Sometimes, it’s hard to remember that from our kids’ perspective, the disorder in their family is their normal. They don’t know any different. And by that standard, their affected sibling shouldn’t be treated any differently, either. Sometimes, having the affected sibling means gaining the ability to understand people at a much younger age. It means being empathetic and kind. And it means learning to taking a stand against injustices in our lives, be they huge societal issues like the right to sit in a restaurant unmolested or small things such as walking through a store without some busybody making remarks.

Our friend Darin Dillehunt, who has a form of ARCI,  shares a childhood story that illustrates this perfectly.

Ally-Gator Skin

Family members are naturally protective. Older siblings grow up feeling responsible for their brothers and sisters. It’s like having an extra set of parents. One Saturday, after devouring happy meals at McDonalds, my sister and I vacated to the children’s depository Playplace.

A boy of six or seven approached me in the ball pit. He stood not four feet tall, wearing a mask from the Rocketeer movie. He looked me up and down and pointed at my face. “You…have ally-gator skin.” A silent hush fell over the ball pit. I shrugged and walked away from this small boy in a Rocketeer mask, snot dripping from his nose.

Not a minute later, the silence was broken by a sudden wailing. It didn’t come from me, or from snot-boy, who continued to point like the missing guide from a Louis and Clark statue. The cacophonous racket came directly from my sister’s mouth.

Snot-boy’s invective didn’t bother me. Unharmed, I continued to play.   For those with Ichthyosis, there is essential strength inherent in our lives.  These encounters with the misinformed are routine.  Contrary to popular opinion, we are not tragic victims, or brave, stiff upper-lipped individuals. We live complex, if sometimes predictable, lives.

Ironically, my sister was visibly (and audibly) upset, unable to cope with the ignorant remark directed at me. Through the loud, high-pitched crying, she screamed “Stupid-head…meanie…fish-face…”

I was able to laugh at snot-boy’s nonsense because my parents urged me to  experience of life’s ups and downs, knowing the risks, but also knowing what waste there is in the world.  I grew up knowing that whatever I desired to do, I could do, guarded by personal strength and my sister.

When we think about living with ichthyosis, it’s all too easy to forget that children can gain a lot from the adversity placed in front of them. And maybe facing that adversity helps them grow into wonderful, caring, considerate adults who will make the world a better place for the next generation.

In early April, we went to the National Cherry Blossom Festival up in DC. We took some amazing family pictures along the Potomac and at the MLK Memorial. What the pictures of the children playing amidst the cherry blossoms and by the Potomac River don’t show, though, were some of the skin-related meltdowns we had to deal with.

Reading some of our descriptions on Facebook, one of Rachel’s friends jokingly asked about our “Cherry Blossom Death March”. Read on for our full story.

Now that we live within driving distance of Washington DC, we head up that way when the kids have days off from school. We take the train in from the suburbs, which make Monkey and Momo, our train fanatics, very happy. Then we head off to a museum and spend the afternoon checking out dinosaur fossils or locomotives or Civil War artifacts. During the kids’ spring break, we spent a long weekend up in DC right around the time when the cherry blossoms started blooming.

The Cherry Blossom festival, if you aren’t familiar with it, is a 3 week long carnival with music and dance performances, kite flying around the Washington Monument, stalls selling overpriced funnel cakes, a parade, Japanese culture events, a beauty contest and other various attractions. The cherry trees bloom and turn the entire Potomac River shore and the Tidal Basin area a gorgeous pink.

Cookie was in the middle of battling his mystery abdominal pain since the initial doctor visit two weeks before, and his pain was flaring up on Saturday after brunch. Rather than consign the entire family to watching him moan in pain, we decided to split the party. Rachel took Kitty and Monkey into DC, and Jennifer stayed with Cookie and Momo (and, once Cookie’s pain subsided, she got in some extra shopping time).

Rachel took the middle two children via the DC Metro to Arlington National Cemetery, walked with them over the  Memorial Bridge towards the Lincoln Memorial, and then walked past the Reflecting Pool and the Washington Monument, finally riding the Metro back from the Smithsonian station. It was a leisurely afternoon stroll, and Kitty enjoyed it. Rachel had to cajole the children towards the end of the stroll by promising them a visit to an ice-cream shop afterwards, but generally, the kids did just fine.

Kitty and Monkey relaxing by the Washington Monument after our Saturday afternoon stroll

On Sunday, Cookie was feeling a bit better, and after brunch he said that he would like to see the cherry blossoms. So off we all set, intending to replicate the stroll Rachel did with Kitty and Monkey the day before.

We had our first sign that things would be different on the train to Arlington, when Momo tugged at my sleeve and said, “I have to go potty.” We realized that restrooms were few and far between, so we headed off into the Arlington visitor center.

After this lengthy detour, we turned around and started walking over the Memorial Bridge. The children were amused by the eagle statues over the Memorial Bridge. Coming at them from the back, all you could see was the wings, and Kitty and Monkey warned us not to blink – they look like Weeping Angels from the back! At the other end, two golden horses stand on their plinths, looking out regally. But from the kids’ point of view, they looked up and got an eyeful of the horses’ undersides. Which were, shall we say, anatomically correct. Snicker, snicker! (and no, we didn’t get a picture of that!)

We took a break after crossing the bridge, and, instead of walking by the Reflecting Pool like Rachel did the day before, we decided to veer off towards the Potomac River. By the river, the kids had fun gathering and building stuff out of driftwood, and they tried out the newfound skill of counting tree rings on a nearby stump. While crossing the bridge, Monkey kept on asking if there were any alligators in the Potomac. We kept on telling him “no,” but as the children were playing with driftwood we took the photo below. What do you think?

What unknown danger lurks in the Potomac?

We eventually abandoned the children’s stack of driftwood and took a few pictures by the lone cherry tree in full bloom.

You can’t tell, but this was the only tree in full bloom in that area!

As we started walking toward the Tidal Basin, Cookie complained that his feet were hurting. At this point, we belatedly realized that we hadn’t taken a lot of supplies (cream, plastic, liquids, strollers) for a full-scale family outing. But we trudged onward.

We wandered through the Martin Luther King, Jr. Memorial and got the entire family sitting underneath one of Dr. King’s very significant quotes.

And as we were walking through the memorial, we saw the participants in the Cherry Blossom Princess Program dispersing, so we waited by the fountains for the crowd to thin.

Things were rapidly deteriorating and descending into full meltdown mode. The meltdown truly commenced as we walked over the Kutz Bridge.

“My feet hurt.”
“I’m thirsty.”
“Where are the boats? I want to ride the boats.”
“My feet really hurt.”
“This is boring.”
“You said we could ride on the boats!”
“I can’t walk any more.”

Not included in that list of complaints was Momo’s experience. The Kutz Bridge carries auto traffic from Independence Avenue across an edge of the Tidal Basin, and it has a narrow pedestrian walkway on the side. With all the tourists looking to attend the Cherry Blossom Festival, the bridge was extremely crowded with foot traffic. And as we were crossing, the wind started picking up.

So picture little Momo at age 4. He is toddling along next to Rachel. But people are pushing by him on the bridge because he’s not walking as fast as they are. On your left (fortunately behind a concrete barrier) are cars zooming by, kind-of-close. You could definitely feel the “ka-whoosh” as they passed by. And on your right (also behind a concrete rail) is a drop to the water. Now picture some gusts of wind blowing across the bridge, and the little guy losing his footing (and being blown into the people behind him who were already impatient with his slow pace). And add onto that the start of overheating unpleasantness.

Meltdown.

Rachel scooped up Momo and carried him across the bridge. It was hard to tell if his bawling was because of the cars, the wind, the crowd, fear of being lost in the crowd, his feet, the overheating, or some unholy combination of all of that. Cookie, clearly in pain, made it across the bridge on his own power, hanging heavily onto my arm, and then plopped down in relief on the grass by the shore. In between Momo’s sniffling, we realized that he was very clearly showing signs of overheating, so I raced off with Kitty to buy some cold beverages while Rachel rested with the boys.

After cooling Momo down with some water, we looked at our options. The Smithsonian Metro was a few blocks away, but given the crowd it wouldn’t be easy to hail a taxi. We briefly considered calling in a sedan from Uber or further splitting the party. After talking more with Cookie and further contemplating the map, we decided to try to walk to the Metro station.

And thus began the last leg of our poorly planned outing. I scooped up Cookie and lugged him for most of the way. (He was very much unable to take more than a few steps on his own power.) And Rachel carried Momo and our coats, while listening to Kitty and Monkey complain that they had been promised — PROMISED, I tell you — a paddle-boat ride.

We made it to the Metro after several breaks (despite being on the low-end of the growth chart, Cookie is a hefty 70 pounds), thus ending the Cherry Blossom Death March. The next day, Rachel plotted out our walking path. Accounting for the detour into the Arlington National Cemetery visitor’s center, we walked nearly 3 miles with the children, carrying two of them for a not-insignificant portion.

In retrospect, we made two big mistakes. First, we hadn’t planned for overheating. The day before (Saturday) was a perfect day, near 60F, and overheating wasn’t an issue for either Rachel or Monkey. But unfortunately, Sunday was warmer, still only 70F and well below the usual misery threshold. Momo, being a smaller kid, was prone to overheating much easier than the larger siblings or an adult.

Second, because Monkey hadn’t had any issues on Saturday, we didn’t double-check the condition of Cookie’s feet before setting out, and we didn’t make contingency plans. Because he had been miserable with the mystery abdominal pain, Cookie hadn’t been spending a lot of time in the shower (and afterwards) dealing with his skin, and it had been too long since the skin on his feet had been filed down. Add to that a sloppy creaming job on Sunday morning and the result was even less mobility than usual.

Last-minute planning also complicated things. We didn’t bring a stroller, extra cream, plastic wrap or beverages, because our decision to head back out to the cherry blossoms on Sunday was a last-minute decision. And finally, by veering off away from the Mall and the Reflecting Pool (and towards the Potomac), we wound up taking a much longer route on Sunday than Rachel had taken the day before with Kitty and Monkey.

So yes, as parents, we messed up. And given all the planning we usually do, and the experience we’ve had with skin issues, we really should have known better.

Read on for what Laurie wrote about  the myth of having an “unaffected” child and our own reaction and experience.

As you know my two oldest children do not have ichthyosis. This makes them, by conventional wisdom, unaffected siblings. When my son was born, Julianna had just turned four and Jackie was just shy of turning seven. Just a day or so after being born and placed in the NICU, I had to choose between leaving my son, who we were told was going to die, and taking my Julianna to the pediatrician because she had pneumonia. Thankfully, her doctor had an office in the same hospital. From the earliest moments, my daughters have been affected by ichthyosis. Life became a series of difficult choices, doctor’s appointments, visiting nurses, new language, care products, fear, tears, and not so subtle blame directed at me from those unwilling to accept the meaning of congenital.

They are affected by the stares, the unkind words, the feeling of needing to protect the little ones from the creepos of the world. When Faith was born, she didn’t come easy. She tried to be born for weeks. It was summer time and the children came to every appointment with me. Little three year old Joseph in tow, people would loudly proclaim their shock and apparent horror at my son’s appearance. All in front of my big girls who at the time were only 7 and not quite 10. They absorbed the painful words from others and sometimes were even asked direct questions from strangers about why their brother was burned. Would you ask a 7 year old the same question?

Then when Faith came, it was more time in the NICU, more doctors, more stares and unkind words. Getting good at something is not the same as being happy about it. My husband Dan and I grew a lot and changed in the past seven years. I have completely accepted Christ as my savior (oh dear! I’ve lost some of you J), I’ve learned that acceptance is a gift and I’ve made friends, all of you, in the ichthyosis community that make things better for everyone. By changing myself, I’ve improved the lives of my children. They see a better model and have clearer guidelines.

But they’re not unaffected siblings. That is a gift saved for all the other children in the world who’s siblings are not affected by some disease, illness, condition. All four of mine are very much affected siblings. And its time we stopped expecting more from them. ♥

Our own “unaffected” child most certainly has had her life changed because of her brothers’ ichthyosis. Family outings that have to take overheating and decreased mobility into account aren’t as much fun!

Even though she’s a year younger than Cookie (age 10), Kitty (age 9) has been quick to defend him on the school bus when other kids have been mean to him. And she’s been a wonderful help with her little brothers — we’ve asked her to help with their cream and other parts of their skin-care routine, and she really can be a life-saver.

But even so, there are times when she pushes back. “Why do I have to cream my stupid little brother?” “Why can’t I ride in the cart?” “Why can’t I go to the park today?” “Why can’t they come out and ride bikes with me?” “Why can’t Cookie do the stinky, wet chore?”

Even school is a challenge. Countless times, she’s been late to school because of her brothers’ skin-care needs, yet she gets a tardy slip while they are excused. Because, you see, the affected boys’ IEP and 504 plans don’t apply to their “unaffected” sister, even though her life is directly impacted by the realities of the bus schedule. I suppose it makes sense, in an administrative sense, to say that Kitty should just ride the bus while her brothers take a different route to school, or that everyone just should get up a few hours earlier so they can be on time to school. But that assertion just doesn’t fit in with the realities of our schedule.

So, yes — all our children are affected by ichthyosis. Readers, how are the lives your your “unaffected” children different because of  their “more affected” siblings?

EDIT 5/10/2013: While sharing this post, our friend Darin Dillehunt wrote up her own story about how her “unaffected” sibling reacted to a mean kid. It’s a must-read.

Our form of ichthyosis (ichthyosis-en-confetti type 2) has many symptoms that look like palmoplantar keratoderma (PPK), a type of epidermolytic ichthyosis. The skin grows fast, gets thick everywhere, turns gray and itches, and in an injury, comes off in sheets instead of bruising. But unlike many with types of epidermolytic ichthyosis, Rachel and the boys usually don’t get blisters.

The worst parts for us to manage is the hands and feet, as those two areas get the fastest growth and thanks to all the nerve endings, are sometimes painful to deal with. As the skin gets thicker, the color changes from pink to white, and anywhere there is a crease (like at the bend in each finger, the wrists, the base of the thumbs and the lines all along the palms) or anywhere you put pressure (like your fingertips or just walking around), the skin will dry out over the course of the day and crack open with a deep fissure or a small paper cut, depending on how thick we let it get.

We’ve tried everything.

As a child, Rachel would sit on the counter while her parents would clip away at the thick callouses on her feet with a pair of cuticle nippers. She was an avid reader and spent a lot of time watching Star Blazers (does anyone remember the theme song?) – what else do you do to occupy several hours each weekend for foot maintenance? Rachel describes it as basically an hour of boredom punctuated by shocking pain when the cutter accidentally went too deep. Also, leaving them exposed to the air made them dry out faster.

Growing up, Rachel used tretinoin (Retin-A), a topical retinoid, on her palms, feet, knees, and other thickened areas. We’ll write at length about retinoids in the future, but the very short answer is that there are some serious side effects to consider. Rachel’s experience with Retin-A as a child was that on the thin parts of her skin (like the back of her hands or her neck), too much of the skin would shed off and become raw. And on the thickest parts like the heel of her foot, where action was most needed, the Retin-A didn’t seem to penetrate and do anything, even after applying the ointment and wrapping the hand or foot in plastic wrap and leaving it on overnight. 

In high school Rachel had some luck with a Swiss Army knife. She’d sit in the bathtub and literally saw off the clumps of built up skin. It was crude and somewhat effective, but it never really got rid of that massive crack in the center of each heel.

In high school, she also discovered that sandpaper and thick manicure files (100/120 grit) were excellent tools for managing her hand and foot care.

When we got married, I went to a salon and had acrylic nails done, and kept them on for a good while. Every time my nails had to be touched up, the manicurist would use a Dremel to rough them up before applying a new coat of acrylic. Sometimes the tool would bump against skin, and it didn’t really hurt. And thus, an idea was born.

I went off to the local hardware store and picked up a Dremel for Rachel. She was pretty skeptical when I pulled it out. It’s noisy and the idea of coming at your foot with a handheld power tool can be intimidating! She took a few days before getting the courage up to try it, and it took a while to figure out the best technique. We tried it dry at first, but quickly discovered that dry sanding generated too much heat, which caused pain. So for a while, we would bounce it like a conductor’s baton.

Later, we figured out that showering first made the skin softer and generated less heat, and she would call me into the shower to pass an electric tool to her (water turned off, of course!) We would block the shower door with towels to catch all the dust, and 15 minutes later, I’d be back to collect the Dremel and the towels so she could clean up the dust on herself. 

Now, her usual routine is on “Dremel days”, she takes a shower and, after her skin has softened up in the shower, and then she turns the water off and leaves one foot in a bucket of water in the shower. She sits on a stool and fires up the Dremel on the other foot. When that one starts drying out, she switches feet. Doing this IN the shower keeps the dust contained better than letting it spray all over the bigger bathroom.  (Using a plugged-in power tool in a wet shower with your foot in a bucket of water is probably a horrible, horrible OSHA violation of some sort but hopefully it doesn’t rise to the level of any of these pictures.)

When the kids were born, we introduced them all to the Dremel. By this point, we went and purchased a multi-speed version, and for the kids, we made sure it was always on the lowest setting and we use it on them after (not in) the shower. Lower noise meant less drill bit scariness, and even now, I’m able to use it on 4yo Momo without him flinching. He actually prefers it to the file.

And that nasty crack on their heels? Most days, it’s history.

• We talked about this once before, back on Meet the Teacher Night. Basically, there are 3 overlapping issues.
• First, the thick skin functions like an extra layer of clothes, trapping heat simply by being thick.
• Second, the broken water barrier passively lets out water at a constant rate, called transepidermal water loss, that goes up and down with the temperature. The hotter it is, the more evaporation takes place, just like turning up a pot of water on a stove makes it boil faster and turn to steam quicker.
• Third, overheating is compounded by blocked sweat glands that cannot actively pump out sweat  to keep up when the temperature climbs.
• On top of all that, the smaller the person, the more skin they have relative to their size. Bigger people tend to have a lot of mass and a comparatively small skin surface, while an infant at birth can have as much as 6 times the surface that an adult does compared to its weight. This goes down as we grow, but remains a problem especially for younger or undersized children.

Staying hydrated is vital to summer comfort for anyone, but most people don’t have to deal with 4 or more times the water loss through their unimpaired skin. So we often have to go a step further and find another solution. One way to extend our comfort level outside is to wear a cooling vest. There are 3 general types of vests, plus a hybrid style, that you can buy, and all have their benefits and challenges.

The first type is the Evaporative Cooling Vest. You may have seen small versions of this as neck wraps or wrist bands in sporting goods stores. The vest is made of a special material or has small pockets with water-absorbing beads in them.  If you get the vest wet, the beads absorb large amounts of water.  As it slowly dries, the evaporating water takes heat from your body with it. Once it dries, it takes only minutes – a drinking fountain, a bottle of water, or a sink – to get it wet again, so it can be used for longer afternoons. They are also relatively lightweight, last around 5 hours (some vests claim up to 10 hours!) and cheap – around $40-$100.  However, the feeling of being damp may not be appreciated by sensitive skin, and they don’t work very well in high humidity places because there’s nowhere for the water to go in the saturated air, or really dry areas where they dry too fast. These are the ones you usually see on bikers and runners.

The second type is a vest with pockets for ice packs, called a Phase Change Vest, or sometimes a Cold Pack Cooling Vest. These vests are full of Velcro pockets where you can slide in ice packs or gel packs. Ice packs are just like the ones you put in your cooler or lunch bag. They freeze in your freezer. The upside is that they are nice and cool, but they can also be bulky and even painful for sensitive people or damage skin from frostbite if exposed too long. Many vests have gel packs instead, shaped to fit the vest. The chemical that makes the gel doesn’t need to be as cold to freeze, and are often slower to change phase from frozen to liquid, so they last longer and freeze faster than ice packs. Both styles work fine in higher humidity because the packs absorb your heat as they defrost, not attempt to evaporate it into the air. The big downside is that they tend to be heavy and more expensive – most seem to be around $150-$300 and include one set of inserts that stay cool around 2-3 hours. A second set of inserts doubles that time.

Hybrid vests are made with both the beads in the fabric and extra pockets for gel packs, so you can use either function, but they are also at the top end of the price range, closer to $250+

The third kind has a pump system where cooled water flows through tubes in the vest like a refrigerator, powered by a battery pack. They work well, the vest is pretty light, and cooling can last 4-8 hours, depending on the battery and the size of the container with the ice in it, but they tend to be expensive ($300 is the low end) and you have to drag the pump in a cart or bag or backpack, so not as useful for playing soccer, but would be okay for sitting in a wagon for an afternoon at the zoo. Because of the price point, this type is pretty hard to find for children’s sizes.

Expect to replace the vest every 2-3 years in a growing child. If you ask around places such as the Facebook communities, you may be able to find a hand-me-down from a family with an older child.. If you have a vest to pass on, please share your info with FIRST or leave a comment here. Finally, while this won’t help this year, at next year’s FIRST National Family Conference, the raffle usually has several vests as prizes, and often attending families will bring their old vests to pass down to younger kids.

If you’re looking for a new vest, FIRST has a list of companies that have generously donated to the conference raffle in the past on their Overheating Resource Sheet.