A Life of Pain

Jen Depeppe/Times photos
By: Sunshine Werbock/ Times staff writer

The pain of Reflex Sympathetic Dystrophy keeps Diane Ivone confined to her Mayfair home

Little medical attention has been devoted to treatment of a disease known as RSD. For Diane Ivone, who is coping with it, her days just get more painful…and there is little relief in Site.

Pain is a miraculous thing. It's your body's tireless watchdog, alerting you when something is wrong. Without it, you would be walking around with weeping blisters from backing up against steaming radiators, grasping hot pot handles or grilling your flesh under a scalding summer sun. Broken bones, heart attacks and erupting appendixes would go unnoticed.

You could die of food poisoning, the flu, cold weather, starvation without your nervous system's intelligent arsenal of pangs, aches, stabs, pins and needles and dozens of levels of discomfort.

But imagine that your nervous system has gotten confused, like a car whose broken computer keeps sending "overheat" messages to an engine that's actually fine, causing it to shut down for no reason.

Imagine that after bumping, bruising or cutting your leg, your body sends excruciating signals to the area.

Imagine that the signals don't stop. In fact, over time they get worse, assaulting the skin, muscle bone and joints of the area until it is racked with agony.

And then it spread-malignant pain inching it's way up so that, eventually, your whole body is paralyzed with ravenous anguish.

You can't move. You can't be touched. Even more agonizing is that there isn't any cure.

You call every doctor you can find. None of them will see you. You're not eligible for Social Security benefits. That's because, they tell you, "it's all in your head".

When you're able to do some research and get in touch with people who suffer the same tormenting symptoms, you discover that others have killed themselves because of the pain.

It sounds like a nightmare but for Mayfair resident Diane Ivone, it's a horrifying reality. Ivone has Reflex Sympathetic Dystrophy, or RSD, a little-known degenerative disease that has trapped her inside of her own body and house for years.

It all started in 1987, when Ivone had surgery on her right foot to remove a tumor and repair bone fractures. Until that point, everything had been fine. "I had been working, dancing, gardening, doing all the stuff I love" recalled Ivone, now 40. "I loved the outdoors."

But suddenly, Ivone began experiencing excruciating, burning pain, and then freezing pain and twitching muscle spasms in her right foot. Baffled and frightened, she started calling and visiting area doctors, about 20, who insisted she was fine, said they had no idea what was wrong, or shift her to other doctors.

Finally, that same year, Ivone met DR. Robert Knobler at Thomas Jefferson University Hospital, the only doctor that knew about RSD. Knobler was willing to try to treat Ivone, prescribe pain medication and testify for her Social Security benefits hearing.

But he could not make the pain stop. It soon traveled up Ivone's right leg, down the left, up through her groin, bladder, hips, back, arms, hands, chest, shoulder and jaw.

Today, Ivone can't even walk from her couch out to the garden that she loved so much. She can't sleep even in her special hospital bed. Her feet are swollen twice-there normal size covered in a scale like sheath of powdery white flesh. It's difficult for her to talk or to move her hands.

Until she got an ambulance to take her to a doctor's appointment two weeks ago, Ivone has not been out of her home in a year.

"It's torture. I just can't do anything"

Ivone has tried nerve block treatments, in which a needle is inserted into the nerve area to kill or deaden it.

It only worked for two hours.

As if Ivone's pain hasn't been enough, has had to deal with the devastating humiliation of being told time and time again that she's not sick. Or having doctors refuse to see her because treating RSD patients is so difficult and because it's not really studied in medical school. Even neurologists, she says refuse to see her. And because RSD is such a little-known malady, drug companies aren't willing to pour money into drug research to cure it, Ivone noted.

Ivone has been unable to acquire special equipment to help mobilize her, and in 1997, she lost a Social security hearing for disability benefits, even after the judge saw her feet and heard Dr. Knobler's testimony. She will be appealing that decision.

But even with the diligent help of friends, who have been making calls, getting information on her behalf, Ivone hasn't been able to get anywhere. Literally.

And she needs medical attention. Like a diabetes or cancer patient, Ivone needs to see nutritionists, eyecare specialist, urologist, dermatologist and bone doctors.

But leaving the house is an excruciating ordeal involving ambulances and attendants who have no idea how to handle an RSD patient. (If you touch Ivone's leg, for instance, she will be in additional pain for days).

"The world's open to you, if you can walk," she said. "If I could just get out that door…. You're just stuck here in these four walls, in excruciating pain. I can't even get out to get my blood pressure checked".

As a result little infections become major infections. And none of the general practitioners who Ivone has contacted are willing to write referrals for her so that she can get basic analgesic skin creams.

Although finding other RSD sufferers on-line has helped to alleviate some of Ivone's isolation, her hands have been in too much pain lately for her to use the computer. She spends most of her day exhausted, in a daze of pain the groggy side effects of morphine, which is used to numb it.

The morphine offers a little relief, but it also contributing to Ivone's degeneration, since use of the drug limits patient's life span. And the more she takes, the closer she is to death.

It is undoubtedly a depressing way to live. And battling depression has been tough for Ivone, especially since her mother died five years ago. Now Ivone's sole caretaker is her father, John Ivone, a retired baker.

Knobler, her doctor at Jefferson, explained that pervasive depression is typical in RSD sufferers. Knobler who founded the Knobler Institute of Neurological Disease, care for patients who suffer from multiple sclerosis, RSD and other forms of chronic pain.

Most physicians, he said, refuse to treat chronic pain, other than that suffered by terminal cancer patients.

And most doctors won't treat RSD victims because so little is known about the disease, Knobler said.

"It was not covered in medical school," he said.

But Knobler became fascinated with chronic pain while he was in medical school and began treating multiple-sclerosis sufferers in 1969. Then he started to study RSD and see RSD patients.

Knobler explained that a simple trauma, such as a sprain, drain or operation, can cause damage to nerve endings. Those nerves trigger chemical signals that stimulate cells of the sympathetic nervous system, resulting in pain, swollen, skin problems and movement control.

"It tends to take over," he said. "There are people who commit suicide because the pain is intolerable.

Because Knobler is the one doctor in the area who will treat RSD patients, he has hundreds of them, including Ivone, whom he called "a prisoner of her own body" but there are no concrete ratios for statistics available about the disease and how many people it affects. Knobler firmly reiterated that why there is no test for RSD and no cure (even amputation can't stop the spread of pain or swelling from limb to limb), RSD can become more manageable if it's caught early and the patient forces himself to continue to be mobile.

But it can't be caught early if sufferer's se doctors who refuse to treat them.

"The worse is when a doctor will say, it's all in your head" said Knobler. "That leaves the patient in agony. Who do you turn to?"

Ivone and her father turn to faith. John Ivone, who has tried to remain as strong and optimistic as he can be, keeps telling his daughter that she must have faith. He believes that there is a reason for everything, even RSD.

"Without faith, you don't have hope," he said. "Without hope you have nothing."