat normally. I try to drink a lot because I have been advised to do so. […] I have learnt what signs to look out for to understand how to change something , P009). Among caregivers, 50% reported that the patient had a highly restricted diet. In contrast, in 21% of parallel charts, nephrologists reported that the patient had a complex relationship with food (– I’m going to put him on a low protein diet of 0.6g/kg/day and hope to gain time and opportunity... It will be difficult to get him to do this as it will make him feel ill and tear down the curtain of 'there is no problem' that he hides behind , PC006). Looking to the future, 54% of patients and 82% of caregivers expressed a desire for a good quality of life (– For tomorrow I am confident... I hope I can get back to my old self, even though I know I will never be the same again, but at least I can regain the strength I had before , P005). C3G illness experience in narratives Restitution [ 33 ] characterises 75% of patient narratives, 46% of caregiver narratives, and 47% of parallel charts (Fig. 2 ). Quest characterises only patients’ and caregivers’ narratives (17% and 23%, respectively), whereas chaos is predominant in parallel charts (53%). [Figure 2 ] Participants used various metaphors to describe C3G, with two groups being prevalent: (a) 22% of patient narratives, 18% of caregiver narratives, and 47% of the parallel charts contained nature-related images (– I see myself flanked by a white wolf , P003; – A black spider , C007; – A slow approaching tide , N014); (b) 33% of patient narratives, 72% of caregiver narratives, and 53% of the parallel charts contained images of limitation or threat (– A path covered in mist , P011; – A black cloud above me , C002; – A ghost , N001). Discussion Given the lack of literature on the humanistic burden of people living with C3G, the project represented the first attempt to investigate the illness experience and impact on QoL of C3G in Italy, while incorporating the viewpoints of adult patients, informal caregivers and treating nephrologists. Consistent with a previous study [ 14 ], the symptoms most frequently reported by patients, such as swelling and fatigue, have received minimal attention in the existing literature. In particular, fatigue remains poorly addressed despite its crucial impact on work activities and overall quality of life. The diagnosis and treatment of C3G elicit emotions of fear, anxiety, and disbelief in both patients and caregivers. Nevertheless, narratives illustrate that both patients and caregivers can establish a new daily routine while living with the disease, evidenced by the prevalence of restitution [ 33 ] narratives. Despite this, C3G maintains a significant impact on patients’ QoL, particularly relatively to social and work activities, as highlighted by the self-reported loss of an average 52.7% of working days per year, in line with findings from previous studies [ 14 ]. Interestingly, treating nephrologists show more chaos narratives [ 33 ] and express feelings of frustration concerning the C3G management while waiting for new therapeutic strategies. This observation highlights their emotional involvement and, at the same time, raises concerns about their potential oversight of the improved everyday life that patients and caregivers report in narratives. On the other hand, the prevalence of chaos narratives among treating nephrologists may be due to their awareness of the disease progression and suggests that caring for C3G substantially impact their professional lives: this issue should be further investigated, also in the light of previous studies suggesting that nephrologists are susceptible to burnout with specific characteristics due to the disease and the chronic care relationship [ 35 ]. Continuing scientific research to discover new and targeted treatments for C3G is of great importance to all those involved in this project. Patients often report enduring intensive treatments for C3G, some of which are experimental. However, patients emphasise that these treatments offer hope of stabilising the disease and potentially allowing a return to their previous life or ensuring a good QoL. Suggestions for improving the care pathway for people with C3G emerged from the narratives: Organizational and structural improvements: encouraging systematic collaboration among healthcare centres in the area and facilitating early diagnosis were highlighted. This reinforces findings from a previous survey [ 14 ] where patients and caregivers perceived delayed diagnosis. Lack of detailed information about C3G was also mentioned by patients and caregivers, leading to emotional stress. Efforts aimed at disease awareness and social understanding could alleviate this stress, potentially reducing the fear, anxiety, and disorientation experienced by patients and caregivers upon diagnosis. Emotional and psychological support: offering opportunities for caregivers to connect with others who share similar experiences, such as family members of individuals with C3G, could provide valuable support. Similarly, facilitating avenues for patients to engage with others about their condition, addressing their fear of not being comprehended, could be beneficial. It is worth noting that other kidney diseases requiring dialysis or transplantation also significantly impact patients’ psychological well-being [ 36 – 38 ]. While further studies are warranted to explore the humanistic burden and unmet needs of both patients with C3G and their informal caregivers, and to explore the patient’s journey through the care continuum, findings suggest the potential of NM as a valuable tool in understanding the illness experience of individuals living with C3G and the caregiving burden. Furthermore, findings highlight the importance of involving treating nephrologists in research into the overall impact of C3G. Since the participation in the project was voluntary, the researchers could not quantify the agreements between patients, informal caregivers, and treating nephrologists. Also, as the project only included patients who were followed up in expert centres or supported by PAs, the situation of those who did not have access to these centres may be more critical. Conversely, the lack of validated scales for QoL assessment in C3G allowed a more spontaneous and qualitative experience of the disease to be captured through narratives. Conclusion The TIGRE project investigated the humanistic burden associated with C3G as perceived by patients, informal caregivers and treating nephrologists, uniquely integrating these viewpoints, and marking the first Italian NM initiative on C3G. The findings suggest that NM is a valuable tool for understanding the C3G illness experience, while highlighting the need to involve nephrologists in more comprehensive research. Overall, narratives provided valuable insights aimed at raising awareness about the living with C3G. Abbreviations C3 Complement 3 C3G Complement 3 glomerulopathy C3GN Complement 3 glomerulonephritis COREQ Consolidated criteria for reporting qualitative research DDD Dense deposit disease ESRD End stage renal disease ISTUD Istituto Studi Direzionali NM Narrative Medicine PA Patient association PROs Patient reported outcomes QoL Quality of life WHO World Health Organisation Declarations Ethics approval The project was conducted according to the declaration of Helsinki. Consent to participate Participants provided a web-based informed consent before their involvement and after being briefed on the purposes of the research and the procedures for the processing of personal data, according to General Data Protection Regulation of the European Union 2016/679 and the Italian Law 196/2003. The clinicians involved obtained a written informed consent to participate from the parents of underage patients during the first briefing on the project’s methods and purposes. Data sharing All datasets used and analyzed during the current research are available in Italian from the corresponding author, upon reasonable request. Competing interests EDS have received honoraria from Alexion and AstraZeneca for holding webinars. LG received grant support from Abionyx and Sanofi to his university department (DIMEPRE-J); he also has been a member of advisory boards for AstraZeneca, Baxter, Chinook, GSK, Mundipharma, Novartis, Pharmadoc, Roche, Sanofi, Travere, Vifor Pharma, and an invited speaker at meetings supported by AstraZeneca, Astellas, Estor, Fresenius, Werfen, Medtronic, Travere, GSK. A.C., E.D., A.P., A.M., G.L, F.T, E.T., A.P., M.F., A.P., A.M., F.S., C.D., E.G., M.G.M., M.V. don't have financial interests in relation to the work described. Funding statement Novartis Farma unconditionally supported ISTUD Srl for realising the project. Authors’ contribution statement AC, EG, MGM, CD were involved in the project’s conceptualisation. AC, EG, MGM, MV, ED were involved in the methodology. All authors contributed to the project’s investigation.AC, EG, MGM contributed to data analysis. AC, EG, MGM, MV, ED, FS contributed to data validation. All authors were involved in writing; all authors contributed to the manuscript review and approved the final draft for submission. MGM is responsible for the overall content as guarantor. Acknowledgements The authors wish to thank Novartis Farma Italia that sponsored and funded this work, especially Vincenza Vinaccia for her editorial assistance. The authors would also thank Erica Daina for the support provided for the Ethical Committee’s approval of the project, as well as the researchers of ISTUD Healthcare Area for their useful role throughout the project, as well as all the people suffering from C3G, their caregivers, the healthcare professionals and the Patient Association representatives who took part in the research. All the authors want to thank Alessandra Fiorencis for her professional contribution to the paper. References Smith RJH, Appel GB, Blom AM, et al. C3 glomerulopathy - understanding a rare complement-driven renal disease. Nat Rev Nephrol. 2019;15(3):129–43. Bomback AS, Kavanagh D, Vivarelli M, et al. Alternative Complement Pathway Inhibition With Iptacopan for the Treatment of C3 Glomerulopathy-Study Design of the APPEAR-C3G Trial. Kidney Int Rep. 2022;7(10):2150–9. Pickering MC, D'Agati VD, Nester CM, et al. C3 glomerulopathy: consensus report. Kidney Int. 2013;84(6):1079–89. Heiderscheit AK, Hauer JJ, Smith RJH. C3 glomerulopathy: Understanding an ultra-rare complement-mediated renal disease. Am J Med Genet C Semin Med Genet. 2022;190(3):344–57. Donadelli R, Pulieri P, Piras R, et al. Unraveling the Molecular Mechanisms Underlying Complement Dysregulation by Nephritic Factors in C3G and IC-MPGN. Front Immunol. 2018;9:2329. Servais A, Noël LH, Roumenina LT, et al. Acquired and genetic complement abnormalities play a critical role in dense deposit disease and other C3 glomerulopathies. Kidney Int. 2012;82(4):454–64. Medjeral-Thomas NR, O'Shaughnessy MM, O'Regan JA, et al. C3 glomerulopathy: clinicopathologic features and predictors of outcome. Clin J Am Soc Nephrol. 2014;9(1):46–53. Athanasiou Y, Voskarides K, Gale DP, et al. Familial C3 glomerulopathy associated with CFHR5 mutations: clinical characteristics of 91 patients in 16 pedigrees. Clin J Am Soc Nephrol. 2011;6(6):1436–46. Lu DF, Moon M, Lanning LD, et al. Clinical features and outcomes of 98 children and adults with dense deposit disease. Pediatr Nephrol. 2012;27(5):773–81. Schena FP, Esposito P, Rossini M. A Narrative Review on C3 Glomerulopathy: A Rare Renal Disease. Int J Mol Sci. 2020;21(2):525. Bomback AS, Santoriello D, Avasare RS, et al. C3 glomerulonephritis and dense deposit disease share a similar disease course in a large United States cohort of patients with C3 glomerulopathy. Kidney Int. 2018;93(4):977–85. Feldman LDB, Bomback A, Nester C. Voice of the patient: report of externally-led patient-focused drug development meeting on: complement 3 glomerulopathy (C3G). National Kidney Foundation, 2018. Accessed August 17, 2022. https://www.kidney.org/sites/default/files/C3G_EL-PFDD_VoP- Report_3-29-18.pdf. Goodship TH, Cook HT, Fakhouri F et al. Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference. Kidney Int . 2017;91(3):539–551. Tyagi N, Aasaithambi S, Chauhan J, et al. Patient insights for complement 3 glomerulopathy (c3g) using social media listening. Value Health. 2019;22(3):S919. George AT, Zaour N, Nic Lochlainn EM. The burden associated with complement 3 glomerulopathy (C3G). Value Health. 2018;21(3):S480. Fletcher BR, Damery S, Aiyegbusi OL, et al. Symptom burden and health-related quality of life in chronic kidney disease: A global systematic review and meta-analysis. PLoS Med. 2022;19(4):e1003954. Finkelstein FO, Arsenault KL, Taveras A, et al. Assessing and improving the health-related quality of life of patients with ESRD. Nat Rev Nephrol. 2012;8(12):718–24. Pierret J. The illness experience: state of knowledge and perspectives for research. Sociol Health Illn. 2003; 25. Greenhalgh T. Cultural contexts of health: the use of narrative research in the health sector. Copenhagen: WHO Regional Office for Europe; 2016, Health Evidence Network (HEN) synthesis report 49. http://www.euro.who.int/__data/assets/pdf_file/0004/317623/HEN-synthesis-report-49.pdf , last accessed on February 20, 2024. Marini MG. Narrative Medicine: Bridging the Gap between Evidence-based Care and Medical Humanities. London: Springer International Publishing; 2016. Kleinman A. The Illness Narrative, Suffering and Healing the Human Condition. New York: Basic Book; 1989. Greenhalgh T, Hurwitz B, Why study narrative?. BMJ 1999; 318. Marini MG. Languages of Care in Narrative Medicine. Words, Space and Time in the Healthcare Ecosystem. London: Springer International Publishing; 2019. Simonelli F, Sodi A, Falsini B, et al. Narrative medicine to investigate the quality of life and emotional impact of inherited retinal disorders through the perspectives of patients, caregivers and clinicians: an Italian multicentre project. BMJ Open. 2022;12(9):e061080. Ragusa L, Crinò A, Grugni G, et al. Caring and living with Prader-Willi syndrome in Italy: integrating children, adults and parents' experiences through a multicentre narrative medicine research. BMJ Open. 2020;10(8):e036502. Reid K, Soundy A. A qualitative study examining the illness narrative master plots of people with head and neck cancer. Behav Sci 2019; 9. Peeters B, Marini M. Narrative medicine across languages and cultures: using minimal English for increased comparability of patients’ narratives. In: Goddard C, editor. Minimal English for a Global World: Improved Communication Using Fewer Words. Basingstoke, UK: Palgrave Macmillan; 2018. pp. 259–86. Charon R. The patient-physician relationship. Narrative Medicine: a model for empathy, reflection, profession, and trust. JAMA 2001; 286. Personal data code protection. Legislat. Decree no. 196 of 30 June 2003. Published on the Italian Official Journal n. 174, July 29, 2003, Supplementary n. 123. http://leg13.camera.it/parlam/leggi/deleghe/testi/03196dl.htm , last accessed on February 20, 2024. Regulation (EU) 2016/679 of the European Parliament and of the Council of 27 April. 2016 on the protection of natural persons with regard to the processing of personal data and on the free movement of such data and repealing Directive 95/46/EC (General Data Protection Regulation. Published Official J Eur Union L 119, May 4, 2016. https://eur-lex.europa.eu/legal-content/EN/TXT/PDF/?uri=CELEX:32016R0679 , last accessed on February 20, 2024. Software VERBI. MAXQDA 2022. Berlin: VERBI Software; 2021. Hsieh H-F, Shannon SE. Three approaches to qualitative content analysis. Qual Health Res. 2005;15:1277–88. Frank AW. The wounded storyteller: body, illness, and ethics. Chicago: University of Chicago Press; 2013. (second edition). Tong A, Sainsbury P, Craig J. Consolidated criteria for reporting qualitative research (COREQ): a 32-item checklist for interviews and focus groups. Int J Qual Health Care. 2007;19:6. Kotzabassaki S, Parissopoulos S. Burnout in renal care professionals. EDTNA ERCA J. 2003 Oct-Dec;29(4):209–13. Chilcot J, Wellsted D, Da Silva-Gane M, et al. Depression on dialysis. Nephron Clin Pract. 2008;108(4):c256–64. King-Wing Ma T, Kam-Tao Li P. Depression in dialysis patients. Nephrol (Carlton). 2016;21(8):639–46. Hussien H, Apetrii M, Covic A. Health-related quality of life in patients with chronic kidney disease. Expert Rev Pharmacoecon Outcomes Res. 2021;21(1):43–54. Additional Declarations Competing interest reported. E.D.S. have received honoraria from Alexion and AstraZeneca for holding webinars. L.G. received grant support from Abionyx and Sanofi to his university department (DIMEPRE-J); he also has been a member of advisory boards for AstraZeneca, Baxter, Chinook, GSK, Mundipharma, Novartis, Pharmadoc, Roche, Sanofi, Travere, Vifor Pharma, and an invited speaker at meet-ings supported by AstraZeneca, Astellas, Estor, Fresenius, Werfen, Medtronic, Travere, GSK. A.C., E.D., A.P., A.M., G.L, F.T, E.T., A.P., M.F., A.P., A.M., F.S., C.D., E.G., M.G.M., M.V. don't have financial interests in relation to the work described. Supplementary Files Supplementaryfiles.docx Cite Share Download PDF Status: Posted Version 1 posted 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. 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E.D.S. have received honoraria from Alexion and AstraZeneca for holding webinars. L.G. received grant support from Abionyx and Sanofi to his university department (DIMEPRE-J); he also has been a member of advisory boards for AstraZeneca, Baxter, Chinook, GSK, Mundipharma, Novartis, Pharmadoc, Roche, Sanofi, Travere, Vifor Pharma, and an invited speaker at meet-ings supported by AstraZeneca, Astellas, Estor, Fresenius, Werfen, Medtronic, Travere, GSK.\nA.C., E.D., A.P., A.M., G.L, F.T, E.T., A.P., M.F., A.P., A.M., F.S., C.D., E.G., M.G.M., M.V. don't have financial interests in relation to the work described.","formattedTitle":"Unveiling the humanistic burden of C3 glomerulopathy in Italy: a narrative medicine multicentre project","fulltext":[{"header":"Introduction","content":"\u003cp\u003eThe term \u0026lsquo;C3 glomerulopathy\u0026rsquo; (C3G) refers to a group of rare and chronic kidney diseases characterised by the accumulation of the third component (C3) of complement in renal tissue [\u003cspan additionalcitationids=\"CR2\" citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e]; this C3 accumulation is the primary diagnostic criterion [\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e]. The electron microscopic examination of kidney biopsy distinguishes two subtypes of C3G, namely dense deposit disease (DDD) and C3 glomerulonephritis (C3GN), based on the location and density of deposits [\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e, \u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eIn Europe, the estimated number of C3G cases per 1,000,000 ranges from 0.2 to 1.0, with prevalence ranging from 14 to 140 cases per 1,000,000 [\u003cspan additionalcitationids=\"CR7\" citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e]. C3G typically presents in children and young adults [\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e] and is characterised by micro- or macroscopic haematuria, light or heavy proteinuria, and a progressive reduction in kidney function [\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e, \u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e]. Following the development of terminal kidney failure, patients require maintenance dialysis or kidney transplantation [\u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e], with a significant risk of disease recurrence after transplantation [\u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e]. Although C3G poses one of the highest risks for kidney failure among all primary glomerular diseases, there are currently no approved therapies that target the underlying cause of the disease [\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e].\u003c/p\u003e \u003cp\u003ePrevious research has highlighted the lack of evidence on the humanistic burden of C3G in the literature [\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e, \u003cspan citationid=\"CR15\" class=\"CitationRef\"\u003e15\u003c/span\u003e]; additionally, studies on other kidney diseases have emphasised the critical importance of systematically evaluating the impact of these conditions on quality of life (QoL) [\u003cspan citationid=\"CR16\" class=\"CitationRef\"\u003e16\u003c/span\u003e], including the use of patient-reported outcomes (PROs), as healthcare professionals may face challenges in recognising and understanding symptoms that are not reported in the literature and patients\u0026rsquo; coping mechanisms with the disease [\u003cspan citationid=\"CR17\" class=\"CitationRef\"\u003e17\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eThe World Health Organisation (WHO) has endorsed narrative research as a means of investigating the illness (subjective) experience related to a medical condition [\u003cspan citationid=\"CR18\" class=\"CitationRef\"\u003e18\u003c/span\u003e] within a real-world context [\u003cspan citationid=\"CR19\" class=\"CitationRef\"\u003e19\u003c/span\u003e]; the aim is to reveal personal and societal aspects that could be relevant to improving the care continuum. Specifically, Narrative Medicine (NM), which is based on illness narratives [\u003cspan citationid=\"CR20\" class=\"CitationRef\"\u003e20\u003c/span\u003e], pursues the integration between the biomedical domain, focusing on the \u003cem\u003edisease\u003c/em\u003e, and the individual and social experiences of a condition \u0026ndash; \u003cem\u003eillness\u003c/em\u003e- and \u003cem\u003esickness\u003c/em\u003e-centred approaches, respectively [\u003cspan citationid=\"CR21\" class=\"CitationRef\"\u003e21\u003c/span\u003e]. NM considers the viewpoints of all actors involved in the care pathway and proposes practical interventions for a specific disorder [\u003cspan citationid=\"CR22\" class=\"CitationRef\"\u003e22\u003c/span\u003e, \u003cspan citationid=\"CR23\" class=\"CitationRef\"\u003e23\u003c/span\u003e] to improve the quality of care, as discussed in previous research on other rare diseases [\u003cspan citationid=\"CR24\" class=\"CitationRef\"\u003e24\u003c/span\u003e, \u003cspan citationid=\"CR25\" class=\"CitationRef\"\u003e25\u003c/span\u003e].\u003c/p\u003e \u003cp\u003e The \u0026lsquo;TIGRE \u0026ndash; Finding the identity of C3 glomerulopathy through the experiences of patients, family members and healthcare professionals\u0026rsquo; project collected and analysed narratives of patients with C3G, their informal caregivers, and their treating nephrologists to address their perception of QoL and humanistic challenges associated with C3G, and to gain a deeper understanding of C3G and patients\u0026rsquo; unmet needs. To the best of our knowledge, this is the first research project to use NM to investigate the humanistic burden of C3G from all three viewpoints simultaneously.\u003c/p\u003e"},{"header":"Methods","content":"\u003cp\u003eThe project was conducted from January to October 2023 and focused on adult patients at different stages of C3G, their informal caregivers, and their treating nephrologists. Enrolment occurred across eight hospital-based centres expert in managing C3G and two patient associations (PAs) (Supplement 1). Nephrologists from the involved centres attended a webinar organised by ISTUD (Istituto Studi Direzionali) researchers to receive training in NM and discuss the project\u0026rsquo;s purpose, design, and data collection tools; they were then invited to involve patients with C3G and their informal caregivers by directing them to the project\u0026rsquo;s webpage \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://www.medicinanarrativa.eu/tigre\u003c/span\u003e\u003cspan address=\"https://www.medicinanarrativa.eu/tigre\" targettype=\"URL\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/p\u003e \u003cp\u003eA diagnosis of C3G, at various stages and irrespective of follow-up time postdiagnosis, or the caregiving of a person diagnosed with C3G constituted the inclusion criteria for patients and informal caregivers, although the ability to write in Italian was crucial for the inclusion.\u003c/p\u003e \u003cdiv id=\"Sec3\" class=\"Section2\"\u003e \u003ch2\u003eNarrative and data collection\u003c/h2\u003e \u003cp\u003e Anonymously written narratives were collected through the Alchemer platform, which was accessible to participants via the project webpage. Patients and caregivers were asked to complete a sociodemographic and QoL survey and to write their narrative supported by an illness plot [\u003cspan citationid=\"CR26\" class=\"CitationRef\"\u003e26\u003c/span\u003e]. The plot was composed of evocative words encouraging personal expression and was chronologically structured, thus enabling the identification of changes experienced over time [\u003cspan citationid=\"CR27\" class=\"CitationRef\"\u003e27\u003c/span\u003e]. Researchers collected treating nephrologists\u0026rsquo; narratives through the parallel chart [\u003cspan citationid=\"CR28\" class=\"CitationRef\"\u003e28\u003c/span\u003e], i.e., a personal block, parallel to the clinical chart, where considerations and emotions related to the care relationship are noted in plain language. The patients described in parallel charts could not coincide with patients participating in the project. The investigation tools (Supplement 2) addressed two common aspects: (a) the personal, emotional and care experience of C3G from the onset of symptoms onwards, and (b) the perception of QoL, current daily life, and care pathway with C3G.\u003c/p\u003e \u003cp\u003eThe data collection tools were designed by two ISTUD researchers with different academic backgrounds and reviewed by the project Steering Committee, including three experienced nephrologists in C3G, as well as one member from each of two Italian C3G PAs (Supplement 1), to reduce cognitive bias.\u003c/p\u003e \u003c/div\u003e \u003cdiv id=\"Sec4\" class=\"Section2\"\u003e \u003ch2\u003eEthical considerations\u003c/h2\u003e \u003cp\u003e The project was conducted in accordance with the Declaration of Helsinki and received an ethical waiver by the Ethical Committee of the Mario Negri Research Institute (Bergamo, Italy). Participants provided their written informed consent before their involvement and after being briefed on the project purposes and personal data handling procedures, in accordance with the General Data Protection Regulation of the European Union 2016/679 [\u003cspan citationid=\"CR29\" class=\"CitationRef\"\u003e29\u003c/span\u003e] and the Italian Law 196/2003 [\u003cspan citationid=\"CR30\" class=\"CitationRef\"\u003e30\u003c/span\u003e]. The informed consent included the publication of anonymised responses and narratives.\u003c/p\u003e \u003c/div\u003e \u003cdiv id=\"Sec5\" class=\"Section2\"\u003e \u003ch2\u003eNarrative and data analysis\u003c/h2\u003e \u003cp\u003eThe sociodemographic data were analysed using descriptive statistics. Answering survey questions or filling in fields in the illness plots and parallel charts was not mandatory; non-respondents were considered as a separate category.\u003c/p\u003e \u003cp\u003eNarratives were entered into MAXQDA (VERBI Software) qualitative analysis software [\u003cspan citationid=\"CR31\" class=\"CitationRef\"\u003e31\u003c/span\u003e] for coding and content analysis [\u003cspan citationid=\"CR32\" class=\"CitationRef\"\u003e32\u003c/span\u003e]. To assess consistency across the research team, two narratives from each group were collectively coded; then, the narratives were separately coded and reviewed during weekly peer debriefings to limit any interpretation bias.\u003c/p\u003e \u003cp\u003eOpen interpretive coding was used to identify and analyse the emerging themes in all narratives. Additionally, narratives were retrospectively classified following Frank\u0026rsquo;s classification [\u003cspan citationid=\"CR33\" class=\"CitationRef\"\u003e33\u003c/span\u003e], which distinguishes between three types: (a) \u003cem\u003echaos\u003c/em\u003e narratives, which are anti-narratives revealing the narrator\u0026rsquo;s vulnerability, (b) \u003cem\u003erestitution\u003c/em\u003e narratives, in which the narrator explores the experience and meaning of the care pathway, and (c) \u003cem\u003equest\u003c/em\u003e narratives, in which the disease becomes a motivation for change.\u003c/p\u003e \u003cp\u003eThe Steering Committee collectively discussed the results to address the emerging issues and data interpretation. The research team followed the COREQ reporting guidelines [\u003cspan citationid=\"CR34\" class=\"CitationRef\"\u003e34\u003c/span\u003e].\u003c/p\u003e \u003c/div\u003e"},{"header":"Results","content":"\u003cp\u003eSeventeen adult patients diagnosed with C3G and 14 informal caregivers participated in the project; 12 treating nephrologists wrote 15 parallel charts. Table\u0026nbsp;\u003cspan class=\"InternalRef\"\u003e1\u003c/span\u003e shows their sociodemographic data, as well as those of the patients described in the parallel charts, including non-responders as a separate category.\u003c/p\u003e\n\u003cdiv class=\"gridtable\"\u003e\u0026nbsp;\u003ctable id=\"Tab1\" border=\"1\"\u003e\n \u003ccaption language=\"En\"\u003e\n \u003cdiv class=\"CaptionNumber\"\u003eTable 1\u003c/div\u003e\n \u003cdiv class=\"CaptionContent\"\u003e\n \u003cp\u003e\u0026ndash; Sociodemographic data of participants\u003c/p\u003e\n \u003c/div\u003e\n \u003c/caption\u003e\n \u003ccolgroup cols=\"5\"\u003e\u003c/colgroup\u003e\n \u003cthead\u003e\n \u003ctr\u003e\n \u003cth align=\"left\"\u003e\u0026nbsp;\u003c/th\u003e\n \u003cth align=\"left\"\u003e\n \u003cp\u003ePatients with C3 glomerulopathy\u003c/p\u003e\n \u003cp\u003e(N\u0026thinsp;=\u0026thinsp;17)\u003c/p\u003e\n \u003c/th\u003e\n \u003cth align=\"left\"\u003e\n \u003cp\u003eCaregivers\u003c/p\u003e\n \u003cp\u003e(N\u0026thinsp;=\u0026thinsp;14)\u003c/p\u003e\n \u003c/th\u003e\n \u003cth align=\"left\"\u003e\n \u003cp\u003eTreating nephrologists (N\u0026thinsp;=\u0026thinsp;12)\u003c/p\u003e\n \u003c/th\u003e\n \u003cth align=\"left\"\u003e\n \u003cp\u003ePatients in parallel charts (N\u0026thinsp;=\u0026thinsp;15)\u003c/p\u003e\n \u003c/th\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003cth align=\"left\"\u003e\n \u003cp\u003eGender\u003c/p\u003e\n \u003c/th\u003e\n \u003cth align=\"left\"\u003e\u0026nbsp;\u003c/th\u003e\n \u003cth align=\"left\"\u003e\u0026nbsp;\u003c/th\u003e\n \u003cth align=\"left\"\u003e\u0026nbsp;\u003c/th\u003e\n \u003cth align=\"left\"\u003e\u0026nbsp;\u003c/th\u003e\n \u003c/tr\u003e\n \u003c/thead\u003e\n \u003ctbody\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eFemale\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e11 (64%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e11 (79%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e6 (50%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e9 (60%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eMale\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e3 (18%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e2 (14%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e6 (50%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e6 (40%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eNon-responders\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e3 (18%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e1 (7%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e-\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e-\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e\u003cstrong\u003eAge\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e26 (19\u0026ndash;35)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e49 (22\u0026ndash;59)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e41 (27\u0026ndash;66)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e26 (11\u0026ndash;60)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e\u003cstrong\u003eNationality\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\u0026nbsp;\u003c/td\u003e\n \u003ctd align=\"left\"\u003e\u0026nbsp;\u003c/td\u003e\n \u003ctd align=\"left\"\u003e\u0026nbsp;\u003c/td\u003e\n \u003ctd align=\"left\"\u003e\u0026nbsp;\u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eItalian\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e12 (70%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e13 (93%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e12 (100%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e15 (100%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eNon-responders\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e5 (30%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e1 (7%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e-\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e-\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e\u003cstrong\u003eEducation\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\u0026nbsp;\u003c/td\u003e\n \u003ctd align=\"left\"\u003e\u0026nbsp;\u003c/td\u003e\n \u003ctd align=\"left\"\u003e\u0026nbsp;\u003c/td\u003e\n \u003ctd align=\"left\"\u003e\u0026nbsp;\u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eElementary school\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e-\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e-\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e-\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e1 (7%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eMiddle school\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e1 (6%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e1 (7%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e-\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e3(15%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eHigh school\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e8 (46%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e3 (21%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e-\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e8 (53%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eBachelor or higher degree\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e3 (18%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e9 (64%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e-\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e3 (15%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eNon-responders\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e5 (30%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e1 (7%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e-\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e-\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e\u003cstrong\u003eEmployment status\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\u0026nbsp;\u003c/td\u003e\n \u003ctd align=\"left\"\u003e\u0026nbsp;\u003c/td\u003e\n \u003ctd align=\"left\"\u003e\u0026nbsp;\u003c/td\u003e\n \u003ctd align=\"left\"\u003e\u0026nbsp;\u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eStudent\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e3 (18%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e1 (7%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e-\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e7 (47%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eWorking\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e8 (46%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e11 (79%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e-\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e7 (47%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eNot working\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e1 (6%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e1 (7%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e-\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e1 (6%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eNon-responders\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e5 (30%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e1 (7%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e-\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e-\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e\u003cstrong\u003eMarital state\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\u0026nbsp;\u003c/td\u003e\n \u003ctd align=\"left\"\u003e\u0026nbsp;\u003c/td\u003e\n \u003ctd align=\"left\"\u003e\u0026nbsp;\u003c/td\u003e\n \u003ctd align=\"left\"\u003e\u0026nbsp;\u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eSingle\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e7 (41%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e1 (7%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e-\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e-\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eMarried or cohabitant\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e4 (24%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e10 (71%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e-\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e-\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eSeparated\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e-\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e2 (14%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e-\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e-\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eNon-responders\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e6 (35%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e1 (7%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e-\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e-\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e\u003cstrong\u003eProfessional activity\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e-\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e-\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e11.5 (2\u0026ndash;41)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e-\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e\u003cstrong\u003eWorkplace\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\u0026nbsp;\u003c/td\u003e\n \u003ctd align=\"left\"\u003e\u0026nbsp;\u003c/td\u003e\n \u003ctd align=\"left\"\u003e\u0026nbsp;\u003c/td\u003e\n \u003ctd align=\"left\"\u003e\u0026nbsp;\u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eResearch and Care Institute\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e-\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e-\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e8 (67%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e-\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eHospital\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e-\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e-\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e1 (8%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e-\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eUniversity Hospital\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e-\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e-\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e3 (25%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e-\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\" colspan=\"5\"\u003e\n \u003cp\u003e\u003cem\u003eData are presented as N (%) or median (range).\u003c/em\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003c/tbody\u003e\n \u003c/table\u003e\n\u003c/div\u003e\n\u003cp\u003e[Table\u0026nbsp;\u003cspan class=\"InternalRef\"\u003e1\u003c/span\u003e]\u003c/p\u003e\n\u003cp\u003eResults are presented by focusing on three main areas: (a) the C3G care pathway from early symptoms to current treatments; (b) patients\u0026rsquo; and informal caregivers\u0026rsquo; perception of QoL, including the impact of C3G on daily life and the associated emotional burden; and (c) the C3G illness experience as analysed through Frank\u0026rsquo;s classification and metaphors.\u003c/p\u003e\n\u003cp\u003eNarratives informed (a) to (c), while both narratives and quantitative survey data were used to investigate (a) and (b). Figure\u0026nbsp;\u003cspan class=\"InternalRef\"\u003e2\u003c/span\u003e and Tables\u0026nbsp;\u003cspan class=\"InternalRef\"\u003e2\u003c/span\u003e\u0026ndash;\u003cspan class=\"InternalRef\"\u003e3\u003c/span\u003e provide supporting quotes from narratives; additionally, Supplementary file 3 contains three narratives in English.\u003c/p\u003e\n\u003cp\u003eThe majority of patients (92%), caregivers (63%), and nephrologists (86% of parallel charts) reported positive experiences with writing (\u0026ndash;\u003cem\u003eI\u0026rsquo;m happy when my testimony gives strength to another person\u003c/em\u003e, P006; \u0026ndash;\u003cem\u003eI hope it helps similar cases and research\u003c/em\u003e, C007). Conversely, 8% of patients, 37% of caregivers, and nephrologists in 14% of parallel charts considered it a challenge (\u0026ndash;\u003cem\u003eThe memory of this journey gave me strong emotions, because the suffering of this patient due to an untreatable, relapsing disease, which led him to dialysis and the failure of the kidney transplant, is also a suffering of the doctor who takes care of the patient\u003c/em\u003e, PC011).\u003c/p\u003e\n\u003cdiv\u003e\n \u003ch2\u003eThe C3G care pathway from early symptoms to current treatments\u003c/h2\u003e\n \u003cp\u003eSeventy-three per cent of patients discovered the condition because of symptoms, e.g., swelling, fever, tiredness (\u0026ndash;\u003cem\u003eI remember one summer evening when my parents were very worried about my very swollen ankles and feet. It had been a few weeks since we had noticed widespread oedema, first around the eyes and then suddenly in my lower limbs\u003c/em\u003e, P008), while 27% discovered it from the results of analyses carried out in other circumstances; the same was reported by 45% and 55% respectively of caregivers (\u0026ndash;\u003cem\u003eWe became aware of the disease purely by chance by doing routine tests required for sporting activity, no symptoms of the disease had ever appeared\u003c/em\u003e, C003). Nephrologists highlighted that some patients \u0026lsquo;denied\u0026rsquo; C3G symptoms in 40% of parallel charts (\u0026ndash;\u003cem\u003eOne could see how his will/need to deny change and therefore the presence of an \u0026lsquo;illness\u0026rsquo; shone through. The feigned indifference was a sign of concern and fear of something he could not define but did not want to be confronted with. A desire to get rid of the problem so as not to be upset by his certainties and habits\u003c/em\u003e, PC004).\u003c/p\u003e\n \u003cp\u003eThe mean age of onset of C3G symptoms in patients was 18.8 years (\u0026plusmn;9.1); of the 17 patients, five reported consulting a nephrologist as their first specialist after symptom onset. The diagnosis was communicated to all patients by the nephrologist.\u003c/p\u003e\n \u003cp\u003eWhen patients were asked to write about their biopsy experience, only 17% described it as traumatic (\u0026ndash;\u003cem\u003eI will never forget the first biopsy because of the pain I felt when the anaesthetic wore off\u003c/em\u003e, P001; \u0026ndash;\u003cem\u003eIt was not too painful, but I felt some discomfort for the next few days\u003c/em\u003e, P002); patients were more likely to describe the procedure itself (50%) and express gratitude towards the healthcare professionals who carried it out (\u0026ndash;\u003cem\u003eThey all always took care of me\u003c/em\u003e, P004). Thirty-six per cent of caregivers described the diagnosis as a traumatic experience (\u0026ndash;\u003cem\u003eThe day of the kidney biopsy, the only way to diagnose the disease without any doubt, was perhaps one of the most difficult days of my life\u003c/em\u003e, C008), as also highlighted in parallel charts (\u0026ndash;\u003cem\u003eLuckily, each time we did it, it was an uncomplicated procedure. Nevertheless, I could not accept that a child could be so unfortunate as to be subjected to such invasive acts\u003c/em\u003e, PC011).\u003c/p\u003e\n \u003cp\u003eOn receiving the diagnosis, 36% of patients and 20% of caregivers reported feeling anxious, while 46% of patients and 50% of caregivers expressed disbelief (Table\u0026nbsp;2). Similarly, nephrologists experienced anxiety in 20% and found the diagnosis a challenging moment in 26% of parallel charts. Twenty-five percent of patients and 40% of caregivers and nephrologists reported ongoing concerns about the potential development of C3G. Meanwhile, 42% of patients and 60% of caregivers and nephrologists in 20% of parallel charts reported feeling reassured. In addition, 33% of patients expressed gratitude for their treatment, while nephrologists in 40% of parallel charts reported feeling frustrated.\u003c/p\u003e\n \u003cp\u003e[Table 2]\u003c/p\u003e\n\u003c/div\u003e\n\u003cdiv class=\"gridtable\"\u003e\u0026nbsp;\u003ctable id=\"Tab2\" border=\"1\"\u003e\n \u003ccaption language=\"En\"\u003e\n \u003cdiv class=\"CaptionNumber\"\u003eTable 2\u003c/div\u003e\n \u003cdiv class=\"CaptionContent\"\u003e\n \u003cp\u003e\u0026ndash; Emotions experienced by participants at diagnosis and today: quotes from narratives\u003c/p\u003e\n \u003c/div\u003e\n \u003c/caption\u003e\n \u003ccolgroup cols=\"3\"\u003e\u003c/colgroup\u003e\n \u003cthead\u003e\n \u003ctr\u003e\n \u003cth align=\"left\"\u003e\u0026nbsp;\u003c/th\u003e\n \u003cth align=\"left\"\u003e\n \u003cp\u003eEmotions at diagnosis\u003c/p\u003e\n \u003c/th\u003e\n \u003cth align=\"left\"\u003e\n \u003cp\u003eEmotions today\u003c/p\u003e\n \u003c/th\u003e\n \u003c/tr\u003e\n \u003c/thead\u003e\n \u003ctbody\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e\u003cstrong\u003ePatients\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e\u0026ndash;\u003cem\u003eAt first I did not know what it was all about. I was very scared and had a hard time processing it, also because they told me that I would also have to start a special course on nutrition\u003c/em\u003e. (P011)\u003c/p\u003e\n \u003cp\u003e\u0026ndash;\u003cem\u003eAt the time of diagnosis, the name C3 glomerulopathy did not exist [...]. Despite the limited knowledge at the time, it was already known that the disease leads to terminal kidney failure in a good percentage of patients, so the news was obviously terrible for my parents. For me, a seemingly healthy child, the news was certainly frightening, but with faith in the progress of medicine, I saw the option of transplant/dialysis as a distant one\u003c/em\u003e. (P012)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e\u0026ndash;\u003cem\u003eI am grateful because what I have gone through has made me a strong and independent woman. I definitely feel happier and less angry at the world, also thanks to psychotherapy. There are days when the darkness returns and fears take over, but I try to manage the panic by concentrating on the present\u003c/em\u003e. (P002)\u003c/p\u003e\n \u003cp\u003e\u0026ndash;\u003cem\u003eToday, after a kidney transplant, I feel reborn. I still have my fears and anxieties, but it takes little to know how to manage them and live your life harmoniously\u003c/em\u003e. (P004)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e\u003cstrong\u003eCaregivers\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e\u0026ndash;\u003cem\u003eI had read about this disease on the internet. When they explained the disease and the uncertainty of the treatment and its results, the world collapsed on us. It was just me and her in a room 24 hours a day, with no contact with the outside world. It was a very difficult and complex time, but we were always supported with great professionalism and empathy\u003c/em\u003e. (C001)\u003c/p\u003e\n \u003cp\u003e\u0026ndash;\u003cem\u003eWe didn\u0026rsquo;t know what it was all about. In a world where a cure is expected for every disease, I was particularly shocked when the doctors told us that there was currently no cure for C3 glomerulonephritis, and that the child would have to live with the disease for the rest of her life. We did not know (and still do not know) what that meant\u003c/em\u003e. (C005)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e\u0026ndash;\u003cem\u003eI have ups and downs as a parent. In truth, I am always a little afraid of the future and I still find it difficult to accept my child\u0026rsquo;s illness. Thanks to the medical staff, who are always attentive, present, prepared and humane, I always try to see the positive side and think that a definitive cure can be found\u003c/em\u003e. (C003)\u003c/p\u003e\n \u003cp\u003e\u0026ndash;\u003cem\u003eI feel blessed and happy. We keep going back for check-ups, but I feel like a survivor of a shipwreck with my family. When I see my son so strong and beautiful and my family so \u0026lsquo;normal\u0026rsquo;, I am a happy woman\u003c/em\u003e. (C008)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e\u003cstrong\u003eNephrologists\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e\u0026ndash;\u003cem\u003eIn the last biopsy, the damage was so advanced that it was not worth doing anything more. It was difficult to tell her. Of course, she gave us one of her many life lessons: \u0026lsquo;I thought this is the way it\u0026rsquo;s supposed to be.... I\u0026rsquo;d better start dialysis\u0026rsquo;. As usual, she was more oriented than we were. She already knew everything\u003c/em\u003e. (PC005)\u003c/p\u003e\n \u003cp\u003e\u0026ndash;\u003cem\u003eI talked to the parents for about an hour, keeping them busy with the excuse of a diagnostic test where she needed to be alone. Then, in agreement with the parents, we explained to her what had happened, without lying, but without giving up hope of remission\u003c/em\u003e. (PC012)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e\u0026ndash;\u003cem\u003eI have an underlying concern when I think about the uncertainty of the future course of her illness, but I am very happy about the strength and positivity this person shows, even though I am aware of the uncertainties of the future\u003c/em\u003e. (PC003)\u003c/p\u003e\n \u003cp\u003e\u0026ndash;\u003cem\u003eI feel like a boxer in the last round. I must land a hook, just one, and hope I have the right aim and strength. I feel under pressure, but I don\u0026apos;t want to give up. I consult the literature and ongoing trials to find the best drug. But as I do so, I feel the weight of time passing, taking away nephrons and limiting my options\u003c/em\u003e. (PC006)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003c/tbody\u003e\n \u003c/table\u003e\n\u003c/div\u003e\n\u003cp\u003eSix out of 17 patients underwent genetic testing, while 7/17 patients had follow-up visits within 0\u0026ndash;3 months. Nephrologists reported that patients were living with the disease and had stable kidney function at this point in their care pathway in 60% of parallel charts, while reported that patients were experiencing or feared clinical deterioration in 40% of parallel charts.\u003c/p\u003e\n\u003cp\u003eIn the narratives, 33% of patients and 20% of caregivers mentioned experimental treatments. Another 33% of patients described treatments as burdensome, while 17% of patients and 20% of caregivers expressed that current treatments offered hope. Nephrologists stated that their goal was to stabilise or reverse C3G in 60%, mentioned the possibility of transplantation in 20%, and aimed to return patients to their previous lifestyle in another 20% of parallel charts.\u003c/p\u003e\n\u003cp\u003e[Table 3]\u003c/p\u003e\n\u003cdiv class=\"gridtable\"\u003e\u0026nbsp;\u003ctable id=\"Tab3\" border=\"1\"\u003e\n \u003ccaption language=\"En\"\u003e\n \u003cdiv class=\"CaptionNumber\"\u003eTable 3\u003c/div\u003e\n \u003cdiv class=\"CaptionContent\"\u003e\n \u003cp\u003e\u0026ndash; The experience of C3G current treatments according to participants: quotes from narratives\u003c/p\u003e\n \u003c/div\u003e\n \u003c/caption\u003e\n \u003ccolgroup cols=\"2\"\u003e\u003c/colgroup\u003e\n \u003cthead\u003e\n \u003ctr\u003e\n \u003cth align=\"left\"\u003e\n \u003cp\u003ePatients\u003c/p\u003e\n \u003c/th\u003e\n \u003cth align=\"left\"\u003e\n \u003cp\u003e\u0026ndash;\u003cem\u003eTherapies are always present in our lives and cooperation with the doctor is essential. Dialysis removes most of the medications and restrictions of the terminal period, but it also introduces new ones. In my experience, having to feel attached to the dialysis session has a much stronger negative psychological impact than any therapy I might undergo\u003c/em\u003e. (P012)\u003c/p\u003e\n \u003cp\u003e\u0026ndash;\u003cem\u003eThere are so many treatments these days, especially after a kidney transplant... There are days when I feel tired and think about all the therapies I have to do, but I know it is for my own good and especially for the gift I have received\u003c/em\u003e. (P004)\u003c/p\u003e\n \u003c/th\u003e\n \u003c/tr\u003e\n \u003c/thead\u003e\n \u003ctbody\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e\u003cstrong\u003eCaregivers\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e\u0026ndash;\u003cem\u003eOver time, thanks to the new avenues opened up by scientific research, treatments are giving people more confidence, hope and the ability to live as normal a life as possible. Treatments are becoming less invasive and more manageable\u003c/em\u003e. (C003)\u003c/p\u003e\n \u003cp\u003e\u0026ndash;\u003cem\u003eCures are possible, difficult to detect and potentially very long, but they are improving\u003c/em\u003e. (C006)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e\u003cstrong\u003eNephrologists\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e\u0026ndash;\u003cem\u003eI would like him to be able to perform a kidney transplant with real success. Unfortunately, there is no guarantee of this; on the contrary, he has a high risk of further transplant failure. Now, our knowledge does not provide us with a definitive solution or a stable approach to this pathological condition. Today, as yesterday, there are the same complaints and the same predictions of failure, albeit to a lesser extent\u003c/em\u003e. (PC011)\u003c/p\u003e\n \u003cp\u003e\u0026ndash;\u003cem\u003eWe have fought, but we have lost. For the future, I am already looking for the \u0026lsquo;best possible medicine\u0026rsquo;, but I am not sure I will find it. In the meantime, I pray that the dragon will not return\u003c/em\u003e. (PC005)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003c/tbody\u003e\n \u003c/table\u003e\n\u003c/div\u003e\n\u003cdiv id=\"Sec7\" class=\"Section2\"\u003e\n \u003cp\u003e\u003cbr\u003e\u003c/p\u003e\n \u003cp\u003eLooking back on the care pathway, 58% of patients and 60% of caregivers reported satisfaction (\u0026ndash;\u003cem\u003eI wouldn\u0026rsquo;t change a thing, because to this day I am proud of who I am and the scars I carry, especially when you are an inspiration to your generation\u003c/em\u003e, P004); in 60% of parallel charts, nephrologists stated that they had done everything possible (\u0026ndash;\u003cem\u003eIn this case, I have no regrets or remorse about what we did medically or what we communicated in terms of our relationship with the family and the patient. [...] The time available should be more than we can offer. Proper and thorough communication is also part of the process of caring for a patient. Not talking enough becomes a source of regret, especially in situations where the outcome is not positive\u003c/em\u003e, PC004); 33% of patients, 20% of caregivers and nephrologists in 60% of parallel charts expressed some regret about the care pathway (\u0026ndash;\u003cem\u003eI think that if there had been more cooperation between all the centres, given the rarity of the disease, things might have been different\u003c/em\u003e, P012; \u0026ndash;\u003cem\u003eI would have liked to have been diagnosed earlier and to have experienced less suffering and anxiety\u003c/em\u003e, P010; \u0026ndash;\u003cem\u003eMaybe I could have shared my experience with other parents of people with the same condition\u003c/em\u003e, C005). Finally, 8% of patients and 20% of caregivers reported that the care pathway was complex (\u0026ndash;\u003cem\u003eI hope everything goes well, but I am very afraid of another relapse\u003c/em\u003e, P008).\u003c/p\u003e\n \u003cp\u003eForty-five per cent of patients, 18% of caregivers and nephrologists in 40% of parallel charts expressed a desire for more research funding and more available therapies for C3G (\u0026ndash;\u003cem\u003eI hope that research will continue and that over the years we will learn more about the disease\u003c/em\u003e, P001; \u0026ndash;\u003cem\u003eI wish there was more money for research! Not just for this disease, but for all diseases\u003c/em\u003e, C009; \u0026ndash;\u003cem\u003eI hope that new drugs will be developed that will at least further reduce the risk of transplant failure, or at least delay the need for dialysis for as long as possible\u003c/em\u003e, PC011).\u003c/p\u003e\n\u003c/div\u003e\n\u003cdiv id=\"Sec8\" class=\"Section2\"\u003e\n \u003ch2\u003eLiving with C3G and quality of life\u003c/h2\u003e\n \u003cp\u003eFigure \u003cspan class=\"InternalRef\"\u003e1\u003c/span\u003e shows the self-assessment of the impact of C3G on work activities by the patients; ten out of 17 patients responded. Specifically, 50% of respondents reported that C3G impacts \u0026lsquo;quite a lot\u0026rsquo; and 20% \u0026lsquo;a lot\u0026rsquo; on what they do at work, while 40% reported being \u0026lsquo;quite a lot\u0026rsquo; and 20% \u0026lsquo;a lot\u0026rsquo; less focused than they would like at work.\u003c/p\u003e\n \u003cp\u003e[Figure \u003cspan class=\"InternalRef\"\u003e1\u003c/span\u003e]\u003c/p\u003e\n \u003cp\u003ePatients reported losing an average of 133 working days per year due to C3G, which accounts for 52.7% of the total working days; additionally, they spend an average of EUR 400 per year on medication and transport. Family members report losing an average of one working day per month to care for their loved one, with the main impact being on their social life.\u003c/p\u003e\n \u003cp\u003eForty per cent of patients reported a low impact on social activities and relationships due to C3G, while another 40% reported a moderate to high impact. In terms of the patient\u0026apos;s relationships with others, 70% of caregivers and only 11% of patients reported improvement since the onset of C3G; conversely, 55% stated that they often did not disclose the disease to others (\u0026ndash;\u003cem\u003eOnly a few of my friends know about my illness. I was always reserved; I did not like to tell people about my illness. I didn\u0026apos;t want to change my image and I was afraid that not everyone would understand\u003c/em\u003e, P009), as also highlighted in 21% of parallel charts (\u0026ndash;\u003cem\u003eHe is always in a good mood, although he may be hiding the anguish associated with his condition\u003c/em\u003e, PC001) and in 10% of caregiver narratives.\u003c/p\u003e\n \u003cp\u003eRegarding their relationship with food, 55% of patients reported having a highly restricted diet, while 45% reported less restriction (\u0026ndash;\u003cem\u003eAt home I try to limit the use of salt, outside I eat normally. I try to drink a lot because I have been advised to do so. [\u0026hellip;] I have learnt what signs to look out for to understand how to change something\u003c/em\u003e, P009). Among caregivers, 50% reported that the patient had a highly restricted diet. In contrast, in 21% of parallel charts, nephrologists reported that the patient had a complex relationship with food (\u0026ndash;\u003cem\u003eI\u0026rsquo;m going to put him on a low protein diet of 0.6g/kg/day and hope to gain time and opportunity... It will be difficult to get him to do this as it will make him feel ill and tear down the curtain of \u0026apos;there is no problem\u0026apos; that he hides behind\u003c/em\u003e, PC006).\u003c/p\u003e\n \u003cp\u003eLooking to the future, 54% of patients and 82% of caregivers expressed a desire for a good quality of life (\u0026ndash;\u003cem\u003eFor tomorrow I am confident... I hope I can get back to my old self, even though I know I will never be the same again, but at least I can regain the strength I had before\u003c/em\u003e, P005).\u003c/p\u003e\n\u003c/div\u003e\n\u003cdiv id=\"Sec9\" class=\"Section2\"\u003e\n \u003ch2\u003eC3G illness experience in narratives\u003c/h2\u003e\n \u003cp\u003e\u003cem\u003eRestitution\u003c/em\u003e [\u003cspan class=\"CitationRef\"\u003e33\u003c/span\u003e] characterises 75% of patient narratives, 46% of caregiver narratives, and 47% of parallel charts (Fig.\u0026nbsp;\u003cspan class=\"InternalRef\"\u003e2\u003c/span\u003e). \u003cem\u003eQuest\u003c/em\u003e characterises only patients\u0026rsquo; and caregivers\u0026rsquo; narratives (17% and 23%, respectively), whereas \u003cem\u003echaos\u003c/em\u003e is predominant in parallel charts (53%).\u003c/p\u003e\n \u003cp\u003e[Figure \u003cspan class=\"InternalRef\"\u003e2\u003c/span\u003e]\u003c/p\u003e\n \u003cp\u003eParticipants used various metaphors to describe C3G, with two groups being prevalent: (a) 22% of patient narratives, 18% of caregiver narratives, and 47% of the parallel charts contained nature-related images (\u0026ndash;\u003cem\u003eI see myself flanked by a white wolf\u003c/em\u003e, P003; \u0026ndash;\u003cem\u003eA black spider\u003c/em\u003e, C007; \u0026ndash;\u003cem\u003eA slow approaching tide\u003c/em\u003e, N014); (b) 33% of patient narratives, 72% of caregiver narratives, and 53% of the parallel charts contained images of limitation or threat (\u0026ndash;\u003cem\u003eA path covered in mist\u003c/em\u003e, P011; \u0026ndash;\u003cem\u003eA black cloud above me\u003c/em\u003e, C002; \u0026ndash;\u003cem\u003eA ghost\u003c/em\u003e, N001).\u003c/p\u003e\n\u003c/div\u003e"},{"header":"Discussion","content":"\u003cp\u003e Given the lack of literature on the humanistic burden of people living with C3G, the project represented the first attempt to investigate the illness experience and impact on QoL of C3G in Italy, while incorporating the viewpoints of adult patients, informal caregivers and treating nephrologists.\u003c/p\u003e \u003cp\u003eConsistent with a previous study [\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e], the symptoms most frequently reported by patients, such as swelling and fatigue, have received minimal attention in the existing literature. In particular, fatigue remains poorly addressed despite its crucial impact on work activities and overall quality of life.\u003c/p\u003e \u003cp\u003eThe diagnosis and treatment of C3G elicit emotions of fear, anxiety, and disbelief in both patients and caregivers. Nevertheless, narratives illustrate that both patients and caregivers can establish a new daily routine while living with the disease, evidenced by the prevalence of \u003cem\u003erestitution\u003c/em\u003e [\u003cspan citationid=\"CR33\" class=\"CitationRef\"\u003e33\u003c/span\u003e] narratives. Despite this, C3G maintains a significant impact on patients\u0026rsquo; QoL, particularly relatively to social and work activities, as highlighted by the self-reported loss of an average 52.7% of working days per year, in line with findings from previous studies [\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eInterestingly, treating nephrologists show more \u003cem\u003echaos\u003c/em\u003e narratives [\u003cspan citationid=\"CR33\" class=\"CitationRef\"\u003e33\u003c/span\u003e] and express feelings of frustration concerning the C3G management while waiting for new therapeutic strategies. This observation highlights their emotional involvement and, at the same time, raises concerns about their potential oversight of the improved everyday life that patients and caregivers report in narratives. On the other hand, the prevalence of \u003cem\u003echaos\u003c/em\u003e narratives among treating nephrologists may be due to their awareness of the disease progression and suggests that caring for C3G substantially impact their professional lives: this issue should be further investigated, also in the light of previous studies suggesting that nephrologists are susceptible to burnout with specific characteristics due to the disease and the chronic care relationship [\u003cspan citationid=\"CR35\" class=\"CitationRef\"\u003e35\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eContinuing scientific research to discover new and targeted treatments for C3G is of great importance to all those involved in this project. Patients often report enduring intensive treatments for C3G, some of which are experimental. However, patients emphasise that these treatments offer hope of stabilising the disease and potentially allowing a return to their previous life or ensuring a good QoL.\u003c/p\u003e \u003cp\u003eSuggestions for improving the care pathway for people with C3G emerged from the narratives:\u003c/p\u003e \u003cp\u003e \u003col style=\"list-style-type: lower-alpha;\"\u003e \u003cspan\u003e \u003cli\u003e \u003cp\u003eOrganizational and structural improvements: encouraging systematic collaboration among healthcare centres in the area and facilitating early diagnosis were highlighted. This reinforces findings from a previous survey [\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e] where patients and caregivers perceived delayed diagnosis. Lack of detailed information about C3G was also mentioned by patients and caregivers, leading to emotional stress. Efforts aimed at disease awareness and social understanding could alleviate this stress, potentially reducing the fear, anxiety, and disorientation experienced by patients and caregivers upon diagnosis.\u003c/p\u003e \u003c/li\u003e \u003c/span\u003e \u003cspan\u003e \u003cli\u003e \u003cp\u003eEmotional and psychological support: offering opportunities for caregivers to connect with others who share similar experiences, such as family members of individuals with C3G, could provide valuable support. Similarly, facilitating avenues for patients to engage with others about their condition, addressing their fear of not being comprehended, could be beneficial. It is worth noting that other kidney diseases requiring dialysis or transplantation also significantly impact patients\u0026rsquo; psychological well-being [\u003cspan additionalcitationids=\"CR37\" citationid=\"CR36\" class=\"CitationRef\"\u003e36\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR38\" class=\"CitationRef\"\u003e38\u003c/span\u003e].\u003c/p\u003e \u003c/li\u003e \u003c/span\u003e \u003c/ol\u003e \u003c/p\u003e \u003cp\u003eWhile further studies are warranted to explore the humanistic burden and unmet needs of both patients with C3G and their informal caregivers, and to explore the patient\u0026rsquo;s journey through the care continuum, findings suggest the potential of NM as a valuable tool in understanding the illness experience of individuals living with C3G and the caregiving burden. Furthermore, findings highlight the importance of involving treating nephrologists in research into the overall impact of C3G.\u003c/p\u003e \u003cp\u003eSince the participation in the project was voluntary, the researchers could not quantify the agreements between patients, informal caregivers, and treating nephrologists. Also, as the project only included patients who were followed up in expert centres or supported by PAs, the situation of those who did not have access to these centres may be more critical. Conversely, the lack of validated scales for QoL assessment in C3G allowed a more spontaneous and qualitative experience of the disease to be captured through narratives.\u003c/p\u003e"},{"header":"Conclusion","content":"\u003cp\u003e The TIGRE project investigated the humanistic burden associated with C3G as perceived by patients, informal caregivers and treating nephrologists, uniquely integrating these viewpoints, and marking the first Italian NM initiative on C3G. The findings suggest that NM is a valuable tool for understanding the C3G illness experience, while highlighting the need to involve nephrologists in more comprehensive research. Overall, narratives provided valuable insights aimed at raising awareness about the living with C3G.\u003c/p\u003e"},{"header":"Abbreviations","content":"\u003cdiv class=\"DefinitionList\"\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eC3\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003eComplement 3\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eC3G\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003eComplement 3 glomerulopathy\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eC3GN\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003eComplement 3 glomerulonephritis\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eCOREQ\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003eConsolidated criteria for reporting qualitative research\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eDDD\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003eDense deposit disease\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eESRD\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003eEnd stage renal disease\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eISTUD\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003eIstituto Studi Direzionali\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eNM\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003eNarrative Medicine\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003ePA\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003ePatient association\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003ePROs\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003ePatient reported outcomes\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eQoL\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003eQuality of life\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eWHO\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003eWorld Health Organisation\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003c/div\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003eEthics approval\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe project was conducted according to the declaration of Helsinki.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConsent to participate\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eParticipants provided a web-based informed consent before their involvement and after being briefed on the purposes of the research and the procedures for the processing of personal data, according to General Data Protection Regulation of the European Union 2016/679 and the Italian Law 196/2003. The clinicians involved obtained a written informed consent to participate from the parents of underage patients during the first briefing on the project\u0026rsquo;s methods and purposes.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eData sharing\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eAll datasets used and analyzed during the current research are available in Italian from the corresponding author, upon reasonable request.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eCompeting interests\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eEDS have received honoraria from Alexion and AstraZeneca for holding webinars. LG received grant support from Abionyx and Sanofi to his university department (DIMEPRE-J); he also has been a member of advisory boards for AstraZeneca, Baxter, Chinook, GSK, Mundipharma, Novartis, Pharmadoc, Roche, Sanofi, Travere, Vifor Pharma, and an invited speaker at meetings supported by AstraZeneca, Astellas, Estor, Fresenius, Werfen, Medtronic, Travere, GSK. A.C., E.D., A.P., A.M., G.L, F.T, E.T., A.P., M.F., A.P., A.M., F.S., C.D., E.G., M.G.M., M.V. don\u0026apos;t have financial interests in relation to the work described.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eFunding statement\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eNovartis Farma unconditionally supported\u0026nbsp;ISTUD Srl for realising the project.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAuthors\u0026rsquo; contribution statement\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eAC, EG, MGM, CD were involved in the project\u0026rsquo;s conceptualisation. AC, EG, MGM, MV, ED were involved in the methodology. All authors contributed to the project\u0026rsquo;s investigation.AC, EG, MGM contributed to data analysis. AC, EG, MGM, MV, ED, FS contributed to data validation. All authors were involved in writing; all authors contributed to the manuscript review and approved the final draft for submission. MGM is responsible for the overall content as guarantor.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAcknowledgements\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe authors wish to thank Novartis Farma Italia that sponsored and funded this work, especially Vincenza Vinaccia for her editorial assistance. The authors would also thank Erica Daina for the support provided for the Ethical Committee\u0026rsquo;s approval of the project, as well as the researchers of ISTUD Healthcare Area for their useful role throughout the project, as well as all the people suffering from C3G, their caregivers, the healthcare professionals and the Patient Association representatives who took part in the research. All the authors want to thank Alessandra Fiorencis for her professional contribution to the paper.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\u003cli\u003e\u003cspan\u003eSmith RJH, Appel GB, Blom AM, et al. C3 glomerulopathy - understanding a rare complement-driven renal disease. Nat Rev Nephrol. 2019;15(3):129\u0026ndash;43.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eBomback AS, Kavanagh D, Vivarelli M, et al. Alternative Complement Pathway Inhibition With Iptacopan for the Treatment of C3 Glomerulopathy-Study Design of the APPEAR-C3G Trial. Kidney Int Rep. 2022;7(10):2150\u0026ndash;9.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003ePickering MC, D'Agati VD, Nester CM, et al. C3 glomerulopathy: consensus report. Kidney Int. 2013;84(6):1079\u0026ndash;89.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eHeiderscheit AK, Hauer JJ, Smith RJH. C3 glomerulopathy: Understanding an ultra-rare complement-mediated renal disease. Am J Med Genet C Semin Med Genet. 2022;190(3):344\u0026ndash;57.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eDonadelli R, Pulieri P, Piras R, et al. 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Clin J Am Soc Nephrol. 2011;6(6):1436\u0026ndash;46.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eLu DF, Moon M, Lanning LD, et al. Clinical features and outcomes of 98 children and adults with dense deposit disease. Pediatr Nephrol. 2012;27(5):773\u0026ndash;81.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eSchena FP, Esposito P, Rossini M. A Narrative Review on C3 Glomerulopathy: A Rare Renal Disease. Int J Mol Sci. 2020;21(2):525.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eBomback AS, Santoriello D, Avasare RS, et al. C3 glomerulonephritis and dense deposit disease share a similar disease course in a large United States cohort of patients with C3 glomerulopathy. Kidney Int. 2018;93(4):977\u0026ndash;85.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eFeldman LDB, Bomback A, Nester C. Voice of the patient: report of externally-led patient-focused drug development meeting on: complement 3 glomerulopathy (C3G). 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Expert Rev Pharmacoecon Outcomes Res. 2021;21(1):43\u0026ndash;54.\u003c/span\u003e\u003c/li\u003e\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":true,"highlight":"","institution":"","isAcceptedByJournal":false,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true},"keywords":"C3 glomerulopathy, quality of life, humanistic burden, narrative medicine, illness experience","lastPublishedDoi":"10.21203/rs.3.rs-4020752/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-4020752/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003ch2\u003eBackground.\u003c/h2\u003e \u003cp\u003eEvaluating the impact of C3 glomerulopathy (C3G) on quality of life (QoL) and its humanistic burden is critical to a deeper understanding of the condition in clinical practice. This Narrative Medicine (NM) project collected C3G narratives to investigate QoL and humanistic challenges associated with C3G and to gain insights for a more profound knowledge of this disease.\u003c/p\u003e\u003ch2\u003eMethods.\u003c/h2\u003e \u003cp\u003eThe project was conducted in Italy from January to October 2023 and involved eight hospital-based centres and two C3G patient associations. Illness plots and parallel charts were collected through the project website, along with a survey to collect sociodemographic and QoL data. Narratives were analysed using MAXQDA software and interpretive coding.\u003c/p\u003e\u003ch2\u003eResults.\u003c/h2\u003e \u003cp\u003eSeventeen adult patients with C3G and 14 informal caregivers participated in the project, along with 12 treating nephrologists who wrote 15 parallel charts. On receiving the diagnosis, 36% of patients and 20% of caregivers reported feeling anxious, while 46% of patients and 50% of caregivers expressed disbelief. Nephrologists experienced anxiety in 20% and found the diagnosis a challenging moment in 26% of parallel charts. Concerns about C3G evolution were reported by 25% of patients and 40% of caregivers and nephrologists, while reassurance was expressed by 42% of patients and 60% of caregivers and nephrologists. Treatments were defined as burdensome by 33% of patients and hopeful by 17%. Patient and caregiver satisfaction with the care pathway was reported by 58% and 60%, respectively, while 45% of patients, 18% of caregivers and nephrologists in 40% of parallel charts called for more research funding and therapies. C3G significantly impacted work for 50% of patients, resulting in an average loss of 52.7% of working days. The impact on social activities and relationships varied, with 40% of patients reporting a low impact and 40% a moderate to high impact.\u003c/p\u003e\u003ch2\u003eConclusions.\u003c/h2\u003e \u003cp\u003eThis project marked the first Italian NM initiative on C3G. NM has shown to be a valuable tool for understanding the C3G illness experience and raising awareness about the living with this condition.\u003c/p\u003e","manuscriptTitle":"Unveiling the humanistic burden of C3 glomerulopathy in Italy: a narrative medicine multicentre project","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2024-03-11 19:15:15","doi":"10.21203/rs.3.rs-4020752/v1","editorialEvents":[{"type":"communityComments","content":0}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"44cda01f-5d4e-4ae3-b486-e9cc8b22d7c7","owner":[],"postedDate":"March 11th, 2024","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[],"tags":[],"updatedAt":"2024-07-03T06:35:37+00:00","versionOfRecord":[],"versionCreatedAt":"2024-03-11 19:15:15","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-4020752","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-4020752","identity":"rs-4020752","version":["v1"]},"buildId":"qtupq5eGEP_6zYnWcrvyt","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}