Coping with lifelong pain
April 30, 2009
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Elvira Cordileone
STAFF REPORTER
An inherited condition dealt Mariah Pequegnat a life laced with acute pain. For many of her 13 years, she has contended with a growing number of joint dislocations and increasing agony.
Then, about two years ago, Mariah and her sister Olivia, 16, both of whom have a rare connective tissue disorder, fell into the soothing hands of SickKid's team of chronic pain specialists. (See a photo gallery of the girls' day at SickKids.)
Today, Mariah's pain has dropped by 75 per cent, and with less pain has come a vibrant new life.
Mariah, Olivia and their mother, Carol Anne, all have Ehlers-Danlos syndrome, which can affect every area of the body, from joints and skin to blood vessels and internal organs.
For Mariah, pain came from frequent joint dislocations, as well as less severe internal problems. Olivia also experiences dislocations but not as frequently. However, she has abnormally stretched bowels that don't work as efficiently as they should and require ongoing treatment, and her skin is extremely fragile and prone to bruising.
No cure exists. Treatment focuses on preventing joint deterioration and managing pain. The pain clinic uses a combination of drug therapy, physical therapies and counselling, says Dr. Fiona Campbell, a pain specialist and anesthesiologist.
"I don't think there's one single thing (that works). It's really the (team) approach and the combination of strategies we use that's important," she explains.
In Mariah's case, they modified her drug regimen to give her optimum relief while reducing some of the side effects she'd experienced, then customized the other therapies, since no drug, however effective, completely eliminates pain.
Meanwhile, physiotherapist Anne Ayling Campos introduced Mariah to the benefits of ice. A transcutaneous electrical nerve stimulation unit (TENS), which sends an electrical current through pain pathways via electrodes on the skin, also helped.
Equally important, Campos says, is that Mariah learned to pace herself, to recognize when to be active and when to rest. They also taught her to reset some minor dislocations on her own.
Advanced Practice nurse Jennifer Stinson liaised with Mariah's school to ensure she could take breaks as needed, use her medication on site, and have a private place to go to reset her bones.
A growth spurt between the ages of 10 and 12 shifted Mariah's centre of balance, increasing the number of daily dislocations she endured. Pain caused her to miss three-quarters of Grades 5 and 6.
Even on the days she did attend class, she spent much of the time lying on a special bed. And no school day went by without her mother having to come at least twice to reset a popped joint.
The huge reduction in pain means Mariah can now attend most of her Grade 8 classes. She has new friends and an active social life. And, because she has learned how to take care of herself, she is trying new things, such as sports.
Olivia says little during our interview with the family, except that the clinic has helped her neck pain. She has autism, a disorder often linked with Ehlers-Danlos, according to Carol Anne, a former nurse.
Because the disorder affects every part of the body to varying degrees, her daughters have visited just about every specialty at SickKids over the years: cardiology, urology, ophthalmology, gastroenterology and even dentistry, as gum tissue is fragile and doesn't support their teeth well.
But since they've started working with the pain clinic, the number of hospital appointments has dropped to less than six a year, including two at the pain clinic. When pain concerns arise between visits, such as a drug side effect, the clinic arranges telephone conferencing so they don't have to drive in from their home in Coburg.
Ehlers-Danlos syndrome forced Carol Anne into a wheelchair 11 years ago. But she hopes the advice from the pain clinic will let her daughters stand on their own two feet for the rest of their lives.
See a photo gallery of the girls' day at SickKids.
Toronto Star