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Staying out of the sun

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Thursday, January 29, 2009

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The children in one Washington City neighborhood know it's almost bedtime when the Feltner kids come out to play.

That's because three of the five children in Todd and Jennifer Feltner's family have xeroderma pigmentosum, or XP, a rare inherited disease that causes extreme sensitivity to ultraviolet radiation.

"If the sun's out - clouds or not - they can't be outside," Todd says.

Even some indoor lighting can be dangerous for their three children with the disease, Paris, 10; Paxton, 5; and Payton, 3. The couple's oldest son, Parker, 13, does not have the disease. Neither does their youngest, Porter, who is 18 months old.

XP comes from a double recessive gene, meaning both parents have to have the gene. Even then the chance of their children getting the disease is only one in four. Yet three of the five Feltner children have XP. They only know of one other family in the nation with three XP children and they live in Florida.

The disease makes people with XP about 1,000 times more susceptible to skin cancer and significantly reduces the skin's ability to repair itself.

Obviously it has greatly altered how the entire family lives. Their home has tinted windows and extra blinds and curtains. Even the lighting inside is dim - often nothing more than 40-watt bulbs.

The children do not stay at home all the time. Those with XP have to wear special hoods and cover their bodies from head to toe when venturing out in sunlight. They also have to limit their time outside because the protective clothing is not 100 percent effective.

Their elementary school and church also have the tinted windows so the children are able to attend school and worship with everyone else. About 10 of their neighbors have also had their home windows tinted so the Feltner children are able to come over and play.

Paris, the oldest Feltner child with XP, says one of her friends has had the tinted windows for a few years. She likes knowing that it's safe for her to play there.

When they venture out of safe environments like their home, church and school, the family uses an ultraviolet meter. Todd says they do not allow their children to go anywhere without their protective clothing if the meter registers any ultraviolet light.

The meter has helped them determine restaurants where they can eat during the day and how early they are able to go outside after sunset. It even added 15 minutes to the time the children were able to go outside. It may not sound like much but it's a big deal to energetic children who just want to play.

Summertime can be especially difficult because it gets dark so late.

"Summer is hard," Jennifer says. "They sit at the window and say, 'Is it time?'"

The meter has also eliminated some places they once visited - places they thought were safe but actually had too much ultraviolet light.

One place they know is safe is their indoor "backyard." It's an 850-square-foot area with 16-foot high ceilings. There's a swing set and trampoline and plans for a basketball hoop since Paxton just began playing the sport.

"When I swing it feels like I'm outside," Paris says. "It's fun because we get to do what all the kids get to do except it's inside."

Jennifer says her daughter loves to swing. She's been swinging ever since they built the indoor playground with donated funds nearly six years ago.

After the sun is down, it's time to play outside. Paxton likes to ride his scooter and Paris rides her bike. Despite the often-late hours, many of the neighborhood kids will come out to play with them. Local parents will dim their headlights if they see the Feltner kids playing on the neighborhood streets.

"I think it's really nice that they turn (their headlights) off because they know they can hurt us," Paris says.

XP support

Because XP is so rare finding information on the disease can be a challenge. The Feltners say they know of only two other families in Utah that have someone with XP.

Despite the rarity of the disease, the family's late dermatologist, Dr. Lansing Ellsworth, took the initiative to research the disease and help them as much as he could. Unfortunately, Ellsworth was part of the medical team from Cedar City that was killed in a plane crash in 2008 in Moab.

Otherwise, much of what they know about XP has come from their own research and from networking with other families through a group they all started called the XP Family Support Group. The group's mission is to improve the quality of life for those with XP and other diagnosed ultraviolet light conditions.

"To find out your child can never be exposed to UV rays can be a big deal at first," Jennifer says.

The group provides support through a variety of efforts, including the chance to network with other families dealing with XP. The group also provides financial support to purchase protective equipment and to aid in medical research. Todd says the basic idea is to help their children live as normal as possible.

The group also sponsors children with XP so they can attend annual camps in a protected environment.

Paris recently attended a camp in Texas that hosted children with a variety of skin disorders. Jennifer says it was a great opportunity for her daughter, who will not be able to attend other types of camps.

"There were cabins and you got to know about everyone's disabilities," Paris says. "It was so fun. I met lots and lots of new friends."

Because there were children like her who could not participate in outside activities during the day the camp offered a variety of nighttime activities, including swimming, horseback riding and even a zip-line.

"It was really scary at night because you couldn't see," Paris says of the zip-line.

The Feltner family has also attended a variety of retreats with other XP families in places like Sacramento and Disneyland. These retreats allow the Feltner children to interact with others who face the same challenges as they do.

Following the retreat in Sacramento, Paxton reflected on his experience: "That was cool," he told his family. "Everyone in this town has XP."

The next retreat is scheduled for November at the University of Pittsburgh. It may not sound like an exciting destination but Jennifer says the university is leading the way with XP research.

The support group has also funded some international work, which included sending a medical team to visit a primitive tribe in Guatemala that has a high percentage of children with XP. Because many of the tribal members don't understand the disease, education about the condition can drastically improve their quality of life.

The medical team teaches the tribal members how to protect themselves better with sunscreen and even performs operations to remove tumors.

Yet because of their way of life, most of the tribal children will eventually die from skin cancers.

"We're just trying to extend their lives," Todd says.

Even though he doesn't have XP, the oldest Feltner child, Parker, is also getting into the international outreach with his Eagle Scout project.

For the project Parker plans to canvass his neighborhood for donations to help people with XP in Tanzania. The donations will go toward medication and instructional materials.

"I'm going to make a DVD showing them how to put sunscreen on and protect themselves," Parker says.

All the funding for the XP Family Support Group comes through donations and fundraisers. Jennifer is currently heading a local fundraiser selling a dozen Krispy Kreme Doughnuts for $10 with a goal to sell 1,500 dozen donuts.

There will also be a drawing at the end of April to raise funds for the support group. A Harley-Davidson motorcycle is the grand prize. Tickets are $20 each or six for $100.

To participate in these fundraisers or to donate to the XP Family Support Group call Jennifer at 680-1379. For more information visit www.xpfamilysupport.org.

Copyright ©2009 The Spectrum

Author: By Brian Passey
Source: The Spectrum
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2.0 from 7 votes
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