It doesn`t satisfy her father, however. Jake DeMarco wants to go back and finish the education he started before he got sick, marry his girlfriend and be there to raise his daughter. The 20-year-old aspiring chef wants to play his guitar, go to concerts and see his friends, but for that he needs surgery.

The town resident and Lyman Hall High School graduate suffers from a rare genetic disorder known as Chiari malformation. His cerebellum and brain stem have been displaced and herniated a dangerous 22 millimeters into his neck. This puts pressure on his spinal cord, causing debilitating headaches, neck pain, numbness in the limbs, balance problems, visual disturbances and a host of other symptoms, according to the National Institutes of Health. DeMarco has almost all of these symptoms, in addition to body pain, extreme sensitivity to light and motion, and vomiting as often as a dozen times a day.

To control his symptoms, DeMarco must take 38 shots a day. For the pain, he wears a patch that dispenses 100 micrograms of Fentanyl, a narcotic painkiller normally given to terminal cancer patients. And even then the pain is never completely gone.

"On a scale of one to 10, it`s a matter of going from a seven to four or so," DeMarco said. "It`s never a zero. It`s always there."

Chiari malformation affects as few as one in 1,000 people. Symptoms may appear at different points in life and the condition is hard to diagnose. This is part of the reason many doctors are not familiar with it and even less familiar with the surgery to relieve pressure on the spinal cord, known as posterior fossa decompression surgery. While the surgery isn`t a cure, it has been shown to alleviate many of the symptoms, according to the National Institutes of Health.

DeMarco is enrolled in the state-financed HUSKY health insurance plan, which will cover the surgery if it is done at the University of Connecticut Health Center. But DeMarco`s grandmother and legal guardian has mounted a campaign to allow him to go to the Chiari Institute in Great Neck, N.Y., for surgery. The institute, founded in 2001, specializes in treating disorders of the junction of the brain and spinal cord and recently completed its 2,000th decompression surgery with one of the highest success rates in the nation.

"Even they say he should be there, and that he should have been there yesterday," JoAnn DeMarco said.

But the DeMarcos` insurance provider through HUSKY won`t cover surgery at the Chiari Institute. According to a May 12 letter from the Community Health Network of Connecticut, the UConn Health Center is "an equally safe and effective alternative that will meet your needs. An out-of-state second opinion is not medically necessary when this can be obtained in the State of Connecticut."

The Community Health Network declined to comment for this story, citing confidentiality laws.

The DeMarcos have been working with state Rep. Vincent Candelora, R-North Branford, to appeal the decision. Candelora said he was going to submit a letter to the state Department of Social Services requesting that it consider the appeal request quickly.

"In Jake`s case, there are extenuating circumstances that require out-of-network care," Candelora said. "His issue is so unique, and based on information I have received from his family, I don`t believe that there is an institution in Connecticut capable of handling his condition."

Dr. Patrick Senatus, a neurosurgeon with the UConn Health Center, disagrees. Senatus said the hospital generally sees about 20 patients a year with Chiari malformation and does about 10 decompression surgeries. He alone has performed three to date this year, Senatus said.

But complicating matters in DeMarco`s case is his size. When he was first diagnosed, the 6-foot college student weighed more than 400 pounds. He has slimmed down because of the disorder, but still weighs close to 350 pounds.

Obese people face additional risks in any surgery, but even greater risks from surgeries performed when the patient is in the prone position. A 2007 study by two University of Kentucky neurosurgeons showed that the obese were more negatively affected than others by the combination of stress from surgery and the pressure of extra weight on the ribcage from lying on one`s chest for long periods.

Decompression surgery is done in the prone position because doctors need access to the brain stem and cerebellum in the back of the brain in order to operate.

Most patients who have surgery see a reduction in their symptoms or prolonged periods of relative stability, sometimes both, according to the National Institutes of Health. However, in order to achieve this, "more than one surgery may be needed."

Making plans

Even though there are still a lot of unanswered questions about when and where his surgery will occur, the DeMarcos remain optimistic about Jake`s future.

While surgery won`t necessarily alleviate all of DeMarco`s symptoms, JoAnn DeMarco hopes it can make them more manageable and less frequent.

"If we can make it so that he`s only in pain and only vomiting maybe one day a month, that would be success," she said.

Over the past six months, Jake DeMarco has lost much of the sight in his right eye, developed a sensitivity to light that requires he be in dark surroundings, and sometimes forgets what he is saying in the middle of sentences.

He said his fighting spirit has dwindled since he was diagnosed and that he had been depressed for a few months, but has been working on pulling himself out of it. He`s even making plans for life outside his dark bedroom.

He wants to marry his girlfriend, Courtney Varney, 21, also of Wallingford, so they can raise their budding family together. He can`t wait to chase his daughter and watch her grow.

"My little girl is my life," he said. "I want to raise her right."

DeMarco anticipates the day he can go back to college and
finish his degree so he can be a chef and open up his own restaurant. Before he got sick, the 2005 Lyman Hall graduate had studied culinary arts on scholarship at Johnson & Wales University in Rhode Island, and business at Post University in Waterbury. DeMarco is also a self-taught guitarist and keeps his instrument at the foot of his bed waiting for the day he can play again.

But while the family`s optimism abounds, much of it is
guarded.

A friend of Jake DeMarco`s who also had the disorder died recently from complications associated with it. The two had met 10 months earlier through a social networking Web site and moved from the computer to the telephone to communicate as the disorder progressed and each found it difficult to type. DeMarco does not believe he will share his friend`s fate, but acknowledges it is a possibility.

Outside support

Jake DeMarco`s cooking teacher at Lyman Hall, Andrew Candido, now the school-to-career coordinator for Wallingford, remembers DeMarco as a natural leader of the class and someone with a lot of talent.

When Candido was out on medical leave during DeMarco`s senior year, he trusted DeMarco and a classmate to assist with the class.

Candido and DeMarco still try to keep in touch almost four years after being in the classroom together. Candido said he visits DeMarco as often as he can though he finds it painful watching him deteriorate.

Candido, whom JoAnn DeMarco referred to as a father figure to her grandson, has also taken up her grandson`s cause, soliciting help, information and donations from anyone he can.

The donations are to pay for out-of-pocket expenses associated with surgery at the Chiari Institute. He estimates the family will need $5,500 even if HUSKY covers the procedure there.

Candido set up a trust fund for DeMarco and sent a letter to employees of the Wallingford schools seeking donations and explaining the plight of his former star pupil.

DeMarco`s friend Josh Carroll also ran a "Bowl for a Cure" event May 31 that raised $800.

According to Carroll, the two have been friends nearly all their lives.

"He`s my best friend. I just want to get him better so we can hang out again."

To contribute to the Jake DeMarco Chiari Fund, send donations to "Jake DeMarco Chiari Fund," care of Heather Abel, People`s United Bank, 930 North Colony Road, Wallingford, CT, 06492.
ashea@record-journal.com
(203) 235-1661

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