Fabry patients take a beating when rising temperatures kick in
Summer heat causes symptoms to intensify for my sons with the disease
The sizzle of burgers on backyard grills, the plop of fishing lures cast into lake waters, and the crack of the home run ball are sounds announcing “summer is here!” Across America, ballparks, amusement parks, zoos, lakes, forests, and beaches are coming alive as people brave the beating sun to make memories outdoors.
These iconic summer pastimes were a big part of our family culture when my kids’ lives were unencumbered by heat intolerance. However, gone are the days of being around a campfire on a warm summer night, stringing a clover chain that could encircle our house, taking long bike rides, or enduring blistering hours of coaster-riding at Six Flags Over Texas.
“Beating the heat” isn’t possible for those with classic Fabry disease. As temperatures rise, so do headaches, stomach pains, and burning sensations in the hands and feet.
My oldest sons, twins Michael and Anthony, lived with Fabry symptoms long before being diagnosed, but thankfully the slow progression of the disease gave them the freedom to experience fun in the sun before it became intolerable.
As children, my boys spent long summer days on family land, wandering the swamp in knee-high waders. They loved playing at golf camps, selling lemonade, biking, and gathering throngs of neighbor kids to play football in the yard. For money, they could mow up to 25 lawns in a day, and for fun, they could stand for hours in the sun awaiting the high-speed twists and turns of the Batman or the Texas Giant roller coasters.
The joy of adventure outweighed the foot pains, the vomiting, the lightheadedness, and the other ailments for which we had no explanation.
As the twins entered their teen years, we were still clueless about the source of many of their “sicknesses,” but Fabry was progressing and their symptoms were intensifying, especially in the summer. The deficiency of alpha-galactosidase causes buildup of the GL-3 fatty acid; that continues throughout a Fabry patient’s life, resulting in various symptoms that get progressively worse.
Hypohidrosis, or an inability to sweat, makes heat unbearable for those with Fabry disease. Eventually, the boys’ diagnosis shed light on the heat intolerance they were experiencing, but along the way there were a few clear markers that my sons were unable to handle summer temperatures.
When the heat is on
An all-time favorite family trip was to San Diego in 2016. We visited the majestic Grand Canyon and marveled at the painted rock formations and beautiful forests of Sedona, Arizona, on the way to California. We relished time on the beach, and the older boys learned to surf.
On our last evening there, we decided to go to church after a full day of riding waves in the sun. Michael passed out, his body crumpling over the pew in front of him. Thankfully, his blackout didn’t last long, but the curious memory stuck with us. He was certainly beaten by the heat that day.
When Michael and Anthony turned 16 and got their driver’s licenses, they were so eager to play chauffeur or run errands for their mama. With the power to drive also came the power to expand their business. They were excited to take on more and more lawns.
When the summer months came, however, they began to struggle with their landscape work. Dominic, their brother who does not have Fabry, worked like the Energizer bunny compared with the twins, who would get lightheaded regularly and need frequent breaks. My enthusiastic new drivers also started to wince when I asked one of them to run to the store. “How big is the parking lot?” was their common and unsettling question. They explained that walking across hot asphalt was torture.
Now, years into our Fabry journey, we’re well aware of the correlation between sweltering temperatures and the misery they bring. My kids with Fabry have thus had to find ways not to “beat the heat,” but to escape it.
The VanVickle boys use a few strategies to survive the extreme Texas summers. For one thing, they stay inside air-conditioned spaces as much as possible.
When running errands or going out, we plan ahead. Since crossing the Walmart parking lot can cause them lightheadedness, my sons often go in pairs. One twin can drop his brother at the door and wait in the car. When eating out or going out, they choose indoor seating and avoid overcrowded places where standing room and body heat can be a dismal combination.
Of course, outdoor fun is something we don’t want to miss, so if the VanVickles are going to be outside, it’ll be in a pool or lake. The twins discovered that not wearing socks or keeping a cool, wet towel about the neck can help regulate their body temperature. Staying hydrated and taking breaks from the heat are important, too. Lastly, we take advantage of the cool early-morning or late-evening hours to take walks or sit out on the patio.
Note: Fabry Disease News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Fabry Disease News or its parent company, Bionews, and are intended to spark discussion about issues pertaining to Fabry disease.
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Elizabeth’
I’m
Wondering if the boys are getting infusion therapy and if so if that has lessened their heat intolerance via an increased sweating.
Susanna VanVickle
Hi, Elizabeth.
My boys have gone through 4 and a half years of ERT infusions. Currently they are on a clinical trial, which is administered orally. Unfortunately, treatment has not helped their symptoms nor caused an increase in sweating.