It took only a little sunlight to launch a deadly disease in several children on the Navajo Nation.
Afflicted with a rare and fatal genetic condition called XP, or Xeroderma Pigmentosum, the children’s bodies couldn’t repair skin damage caused by the sun’s ultraviolet rays. Instead, sunlight made their bodies rebel. Their skin freckled, then came skin cancer and a slow neurological decline.
XP occurs in about one of every 250,000 children worldwide and as few as one in a million in the United States, according to several studies. But among a scattered group of communities in the Navajo Nation around Coyote Canyon, N.M., the incidence could be as high as one in 30,000, according to a new documentary, Sun Kissed, by filmmakers Maya Stark and Adi Lavy, which is showing Wednesday, Oct. 17, during the Santa Fe Independent Film Festival.
The next-highest XP incidence known to researchers is one in 40,000, seen among Japanese children.
Stark and Lavy, the film’s co-producers and directors, spent four years following the devastating impacts of XP on Dorey and Yolanda Nez, who had two children affected by the disease. Their film documents the couple’s struggle to understand why their son and daughter both were afflicted. Their heartrending journey means the couple must navigate some of their own tribe’s taboos and beliefs about disease, history and marriage. They talked to medicine men, elders, family and scientists. Along the way, they discovered many more children in the area where they lived also had XP.
Eventually, the Nezes’ search led the couple and the filmmakers to Fort Sumner, N.M., and a historian who told them about the Long Walk, a seminal event in Navajo culture, which elders are reluctant to talk about. The Long Walk was the forced march of 8,000 to 10,000 Navajos in 1864 by the U.S. military to Fort Sumner. Half died during the forced march or from disease and cold after arriving at the encampment. The Long Walk could have created a “genetic bottleneck” for XP, one geneticist tells the couple.
XP was the first DNA-repair disorder described by medical researchers decades ago. It is an autosomal recessive genetic disorder, which means both the mother and father must carry the XP gene for the disorder to show up in their children. The gene itself is rare, and so the chances of both parents carrying it is even smaller.
The Long Walk’s ultimate tragedy may have been in forcing together a small group of Navajos who likely had more than a couple of XP gene carriers. It increased the chances that over time, their descendants, also carriers, would meet and marry.
Dr. Alan Lehmann, a longtime XP researcher now semi-retired with the Genome Damage and Stability Centre in England, said early diagnosis and strict protection from sunlight remains the best options for managing XP along with quick removal of an skin cancer lesions that develop. “Some drugs are being tried, but [with] no definitive results yet,” he said.
Ultimately, most children with XP die before they are 20.
XP has been portrayed in dramatic films and books. Brad Pitt’s character in the 1988 film Dark Side of the Sun has XP, as does the main character in the 2008 Spanish film Eskalofrio (Spanish for Shiver) and in the Japanese film Midnight Sun. Dean Koontz writes about XP in his Moonlight Bay Trilogy, and Angela Johnson’s novel A Cool Moonlight is about a family’s efforts to help their 8-year-old daughter who has XP feel she has a normal life.
Sun Kissed is the first full-length documentary of the disease and its impacts.
Lavy, a photographer, met the Nez family on a taxi ride to a New York summer camp for children with XP and their families. Lavy had already spent two summers at Camp Sundown, documenting children with XP and learning about the disease. “My initial interest was personal. I had moved to New York from Israel, and after awhile, I found I was affected by the lack of sunlight. I started thinking about people who can’t live with sunlight at all.”
Yolanda and Dorey Nez had flown across country with their daughter, Leandra, to attend the camp. Their young son had already died from the condition. “Pretty much from the get-go, I was stunned by their story,” Lavy said.
The Nez family’s situation with XP had the additional layers of culture and history. Yolanda and Dorey were open to sharing their journey and story with the filmmakers. The result is a heartbreaking look at their day to day lives as they care for Leandra and search for answers.
Lavy and Stark filmed more than 350 hours of the Nez family and other Navajo families whose children have XP. Health professionals on the reservation seemed baffled early on by the condition; XP can be confused with many other illnesses and diseases early on. In addition, there are several mutations of the gene, which lead to varying symptoms. Other Navajos blamed things the couple had done in the past for what was happening to them now. “What my sense was these parents were being exposed to a lot of misinformation,” said Stark, who edited the film.
The filmmakers heard stories of parents who took their XP afflicted kids to the hospital, only to have health professionals accuse them of hurting their children and social services investigate them. “That’s why we feel this film is really important,” Stark said. “This puts a face on this disease. We know there are probably more undocumented cases on the reservation.”
To learn more about XP, see The XP Society, www.xps.org.
If You Go
What: Screening of the film Sun Kissed at the Santa Fe Independent Film Festival
When: Wednesday, Oct. 17, 7 p.m.
Where: Warehouse 21. Seating is limited to 240 people.
The film also will show on PBS as part of the award-winning Point of View (POV) series on Oct. 18 at 10 p.m. The trailer can be viewed at santafeindependent filmfestival.com.ContactStaci Matlock986email@example.comTwitter: @StaciMatlock