I live in Schenectady, New York, where it snows. A lot. And I have a rare condition called “cold-induced urticaria and anaphylaxis.” Which basically means I am allergic to cold conditions. I really should look into moving.
If I’m outside in the damp cold, or handle a frozen steak more than two minutes or so, I develop big, red, itchy splotches. If hives happen over a large enough swath of my body, as it did on December 26, 2000, in the locker room of the local YMCA after swimming in cold water, I go into anaphylactic shock.
Because I can control my condition with anti-histamines, an Epi-pen, and avoiding dangerous situations, and because I write so often about horrible rare diseases, I never counted mine. But inspired by the diversity of diseases represented at the National Organization for Rare Disorders (NORD
) meeting last fall, I looked it up on their registry. And there it was!
Stress as a Trigger?
It began the week before Christmas, 2000. My mother had died a few months earlier, and my sister and I were hauling my father, in the throes of psychotic depression, from their home in Brooklyn to a facility hours away near my sister. The last time I’d seen my dad before my itching began, he’d thrown a chair at me and given all my mother’s possessions to the movers. I had a few weeks to calm down while my sister got him settled, then was to see him on December 25th
A few days before, I started to itch like crazy every time I came back inside from being outside in the snow. The itching would last about an hour, my skin bright red. Sometimes I got dizzy.
On Christmas day, seeing my dad, I began to itch so badly, body-wide, that my daughter and nephew went out and got me Benadryl.
The next day, back home and in the pool at the Y, I knew instantly that something was wrong. In the shower, I saw enlarging black splotches. Apparently I collapsed before I could tell that my throat was closing. I awoke briefly on a bench as a paramedic gave me oxygen, remember my shirt being yanked off in an ambulance, and finally came around when someone in the ER put Claritin in the IV.
Diagnosis By Ice Cube
Soon I saw an allergist, prepared to have 50 little needles stuck in my arms to find the triggers. But she was so excited that she immediately herded up the other docs to watch her perform the diagnostic test: an ice cube on my inner arm. A few minutes of cold, and bingo, there was a big red wheal that took awhile to dissipate. The doc had only seen it in textbooks.
So I looked up cases. A little girl could no longer eat ice cream. A young man had died after a paramedic applied a coldpak to his swollen throat. The medical literature has few reports
and I don’t know the prevalence.
What caused the sudden onset of my weird condition? One idea was cryoglobulinemia, antibody parts that precipitate when body temp drops. Four percent of patients with cold urticaria (CU) have cryoglobulinemia, and 3% of people with cryoglobulinemia have CU. That wasn’t me.
Nor did I have “familial cold autoinflammatory syndrome
,” linked to mutations in 3 genes. Mine is acquired, not inherited, and I don’t also have muscle pain, fever, and chills.
But autoimmune? That was certainly possible, considering my age and gender. Remembering the father-induced itching, I hypothesized stress as the trigger. No, can’t be, said three different MDs.
Whatever the cause, if my CU stuck around for more than 9 years, I’d have it forever.
An Unexpected Adverse Effect
My allergist put me on an old anti-histamine, Periactin
. I got a handicapped parking tag after a downpour impaired my ability to shop at a row of designer outlets taunting me with their proximity. I wore a series of medicalert bracelets that all fell apart, in case I passed out on a Starbucks line.
I experimented with my limitations and threshholds. Three factors determine reaction time and extent: temperature, duration of exposure, and extent of exposed body part. Dampness is a catalyst. However, I can be exposed at the ankle and turn red up to my thigh, even under clothing; it spreads. I react after a 45 minute walk at 60 degrees, but under a minute at 0 degrees. And up here, we don’t bother with “wind chill.” When it is below zero, it is below zero and can stay that way for weeks.
Folks with my condition can never go in a boat smaller than a ferry. I can go in a pool for no more than 15 minutes, only if the air temperature exceeds 90, and I’ll still itch after. Heating the pool makes no difference and my doc doesn’t want me going in at all. I must wear layers in air-conditioning, cashmere in July. And if there’s any environmental threat, I pop a Benadryl. If I were stuck in the storm hammering the east coast this very weekend, I could die.
Unlike other rare diseases, mine brought on some funny situations. My husband began dreaming up ways to murder me, like dumping snow on me while I slept. The evidence would melt. I thought of spelling things out on my stomach with ice cubes.
But the funniest thing was the day I was on the elliptical trainer at the same Y where I’d collapsed stark naked in the locker room months earlier. I was doing ok avoiding reacting, but had mysteriously gained 10 pounds.
I perused the magazine rack and “Periactin” caught my eye on the cover of “O.” I wouldn’t normally seek Oprah for medical advice, but I grabbed it, got on the elliptical, and started to read.
It seems that brides-to-be in Nigeria, when they want to grow their derrieres for their wedding nights, take Periactin.
Mysterious 10-pound weight gain!
I glanced back at my hindquarters, always the smallest part of me, and fell off the machine.
The next day I was at the allergist’s.
“Why didn’t you tell me about this adverse effect?????”
Slight smile. “Would you have taken it if I told you?”
“Of course not.”
“Well, you’ll be happy to know there’s a new drug without that side effect. Zyrtec.”
A Glimpse of What It’s Like to Have a Rare Disease
My cold-induced urticaria and anaphylaxis is here to stay, and I’ve adapted pretty well. The biggest problem: no one believes me.
“You’re allergic to the cold? Right, me too.”
It makes for some awkward situations. Early on an angry dad challenged my “excuse” of not being able to pick up kids after an outdoor concert that went overtime, because it was pouring rain. Another time, stranded in torrential rain on a tarmac, no one, not even police, believed that my life was in danger. I learned to avoid having to explain my limitations, which usually entails wearing a lot of layers and stuffing my pockets with little pink pills.
I’m embarrassed to be on NORD’s registry, but my experiences have provided glimpses into what it must be like to have a much more serious rare disorder that has an unusual symptom. Life puts you on the defensive, especially if your symptoms aren’t obvious – I’ve had more than one confrontation with a stranger challenging my need for a handicapped tag for parking (and I have needed it for more than shopping). And like so many of the parents, especially moms, of rare disease kids, I’ve had to try to convince doubting docs that something is actually wrong.
To all of you enjoying the winter season this year, I wish I could join you!
Image of Route 7 in Schenectady By Otto Yamamoto. https://commons.wikimedia.org/w/index.php?curid=3654690