Skin and mucosa care in systemic sclerosis - patients' and family caregivers' experiences and expectations of a specific education programme: a qualitative study

BACKGROUND Skin and mucosal manifestations such as skin thickening, pruritus, reduced microvascular circulation, digital lesions, appearance-related changes, and dryness of the eyes and mucosa are common in systemic sclerosis (SSc). A specific skin and mucosa care education programme for patients and their family caregivers should increase their self-efficacy and improve coping strategies. AIMS The aims of this qualitative study were to explore the participants' experiences of both everyday life with skin and mucosal manifestations and the programme itself, while identifying unmet needs for programme development. METHODS Narrative interviews were conducted with eight SSc patients and two family caregivers of individuals with SSc. Using qualitative content analysis techniques, the transcribed interviews were systematically summarized and categories inductively developed. RESULTS The findings illustrated participants' experiences of skin and mucosal symptoms and revealed them to be experts in finding the right therapy mix alone (before diagnosis) and also in collaboration with health professionals (after diagnosis). Participants emphasized that the programme gave them useful education on skin and mucosa care. They described how they had to cope alone with the lack of information on pathophysiology, people's reactions, and the impact on their family and working lives. Nevertheless, participants said that they maintained a positive attitude by not dwelling on future disabilities. CONCLUSIONS Patients and family caregivers benefited from the individualized and SSc-specific education on skin and mucosa care. Future improvements to the programme should focus on imparting understandable information on SSc pathophysiology, dealing with disfigurement and seeking reliable disease information, as well as facilitating peer support.

Oxidative-nitrosative stress and systemic vascular function in highlanders with and without exaggerated hypoxemia

BACKGROUND Acute exposure to high altitude stimulates free radical formation in lowlanders, yet whether this persists during chronic exposure in healthy, well-adapted and maladapted highlanders suffering from chronic mountain sickness (CMS) remains to be established. METHODS Oxidative-nitrosative stress (as determined by the presence of the biomarkers ascorbate radical [A •- ], via electron paramagnetic resonance spectroscopy, and nitrite [NO 2 2 ], via ozone-based chemiluminescence) was assessed in venous blood of 25 male highlanders in Bolivia living at 3,600 m with CMS (n 5 13, CMS 1 ) and without CMS (n 5 12, CMS 2 ). Twelve age- and activity-matched, healthy, male lowlanders were examined at sea level and during acute hypoxia. We also measured fl ow-mediated dilatation (FMD), arterial stiffness defined by augmentation index normalized for a heart rate of 75 beats/min (AIx-75), and carotid intima-media thickness (IMT). RESULTS Compared with normoxic lowlanders, oxidative-nitrosative stress was moderately increased in the CMS 2 group ( P , .05), as indicated by elevated A •- (3,191 457 arbitrary units [AU] vs 2,640 445 AU) and lower NO 2 2 (206 55 nM vs 420 128 nM), whereas vascular function remained preserved. This was comparable to that observed during acute hypoxia in lowlanders in whom vascular dysfunction is typically observed. In contrast, this response was markedly exaggerated in CMS 1 group (A •- , 3,765 429 AU; NO 2 2 , 148 50 nM) compared with both the CMS 2 group and lowlanders ( P , .05). This was associated with systemic vascular dysfunction as indicated by lower ( P , .05 vs CMS 2 ) FMD (4.2% 0.7% vs 7.6% 1.7%) and increased AIx-75 (23% 8% vs 12% 7%) and carotid IMT (714 127 m M vs 588 94 m M). CONCLUSIONS Healthy highlanders display a moderate, sustained elevation in oxidative-nitrosative stress that, unlike the equivalent increase evoked by acute hypoxia in healthy lowlanders, failed to affect vascular function. Its more marked elevation in patients with CMS may contribute to systemic vascular dysfunction.