When people first learn that Anna has XP, we are often asked the same questions about the rare genetic condition. It’s usually the first time people have heard about it, so naturally there are questions. We’ve learned a lot about the condition, but we aren’t doctors – so please don’t take these answers as absolute truth. It’s just what we’ve come to understand so far.
Q. How did you find out she has XP?
Anna got a bad sunburn at a young age. Learn what happened next and how we confirmed the diagnosis in our first blog posts.
Q. What is that funny looking hat she’s wearing?
We’ll also hear comments like “Why is she dressed like a beekeeper?” When outside, Anna wears a cute hat that has a denim drape in the back to protect her neck, and UV-blocking plastic shield over her face and neck. We think the hat looks fantastic.
Q. Will she ever grow out of it?
No. XP is a genetic disorder, and there is no cure. Anna will need to be kept safe from the sun and UV rays for her entire life.
Q. Is she allergic to the sun?
No. An allergy is an overcompensation of the body’s immune system when exposed to a food, a drug, a bee sting, etc that the immune system treats as an invader. XP is a is a rare inherited disease that stops her DNA from repairing itself when exposed to sunlight or other UV radiation. Allergies have to do with the immune system. XP is about DNA repair.
Q. Will Anna have any neurological problems?
Since Anna has mutations in XP-D she is at an increased risk for the neurological issues with XP, however it is not possible to say if or when she would develop the issues. So far, the best indicator of risk for neurological issues developing is doing hearing tests. Anna’s last hearing tests went well. We are hoping that Anna will not develop these problems, but we will watch and be prepared.
Q. Is that the same condition the kids have in the movie “The Others?”
No. In that movie, the children are ghosts. Anna is alive and well. (Actually, I’m not sure if the movie ever states what disease the mother says the children have, other than a rare aversion to sunlight.)
Q. What does XP do to Anna?
Basically, when Anna’s skin is exposed to sunlight or other UV rays, DNA strands are damaged. XP means that her DNA is unable to repair itself from that damage. The damaged DNA builds up and can become cancer. For that reason, her skin must not be exposed to sunlight.
Q. What does XP stand for?
Xeroderma Pigmentosum. In ancient greek, it means dry, pigmented skin
Q. How many people have XP?
XP affects about one person in every 1.5 million people in the United States. There are about 250 people in the US. There are 3 families in the state of Utah where XP is found.
Q. How did she get it?
Because XP is a recessive genetic disease, both Anna’s mom and dad had to be carriers of the mutated gene. Her parents each carry one of the mutated genes, so they are not affected. Each future sibling that may coming along to join Anna has a 25% of having XP.
Q. Is she safe in the shade?
No. Shade is insufficient to block enough of the harmful UV radiation. This is especially true due to the bounce-back effect the sunlight can have in the areas outside of the shade.
Q. Do windows block the UV rays?
Most glass does not block the UV radiation. Our home and car windows have been treated with a special UV blocking film. Each window of our home and car has been treated with a UV-blocking film, thanks to the generosity of 3M, Glamour Glaze, and the XP Family Support Group. UV blocking plastic sheets are used to cover her stroller and windows at places such as Church and other family members’ homes.
Q. Is it really hard to live with?
It’s all about adjustments. Some days are harder to deal with than others. But with all of the help and support that we have, we are doing pretty well.
Q. does Anna have to wear her gear when she goes outside at nighttime? (Mia, 5/14)
No. We enjoy the warm summer nights after the sun goes down. Sweet freedom!
Q. If she is exposed to sunlight, will it cause external damage that can be seen on her skin? Or only cause internal damage that will lead to cancer? (Sara, 5/14)
Yes, external damage would be seen if she were exposed to sunlight. It’s the same skin cancer anyone would get later in life…but it happens thousands of times more quickly.
Q. Does her skin literally “melt” in the sun? (Sara, 5/6/14)
No. That was just a headline used by some papers when describing what skin cancer looks like after years of exposure.
Q. Cant you move to places that aren’t sunny to enable her to have more freedom outside? (Emma, 5/14)
Cloudy places still have way too much sunlight to play in the daytime. Clouds do not block the harmful UV. Besides, Anna enjoys light as much as anyone. We dont’ want to have her live in the dark all of the time. We’d rather protect her with her plastic shield, etc.
new! Q. What does she like to do for fun while she is inside all the time? (Trinity, 10/14)
Anna loves to climb on anything and everything – mostly her dad! She loves puzzles, dolls, and watching her favorite TV shows.