Meet Louie
Louie is a twin, and is soon to celebrate his 2nd Birthday! Louie is affected with KRT1 EI with PPK, but his twin brother is unaffected. The family are from Taiwan, and so they struggle with a very hot and humid climate in the summer. The heat and humidity causes skin to be more fragile and prone to blisters, but the air conditioning used to control the temperature can be very drying for the skin also, so they are always trying hard to get the balance right. Having twins is always a blessing, but parenting twins brings unique challenges. Having an added complication of EI fragility for one twin meant that Mum Patty and Dad Wang had to quickly find ways to adapt to being at home with one new born and being in the NICU with little Louie.
Louie’s Care Routine
Like most children with EI and PPK, Louie gets thick skin which can physically limit mobility, cause pain, and can catch or snag on clothing or other materials and cause tears. The beginning of the care routine for Louie includes trimming any loose or protruding bits of thick skin. Following this, Louie soaks in a bath for around 10 to 15 minutes. If his skin has wounds, shows signs of infection, or just seems particularly red and inflamed, he has a diluted bleach bath for 10 to 15 minutes to try to prevent or treat any skin infections.
Following his bath, Mum or Dad apply lotion all over Louie’s body and this must be re-applied 3 to 4 times daily. Any wounds are first managed with cornstarch to help dry them out, followed by antibiotic ointment, and zinc paste to reduce the risk of infection, and help the wound to dry up in a controlled manner. Dressings are often not used for wounds because they cause the wound to retain moisture, and when this is combined with heat, EI skin blisters. This means it is important to try to seal the edge of the wound to stop blisters from spreading.
In the image gallery above, it is quite clear that Louie is smaller in size and weight than his twin brother. Like many of those affected with EI, the excess production of skin cells, along with the demands on the body’s energy caused by skin inflammation and the constant need to fight infection, results in kids affected with EI suffering with malnutrition. Louie needs to supplement his diet to help maintain his weight.
The thick skin associated with EI, and in particular with PPK, can limit mobility. As shown in the image gallery, Louie gets much thicker skin on his palms and soles, but he also has thicker skin on the joints and flexures, all of which make it harder for Louie to move. Louie has also seen a physical therapist since birth to help Louie develop his muscles properly, and to help improve flexibility in general.