Meet Paetynn

Paetynn is 5 years old and has KRT10 EI without PPK. She is the youngest of 5 children in the family, and she is the first in the family to be affected with EI. They live in the US. Paetynn’s parents had a video made of their journey through the early years with Paetynn. The first two years are typically the hardest for new families with a baby with EI. The skin is extremely fragile and prone to blistering, making everyday life very challenging and often families feel very isolated. The video perfectly summarises the experiences of having a baby with EI, and I want to thank Paetynn’s parents for giving permission to share it here. It certainly brought tears to my eyes as it invokes all my memories of our early years too. A very powerful and moving video with such a great sound track “This is me”.

Paetynn’s Care Routine

Mum Aubrey applies Vaseline or Aquaphor ointment to Paetynn head to toe once daily, but she also needs lotion to be applied to some of the drier or more painful areas throughout the day. She has a special bath (Microsilk) that helps with tissue debridement and antimicrobial cleansing 4 times a week, but she sometimes needs this more frequently.

Twice per week Aubrey soaks Paetynn’s scalp with oil, leaves it to soak for 20 minutes, and then uses a fine lice comb to try to remove the loose scales. This is often painful, so is hard to do, and takes a long time. Any blisters need to be lanced (fluid removed using a sterile needle), and any wounds are bandaged to protect the raw skin.

Paetynn gets very thick and dark skin over her joints and flexures, and this can lead to stiffness and pain in the joints, which also limits her mobility. On extremely painful days, she must use a wheelchair, but tries her best to stay active. She loves the outdoors, but Mum says she often pays the price of her adventures, with long nights full of blisters and pain. Paetynn loves animals and chocolate! Often these are the motivators Mum uses to get through the more difficult days, and the more challenging aspects of their care routine.

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Meet Louie