Who Am I?

KOURTNEY CHARLENE KUJAWA

I can do all things through Christ who strengthens me.
Phillipians 4:13

Recessive Dystrophic Epidermolysis Bullosa(EB) is a genetic skin disorder. There are 4 types of EB, ranging from mild to very severe. Kourtney's type is severe.

EB is characterized by blistering of the skin with only a slight amount of friction. Kourtney's EB also affects her mucous membranes such as the eyes (cornea), the mouth, esophagus and rectal area.

Kourtney's blisters are significant in the fact that her blisters cause scarring. Repetitive blistering and scarring of affected areas cause contractures of the hands, webbing of the toes and strictures (or narrowings) in her mouth and esophagus.

How does it affect Kourtney:

• Kourtney has dressings on all her extremities to protect her skin. The bandages are changed twice a day, taking 1 hour each time

• Kourtney's hands have developed contractures and her fingers have become fused together. She will require plastic surgery for the rest of her life to maintain proper hand function. The plastic surgery requires skin grafts from Kourtney's thigh or abdomen and placing them onto Kourtney's hands. Surgery is painful and intense.
• Kourtney has had a difficult time eating due to being tongue tied, and having a small mouth. People with EB have very high nutritional needs as it takes many calories to heal. In the past many children with EB died because of malnutrition. At 2 1/2 Kourtney ended up getting a feeding tube placed into her to help meet her daily calorie requirements. Kourtney can eat small amounts of food by mouth. We call her our social eater (chocolate is her favourite).
• Kourtney's esophagus is also prone to blistering. Sometimes she cannot even swallow her own saliva. Kourtney's airway is not challenged but swallowing is painful and uncomfortable.
• Kourtney's corneas have also had blisters on them. The cornea refracts the light coming into the eye, so when there are blisters on her cornea, her eyes become very sensitive to light. The pain is excruciating. We have been able to relieve the eye problems by using many lubricants in her eyes at night.
• Kourtney uses an electric wheel chair for long distances because she gets blisters on her feet in addition to lacking the energy to walk long distances.

What Kourtney can do:

• She can walk short distances and likes to run and play soccer with a beach ball or play volley ball with a balloon
• Loves to swim, the chlorine does not bother her; neither does the sun
• Can sing and dance; has been helping out with the worship band at church for 2 years
• Great sense of humour and ability to converse with adults
• Very intellectual and bright
• Has learned to knit
• Loves to do crafts
• Plays computer games and game boy

Despite the constant challenges, Kourtney remains a very positive and happy child.