When you hear the acronym “XP”, you probably think about the Windows platform, but it also stands for xeroderma pigmentosum, which is a genetic disorder found in only one in every one million people in the world. There are plenty of unfortunate, cruel and inconvenient diseases and disorders plaguing humanity, but XP is certainly at the head of its class. Those who suffer from xeroderma pigmentosum don’t have the luxury of spending time in the sun or even under the deadly glare of fluorescent lights.
In most cases, symptoms of XP appear before a child has reached his or her third birthday, and they are often visible from infancy. An intolerance for sunlight is the first indication of xeroderma pigmentosum: Severe sunburns and blistering as well as an ocular aversion to light. Children with XP often have a greater concentration of freckles than other people and may even develop melanomas or skin cancer before they turn five. Neurological problems are also associated with this disorder, though ataxia and confusion usually follow several years after the physical symptoms of XP set in.
This is one of the many genetic disorders that scientists and doctors don’t fully understand. In people without XP, damage to our skin and eyes caused by ultra-violet light is repaired by enzymes released naturally. With xeroderma pigmentosum, those same enzymes are not excreted and cellular damage is irreparable. A severe sunburn can have drastic consequences, and people with XP are encouraged never to venture out into the sunlight. Their lives are expected to be led indoors and away from even artificial sources of UV light.
The worst part of this disorder is that UV damage to the skin and eyes is cumulative. This means that damaged cells are never repaired and that prolonged sun exposure is not the only problem. An hour-long excursion into the sun has the same effect as sixty separate minutes spent in direct light over the course of three years.
One of the major problems associated with xeroderma pigmentosum is medical care. Most XP patients must spend a great deal of time at the hospital and in doctor’s offices. The disorder itself requires monitoring, but is also a source of secondary diseases such as cancer and the aforementioned neurological disorders, all of which must be treated by qualified health care professionals. The issue, of course, is that hospitals and clinics are dangerous for patients with XP because they are well-lit by dangerous fluorescent lighting.
Not only will the patient be in danger just by visiting the hospital, but it is also likely that his or her eyes will not be able to tolerate the light. For this reason, special arrangements must be made with health care providers in order to ensure his or her safety. Surgery is obviously a serious problem because surgeons need sufficient light to work. The majority of the patient’s body will be covered during the procedure and light is never direct.
As you can probably imagine, living with xeroderma pigmentosum is quite difficult, but patients are lucky to live past their teens. Unfortunately, many children aren’t diagnosed with XP until they are four or five years old (sometimes as old as thirteen), which means that they haven’t limited their sun exposure. This increases their chances of developing skin cancers early on, which often leads to premature death. If, however, a patient with XP avoids light from infancy, life expectancy dramatically increases into the mid-forties.
Fortunately, a diagnosis of xeroderma pigmentosum doesn’t have to mean that life stops. XP isn’t an immediate death sentence and children (as well as adults) can learn how to cope with the disorder just like with any other affliction. The XP Society (XPS.org) is an excellent resource for parents of children with XP, and even offers a year-round retreat and social event called Camp Sundown. People with XP can learn to live on a different schedule and can often venture out into the world after dark as long as they don’t patronize well-lit establishments.
