Volunteering with the Children of the Night

Guatemala

Nick Galvin, Hart Crowser environmental scientist, is in Guatemala with his family to volunteer for the Xeroderma Pigmentosum Project.

A bumpy two hours from the nearest “town” of Santa Cruz Barillas, a padlocked chain stretches across the one-lane dirt road. The volunteer team’s diesel Land Cruiser rumbles to a halt. Another Guatemalan road toll. The chain falls and they continue on their way. The small village in the mountainous Huehuetenango department comes into view; the rusting corrugated-steel rooftops of the small village sprawl on the steep mountainsides. The scent of cardamom wafts through the air. Along with maize, cardamom is the local cash-crop.

The volunteer team consists of four. Milo and Dalila, who grew up there and have family in the area, plus Nick Galvin and his wife Bree. Dalila has worked as an un-trained nurse with these families for over fifteen years and Milo is the driver. They serve as translators from Spanish to Canjobal—the native Mayan dialect. Many there know only a few words of Spanish or none at all.

They park on a ridge between two deteriorating wooden school houses. The kids peer between the cracks to get a better look at the two extranjeros (foreigners). They don’t know it yet, but the road to this house will be impassable in two weeks from the incoming rains. From there they walk 15 minutes through hillsides of cardamom, meeting one of the program’s oldest children.

Cardamom fields

Bree en route through the cardamom fields.

At 16 years old, she is hardly a child. But after losing her eyesight and numerous surgeries to remove cancerous growths, she is completely dependent on her family for care. The disease has wracked her body and she suffers daily, but greets the team warmly. Her family’s house consists of two rooms: a smoky cooking area, where an open fire heats a listing metal cooktop surrounded by a couple of plastic chairs; and a sleeping area, where the entire family sleeps on uneven wooden-board beds. The floors are dirt and slope gently with the hillside. There is no electricity, toilet, or running water.

For a little over a month now, the team has been working with several families in this community suffering from Xeroderma pigmentosum or XP. XP is an unimaginably debilitating disease. Those suffering from XP are unable to repair damage from UV-radiation (i.e., the sun). This in turn leads to aggressive skin cancers and extreme photosensitivity, often at a very young age.

XP is an autosomal recessive genetic disease. Think back to freshman-year biology with Punnett Squares and Mendel’s Peas. A person suffering from the condition must have two affected alleles – one from each parent. The inheritance pattern is similar to red hair, blue eyes, or green peas, but with a much more drastic outcome. A person with only one affected allele (a portador, or carrier) can lead a normal life, with no adverse effects.

Punnett Square

Punnett Square showing regular distribution of “regular” (Y) and “affected” (y) alleles and outcome for a recessive trait – green peas. Photo courtesy of Quizlet

The majority of children in Santa Cruz Barillas are not well protected from sun exposure. Often, diagnosis occurs when the child begins to develop hyperpigmentation (darker spots) on the most exposed areas of their body; their face, neck, and arms. Many of these will become skin cancer. Shortly after, if still unprotected, the child will begin to lose his eyesight and exposure to bright light will become extremely painful.

Battling this horrific disease is beyond difficult. The only real “treatment” for those suffering from XP is an early-age diagnosis and vigilant sun protection—avoiding all exposure to sunlight. The only true way to achieve this is to stay indoors in a closed room during all daylight hours. This can have drastic psychological effects. Many houses don’t have electricity or artificial light sources, so this leads to a life sequestered inside, alone and in the dark.

In this village, generations have lived and intermarried, spreading the XP gene throughout the population. However, this is only one reason this disease is so rampant. Many still reject outside aid and chose to believe in archaic explanations; curses, spiritual intervention, or a divine form of punishment. Lack of education about the disease allows these ideas to perpetuate. Some families choose to hide (or worse, abandon) affected children. However, many do not and there is a growing contingent working to eradicate the disease.

Village center

Village center and an omnipresent Land Cruiser shuttle.

In reality, the only true method of prevention is selective reproduction. By knowing who the carriers in the community are, carriers can avoid marrying one another. This slowly dilutes and removes the affected gene from the population. Working with the community, this is a project that their organization has been heavily involved with. Through genetic testing, they have identified the families with the gene and carriers in the village. They have encouraged open communication between the carriers, families, and community, and provided scholarships for carriers to attend college outside of the area. This both elevates the family economically and provides them with an opportunity to find a partner outside of the village. They hope that this information, combined with genetic counselling and education, will allow the village to move closer to eradicating XP from their community.

Other ongoing projects focus on improving the health and quality of life of individuals living with XP. Many of the children have parasitic infections from questionable drinking water sources. Many don’t have sufficient clothing or shoes. All of the children are malnourished. A typical diet consists only of maize tortillas, beans, and the occasional vegetable or fruit. One project the team has spearheaded since they arrived is vegetable gardens. By providing the materials for gardens and classes in fertilizing, composting, and maintenance, they can provide access to a sustainable, healthier diet for the families and children.

Without a doubt, it has been an incredible experience. Staying in the village and working in the families’ homes is a sobering view into their lifestyles and the unimaginable conditions many have to endure. There is an imposing amount of work that could be done to improve the living conditions and quality of life for many of these families. For Nick and his family knowing that they were able to help—even just a small amount—is incredibly rewarding.

Due to the sensitive nature of this disease and the volunteers’ agreement with the community, they could not provide photos of the families or children.

More information about XP

Sources: Up-to-Date, Medscape

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