Background DcSSc is associated with high morbidity and mortality, and reduced quality of life. Patient priorities are rarely discussed, with physicians focusing on organ involvement. Using discursive approaches and ethnography, we evaluated the impact of dcSSc on patients' daily lives, and examined how disease burden shapes patient outlook.
Methods Patients were recruited via healthcare professionals (HCPs) or patient associations. In France, Italy, the UK and USA, patients filmed short (∼15-minute) daily video diaries about their lives over 7 days. On Days 1 and 7, patients participated in a moderator-led discussion of their experiences, and an observation session. In Germany and Spain, patients participated in 60-minute telephone interviews. Video footage and transcribed discussions were reviewed, and the data were categorized and assessed for themes, patterns, and indicators of emotion, ambivalence, and conflict.
Results Twenty-three patients (mean age 54 years; 83% women) participated. Of these, 17 made video diaries, and 6 took part in telephone interviews. The majority of video diaries and interviews took place in patients' homes; on average 5.25 hours of video footage were collected per patient.
Results show that time to diagnosis was often delayed, as patients trivialized symptoms and HCPs attributed symptoms to other causes. Patients had a poor understanding of their diagnosis, and rarely received patient information. DcSSc patients had a high treatment burden; on average this patient sample received 10 tablets of prescribed drugs daily. Importantly, while patients were aware of the seriousness of organ involvement, they reported that skin changes, pain, and fatigue had a dominant effect on daily life, impairing their ability to perform routine tasks. Skin tightening in the lower limbs and feet lead to deformity and loss of mobility, while Raynaud's phenomenon, calcinosis, and digital ulcers caused significant pain and loss of function. Changes in appearance lead to embarrassment, social withdrawal, and depression. Patients experienced dcSSc as a series of losses, including independence and self-esteem, and the unpredictability of the disease made acceptance of the condition difficult (Figure 1). Moreover, patients tended to have small support networks and support services were not offered as part of standard care.
Conclusions Patients with dcSSc had high treatment and emotional burdens, with skin complications, pain, and fatigue profoundly affecting their lives. Patients placed the greatest emphasis on these issues rather than the possibility of organ involvement. There is an unmet need for patient information at the time of diagnosis, and for emotional support services throughout their journey with dcSSc. Directing patients to websites such as SSc patient organizations may be helpful.
Disclosure of Interest D. Khanna Grant/research support from: NIH/NIAMS, NIH/ NIAID, Bayer, BMS, Consultant for: Bayer, BMS, Genetech/Roche, GSK, Genkyotex, Sanofi-Aventis, Actelion, Gilead, Employee of: University of Michigan, Y. Allanore Grant/research support from: Bristol-Myers Squibb, Roche/Genentech, Inventiva, Pfizer, Sanofi, Servier, Consultant for: Actelion, Bayer, Roche/Genentech, Inventiva, Medac, Pfizer, Sanofi, Servier, UCB, C. Denton Grant/research support from: Actelion, GSK, Novartis, CSL Behring, Consultant for: Bayer, Roche, GSK, Actelion, Inventiva, CSL Behring, Takeda, Merck-Serono, Medimmune, Biogen, M. Matucci-Cerinic Consultant for: Actelion, GSK, Pfizer, BMS, J. Pope Grant/research support from: Actelion, Bayer, Consultant for: Actelion, Bayer, B. Hinzmann Shareholder of: Bayer Pharma AG, Employee of: Bayer Pharma AG, S. Briody: None declared, J. de Oliveira Pena Employee of: Bayer HealthCare Pharmaceuticals Inc., O. Distler Grant/research support from: Actelion, Pfizer, Sanofi-Aventis, Bayer, Consultant for: Ergonex, United BioSource, Biovitrium, Novartis, Biogen Idec, Inventiva