A constant struggle

A disease has the capacity to move into your house, bombarding you with prescription bottles, syringes, wheelchairs, nurses and emergencies. Daily routines revolve around medication times rather than meals. Expectations for your children or your future change drastically.

Judy Matthews, a Medical Center nurse, and her 29 year old son, Claiborne "Ivy" Woods know what its like to deal with this type of home. That is, Ivy is terminally ill, constantly fighting a rare degenerative affliction.

At first there were little signs that her baby was not completely healthy, said Matthews. "Have you ever held a floppy baby?" she asked. "They just don't hold you tight." This was the first warning that Ivy's muscles might not be normal. Since infancy, the weakness in his muscles has become more severe.

Specifically, Ivy has hereditary disorder of the mitochondria. The mitochondria, which are often referred to as a cell's "power plant," are the energy producing organs in the cell. In myopathy, the mitochondria do not produce energy effectively.

In Ivy's case, the disease, inherited genetically from his mother, manifests itself as mitochondrial myopathy and mitochondrial encepha- lomyopathy, meaning that it affects both muscle and brain cells. "The disease primarily affects muscle and brain cells because they are the only two types of cells that can't reproduce," said Dr. Rodney Radtke, assistant professor of neurology. The degree of degeneration varies a great deal from case to case because the affected mitochondria are distributed in different tissues all over the body during pregnancy when the fertilized egg begins to split, he added.

"His condition most closely resembles Myoclonus Epilepsy and Ragged Red Fibers," Radtke said.

This type of mitochondrial disorder can be devastating because of the physical as well as mental degeneration. Complications arising from myopathy include weakened muscles and the gradual loss of coordination. Ivy's growing muscular weakness is concentrated in his legs and he is now confined to a wheelchair, though two years ago he was able to walk on his own. He can, however, use his hands and arms to push his wheelchair.

Meanwhile, encephalomyopathy causes emotional and cerebral regression manifested as mental retardation and dementia in some cases. For Ivy, this means that his speech is somewhat slurred and he cannot make the mathematical calculations he was once able to, though he can still read.

As a result, Matthews is now seeking to initiate a chapter of the support group Mitochondrial Disorders Foundation of America, founded by Lynnie Morgan in California. Morgan is the mother of two children afflicted by mitochondrial disorders as well.

The foundation provides a forum for information on mitochondrial diseases and would serve to realize Matthews' desires to educate families in ways that she was afforded. Instead, most of her education about myopathy has come through countless visits to Duke.

With all of the time Ivy spends in the hospital--his most recent extended hospital stay lasted from mid-January through April--Ivy has developed strong ties with many of his care-takers, including one of his nurses, Karmon Strand, an RN in the neurology department.

"Karmon and I, we go way back," Ivy said. "I was in [neurology] intensive care and she took care of me. She was my primary nurse."

Relationships between patients, particularly those with incurable illnesses, and care-takers can be strong, yet emotional at the same time. In the case of a "regular" patient like Ivy, stories develop.

For example, many employees have heard about Ivy's talking bear, given to him by a nurse whom he now refers to as "Nanny." On one hospital stay, Ivy started hiding the bear under his bed-covers and the stuffed animal would repeat what a nurse or doctor said when they entered his room. Ivy would also cause the bear to whistle at female nurses when they walked by his bed.

Because the disease is degenerative, Ivy's childhood was not totally altered. Though he could not hop, jump or skip, Ivy could talk and walk and was even on a swim team, bringing home two ribbons, until an increase in his seizures prohibited the sport.

When Ivy asks questions about his future, his nurse Danny Cockman, who works for Alpha Omega Nursing Services and the Medical Center, responds honestly. "We don't lie about it," Cockman said. "We just say we don't know."

Interaction with Ivy can be unpredictable because you never know what his mental state will be, Matthews said. Sometimes the strain can wear down even this dynamic mother, whose energy and love friends point to as the reason for Ivy's full life despite his disease. "They tell me that he may not live much longer," Matthews said. "But, they have said that before."

Ivy himself takes his disease in stride. Due to a weight gain of 30 pounds since his last hospital visit, he was well enough to realize one of his long-term dreams on a sunny October Saturday: Ivy was able cam overnight at Camp Kanata in Cary, NC, in conjunction with a picnic that Matthews held in appreciation of all of support shown by family, friends and hospital staff.

The atmosphere was festive as attendees reminisced about times spent with Ivy. Ivy himself sparkled as the star guest, sporting his new hiking boots and "hog leg" knife.

When he talks about his disease Ivy admits in a slightly slurred, but alert and intelligent voice, that it has been hard at times. "After the muscle biopsies, I just say `That's enough!'" he said. "It isn't the best feeling. I'm not a pin cushion."

For someone who spent his last birthday and Valentine's day in neurology intensive care, Ivy is remarkably free of bitterness toward his condition. "I'm tender-hearted," he said. "You gotta be."