OA42 Patient experiences of managing their rare rheumatic disease
Authors: Dures Emma , Almeida Celia , Hunt Andrew , Lanyon Peter C , Walsh Nicola , Robson Joanna C 
Published: April 2023

Background/Aims

Systemic lupus erythematosus, systemic vasculitis, inflammatory myositis, scleroderma and Sjogren’s syndrome are rare autoimmune rheumatic diseases (RAIRDs). A survey of people with RAIRDs found 61% were struggling, 45% had reduced/stopped work and 45% reported an impact on family life. A survey of NHS rheumatology departments found 80% do not offer access to self-management support to people with RAIRDs. Aims: To understand the patients’ experiences of their RAIRD and views on interventions to support self-management, including content and structure.

Methods

Online focus groups via Zoom using a topic guide developed with a multi-disciplinary team including patient partners with lived experience. Focus groups were advertised via patient charities’ social media platforms (Vasculitis UK, Scleroderma and Raynaud’s UK, Lupus UK, Myositis UK and the British Sjogren’s Syndrome Association). Sessions were audio-recorded, transcribed, organised using NVIVO, and analysed thematically.

Results

Twenty-six patients, 21 (80.76 %) female, median age 62 (range 34-82), participated in six focus groups. Diagnoses included systemic lupus erythematosus (4), inflammatory myositis (7), ANCA-associated vasculitis (5), mixed connective tissue disease (2), scleroderma (1), undifferentiated vasculitis (2), and primary or secondary Sjogren’s (16). Five patients (19%) were newly diagnosed (&lt;2 years) and 21 patients (81%) reported active disease.

Two related main themes capture the data:“I’m constantly doing medical admin along with surviving and fighting a disease.” A lack of joined-up healthcare (“I’ve got some great people but they’re not working as a team”), difficulty accessing specialists (“Everyone just passes you like the sort of parcel that no one wants to open”) and disease complexity (“she said you’re too complicated”) makes managing RAIRDs hard, resource-intensive work (“I’ve had to push so hard for all of it and I think that only happens with very rare conditions”).“Because you have very rare conditions it’s uniquely isolating.” The psychological impact of having complex autoimmune conditions can be exacerbated when they are poorly understood by family and friends (“I’ve encountered mental health problems having to manage family expectations of me”) and health professionals (“they’ve never seen a patient like me in their life”). Patients described strategies that could be beneficial at an individual level (“acceptance, mindfulness, meditation and to not beat myself up about things”) and the importance of addressing mental health (“you’ve got to build your self-esteem, so you don’t feel rubbish all the time”).

Conclusion

Patients with RAIRDs identified shared experiences to inform the development of a cross-condition self-management intervention. These include negotiating healthcare systems and managing the psychological impact of RAIRDs. Further work is needed with under-represented groups to identify whether their support needs are distinct.

Disclosure

E. Dures: None. C. Almeida: None. A. Hunt: None. P.C. Lanyon: None. N. Walsh: None. J.C. Robson: None.