Exploring self-management of diet and physical activity in CACNA1S-related hypokalemic periodic paralysis: A...

Hypokalemic Periodic Paralysis (HypoPP) is a rare genetic neuromuscular disorder characterized by attacks of skeletal muscle weakness or paralysis with spontaneous recovery. The attacks are frequently triggered by specific factors, and previous studies have identified certain associated lifestyle factors, such as dietary intake and physical activity. However, there is currently no in-dept knowledge on how patients experience and self-manage triggering factors in their everyday life.

In this study, we aimed to explore patients' experiences with dietary intake and physical activity through semi-structured interviews. The research question was: Which strategies do individuals with HypoPP utilize in relation to dietary intake and physical activity to prevent or self-manage attacks of muscle weakness and paralysis?

The study included 14 participants aged 21-58 years with HypoPP due to abnormalities in calcium channel function caused by pathogenic variants in the CACNA1S gene. The interviews were transcribed and analysed using Malterud's systematic text condensation.

The participants experienced that regular meals with controlled portions of complex carbohydrates, limited intake of simple carbohydrates, engaging in regular mild to moderate exercise combined with warm-up and cool-down, and avoiding physical inactivity made them less vulnerable to attacks of muscle weakness and paralysis. Furthermore, participants highlighted the cumulative effect of triggers, by consistently reporting feeling more susceptible to attacks when exposed to multiple triggers simultaneously.

The participants experienced self-management through dietary modifications and adjustments in physical activity to reduce symptoms in CACNA1S-related HypoPP. A novel finding was the experienced cumulative effect of lifestyle-related triggering factors.