Normal cells are usually able to fix DNA damage before it causes problems. However, in people with xeroderma pigmentosum, DNA damage is not repaired normally. As more abnormalities form in DNA, cells malfunction and
eventually become cancerous or die
.
Can you die from xeroderma pigmentosum?
Many patients with xeroderma pigmentosum die at an early age from skin cancers. However, if a person is diagnosed early, does not have severe neurological symptoms or has a mild variant, and takes all the precautionary measures to avoid exposure to UV light,
they may survive beyond middle age
.
What is the life expectancy of a person with xeroderma pigmentosum?
However, the life expectancy is shortened for many individuals with XPC due to the dramatically increased risk for cancers. The average life expectancy of an individual with any type of XP and no neurological features is
approximately 37 years
(29 years if neurological features are present).
Does XP affect the brain?
The XP-A patients developed
neurological and cognitive dysfunction
in childhood. The neurological disease advanced in an orderly fashion through its successive stages, finally affecting the whole nervous system and leading to death before the age of 40 years.
Is XP disease dangerous?
XP dramatically increases the risk of skin cancer
Because the body cannot protect itself from UV light, a person's risk of getting skin cancer skyrockets. It's estimated that XP can raise the risk of getting skin cancer 10,000-fold. Most children who have XP develop skin cancer before reaching 10 years of age.
Are you born with XP?
XP is
a genetic condition that people are born with
. This means that the risk of XP can be passed from generation to generation in a family. Mutations (alterations) in at least 8 different genes are known to play a role in XP.
Is XP disease curable?
There isn't a cure for XP
, but its symptoms can be managed. Staying out of the sun and avoiding other UV light sources is crucial. This means slathering up with sunscreen and covering up completely whenever going out of doors.
How old is the oldest person with XP?
Survival beyond the third decade of life is unusual. The authors present a
46-year-old
patient with proven xeroderma pigmentosum who manifests many characteristic features of this affliction and may be one of the oldest, if not the oldest, living survivor of this unusual disease.
Is XP from Midnight Sun Real?
EclipseRx commends the romantic drama Midnight Sun for bringing awareness to the rare skin condition of Xeroderma Pigmentosum. Xeroderma Pigmentosum or XP is a
genetic disorder
that inhibits the DNA's ability to repair damage afflicted by ultraviolet light.
Is the Midnight Sun a true story?
No,
‘Midnight Sun' is not based on a true story
. The movie is an adaptation of a Japanese film titled ‘Taiyō no Uta,' better known as ‘A Song to the Sun. … Directed by Scott Speer, the storyline of the American film closely follows the 2006 movie.
What are the neurological symptoms of XP?
- Short stature and failure to thrive (ie, cachectic dwarfism)
- Signs of premature aging.
- Progressive retinal atrophy.
- Cataracts.
- Hearing loss.
What are the symptoms of XP?
- Sunburn that does not heal after just a little bit of sun exposure.
- Blistering after just a little bit of sun exposure.
- Spider-like blood vessels under the skin.
- Patches of discolored skin that get worse, resembling severe aging.
- Crusting of the skin.
- Scaling of the skin.
- Oozing raw skin surface.
How is xeroderma treated?
Treatment of xeroderma is focused on
keeping the skin moist
: Frequency of bathing should decrease and tepid, rather than hot, water should be used. Skin moisturizers should be used frequently, particularly immediately after bathing, to decrease transepidermal water loss.
How do you get XP disease?
Xeroderma pigmentosum is
caused by mutations in genes that are involved in repairing damaged DNA
. DNA can be damaged by UV rays from the sun and by toxic chemicals such as those found in cigarette smoke. Normal cells are usually able to fix DNA damage before it causes problems.
How is XP inherited?
XP is an autosomal recessive genetic disorder. Recessive genetic disorders occur when an individual inherits
two non-working copies of a gene for the same trait
. One gene comes from the mother and the other from the father.
Why am I suddenly allergic to the sun?
Research shows that, over time,
repeated exposure to UV radiation can cause the body to develop an immune response to the sun
, similar to environmental pollen and hay fever. This is because sun exposure can produce changes in skin cells that the body's immune system may identify as being foreign, or abnormal antigens.
