Managing Climate Change-Induced Sickle Cell Pain Crises With Frank Gonzalez, MD

Clinicians should be aware of the influence of extreme weather events on pain episodes in high risk pediatric patients with sickle cell disease, according to an analysis of 48,885 emergency department visits.1

A recent study reported an independent association between extreme cold temperatures and increased vasocclusive episodes, as well as a suggested protective effect of extreme heat on acute chest syndrome.1

The retrospective case-crossover study of weather-related emergency department encounters involved low risk and high risk patients 0 to 21 years of age at 40 US children’s hospitals.1

In high risk patients, Gonzalez and colleagues observed elevated rates of emergency department visits for vasoocclusive crisis with increased temperature, heat index, and wet bulb globe temperature. In turn, with acute chest syndrome, these same variables were associated with decreased rates of visits. There were no significant associations in the low risk group.1

“That allows you the opportunity to say, ‘Maybe last week there was a heat wave in the Los Angeles area, and we had patients coming in,’” study investigator Frank Gonzalez, MD, an attending physician in the emergency department at Children’s Hospital Los Angeles, told HCPLive. “You can then make sure patients understand: try to stay hydrated, limit outdoor stress or exertional activity, and essentially create an environmental anticipatory guidance plan, an environmental care plan, to try to limit morbidity.”

Sickle cell disease itself sensitizes patients to cold and heat exposure. Often, the cold triggers vasoconstriction, reducing blood velocity, and increasing the likelihood of sickle-shaped red blood cells forming microvascular occlusions. On the other hand, hot temperatures lead to dehydration, increasing blood viscosity, raising the concentration of hemoglobin and sickle cells. These occlusions lead to local tissue hypoxia and ischemic pain similar to cold-induced crises.2

While these environmental factors allowed investigators to glean insight into proactive clinical management strategies, Gonzalez believes there’s potential for a forecast-informed preventative model to prevent these complications from occurring beforehand, using the example of the Asthma Action Plan.

With the reality of climate change and the rising rates of extreme weather, there are other areas of environmentally related factors and sickle cell disease management left to be explored.

“Moving forward, one important area is wildfire-specific fine particulate matter or air pollution, related to wildfires and air pollution in general,” said Gonzalez. “Another important next step involves looking at multiple levels of risk factors, including patient-level or individual factors that could add to a patient’s vulnerability to environmental stressors.”

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. Brandow AM, Stucky CL, Hillery CA, Hoffmann RG, Panepinto JA. Patients with sickle cell disease have increased sensitivity to cold and heat. American Journal of Hematology. 2012;88(1):37-43. doi:https://doi.org/10.1002/ajh.23341