Tie's story

By Peter Davey, Tie's father.

My son, Tie, is 17 months old and was born with severe Recessive Dystrophic Epidermolysis Bullosa (EB). Because his skin doesn't stick together properly, it comes away or blisters badly when he knocks himself or his clothes rub. This really hurts - but when Tie is tired and rubs his eyes, this causes additional wounds around his eyes and on his nose.

Tie can get up to 40 blisters after rolling over in bed. After drinking he also gets blisters in his mouth or down his throat. When this happens, he struggles to eat or drink because it also affects all his internal linings. His blisters need to be constantly lanced by a needle then dressed to keep infection at bay.

When Tie has a lot of open wounds, he has a special bath. The water stings his wounds but morphine helps a little. Tie's dressings are changed after his bath about every three days and, although he tries to keep still for mum and me, the pain is often too much and he needs to fidget.

Only time will tell how EB will affect my son's life. Tie will probably need a wheelchair because walking will eventually damage his feet. It is also likely that Tie's fingers will web and deform from the age of three onwards. Many children with EB also need feeding tubes inserted into their tummy because the throat can become too narrow to eat as a consequence of blistering and scarring. Tie may also die young if he gets skin cancer at an early age or becomes weakened by infection.

Myself and Tie's mum, Claire, try and make his life as fun as possible and we cherish Tie's smile. We hope that one day it will be safe for us to give Tie a hug without worrying about what damage we may be doing to our son's skin and internal linings.

The local ITV news programme Wales This Week recently featured a news article about Tie which you can watch here.