Have you ever heard of “Children of the Moon”? Behind this rather sweet name hides one very rare disease called Xeroderma Pigmentosumif carriers are children who absolutely must avoid any contact with UV rays, at risk of developing one skin cancer (a risk 4,000 times higher than in the general population). We take stock of this deadly disease, from its symptoms to the schemes of living with it.
Child of the Moon: what are the symptoms of the disease?
Described in 1870 by a Hungarian dermatologist, Dr. Moritz Kaposi, Xeroderma Pigmentosum (XP) is a genetic disease that affects children, both girls and boys (both parents are then healthy carriers of the gene). In Europe, the disease is estimated to affect 1 in a million births (against 1 in 100,000 in Japan, the Maghreb countries and the Middle East) and that between 70 and 80 patients live with it in France, according to the association Enfants de la Lune *.
Children of the Moon has very dry skin and presents one hypersensitivity to ultraviolet rays which, we recall, are constantly present at varying levels, whether the weather is good or not. Some artificial lights can also be a problem.
From the first months of life of the sick child, XP is manifested by the presence of severe redness resembling sunburn after minimal sun exposure, the presence of freckles on the face and neck, hypersensitivity to light (photophobia), which can lead to headaches, eye pain or dry eyes and damage to areas of the skin exposed to the sun. In healthy people, DNA is able to repair itself after sun exposure, but this mechanism is defective in lunar children.
This is why sun damage from the age of 4 can degenerate into skin cancer (mainly of the basal cell carcinoma type) and eye abnormalities can occur. Finally, neurological manifestations and developmental abnormalities may also occur, but they are less common.
Another version of the disease called XP variant (20 to 25% of patients) appears later, between 15 and 40 years, and has a slower progression.
How Long Do Moon Children Live?
The life expectancy of lunar children depends on whether they have been properly protected or not : constantly protected, some may have a “normal” life, go to school and imagine a future after 20, while most children with little or no protection do not go beyond adolescence. Patients with variant XP can expect to live a little longer, but this is highly dependent on the sun exposure received in childhood.
XP is an orphan disease: there is currently no effective treatment. Patients need to adopt a lifestyle where they minimize their exposure to daylight and go out after dark (hence the name Children of the Moon).
To live “normally”, patients must live in an environment that is completely cut off from UV rays, especially by installing UV filters on the windows of their homes and cars. A dosimeter measures real-time UV exposure. They must also wear protective clothing that covers the entire skin, including the head: the protective helmet in the form of a bubble reminiscent of an astronaut is part of the obligatory outfit when leaving home. Very high sun protection is required (never less than SPF50 +), in very large quantities.
ONE very regular medical follow-up (approximately every 3 months) at the dermatologist should be placed and the smallest suspicious lesion should be removed and if necessary covered by a graft of unexposed skin. Dermatological follow-up also aims to prevent vitamin D deficiency through regular intake of free. ONE psychological follow-up can be established for the patient and / or his family.
Good to know: The Disability Act of 11. February 2005 allows the reception of children with the disease in schools, via the installation of anti-UV filters on all windows in buildings, as well as the presence of a school life assistant to accompany the child on a daily basis, especially to help it check its equipment.
ACP: What is the role of the accompanying person for students with disabilities?
Where is the research on Xeroderma Pigmentosum?
In 2022, constant photo protection is still the only option against XP. But research continues (slowly as it is a rare disease …) and hope can come from gene therapy, either replacing the patient’s gene with a healthy gene or correcting the mutation present in patients. However, there is still a long way to go before we can claim victory: many steps, especially to determine the harmlessness of treatments and their effectiveness, must be carried out before considering testing them on humans.
* Source: enfantsdelalune.org