Local family organizes walk to benefit PWS

News September 4, 2012 0 Comments

The McWilliams family (from left) Rebecca, Jackson, Kieran, Blair and Julian, have organized a fundraiser this weekend to raise money to fight Prader-Willi Syndrome, a disease with which Kieran was diagnosed when he was 10 days old.

by Joe Trinacria

Organizing a fundraiser is a difficult enough task without having three children to look after, but one local couple has plenty of experience in working together through trying times.

Becky and Blair McWilliams, 36 and 37, live in picturesque home on leafy Northwestern Avenue in Chestnut Hill with their three small sons. From the outside looking in, it would appear that the McWilliamses are like any other prototypical family. The kids play happily together and have fun with the family dog, Bailey. It would shock those to know that despite Becky and Blair’s optimistic outlook on life, the family is going through a time of serious uncertainty.

The couple’s middle son, Kieran, age 2 ½, was diagnosed with Prader-Willi Syndrome when he was 10 days old. PWS is a rare, non-inherited genetic disorder that occurs in one out of every 15,000 births.

The family started a local race to raise funds for PWS research and are planning the second run this September. The family hopes to raise $50,000 to fight the disease, which for many is unknown.

What is PWS? Babies experiencing Stage 1 PWS exhibit low muscle tone, a deficient sucking reflex, and a delay in developmental milestones. At Stage 2, children with PWS suffer from an insatiable appetite that leaves them unable to feel “full.”

In serious cases, this can require parents to lock refrigerators at all times. Kieran’s family said that kids can now be trained to avoid extreme measures, but it is certain, however, that Kieran and others will never be able to live independently while battling PWS.

“The beginning stage is almost like reverse,” Blair said. “The kids have no interest in food without parental intervention, but between ages two and six they begin to develop a severe appetite.”

“It’s hard as a parent,” Becky said. “We are just here waiting for the next phase and trying to prevent it. It’s like a ticking time bomb.”

As Kieran progresses to Stage 2, Becky and Blair are extremely aware that they need to enforce strict rules regarding food, which can sometimes be difficult on their oldest son, Jackson, age 5.

“No one eats outside of the kitchen,” Becky said. “And there is absolutely no snacking in the car. When Kieran’s meal is over, we make sure he knows it’s over.”

If worrying about their son’s future jump in appetite isn’t enough, there are some potential learning issues that may arise for Kieran when he reaches school age. It has been reported that those suffering from Prader-Willi Syndrome tend to have IQ’s that fall between the low normal and moderate intellectual disability range. Those children with PWS that do score a normal intelligence tend to always have learning disabilities as well.

Recently Kieran has been prescribed human growth hormone (HGH) to help combat the low muscle tone of Stage 1 PWS, and this has paid extreme dividends, according to Becky and Blair. He has been in intense physical therapy sessions for two years, and the doctors believe that the HGH can help both intellectually as well as physically.

Not all is bad news. Because he was diagnosed with PWS so early in life Kieran’s parents are optimistic.

“We are in a much different place with Kieran than we were a year ago,” Becky said. “He has been extraordinary in his progress, but we have seen some of the food issues begin to arise.”

“Knowing what’s going to happen gives you more time to prepare for it,” Blair said. “At age 2 we have a better sense of the disorder, but there are still a lot of open questions about what’s going to happen in the future.”

The McWilliams family has banded together in the face of adversity, and it has become a strong supporter of other area families who are also dealing with Prader-Willi Syndrome. Becky and Blair are sponsoring a local race, called One Small Step, which raises money for crucial PWS research.

The 2k walk will be held on Sept.8 in Ft. Washington State Park and is open to the public. There is free registration, but a $40 donation will get you a One Small Step T-shirt. The McWilliams’ have set a goal of raising $50,000 and encourage the local community to come out and support the cause. Visit http://onesmallstep.fpwr.org/dw/walking/location/238 for more information about the walk.

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