Author(s): BAILEY, D., GOSLING, S., KILBRIDE, C., MANSFIELD, L., HUGHES, D., Institution: BRUNEL UNIVERSITY LONDON, Country: UNITED KINGDOM, Abstract-ID: 1395

INTRODUCTION:
Fabry disease is an inherited metabolic disorder affecting the breakdown of glycolipids in cell lysosomes. A build-up of globotriaosylceramide results in multi-organ disease often affecting the brain, heart and kidneys. The condition leads to a myriad of debilitating symptoms and many individuals with Fabry report impaired mental health and quality of life. Increasing physical activity and reducing sedentary behaviour could support the management of these outcomes. The aim of this study was to explore the feasibility, acceptability, safety and potential efficacy of a novel physical activity and sedentary behaviour intervention in adults with Fabry disease.
METHODS:
The study used a randomised controlled feasibility design. Following baseline measures, participants were individually randomised on a 2:1 ratio to the ACTIVE-FAB (increAsing physiCal acTIVity and limiting sEdentary behaviour in FABry disease) intervention or control group (usual care) for 3 months. The intervention was co-designed and included: (1) three consultation sessions with a physiotherapist to set and review goals, (2) a tailored educational website, (3) a wearable activity tracker, and (4) online peer support. Focus groups were used to explore intervention acceptability. The following measures took place at baseline and 3 months (post-intervention): physical activity and sedentary behaviour using a thigh-worn accelerometer (activPAL4), mental health (Hospital Anxiety and Depression Scale) and quality of life (EuroQol five-dimension questionnaire).
RESULTS:
Forty-five participants aged 54±15 years were recruited. Retention rate was high (96%) and completion rates for study measures were high (ranging from 87-100%). Focus groups with N=13 intervention participants found that the intervention was acceptable overall. Consultation sessions were highly acceptable with support on developing and reviewing goals being instrumental in behavioural changes. The website was useful initially, but most participants rarely used it later in the intervention. Self-monitoring steps using the wearable tracker was a kay factor in increasing physical activity. There was low engagement with the peer support. The trial was safe with <1 adverse event or unplanned healthcare visit per participant during the study. Relative to the control group, the intervention increased step cadence (p=0.01), increased time per day in stepping bouts lasting ≥ 2 and ≥ 5 minutes (by 11 ± 5, p=0.05 and 8 ± 4, p=0.02, respectively) and tended to increase steps/day by 1466 ± 818 (p=0.09).
CONCLUSION:
This study demonstrates the feasibility, acceptability and safety of delivering and evaluating the ACTIVE-FAB intervention in Fabry disease. The intervention also demonstrated potential for improving physical activity behaviour. These findings support evaluation in a definitive randomised controlled trial, which could inform changes in healthcare to improve the management of this condition.