How fourth-grader copes with rare genetic disorder

Other than being small for his age, Cole Spiegel looks and acts like most any other fourth-grader at Avery Coonley School in Downers Grove.

But this young boy's journey through life so far has left his parents, Dan and Beth Spiegel of Wheaton, and grandparents, Duane and Judy Buttell of St. Charles, worried, yet vigilant, about his future.

Cole suffers from basal cell carcinoma nevus syndrome, or BCCNS, a rare genetic disorder that affects multiple systems in the body and manifests itself by making its victims more vulnerable to various forms of cancer, especially basal cell carcinoma skin cancer.

Cole, at 10 years old, has had 139 basal cell carcinomas removed from his body already and has undergone various surgeries in a short period of time. But it wasn't until recently that his family was told exactly what was going on inside of his body.

"Cole's infant skull abnormalities, his skin cancer, vision problems, low muscle tone and low growth hormone are all related to BCCNS," said Duane Buttell, father of Beth Spiegel.

The family is concerned that four of five persons with BCCNS develop aggressive jaw tumors that often require removal of permanent teeth and jawbone, Buttell added.

There was concern that Cole might have his first tumor in the jaw, but in what the family is calling a "small miracle" at this time, after a preliminary image showed a tumor, a second imaging showed it had disappeared.

"We want to call it a miracle because we've been praying a lot for this little boy, but from a medical and technology standpoint, it is also that maybe the second scan was more complete and it showed no tumor," Buttell acknowledged.

Still, it all adds up to quite a struggle for the young boy, as well as his family and friends. They've come to understand that Cole will be in the hospital having skin cancer lesions removed every three to six months.

"Our family is close," Buttell said. "There are no secrets, and everyone is pulling for Cole. His parents are working hard for Cole and they, fortunately, have good insurance, but they are incurring a lot of out-of-pocket expenses."

As for Cole, in most ways he's just a kid who loves school subjects, especially math - as it relates to athletes and their statistics.

He is a high achiever in school and loves sports statistics and geography, and also plays chess and takes French as a foreign language.

Cole's father knows the drill at this point - but his amazement with his son never stops.

"Despite Cole's condition and prior surgeries, he takes life by the horns with such positive spirit and he never questions, 'why me?'" Dan Spiegel said. "From his own observations at hospitals and his parents' reinforcement, he knows other children face far graver challenges than he and, despite his own hardships, he feels that it is he who is the fortunate one."

BCCNS is a rare and fairly new challenge on the medical landscape. But the fight has begun in earnest and the family is sharing the information for those who would want to donate to this challenge.

Donations can be sent to BCCNS Life Support Network, P.O. Box 1322, Kulpsville, PA, 19443.

Right at home:

In his role as president and CEO of the Arcada Theatre, Ron Onesti has taken on many speaking or master of ceremonies engagements over the past decade.

And he'll feel somewhat close to home in taking on that role at TriCity Family Services' 29th annual fundraiser, the Mardi Gras Gala, on Feb. 24 in the Pheasant Run ballroom in St. Charles.

He'll feel at home in part because he's done quite a few events for charitable or service-oriented agencies, but also because he's been spending a little more time at Pheasant Run than in past years.

Last year, Onesti reopened the small theater at Pheasant Run for shows, concerts and special presentations. It had been dark since Fox Valley Repertory closed the year before.

After hearing from those who have attended shows there since Onesti took over, there is no doubt he's got a good thing going. But like anything else competing for consumers' entertainment dollars, the place just needs more people aware of it, and what a nice and intimate setting it is for music and theater.

The annual Golden Heart Award winners will be revealed at the TriCity Family Services event. Those honored this year will be Cris and Tom Anderson, the Shodeen Family Foundation, and Geneva Community Chest.

Arcada's media contact:

Someone really made it easy to keep tabs on the hectic world that is the Arcada Theatre in downtown St. Charles. That was Romi Herron, who operated as Ron Onesti's voice piece and information organizer in her role as a public relations specialist and media contact for the past decade.

And we use the term "hectic" in a good way here, as the theater and Onesti's other operations made for a public relations gold mine - always something to promote, always something to tout, always something new to introduce.

Herron is diving into other projects and other writing worlds, somewhat going back to her roots. Alison Tovato takes over as the media relations coordinator for Onesti Entertainment Corp.

We wish the best for Romi, who was really good at her job, and welcome Alison to her exciting new position.

Downside of 65:

And here I thought that reaching the age of 65 in the coming year would bring with it all sorts of bad signs - slowing down, losing my memory, losing my hair, facing health issues, etc.

But it turns out that it simply means you will be deluged with calls about Medicare supplemental insurance policies.

At last count, it was at least three calls a day. Granted, it's an important topic, but I am still working, so I don't need 20 or more companies seeking my autograph on a policy at the moment.

While on the topic on nonstop calls, have you seen the latest phone scam? My caller ID said "Delnor Community Hospital" and the call was about consolidating my credit card debt, of which I have none, into some super-duper account that is supposed to save my finances.

It had nothing to do with Delnor, but everything to do with being annoying.

By the way, this is happening on both my mobile phone and my landline. Remember the Do Not Call list? I do, as well. Too bad it didn't work.

dheun@sbcglobal.net