For you created my inmost being;
you knit me together in my mother’s womb.
I praise you because I am fearfully and wonderfully made;
your works are wonderful,
I know that full well.
My frame was not hidden from you
when I was made in the secret place,
when I was woven together in the depths of the earth.
Your eyes saw my unformed body;
all the days ordained for me were written in your book
before one of them came to be.
– Psalm 139:13–16 (NIV)
The first thing that most people remark about my skin is “Wow! You have been out in the sun. You’ve got a bad sunburn.” I usually smile and reply that it is a skin condition I was born with. I tell them my skin is very dry and thin, and I explain that I get hurt easily but heal very quickly. I often say, “If I hit the edge of the table with the back of my hand, it would rip the skin off my hand.” They usually respond with an embarrassed apology of some sort. I am not offended though, as it is a question I am quite used to answering.
But I am getting ahead of myself. Let me first explain the basics of the disease, then I will get into my own experiences.
Two in a Million
My skin disease is called ichthyosis (ik-thee-O-sis). That term actually describes over twenty different skin diseases, all of which involve dry, scaly, rough skin. The word ichthyosis comes from the Latin word ichthy which means “fish.” So it makes sense that it can look like fish or alligator scales. Studies estimate that about sixteen thousand people are born with ichthyosis every year. Out of those, about three hundred cases of the disease are considered moderate to severe. It is sometimes present from birth but can develop later in life as well.
I was born with epidermolytic ichthyosis, a type of ichthyosis that occurs in about two out of a million people. As a small child, my friends enjoyed getting me to say the term aloud that doctors in the ’70s had taught me: “epidermolytic ichthyosis hyperkeratosis.” I would say it in short sound bites, and they would try to repeat it. “Epi . . . dermo . . . lytic . . . ichthy . . . osis . . . hyper . . . kera . . . tosis.” We would laugh and giggle as they failed to repeat it correctly every time. They were amazed that someone so young could say such a complicated string of words. Doctors have since added the term bullous variety to my diagnosis as well. If you break the terms down, here is what they mean:
· Epidermolytic refers to the reddish blistering.
· Ichthyosis refers to the fish-like scales.
· Hyperkeratosis refers to the thickening of the skin.
· Bullous refers to blistering.
My skin is dry, red, easily blistered, prone to infection, thick, smelly, and itchy. I’m not sure if you picked up on that word smelly, but as a child, my skin did not smell good. With all the thick scales, it is common for bacteria to grow around and under the scales, causing a distinct odor. As you can imagine, this caused many problems for me growing up. You will hear more about that later.
Dry and Scaly
Ichthyosis skin does not have the barrier it needs to hold in moisture, so patients experience lots of dry, scaly skin. The skin builds up to very thick, almost warty-looking scales. These scales are worse on any joint areas, such as the hands, feet, knees, hips, waist, neck, and elbows. The scales can be very painful and require lots of added moisture to keep them pliable. All skin sheds constantly, but normal skin sheds in tiny microscopic pieces, while ichthyosis skin sheds in visible pieces of considerable size.
Another characteristic of epidermolytic ichthyosis is frequent blistering. This can occur with repetitive motion or as a result of contact. Blisters are often caused by walking, standing, sitting, and contact with clothing, shoes, and any hard surfaces. Even contact with soft surfaces can cause blisters. Blisters often lead to an increased risk of infection, which can be a constant battle.
Ichthyosis affects the body’s ability to regulate body temperature. Think of sweat or perspiration as the skin’s natural way of lowering the body temperature by releasing hot moisture. Since people with ichthyosis do not sweat properly, all the heat gets trapped inside the body. Once we begin to get hot, we have difficulty cooling off. We become almost like a walking sauna. Since getting too hot can cause a heat stroke, ichthyosis patients must be very careful not to overheat. If we do get hot, we must have access to immediate and quick-acting methods of cooling.
Cause and treatment
Doctors tell me that my ichthyosis is a result of a genetic mutation and that there is no cure. It can be genetically inherited, but since there is no history of it in my family, a mutation makes the most sense. Since it is genetic, it can be passed to my future children through my genes. This is part of the reason I have chosen not to have children. (I’ll talk about that in chapter X). And just for the record, you can’t “catch” it from someone.
Because there is no cure for ichthyosis, the goal is to manage the symptoms. Treatment options include the application of a moisture barrier and topical ointments that decrease the thickness of the skin. Exfoliating and oral medications are often routine as well. Although I have never personally met anyone else with ichthyosis, I do have contact with some through social media. From what I can tell, we all have different methods for managing our symptoms. I will describe my own treatment efforts in the next section.
My Experience with Ichthyosis
Now that I have told you a general overview, I want to take some time to tell you about my ichthyosis. I have never met someone that has this disease so I am not sure how others experience it. From what I have read on social media, every case is different.
My mom first noticed something was wrong when her water broke and hundreds of pieces of skin were in it. I was born with a hard shell covering my body. My mom said it felt like tapping a turtle shell. Within a few days, this shell peeled off and left my skin completely raw. My skin was then bright red, raw, and oozing this clear plasma-like substance. (Sorry for the gross stuff—it won’t be the last time.) One doctor told my parents I would die if they took me home. Another said that it was fine to take me home. But after two weeks in the NICU, I came home with my parents and my two older sisters.
When I did come home from the hospital, my parents had no idea what to do. Neither did the doctors. It was such a rare disease that very little was known about it. My parents carried me on a pillow and laid me on a beanbag or a bouncy seat made of cloth. They were not able to hold me because any physical touch hurt my sores or ripped my skin. I wasn’t held while I ate or slept. A great deal of trial and error was involved. There were problems with mattresses, clothes, and even diapers.
My mom has asked me if I remember bath time, and thankfully, I do not. My skin was easily ripped, so typical childhood activities led to lots of deep, raw sores. I could bump a wall or a table with any part of my body and rip large areas of skin. When the water touched my open sores, I screamed in pain. My sisters would go outside and play during my bath time because they could not stand to hear me scream. They confess now that they thought my mom was torturing me. I am grateful that God did not let me remember.
However, I do remember when I was elementary school age and had to get in the bathtub with my socks or pants on because my sores were stuck to the fabric. Until my sores could build the first protective layer, they would ooze. This ooze would soil my socks or clothes. If the sore was in a place that was in constant contact with my clothing, it would stick to the fabric. Several times a month my skin would heal as part of whatever fabric I was wearing. When I got in the tub, I would take a deep breath and brace myself for the pain of the water hitting my sores. Removing the dry fabric would mean ripping off the skin completely, so by wetting the fabric, I stood a better chance of causing less damage. If I took it slow, I could separate skin and cloth, ripping off only about half the healing skin instead of all of it.
Bath time is always followed by immediate head-to-toe moisturizing. My daily routine continues to be showering and covering my body in oil once or twice a day.
Finding clothes that do not hurt my skin has been a big ordeal since day one. My clothes must be loose and soft. I am the person you see in the stores walking around touching everything. The softer the clothing, the better I like it. Cotton or fleece work best. My clothes are a size or two too big because anything tight will rip my skin. My pants must all have an elastic waist, but the elastic cannot be tight. Pants run the risk of ripping my skin around the waist or the seams. Shirts with cuffs on the wrists or around the neck can cause a problem. Socks and other undergarments are major difficulties as well. Although I long to keep up with the latest fashions, I am forced to do what works for me.
The trickiest issue related to clothing has always been shoes. Tennis shoes, boots, dress shoes, heels, and even flip-flops are all out of the question. I remember wearing footies and moccasins to church as a child and transitioning to sandals as I grew up. I have always wanted to fit in and wear what others were wearing. To this day, I am jealous of you ladies that can wear cool, colorful, sneakers or calf-high boots and leggings—I call it “shoe envy.”
I would like to give a shout out to the people that make Crocs because they are the only shoes I can wear right now. Since Crocs are designed to slip on and off, I can get them loose enough not to even touch my foot when I am standing. Let me go ahead and make this official: I would like to take credit for making Crocs cool again! I have Crocs that match almost any outfit and own about thirty pairs. I wear my light-up Christmas Crocs in December, my Tie-Dye Crocs during summer, and my Mickey Mouse Crocs at work. They make me happy. And yes, I buy goofy Jibbitz to decorate many of my Crocs. I have butterflies, flowers, Disney characters, and Alabama Crimson Tide Jibbitz. I get very excited about decorating my Crocs and get quite a bit of joy from doing so. I could talk about my Croc collection all day, so don’t get me started.
One thing I find ironic is that although my skin can be thick and scaly it also heals very quickly. My skin is easily torn or blistered but heals four or five times faster than normal skin. I call this rapid healing my superpower. If there was one cool thing about my disease, this would be it. I can go to bed with blisters and after nine to ten hours of sleep they will be gone. Sleep is critical though. If I don’t get enough sleep and don’t have a super-soft surface to sleep on, my superpower is diminished. Let me give you a couple of stories to illustrate.
One Christmas Eve when I was five, I fell backward over a trash bag in our kitchen, slicing the back of my leg on some broken glass that was in the bag. I ended up in the emergency room of our local children’s hospital, sitting on a towel and waiting for the doctor. There was another child there on that same night who was having some type of severe skin reaction. My dad told me that the doctor came into my room and began asking him and my mom all about my skin.
“What is it called? How long has she had it? What are the symptoms? What medications have you tried?”—question after question about my skin. My parents thought it was rather odd that he was asking so many questions, but they answered them patiently. Finally, the doctor looked at my parents and said, “It’s Christmas Eve. What in the hell do you want me to do about it?” Apparently, he saw my skin and assumed I was the skin reaction patient, an honest mistake. I imagine he felt rather foolish when they told him I was there to have my leg sewn up. We still laugh about that incident today. I have a Donald Duck doll that has the following words written on it: “Kathy got this and 7 stitches on Dec. 24, 1976.”
The craziest part of the story is that my dermatologist had to come to my house the following day (Christmas) and take the stitches out. It seems that in 1976 they did not have dissolvable stitches. In less than eighteen hours, my skin was healing over the stitches.
On another occasion, I woke my dog suddenly, and he jumped up, accidentally cutting my eye with his tooth. When we arrived at the hospital, they told us that I needed stitches but the doctor was busy so it would be a while. By the time the doctor did get to me, I didn’t need stitches anymore. It had healed.
Struggling to Stay Cool
As I mentioned earlier, heat is a huge problem for me. My parents installed a pool when I was small, and that allowed me to spend time outside in the summer. For a few years we had a giant heated dome over our pool, which allowed me to swim in winter. Not only does the pool act as a coolant in hot weather but it also helps replenish the moisture in my skin. However, my skin dries out quickly when I get out of the water. Whether it be the shower or the pool, I have to hurry and cover myself in some type of cold cream or oil to hold in the moisture, otherwise my skin gets tight and painful.
As a child, I constantly feared overheating, which caused me a great deal of anxiety. I remember thinking on school field trips, What if it is too hot, and no one will listen to me? What if I overheat while playing at school, and no one understands the seriousness of the situation? What if I find myself in a situation where I don’t have a way to cool off? It was always on my mind back then and has remained an ever-present issue as my ability to cope with heat has decreased with age.
I still have a pool today, and it allows me to go outside in the summer. Since my husband is in a wheelchair, most of the outside chores fall on my shoulders. If the temperature is too warm, I will have to take frequent dips in the pool to keep cool.
I have tried many things over the years to stay cool that haven’t quite worked out. I experimented with a cooling vest that holds something like ice packs against my skin, but for it to work, it had to be so snug it ripped my skin. Cooling towels and hats aren’t quite enough either. I have, however, found some adaptations that help. For example, long sleeve swim shirts are perfect for me—I wet a shirt, put it on, and mow the grass. I also carry a squirt bottle with me to wet my shirt and hair when I get hot. (If someone out there can please design a way to attach a squirt bottle to a lawn mower, I would appreciate it.)
Not too long ago, I went to Disney World with my family. We were faced with temperatures in the upper eighties. I pretty much stayed wet the entire trip. When I dried, I would go into the bathroom and rewet my swim shirt. There were a few times when my family had to pour ice water, which we kept with us in a cooler, on my head or down my back.
Hot cars are exceptionally dangerous for me, and running errands in the summer can be a problem. I get hot walking to the car, then I have to get into an even hotter car. Most of the time, the car doesn't have time to cool off before I get to my next destination, so my body heats up by the minute. I do have a remote start on my car so I can get the A/C going before I even go outside, but when the temperatures rises to the nineties, my husband must be my chauffeur. He drives and keeps the car cool, dropping me off and waiting for me at the door.
There have been times when I have had to put ice packs under my arms, on the back of my neck, or on my wrists. I have done this on airplanes, in restaurants, and of course, outside. If I am home, the pool is my best friend. If you have a pool, you know that sometimes even the pool gets too warm. In these cases, I end up with my head under a garden hose.
In sum, I can get warm, but if I do not have immediate access to a rapid cooling method, problems will occur. And once I overheat, I will find myself exhausted and wiped out for the rest of the day. I have learned to adapt, think ahead, and plan for potential emergencies. I manage just fine, but needless to say, winter is my favorite time of the year.
Blisters and Sores
Blisters are an everyday occurrence in my world. Sometimes they are caused by infection, but most of the time they are caused by friction. Remember that I mentioned my skin can be thick and scaly? At the same time, it is very brittle and easily ripped. This means that even a slight bump of a table or a counter will rip my skin. I love to work outside in my yard, but I will inevitably have many self-inflicted sores on different parts of my body. The more sores I have, the more likely I will get an infection. There are times when numerous scrapes on my arms and legs led to big problems.
To help prevent sores, I prefer to wear long pants and long sleeves. Of course, that isn’t possible all year round, but I try such things as keeping my house cool so that I can wear clothing to protect me. I cut my fingernails almost daily so I don’t scratch myself. My dogs are trained to keep their paws on the ground so they don’t hurt me. I often have people ask me if my dogs can shake hands. I explain to them that “shake” is a trick I will not teach my dogs because this teaches them to use their paws to communicate with humans. The last thing I need is a dog that scratches me and creates more sores. While I am a dog lover to the core, being around dogs who jump on me or paw at me can ruin my day.
With movement or friction, the outer layers of my skin will separate from my body and fill with liquid. If popped, there is raw skin underneath. Is it painful? Absolutely. Pain is a daily part of my life. My skin can get so sore as it nears the point of blistering that it hurts to move. It is a pain that is difficult for others to understand so I try to hide it as often as I can. Even after twenty years of marriage, my husband will hear me wince as I get up off the couch at night. Not too long ago, he asked, “Does it really hurt that bad?” Even after all this time, he still struggles to understand. I feel my best in the mornings and the worst in the evenings.
The pain is not something I talk about often because it is there all the time. If I mentioned it every time I hurt, it would be all I talked about. By the end of most days, just adjusting my body on the couch or turning over in bed can be excruciating. Sometimes it is an article of clothing that causes blisters. Other times they just happen without any reason. Truthfully, I could be completely naked all day and still get blisters.
As I mentioned earlier, my skin gets thicker around my joints and any repetitive motion can cause blisters. Changing gears in a manual transmission car or riding a bicycle rips the skin on the back of my knees. If I sit too long, I get blisters on my back or the back of my legs. If I stand or walk too long, I get blisters on my feet or my knees. A perfect day for me is a balance of sitting, standing, and lying on a soft surface. Granted, those perfect days don’t happen very often.
Cushions and memory foam mattresses are my friends. Hospital beds are my nemesis. Any time I have to be admitted to the hospital, I can expect to be in more than my usual pain. I have tried laying an air mattress over the hospital mattress with lots of blankets, but the longer I am there, the more pain I am in.
Then there are the times when I have blisters pop out all over my back or my arms for no apparent reason. If I have too many sores and too much infection for my body to fight, blisters will appear out of nowhere. My husband and I have watched as hundreds of blisters have appeared before our very eyes. I was recently in the hospital for ulcers, and the tops of my feet blistered like bubble wrap. I’m still not sure what that was about!
Car rides often cause problems for me because of the friction between my skin and the seat. I can help reduce some of this friction with blankets and pillows, but ultimately, there is only so much I can do. When going on a several-hour car ride, I can plan on being sore and possibly blistered by the time I arrive. On our trips to the beach, I often drive the last hour so that I can use the steering wheel to hold myself away from the seat. Even laying my arm on an armrest will cause blisters. Training seminars, conferences, and airplane rides are a challenge, as is anything fun.
My mother and I often have arguments over my desire to do things that I know are going to hurt. Sometimes it is out of necessity, such as when I have to pull weeds or mow the grass. Mom will often say, “Why do you do that to yourself?” to which I respond, “Who else is going to do it?” Because my husband is in a wheelchair, there are many things he cannot do, despite the fact that we have made our property as handicapped-friendly as possible. So, much to my mother’s chagrin, I often hurt myself doing work that must be done. And until educators earn significantly higher wages, I will continue to be doing the work myself.
But other times, I do things I know will hurt because I want and need to have fun. I roller skate knowing I will pay for it later. I snow ski and jet ski, even though there will be repercussions. My choice is either to stay inside and avoid doing things that are going to hurt or have some fun and take ibuprofen later. My poor mother aches for me during these times, but I want to live! I want to have some fun! (I would like to note here that my philosophy of living life on ibuprofen has led to a few stomach ulcers, so I have had to switch to acetaminophen. Lesson learned.)
Living in a Snow Globe
Have you watched the TV commercials for dandruff shampoo where people complain about embarrassing flakes? Close your eyes and imagine having dandruff over your entire body. Now imagine that the flakes are much, much bigger, hundreds of them coming off all the time.
Let that sink in for a moment.
Flakes are coming out of your hair, your ears, your face, your back, your legs, your hands—everywhere. In my family, we call those flakes snow. Hey, why not use a little humor to cope, right?
Snow is a part of my daily existence. It is everywhere I go. I peel all the time. My skin falls off—constantly. I tell people that I live in a snow globe. Every time I walk or move or stand or sit, it snows. Every time I take off a sock or a shirt, there is a blizzard on the floor. When I was younger, my sisters would come up to me, shake my clothes, and sing “Let it Snow! Let it Snow! Let it Snow!” I can wave my arm while I’m teaching, and snow will fall out on the floor. The other day I went bowling, and a decent-sized dusting of snow was on the ground where I had spent an hour standing.
I buy all my clothes in light colors, especially my shirts, in an attempt to hide the flakes. If people with dandruff struggle with flakes on a black shirt, imagine my experience with dandruff on steroids! It sounds odd, but I long to wear black or navy or red tops. Yet if I did, I would spend the entire day brushing off snow every few minutes.
Snow falls constantly out of my hair, out of my sleeves, and out of my pant legs! I was horrified when my husband and I bought a house that had just been carpeted with wall-to-wall dark green carpet. Ugh! Thankfully, we have since transitioned to a more neutral-tone carpet. However, vacuuming and sweeping are still a frequent occurrence at my house. I am self-conscious when I go to someone’s house and they have dark-colored carpet or a black couch. No matter how hard I try, snow happens.
One time I went to a lunch meeting with the principals in my school district, and the tablecloths were black. I texted a friend and told her my situation, and she responded, “Don’t move! Be very, very still!” On another occasion, I went to a conference where every chair and table and tablecloth was—you guessed it—black. I guess it is a good thing I am not prone to criminal activity, because I leave my DNA everywhere.
It is an instinct for me to turn around and wipe off the seat when I stand up because I know I have left evidence that I was there. And you should see my car—I have to vacuum it out every other day. People will often say, “Don’t worry about it. It’s no big deal.” But to me, it is a huge deal. Even in my mother’s house, I get the broom and sweep up a snowdrift. I mean, no one else I know leaves a snowstorm everywhere they go.
Are there any compulsive peelers out there? I know people who love to peel sunburns. You can’t help yourself. It feels good when it is being pulled off, and it’s kind of fun. I’m not alone in that, right? Imagine that, somewhere on your body, large parts of your skin are peeling all the time. I admit that I do peel my skin often, but I try not to do it in public. It can be hard to resist. Occasionally I’ve been able to peel pieces of skin as long as six inches. Cool, but a little gross.
At times, this peeling causes lots of pain. A few times a year my skin will peel from head to toe. It gets very tight and painful, then it begins to crack all over and come up along the edges of the scales. I imagine a snake might feel like this when its skin molts. I often feel very overwhelmed by the sensation of tight, dry, itchy skin during these times. It is hard not to be cranky because of the pain. The most annoying part of this process is when the palms of my hands peel. It usually starts in a couple of places and proceeds across my entire palm and between my fingers. Talk about a weird sensation!
For the fun of it, I want to share with you a few other oddities about being me. Some of them I consider to be blessings. Others I see as tolerable annoyances. For instance, I cannot shave my legs or any other part of my body. Can you imagine what running a razor up my calf would do to my leg? I tried it once with an electric razor, and it took hours. Not only that, but the process of the hair growing back was itchy—and I don’t need any more itching. If you are around me in the summertime, you likely won’t even notice hair on my legs. Since I have only tried to shave once in my life, the hair on my legs is so soft and light-colored that you can’t see it. Think of the time I save not having to shave my legs.
Makeup is another thing I don’t have to spend my time on. Because my skin overturns so quickly, any blush or eyeshadow I put on is gone in a couple of hours. “Concealer” is a joke for me because I would need enough to cover my entire body, and eyeliner doesn’t stay put. The last time I wore makeup was on my wedding day.
I can paint my nails, but it, too, is a waste of time. Within less than a day, it is peeling off. I have even tried the new stick-on fingernails with all the latest technologies. Once I spent hours with my sisters getting cute Mickey Mouse nails before a trip to Disney World. They peeled off before I even got into the park the next day. Besides, I have to keep my nails extremely short so that I have less of an opportunity to scratch myself in my sleep.
I remind my husband how much money he saves because I cannot wear jewelry. Rings are either too loose to stay on my finger or so snug that they rip my skin. Necklaces bother the skin on my neck. I tried getting my ears pierced as a teenager, but this was not successful. If I took the earrings out for more than a couple of hours, the holes grew together. If I left them in all the time, they got infected. I have recently tried earrings again and have been able to find a balance that works. Nothing makes me happier than to wear my dog or Mickey Mouse earrings!
Everyone has told me I should never attempt to get a tattoo because of the potential for infection. But one day I got so desperate to do something others can do, I went to the tattoo parlor. I selected a small Mickey Mouse tattoo for my chest. And let me tell you, it hurt! It felt like someone was ripping my skin at a slow, excruciating pace. When the gentleman said that he didn’t think it was working, I came flying up out of the chair. It was not worth it. After all that pain, it looks like someone drew on my body with a pen that didn’t work very well. My mom says it serves me right.
My God will meet all your needs according to the riches of his glory in Christ Jesus.
– Philippians 4:19
When I was born, doctors were not sure what I had, much less knew how to treat it. When I was around four years of age, my parents took me to Emory University and National Institute of Health (NIH) in Maryland. Neither of these hospitals were willing to make any recommendations. I have a brief memory of sitting alone in a room on a metal table at NIH. Several doctors in white coats came in and began holding out my hands, turning my arms, looking at my back. I am not sure where my parents were, but I remember feeling scared. The doctors didn’t speak to me or interact with me in any way. They just looked at my skin as if I were a specimen and then left.
There were lots of doctor’s visits, antibiotics, and experimental treatments. Infections occurred often, and I got pretty good at knowing when I needed to seek help from my doctor. My doctors became very familiar with me and listened to what I told them about my body. Even now, my dermatologist will take my phone calls when I am out of town or see me on a moment's notice. He knows that I do not ask for help unless it is an emergency.
My mom tells a story of a time when I was a baby and woke up one morning with infected blisters all over my back. She scooped me up and took me straight to my pediatrician’s office. She was the first one in the door that morning, but the doctor had not yet arrived. The receptionist explained to my mom that the doctor did not have any openings and that I would not be able to see him. Mom went around to the back of the building and waited until he pulled up in his car. He got out of his car, took us straight in the back door, and we never saw that receptionist there again.
Trial and Error
Doctors told us that I might outgrow my disease at puberty or it might be a lifelong thing. But truthfully, they were just guessing. To this day, people with ichthyosis know more about their disease than their doctors do. There are so many different types of the disease, and no two cases seem to be completely alike. Fortunately, my doctors listened. They wondered if it was a vitamin E deficiency and told me to eat a lot of eggs. I can remember eating three scrambled eggs for breakfast for several of my elementary years. You would think that I hate them now, but they are still one of my favorites. Then doctors conjectured I had a vitamin D deficiency, so they had me stand in a UV lightbox (a ’70s version of a tanning bed). All that did was make me hot and scared. I had to stand naked in a room the size of a phone booth with hot lights on three sides, while my mom talked to me through the door.
There is no known cure for ichthyosis, but my doctor left no stone unturned. Every time I left my doctor’s office, I was given with a bag of different creams and ointments to try. None of them worked though. My mom had several people tell her that if she had more faith I would be cured. One lady even followed her into the bathroom to tell her that my disease was a result of her sin. Seriously? I have grown weary of people who suggest to me that whatever brand of supplements they are selling is the answer to my problems. I know that they mean well, but I believe if the answer was that simple, my doctor would have found it by now.
My skin itches constantly, and I am unable to control my scratching when I sleep. My parents would make me wear soft gloves at night so I couldn’t scratch myself. That didn’t work because they would tape them on and I just took them off. To try and hold in the moisture, my parents even coated me with oil then wrapped me in plastic wrap at night. That didn’t work either—I took that off too. I began taking an anti-itching medication to help me control my actions when I sleep. This is still a critical part of my nightly routine. Without it, I am likely to make hand size sores on myself when I sleep.
The scented lotions of today are a waste of time when it comes to keeping my skin moisturized., All they really do is give me a good scent for about thirty minutes, while the moistening effects last only about five minutes. Thicker oils, petroleum jelly, or cold cream work the best. I make my own lotion with a combination of these things. When others use it, their reaction is usually “It’s greasy,” but it’s exactly what I need. When dry skin gets too dry, it can crack and become very painful. So, the more moisture I can add, the better I feel.
Managing my pain and itching has been a constant struggle. I don’t like prescription pain medication, so I rely heavily on over-the-counter pain relievers. But managing the severity of the infection requires a hierarchy of medications I can run through, ramping up my strategy to match the severity. First is Neosporin. A tube of Neosporin, more specifically the kind that has the numbing agent in it, is somewhere on my person at all times. My next option is Betadine. You may recognize it as the red iodine-looking stuff they use to disinfect an area before surgery. It can be helpful to dry out oozing or infected sores. And my last-ditch effort to avoid antibiotics is a powder called Domeboro, which is a medication used to draw out infection in burn patients. It requires mixing a powder with warm water and drizzling the liquid onto the cloth over my sore. It never fails that infections occur in places that I cannot reach. My mom and now my husband often have to step up for me. I worry about what I will do when they are not around.
Covering my sores has always been a problem. The trouble with bandages is that they usually will not stick at all because of the oils I put on my skin. And if they do stick to my skin, they pull off my skin when they are removed. Band-Aids only work on my fingers, where they can wrap around and stick to themselves. If I ever have to get an EKG or the doctor wants me to wear a heart monitor, I put up quite a bit of protest. When those round sticky things come off, so does my skin. I try to slowly remove them so as little skin as possible is ripped, but it is inevitable. One time I had a nurse come into my hospital room, grab all the wires at once, and yank them off. I wasn’t very happy, and I might have punched her if I could have reached her. And if I ever have an IV, I get the nurses to let me remove the sticky stuff that holds it in place.
My point is that my wounds are most often open and uncovered. Many times I have had to miss work because I had a sore in a location that would not allow me to wear clothes. I get very angry when my skin gets in the way of my daily life.
Medication and Side Effects
At the beginning of my school-age years, my parents were told about a new medication. It was being developed but wasn’t approved in the United States yet. Somehow, they were able to get it from Europe. I will never forget the day it arrived at my house. By this point, my dermatologist was my dad's best friend. He came over to my house with this giant wrapped present. My parents helped me open it to find another wrapped box and another wrapped box and another. I don’t remember how many boxes there were, but I do remember that everyone was watching me and laughing. At some point, I got so overwhelmed that I started crying and wouldn’t open any more boxes. I am sure I was expecting some type of present and didn’t consider medication to be a present.
This medication did improve my quality of life some. But though it made the thick scales lessen, it also made my skin thinner—an improvement in appearance but more blisters and potential for injuries. The risks were considerable. My doctor was most concerned about the potential for kidney and liver damage. There was a danger of extra bone growth in the form of bone spurs as well. I went every six months for bloodwork and every year for x-rays. Because of ichthyosis, my veins seem to be very hard to find. To this day, any time I get bloodwork done or need an IV, I say lots of prayers. Frequently, the emergency rooms send for an ultrasound machine to help find a vein.
Although my kidney and liver remain unaffected by the medication, I do have trouble with my cholesterol and triglycerides. But I manage these issues with diet and another medication. The medication for my skin is believed to be so hard on my body that my doctor took me off it during the summer of my sixteenth year. He said it would “give my body a break.” I remember having to take pain medication and antidepressant medications in its place. I developed hard, painful welts and had lots of infections. The depression that came with returning to my pre-medicine skin was difficult. It was not my favorite summer.
The most critical side effect was the high potential (25 percent) for severe birth defects in my future children that included heart defects, eye/ear/facial abnormalities, cleft palate, heart defects, hydrocephalus, unusually small head, intellectual disabilities, and central nervous system malformations. The potential problems are considered so severe that anyone on the medication is required to be on two types of birth control. This, of course, did not apply to me because I was a child. I remember my parents talking to me about the possible birth defects and the strong possibility that I would not be able to have children. Not that I couldn’t, but that I shouldn’t. I was a child and didn’t care. None of the dangers or side effects mattered. If it made my life better, I was ready to try it.
The medication was approved in the US within a few years and was replaced with a slightly less harmful medication a few years later. I am still on this medication today, and I am tested regularly for issues with my kidneys, liver, and bones. As I approach age fifty, I am blessed to say that I have been free of any serious side effects. I do have some annoying minor side effects such as chapped lips, fingertips that peel, dry eyes, and curly hair. They told me that the medication might make my hair fall out, so I will not complain about the curls. Curly hair is better than no hair. And compared to life before the medication, it is well worth it.
When I talk about this medication on social media, I get quite a bit of angry feedback from other ichthyosis patients who have tried it. Many are very passionate when they warn me about how much it has ruined their life with horrible side effects. They describe lots of pain related to skeletal complications and other issues. I am thankful for my experience thus far and continue to take it knowing the risk for potential problems. When I informed my doctor that I would physically fight him or take him to court if he threatened to take me off my medication again, he just chuckles. I’m pretty sure he thinks I’m kidding. It is because of this medication that I can live a semi-normal life, and I cannot imagine life without it.
I hope I have done a decent job of explaining the physical challenges of my disease. Now I want to share with you some stories about how it has affected me socially. As you might imagine, I was aware of my differences, and my differences were noticeable to others. God created us as social beings, and I had a disease that others did not understand. It is because of my social struggles that I developed some very skewed views of myself. But these struggles are the reason my victory is so powerful.