Quality of life in patients with Stiff-Person Syndrome: Evaluation congruent with patient values using the schedule-direct weighting method

The health-related quality of life (HRQoL) of patients with chronic progressive disease is low; however, various compensation mechanisms (medical, informal support, formal support, and environmental mechanisms) can improve and restore quality of life (QoL) even in cases of severe physical disability. In this study, we evaluate the QoL according to patient value by individual QoL assessment and identify the determinants of QoL of patients with Stiff-Person Syndrome (SPS) to consider specific support measures. As of December 2020, 13 patients diagnosed with SPS participated in a mixed study that included questionnaires and semi-structured interviews. The main positive factors cited by patients included ‘family support’ and ‘parental care’, whereas negative factors included ‘physical symptoms’ and ‘relationship with doctors’. Comparing SPS with other diseases revealed ‘parental care’ as a unique determinant of QoL in patients with SPS. ‘Parental care’ was identified as the most important area with the highest weight but low satisfaction; hence, in each case, support is needed to ensure that the patient’s caregiver was satisfactory. Additionally, ‘physical symptoms’ was the strongest negative area and can be alleviated by medical assistance. Furthermore, rehabilitation should address the relief of pain caused by muscle spasms and rigidity.