“The fear of what lay ahead”
Raising children with Erythropoietic Protoporphyria (EPP) is a traumatising experience, one which few parents are able to express or understand.
In EPP, exposure of skin to light, particularly light in the blue spectrum (400 nanometers wavelengths and beyond), causes deep burning, intense pain and scarring skin reactions. Parents helplessly witness the anguish of their children growing up in isolation.
Ralph and Wendy have raised two daughters with EPP. Their eldest daughter Angela experienced symptoms from birth but was not diagnosed until she was three years old. Without understanding the direct cause of her distress and without correct diagnosis, Ralph and Wendy witnessed their daughter’s distress for years.
As one of the most severe photodermatoses, EPP is generally misunderstood; a genetic disorder where severity of skin symptoms (phototoxicity) are not well known. EPP patients are absolutely light intolerant and know the risk of spending as little as five minutes outdoors.
Although their youngest daughter Lyndal was diagnosed with EPP at birth, she still had frequent intolerable reactions to sunlight. There is no effective medicinal treatment available for EPP patients, and to ask patients to avoid the light and sun life-long is an unfathomable task