Diana Ijames was six years old when she was diagnosed with what doctors told her was a rare blood disorder. The doctors told her that they didn't have a name for her condition, that very few people were known to have the disease. The only form of treatment they could suggest for Diana was that she stay out of the sun between the hours of 8am and 6pm. Diana did not find out that her condition is called Erythropoietic Protoporphyria (EPP) until she was 20 years old.
Diana had started having symptoms at age four. Her parents took her to the doctor for allergy testing, which revealed nothing, and then brought her back to the doctor again and again. Like many other EPP patients, Diana's symptoms are not obvious to anyone looking at her. She has the terrible burning, itching pain that is a hallmark of EPP, but she does not get the swelling or redness that often accompany these symptoms. A casual observer would not know that anything is wrong.
Diana recalls that on one family vacation at Lake of the Ozarks in Missouri, she suffered a terrible reaction to the sun from being outdoors all day. Having no idea what was wrong with her and with no means of relief, she started scratching at her skin, trying to soothe the itching and burning. Diana scratched so hard that she split open the skin on her arms. Trying to cool her burns, she started looking frantically for cold liquids she could pour on herself and eventually resorted to grabbing other vacationers' sodas out of their hands to pour them on herself.
Diana remembers her mother telling the doctor of Diana's behavior: "she goes nuts outside," which as any EPP patient knows is about the only possible reaction when stricken by terrible pain that is only relieved by time. The early symptoms of EPP can be especially heartbreaking because the disease usually comes on before age six, and many children with EPP are simply not old enough to understand or describe what is happening to them. The luckiest occurrence of Diana's young life with EPP was when her parents took her to a doctor who told her mother unequivocally that while he did not know what was wrong with her little girl, Diana was clearly not crazy, nor was she making her symptoms up.
Diana was initially pretty angry at the EPP diagnosis and at being advised to stay indoors. She had already suffered for years trying to live like a normal child and having these terrible reactions to the sun over and over again. Both of her parents tan deeply, which made her feel terribly out of place as a child who couldn't tolerate the sun. Diana's father worked as a roofer all his life, spending every day out of doors. But looking back, the family believes Diana must have inherited EPP from her father's mother, who could never stand the sun.
Diana still has flare-ups of EPP from time to time, and says the symptoms on her hands are always the worst. For the most part, Diana says she is able to prevent EPP symptoms from occurring by using sun protective clothing-including hats, jackets and gloves-from coolibar.com, and at one time she also used Lumitene faithfully. Lately she has stopped using Lumitene because where she lives in southern Missouri the summers last from March through September, so with the two months it took her to work up to a full dose of Lumitene each year she wound up taking the medication every day for most of the year and getting only a small amount of relief from the sun.
Now Diana uses tanning beds for five minutes at a time here and there and feels that this helps her acclimate to the sun. She remembers skipping her usual tanning regimen last year and suffering a terrible reaction. When she does have flare-ups there isn't much she can do other than wait it out. While she's waiting she turns the air conditioning in her house down to 65 degrees and uses cold compresses.
Diana and her husband live in Missouri with their child, who has no symptoms of EPP.