Pain assessment: the advantages of using pain scales in lysosomal storage diseases

Routine and standardized assessment of pain should be conducted in patients with conditions, such as Fabry disease, that are associated with chronic pain. Such pain assessments, using validated and reliable pain scales or questionnaires, should cover the severity, location, temporal pattern and quality of the pain and how the pain impacts on quality of life and normal daily activity. The severity or intensity of pain can be assessed on verbal descriptor scales, visual analogue scales and numerical rating scales, which rate pain on a scale from 'no pain' through to 'excruciating pain' or pain as bad as you can imagine'. Three pain questionnaires that include such rating scales are short enough to be used repeatedly in a clinical or research setting: the Memorial Pain Assessment Card, the McGill Pain Questionnaire and the Brief Pain Inventory (BPI). The BPI also measures the effect of pain on daily activity and quality of life, defines the location of pain and assesses the effectiveness of previous pain relief medication.

Reliable instruments are available to assess pain in chronic disease. In Fabry disease, these should be used routinely to aid decisions concerning analgesic/pain control medication and to assess the effect of enzyme replacement therapy.