Aria’s Care Routine

Aria has a bath once every day, she is very sensitive to exfoliation, and so Erica only scrubs lightly with her hands or with a washcloth to try to remove the thick skin. To keep Aria’s skin more comfortable, she must apply lotion all over her body twice every day. Whilst Vaseline can feel too hot, and increase the risk of infection when applied by itself, when Erica has tried to remove it from the care routine, Aria’s skin became too dry, and she suffered more tears. So, to get the necessary vaseline into the skin, whilst helping the skin to breathe, Erica uses a mixture of Vaseline, Eucerin advanced repair lotion, in addition to a lotion Erica makes herself (Ingredients: vegetable glycerin, cocoa butter, shea butter, and jojaba oil). The lotion must be warmed before being applied, because Aria is sensitive to the feel of lotion when it’s cold.

To manage wounds, Erica applies Antibiotic ointment (Mupiricon) for infected wounds, or vaseline for wounds with no signs of local infection. Wounds are covered with Mepilex dressings, and these are held in place with Tubifast bandage.

To try to control the thick skin on the joints and flexures which helps to maintain joint flexibility, Erica uses topical keratolytics once or twice a week, including Tretinoin, Tazorac, or a pharmacy-made compound of 5% urea and N-acetylcysteine. To try to manage the thick skin on Aria’s palms and soles (PPK), Erica must trim any loose segments of skin with sterile scissors every day, in addition to using a hard foot file 3 to 4 days each week on hands and feet. Tazorac must also be applied to hands and feet twice per week to try to reduce the rate of hyperkeratosis on the palms and soles.

To manage scales on the scalp, Erica treats this once or twice a week with a leave-in conditioner mixed with tretinoin. To apply this properly to Aria’s scalp, it takes 1 hour because Erica must divide the hair into sections and massage the mixture well into the scales. The mixture is left on overnight, and is washed off in the morning bath. Both when applying the mixture and after the overnight soak, Erica goes through Aria’s hair with a lice comb to try to lift any loose scales away from the scalp.

Like many other children affected with KRT1 EI, Aria needs special care for her ears in order to prevent the build-up of thick skin which would otherwise cause hearing impairment in addition to increasing her risk of painful ear infections. Erica uses a special light-guided scope to remove thick skin, but also to prevent lotion from building up inside the ear.

Erica also buys a specific brand of underwear for Aria to wear because she discovered that the vaseline and lotions she uses seem to dissolve elastic! At one point she was having to buy new underwear every week because the elastic was degrading so quickly. She now spends $10 a pair for underwear, but has found buying elastic-free underwear designed for kids with sensory issues (Smart kids brand) are able to withstand the vaseline and lotions for much longer.

Like many other children affected with EI, Aria is very prone to blisters caused by seams, edges, and tags. Socks and shoes that don’t cause blisters are always hard to find. Erica typically buys socks one size too big, sometimes snips the top when the elastic feels tight, and turns the sock inside-out to reduce the impact of seams on toes and ankles.

Aria’s care routine takes 2.5 to 3 hours every day.