Despite the availability of ERT, symptomatic therapy continues to play an important role in the management of Fabry disease. Some of the adjunctive therapies used by patients with Fabry disease are shown in the table below.

Table 1. Symptomatic therapies used in patients with Fabry disease.^1-3

Lifestyle modifications can also help manage symptoms. Patients should avoid factors that trigger or exacerbate pain, such as exertion and extremes of temperatures, and maintain hydration to minimise heat exhaustion.²

Self-monitoring of pain is recommended to help patients adopt appropriate lifestyle modifications. A Fabry Pain Diary app has been developed to aid patients with self-monitoring and assist their health care professionals in managing and treating their condition.

Other lifestyle modifications that may help include following a low protein diet in cases of renal insufficiency and avoiding smoking to prevent exacerbating pulmonary symptoms.

Interventions such as renal transplantation and cardiac pacemaker insertion are often necessary to delay serious complications due to organ damage.²