Temperature‐related seizures as a daily challenge in Dravet syndrome: Beyond fevers

Short abstract

This commentary is on the original article by Aledo‐Serrano et al. on pages 1450–1459 of this issue.

Dravet syndrome is a rare, severe developmental and epileptic encephalopathy characterized by the onset of prolonged seizures in an otherwise typically developing infant. ¹ Seizures are treatment resistant, and over time additional symptoms emerge that impact speech, cognition, motor function, sleep, and behavior. ¹ , ² While febrile seizures are a hallmark of Dravet syndrome, a variety of other seizure triggers are also commonly reported, which include temperature‐related stimuli. Warm or hot ambient temperatures are reported to increase the occurrence of seizures for individuals with Dravet syndrome, and rapid transitions between hot or cold environments are also reported as precipitants (https://dravetfoundation.org/dsf‐funded‐research/externally‐led‐pfdd‐meeting/). ¹ , ² Navigating temperature‐related seizure triggers represents a substantial challenge for individuals with Dravet syndrome and their families with wide‐ranging medical, social, and economic consequences.

The study by Aledo‐Serrano et al. explores caregiver experiences related to temperature sensitivity in Dravet syndrome, including common temperature‐related seizure triggers, methods to mitigate temperature‐related seizures, and broader impacts on daily life. ³ Although the study cohort was small, including caregivers to 19 individuals with Dravet syndrome living in Spain, use of open‐ended interviews enabled nuanced insights into the lived realities of managing temperature‐related seizure risk. Common themes emerged that have been reported in caregiver populations in other geographic regions suggesting these experiences are likely to be representative of the broader population with Dravet syndrome.

Navigating the challenges associated with Dravet syndrome affects nearly every aspect of daily life, requiring constant vigilance, extensive planning, and extreme flexibility. ² Although navigating temperature‐related seizure triggers represents only one aspect of disease management, caregiver perspectives in the study by Aledo‐Serrano et al. underscore the disproportionate impacts to daily life. Avoidance of heat and temperature transitions leads to substantial limitations on activities, particularly during warmer months. Common activities like playing outdoors, visiting pools and beaches, going shopping, or eating meals at a restaurant pose too high of a risk due to seizure concerns. Notably, caregivers reported that hot ambient temperatures appear to lower the threshold for other seizure triggers, such as excitement, necessitating further limitations on social activities. Temperature considerations extend to routine aspects of daily care, including bathing, clothing selection, and scheduling of activities to avoid peak hours of heat. Employing additional mitigation measures such as home cooling systems, temperature monitoring devices, and personal cooling devices require advanced planning and financial resources. Despite all these mitigation tactics, caregivers reported increased seizure frequency during hotter months, often accompanied by use of rescue medications and higher rates of hospitalization. Collectively, these challenges contribute to social isolation, increased financial burden, and heightened health risks for individuals with Dravet syndrome.

Rising global temperatures and more severe swings in weather patterns due to climate change could disproportionately impact individuals living with Dravet syndrome as well as other rare diseases characterized by temperature sensitivity. As environmental changes intensify, the medical, socioeconomic, and quality of life burdens are likely to increase, particularly in regions experiencing sustained increases in ambient temperature.

Several priorities for future work emerged from these findings from Aledo‐Serrano et al. Given the pervasive impacts of temperature sensitivity to Dravet syndrome, temperature‐related triggers and risk mitigation strategies should be incorporated into clinical care planning. Caregiver‐reported reliance on antipyretics as a mitigation strategy highlights the need for formal evaluation of effectiveness of mitigation strategies to support evidence‐based recommendations. Investigation of the molecular and physiological mechanisms underlying temperature‐sensitivity may also lead to novel interventions. Lastly, studies assessing quality of life and socioeconomic impacts from temperature‐related stimuli in this population could clarify how shifting climate patterns may exacerbate existing burdens.

DATA AVAILABILITY STATEMENT

Not required.