With a week of Hawaii behind her, 17-year-old Beth Hopkins now faces extensive—and expensive—medical testing and evaluation preceding brain surgery that will help ease some of the symptoms of her rare joint disorder.

Beth was given the Hawaiian vacation by Make a Wish Foundation, but found it surprisingly cold most of the time, windy and rainy.

Apparently Hawaiians are experienced the coldest winter they've had in living memory, so Beth's dream to feel the heat in her bones and swim in the ocean were disappointed—the water was freezing, as were the unheated pools.

It was also a very long trip - 33 hours each way - and in retrospect, says Beth's mother Sue, it was way too much for Beth.

"She was so incredibly exhausted. But having said all that, Hawaii was beautiful and we had a lovely time despite the weather."

Just before Beth and Sue left, they had been to visit a Baltimore specialist, where she learned that in addition to the diagnosis of Ehlers Danlos syndrome—she was seeing the specialist to learn more about that painful disease, which causes her joints to pop out from the least little movement—she also has a Chiari malformation, a condition in which brain tissue protrudes into the spinal canal. It occurs in Ehlers Danlos sufferers when the connective tissue is too weak to hold the brain at the base of the skull in place.

Some of Beth’s symptoms, including her chronic exhaustion, may be the result of the Chiari, and the surgery is expected to improve her quality of life, but it’s difficult to know at this point which symptoms will improve and to what extent, says Beth.

She and her mother will travel to New York to the Chiari Institute for three days of appointments next month, and hope surgery will be scheduled for the summer. They have already been bumped up on a six-month waiting list thanks to the Boston specialist.

The Hopkins received some financial assistance for the trip to Baltimore through a concert fundraiser, and the Chamber of Commerce Candlelight Stroll proceeds helped to pay for training Lexi, Beth's faithful friend ‘special skills service’ dog, but they are still facing significant financial outlays.

The surgery, says Sue, "is a daunting prospect, but we are really eager to get to The Chiari Institute and get things moving so Beth can get it over with and start feeling a bit better."

Thankfully, says Sue, fairly cheap flights are available and they will stay at The Ronald McDonald House.

There is still around $1000 left in Beth's trust fund account set up for donations, but that still leaves them shy of about $2500 to $3000 of the estimated cost, just for this trip.

Once they have more information about the surgery Sue can apply to OHIP and hope they cover the $150,000 to $200,000 for the surgery, but whether covered or not, she is determined it will be done at the New York institute by experts in their field.

As scary as the operation is, says Beth, it helps to know it will be performed by people who are the best in the world at that particular surgery.

She is also looking forward to meeting other teens, and Sue other parents, who are dealing with Ehlers Danlos and Chiari—there is nobody in this part of the country, and maybe right across Canada, with Beth’s combination of illnesses, says Sue.

As for Beth herself, the symptoms caused by the Chiari are worsening fairly quickly now, she says. She has trouble concentrating, she loses words, and feels generally like she has a “brain fog.” And the chronic exhaustion is also worse—a trip to Tim Hortons or a short visit from someone means she must rest the remainder of the day, sometimes too tired to even read.

Yet she is amazingly good at staying positive, says her mom, although she does have her occasional moments of feeling down.

Beth says she misses socializing with her friends and the routine of doing school work, which has been put on hold until after the operation. The only education she gets these days is from shows on the Discovery channel, she says. And it’s difficult that at a time of her life when her friends are graduating from high school and making plans for university next year, she is sitting on the couch watching TV, unable to do much else. But she is optimistic that once she has recovered from her surgery—likely a six-month process—she will finish her Grade 12 work fairly quickly and start applying to university.

“I’m hoping I can do what I planned before I got sick. Just having this appointment to look forward to, knowing that I’m going to be having these tests and getting some answers, has given me so much more hope.”

There is a trust fund set up for Beth Hopkins at the Meridian Credit Union, account number #6972376, to help pay for the expenses of the two trips to New York.

© 2009 Niagara Advance