Summer felt dry, but in comparison with winter, I'd pick the summer humidity any day. However, it was so unbearably hot last year that we felt like prisoners in the house since even just a few minutes outside would have caused Brenna to overheat. Since she can't sweat, she really can't tolerate hot temperatures for very long at all.
Back when Brenna was about 4 months old, I detailed our daily and weekly skin care regimen...and it's been really helpful for me to look back on and also to point other ichthyosis families to, with the information all in one place. We've changed up our routine just a bit, so here goes an update...
We're trying our hardest not to let Harlequin "hold us back" in whatever ways we can, but nothing we do can change the fact that every day is a never-ending battle against Brenna's skin. There is no "we're really busy tonight, let's skip bath time just for tonight." Her skin needs this daily, sometimes hourly care. Without moisturize, it dries out quickly and cracks, leaving areas that are prone to bacterial infections. Without a daily bath to exfoliate, skin just continues to produce and her body can't shed it quickly enough...
We've gotten quite accustomed to our one bath a day. Several times, I've had the opportunity to do a morning bath, but apparently Brenna's skin has adapted enough that there isn't a lot of extra skin ready to come off in just 12 hours, so I just let her soak then.
Our daily bath is intense though. We still wait until Evan gets home from work because it is notsomuch fun for me nor Brenna when I am left to my own devices during bathtime. Around 5:30, Brenna hits the water, and about 5:28, Brenna starts screaming, except on the rare occasion that she'll make it through the soaking before starting her protest. The girl will not tolerate her bath.
Who, me? I don't know what she's talking about...
I was worried for a while that it was really painful, but I have asked older children and adults with Harlequin about it, and everyone reassures me that as long as we let her soak and gently exfoliate, it's not painful. But obviously there is some discomfort (we're rubbing layers of skin off of her entire body after all), and at the least, it's really annoying. And some areas of her body are more sensitive than others, like the bottoms of her feet and her hands, and she really doesn't like her face being messed with either.
To exfoliate, we let her soak for a few minutes, and then begin rubbing with rougher washcloths (we're talking $4-for-a-pack-of-20-from-Walmart washcloths). We use Aquaphor baby wash...we've tried other kinds, but she seemed to look splotchier after with those. Aquaphor is just amazing in general, isn't it?
We used to kill our backs bent over in the big bath tub, then we tried the kitchen table, but that was a huge hassle, and then we found this novel idea of a raised tub, which has relieved our lower backs. We have quite the routine now, and it's probably funny to watch since we can do it all without saying a word - Evan starting with her face, while I tackle her legs...and then we move to her feet, each of us takes an arm and then we sit her up so that I can do her back and Evan takes on her head. He is the expert on the rough layers of her head and working around her hair...when I attempt that area, it's not pretty folks.
It can be tricky because while Brenna's skin is "thick," it's also sensitive and can tear easily in certain areas, especially where there are joints, like behind her knees, her groin and where her fingers connect to her hand. It's a delicate balance of removing the extra skin so it doesn't build up there, and being careful not to create a tear in her skin, or worsen fissures that are already present.
To kill any bad bacteria, we add 2 tablespoons of bleach to her baby bath about every three days; it's about the consistency of a swimming pool. If she's been out around a lot of people, we'll definitely make sure to do a bleach bath that night. We add the bleach toward the end of the bath so that it does its job but doesn't irritate her skin too much.
Our dermatologist also encouraged us to try adding 2 tablespoons of baking soda to her bath, and we have mixed feelings about this so far. It is supposed to make the water a good pH for the skin, but it makes her skin seem more "slimy" when we try to exfoliate. We're trying to get used to this.
Minimizing infection risk is the most important part of skin care - so we wear medical gloves to apply Aquaphor. We wash our hands or use hand sanitizer before touching Brenna or handling anything that touches her, like running her bath or preparing her bottles, to reduce the risk that bacteria from our skin will be transferred to her skin and cause an infection. I literally wash my hands so much that I rarely wear my wedding ring anymore because water gets trapped under it and irritates my skin on my finger. (I think I need to talk to my own dermatologist about this!)
Moisturizing for Brenna is so very important - probably secondary importance behind minimizing infection risk. During the winter, it is so dry, so we've been running the humidifier in Brenna's room almost constantly, and we've been applying Aquaphor over her entire body closer to every 2 hours...whereas in the summer, we can get away with every 3-4 hours.
We go through a 14 oz. jar of Aquaphor every 3-4 days, so thankfully the company that makes Aquaphor, Beiersdorf, has a donation program for people with skin conditions - though it doesn't completely cover Brenna's needs, it hugely helps offset the cost!
Nails: My absolutely least favorite part of Brenna's care. Her nails grow so quickly and so thick, and they sometimes fuse to the skin. It is a PAIN (both literally and figuratively) to trim her nails but something that must be done at least every few days, or else the nails will really fuse with the skin and also when she is particularly dry and has long nails, she has been known to scratch herself bloody while sleeping.
Ears: My absolutely most favorite part of Brenna's care. Harlequin affects the outer layer of skin, so the only "inner" area that builds up with skin is the ear canals. Brenna doesn't mind getting her ears cleaned out (when they are full of skin, they are really itchy) and since I'm really weird, I find it strangely fun to dig all of the dead skin out of her ear canals. Fun for me + comfort for Brenna = win-win situation in the ear department. I have my trusty tools: tweezers to lift out the pieces of skin from her outer ear folds, a scoop to dig out of her canal and a flashlight to help me see.
Oh how we love Aquaphor in our house. And I vary between seeing all of the greasy stains that seem to end up everywhere and wanting to cry...and just laughing about it. It's our life now, and it's not going to change, so we're getting better about just embracing the grease and the bleach stains :)
As for Brenna's clothes, she lives in cotton sleepers - they are the most comfortable and the fabric is breathable for temperature purposes, and they hold up best to the Aquaphor. And really, unless it's for a short time, she has to have outfits that "open" around the middle, whether they snap up, or a pants/shirt outfit that doesn't tuck in, so that we can plug her feeding tube in. And of course, we love us some matching hats - they look cute, keep the Aquaphor on Brenna, keep the Aquaphor off everything else and especially during the winter, keep Brenna from scratching her head like crazy. (In fact, wearing hats keeps her head so moist that I only apply Aquaphor to it three times a day, as compared to six applications for the rest of her body.) Once summer hits, I'm sure Brenna will be rocking her fuzzy head much more often.
So basically what I am trying to say is that I do a lot of laundry.
We soak Brenna's clothes in hot water and OxiClean first - usually just her sleepers and hats, which are coated with Aquaphor. I throw all her towels, burp cloths, etc. in with her clothes into the washer, but I've found if I pre-soak those, they'll become more heavy with Aquaphor than if I just wash them. If the load is particularly greasy, I run it through a second time, but that's rare, because I usually don't want to waste time with it :) I use free and clear detergent (any kind), and some OxiClean in the washer too. After a load of Brenna's clothes, the washer has some leftover Aquaphor remnants, so I run an empty load through to help remove that. (At this point, I'm wondering how long our washing machine will hold up to this kind of abuse...)
When Brenna wears an outfit once or twice, it's usually fine. But her everyday sleepers are more than ready for the garbage after she's outgrown them. I feel sad about having to pitch all that cuteness, but my consolation is that since she grows so slowly, she wears those clothes more than if they had been hand-me-downs between five different kids...
Over the summer, we slowly weaned Brenna off of her oral retinoid that she started taking when she was just about a week old (called acitretin, it was like accutane). It was great for helping her skin shed, but not so great for the liver. Thankfully now in this period of good health she is experiencing, she is only taking a zinc supplement since her zinc has been low and a daily multivitamin (PolyViSol with iron).
Nutrition plays a huge part in the health of children with Harlequin. Their bodies burn so many calories over-producing skin...and sometimes, as in Brenna's case, they just can't keep up. She currently consumes (through George, our trusty feeding pump) around 33 ounces of breastmilk a day with added formula so that it's 27 calories an ounce instead of 20. That is an insane amount for a baby her age, and she's still not even on the charts for height (but 3rd percentile for weight - holla!)
Another regular concern is temperature. People with Harlequin have trouble maintaining their body temperatures, and some tend to be on the warm side while others tend to be on the colder side. Brenna seems to get cold more easily than she overheats. We run a small space heater in her room at night during the winter, and we have to ensure that our house stays pretty constant all the time. Going outside is trickier, and I'm still learning a lot when it comes to adapting to the outside temperatures - I try to bring layers that we can add or remove as needed.
Anddddddd I think I'm done. Is anyone still reading??? This may or may not be even longer than Skin Care, Part 1. This girl definitely keeps us busy :) Hopefully this might provide some helpful information to others in the ichthyosis community (or any med students who are considering dermatology - ha!)
If any others affected by ichthyosis want to comment about parts of your skin care that differ from ours, I'd love to hear them!