Extreme Cold Independently Associated with Pain Episodes in Pediatric Sickle Cell Disease

With rates of extreme weather events rising due to climate change, clinicians may need to take additional measures to prevent pain episodes in pediatric patients with sickle cell disease (SCD), as a new study suggests severe cold temperatures are independently associated with increased risk.1

The findings highlight the association between extreme weather, both hot and cold, and vaso-occlusive crises (VOC), while also suggesting a potential protective effect of higher temperatures on acute chest syndrome (ACS).1

“We've known for a while that there does seem to be some sensitivity to weather changes, but a lot of the studies have been done in adult, single-center populations,” study investigator Frank Gonzalez, MD, an attending physician in the emergency department at Children’s Hospital Los Angeles, said in an interview with HCPLive. “How do these weather-related factors affect the odds of someone coming in with health complications?”

In pediatric patients with SCD, VOC is the leading cause of emergency department visits, with 96% experiencing an episode by age 8, and as many as 60% having ≥ 1 attack per year. In total, the average stay for hospitalization with VOC is 8 days, significantly impacting pediatric patients’ quality of life. These crises can occur suddenly or be triggered by extreme cold, hard work, heat and sun, psychological factors, and deficiency of ingested fluids, according to previous research.2,3

To address the association between weather-related exposures and odds of emergency department visits for acute complications in youth with sickle cell disease, Gonzales and investigators conducted a retrospective case crossover study.1

They examined emergency department encounters at 40 US children’s hospitals from 2015 to 2020. They categorized patients as low risk or high risk based on the number of emergency department visits in the preceding year, with high-risk patients defined a priori as > 3 visits.1

Investigators constructed a single-variable conditional logistic regression model to identify the odds of emergency department visit in association with weather-related exposures of temperature, heat index, and wet bulb globe temperature.1

The study included a total of 48,885 emergency department visits for VOC and/or ACS in patients between 0 to 21 years of age.1

Upon analysis of increased temperature, heat index, and wet bulb globe temperature among high-risk pediatric patients, investigators observed an associated increased odds of emergency department visits for VOC. In contrast, investigators observed a decreased risk for emergency department visit rates in ACS, with increased markers of heat stress or temperature exhibiting a protective effect.1

Notably, there were no significant associations found for low-risk patients.1

“This suggests that colder temperatures are actually a risk factor for developing acute chest syndrome, which is a very important finding,” said Gonzales. “Previous studies have thought that it's the more infectious risk factors during the colder months that are leading to the development of acute chest but we're thinking that just cold weather on its own is an independent risk factor.”

As for Gonzalez’s advice to clinicians managing this pediatric population, he recommends finding ways to prevent pain episodes by advising their patients to adapt incrementally to the extreme weather. For example, during hotter periods, patients should decrease outdoor exertional time, prioritize air conditioning, and ensure they are staying hydrated. During the colder months, clinicians should encourage patients to limit quick changes into cold temperatures by staying bundled up and staying inside during weather advisories.

Future areas of research will continue to evaluate the association between pain episodes in pediatric patients with sickle cell disease and wildfires, particularly in the Los Angeles area.

Editor’s Note: Gonzalez reports no relevant disclosures. 

References

1. Gonzalez F, Hall M, Habre R, et al. Weather-Related Exposures and Emergency Department Visits for Pediatric Sickle Cell Complications. Pediatric emergency care. Published online 2026:10.1097/PEC.0000000000003561. doi:https://doi.org/10.1097/PEC.0000000000003561

2. Jacob SA, Mueller EL, Cochrane AR, Carroll AE, Bennett WE. Variation in hospital admission of sickle cell patients from the emergency department using the Pediatric Health Information System. Pediatric Blood & Cancer. 2019;67(6). doi:https://doi.org/10.1002/pbc.28067

3. Abdo S, Nuseir KQ, Altarifi AA, Barqawi M, Ayoub NM, Mukkatash TL. Management of Sickle Cell Disease Pain among Adolescent and Pediatric Patients. Brain Sciences. 2019;9(8):182. doi:https://doi.org/10.3390/brainsci9080182