Surveying quality of life in patients with Fabry disease by the SF-36 scale

Objective To test the availability of the SF-36 scale for the Chinese patient with Fabry disease (FD), the quality of life(QOL) and its probable influence factors were analyzed. Methods The data were obtained from 50 healthy volunteers and 57 patients with FD enrolled in nephrology department of Ruijin hospital from Jan, 2003 to Jan, 2013. The SF-36 scale was used to evaluate the QOL of patients and to compare the difference between the patientsandcontrols. Furthermore, the influencing factors were estimated by multiple linear regressions. Results Between the patientsandcontrols, the differences of 8 dimensionalitiy's scores had statistical significance, which claimed that the reaction was sensitive. Especially, in the patient group, the Pearson correlation coefficient among each domain of the SF-36 was lower than its Cronbach's α coefficient (0.934), which indicated good internal consistency reliability. The two common factors were much the same to the theory assume, which illustrated the construct validity was available. Even the acceptability of the patient’ group at 100% proved the scale was appropriate for patients with FD. In addition, in role-physical (RP) and bodily pain (BP) , the scores of the male patients were less than the females (P﹤0.01), declared that the QOL of the males was inferior to the females. In the physical function (PF) and the mental health (MH), the scores of the patients with angiokeratoma were less than the group without it, so the patients who had evident clinical symptoms more likely contributed to depression. Using multiple linear regression, age, gender and clinical types were chosen into regression equation by stepwise regression, and the main potential predictor was age. Conclusions The SF-36 scale applies to evaluate the QOL of patients with FD. It is critical to concern and manage the QOL of FD patients, especially in their mental health aspect.