It can be baffling to others, and it’s pretty serious kryptonite for someone who lives through frigid winters in western Canada. But it’s true: I am allergic to the cold. Specifically, I live with cold contact urticaria (CCU), and even though I don’t fall to the ground in a writhing mess when exposed to wintry conditions, I do break out in itchy, mortifying hives. For some however, cold urticaria can even be life-threatening.
When I spoke to cold urticaria expert Dr. Martin Ostro, his first recommendation was for this northern girl to move to a warmer climate. Sadly, relocating to the tropics isn’t in the cards for me, so instead I’ve invested in a gargantuan winter wardrobe along with a steady supply of antihistamines. I’m lucky; the severity of my cold urticaria has waxed and waned over the years since I first had a reaction at age 9.
In childhood and my teenage years, I had to hatch escape plans for when the raised hives would appear – whenever I got chilled or skin was exposed to cold. As an adult, I learned how to manage the condition so that Old Man Winter doesn’t call the shots. But for others managing cold urticaria – with its perplexing age of onset from babies to seniors – the kryptonite can be even tougher to avoid.
Take 8-year-old Adel Schneider, who had her first reaction when she was only 15 months old. Her grandparents had taken her sledding – her first-ever outdoor winter adventure, when within minutes of being outside, her face started swelling. Soon, they couldn’t make out her facial features. A dose of Benadryl helped the swelling subside. Afterwards, her mom, Kara Schneider, took Adel to their local allergist in Appleton, Wisconsin, where she had been diagnosed six months earlier with a severe dairy allergy.
To determine if Adel had CCU, the allergist performed an ice cube test, which typically involves placing ice on the underside of a patient’s forearm for five minutes, or sometimes an ice pack is applied to the back. When Adel’s skin reacted to the ice, she was diagnosed with cold urticaria and angiodema (deep swelling, usually around lips and eyes).
“He was kind of excited since it is rare – I was not,” recalls Schneider. Cold urticaria is an uncommon condition, with an incidence of only 0.05 percent among the general population, according to the few review studies on the subject.
“We live in Wisconsin,” adds Schneider. “My child can’t eat dairy and now she can’t go outside! Are you kidding me? But we just had to adjust to our new normal.”
Adel was prescribed cyproheptadine (a first-generation antihistamine used to treat allergic symptoms including hives and itching by blocking histamines and serotonin), and continues to take it twice daily. Her last outbreak was two years ago, when her hands were exposed to the cold. “They swelled up like balloons,” says Schneider. “Now we don’t let her go out below 40 degrees F. We know when she is about to have a reaction because her skin burns before she gets the hives and swelling.“
The Schneiders work diligently to manage Adel’s condition. She is completely covered up when it’s cold out, and rushed to and from the car. “We really take every precaution and hustle her in,” affirms Schneider. While Adel’s early onset has meant she’s had to deal with cold urticaria her whole life, she also doesn’t know any different. Luckily her school accommodates Adel’s needs, for example by providing indoor recess.
“She’s really fortunate that the school and her classmates have been extremely willing to become educated and adapt for her,” Schneider adds.
Ostro, a clinical associate in the division of rheumatology, allergy and immunology at Massachusetts General Hospital in Boston, says limiting exposure to cold is essentially the only effective treatment for cold urticaria, which is not a typical allergic disease in that there is no specific IgE, the allergy antibody, which responds to a particular substance. Instead, cold urticaria occurs upon “cold activation of a set of immune cells in the skin and elsewhere, called mast cells,” explains Ostro. “On activation, the mast cells release histamine, which causes the skin to become itchy, red and swollen.”
In most cases, the reasons why someone develops cold urticaria aren’t known, although Ostro adds that “up to 50 percent of patients with CCU have a history of allergic conditions,” which certainly holds true for Adel. That means taking extra steps to manage additional risks, her mom notes.
“We do know that the number one cause of death is drowning while having a reaction,” says Schneider. “That’s why we’re very cautious and we always take the EpiPen with us.”
When she swims at the lake at their family cabin, Adel always uses a wetsuit. However, swimming can be very dangerous for those managing cold urticaria. “I typically advise patients to avoid swimming,” says Dr. Jason Lee, a Toronto-based specialist in clinical immunology and allergy. “Swimming can induce urticaria throughout the body, resulting in hypotension or low blood pressure. In some cases, the diffuse urticaria will cause drowning due to loss of consciousness.”
The danger of swimming with cold urticaria is something that Kim Ledin can attest to. Ledin, who lives in Duxbury, Massachusetts, recalls how she would get lightheaded and dizzy when swimming in the ocean before being diagnosed. “I’d be totally off kilter and my chest felt really heavy,” she says.
She remembers one summer when it was scorching hot, she dove head-first into the cold ocean. “I felt like I couldn’t breathe. I got out and it took 15 minutes for the fear to pass, and that’s when I noticed I had great big raised welts, the size of my palms, on my arms and legs.”
She was sent to a rheumatologist who did an ice test. “He put a cold pack on my lower back and it started to itch and burn after four or five minutes. There was a huge weal (or hive), and that’s when he told me I had an allergy to the cold.”
Ledin’s first reaction didn’t occur until adulthood, at the age of 18, and came prior to the ocean swimming incident. “I was holding a plastic party cup with a cold drink, and my fingers started to feel really itchy and were swelling,” she remembers.
Treatment for cold urticaria includes not only avoidance of chilly environments, but also of cold food and drinks. “Occasionally some patients with this condition will experience angioedema with the ingestion of cold beverages. Eating something cold can potentially cause throat constriction,” says Lee. Thankfully, I’ve never had a reaction to cold food and haven’t had to give up the Häagen-Dazs yet, though I do watch for symptoms, like itchiness in my mouth.
Ostro advises consulting an allergy and immunology specialist about medication. “Management may include taking one or more non-sedating antihistamines – up to four times the usual daily dose per day during the winter – to try to prevent the development of hives on cold exposure.”
Ledin was never given a treatment plan beyond avoidance, so she ceased outdoor winter activities more to deal with discomfort rather than to ensure safety. “I was never made to understand how serious this was,” she explains. “I continued to scuba dive, but my face would get all red and swollen. Now that I’m a mom of an allergic kid, I understand the dangers.”
Ledin, who’s now 45, has roughly 30 minutes outdoors in weather cooler than 50 degrees F before her face gets red and itchy. And if it’s damp, she reacts in far less time. These days, she limits swimming to warm water, but still will get “a bit welty.” Her hands have remained sensitive; even handling cold meat will make them tingle. But like me, she has never reacted to eating cold food.
“Finding out your triggers is the key to managing CCU,” affirms Ostro. “Once you know them, you can figure out ways to avoid them or decrease their effect.”
And so we each adapt uniquely to life with a cold allergy. “It’s important to take a step back and get a bigger picture,” says Schneider. “It’s very easy to get caught up in all the things that my child can’t do or challenges she now has. I’ve tried so hard to instill in her all the things she can do, and that there are others with far greater challenges. Even if it requires lifetime management, CCU is manageable, and how fortunate we are that that is the case.” Indeed we are.