Living With - and Without - Pain

Years of living with a neuromuscular disease can take their toll in pain.

"Mental Chaos" by Mandi V. Zeiler

"A Percodan, a Pepsi and a cigarette ... "

These were Claudia's demands each morning even before she fully opened her eyes. These habits took the edge off the intense pain she felt, so she could be dressed and transferred into her power chair each morning.

As the day progressed, so did her self-medication. By midday, she was in a pharmaceutical whirlwind; and by nighttime, she was crashing. This was her daily ritual — all in the attempt to escape the ravages of pain and discomfort of living for more than 30 years with an undetermined neuromuscular disease.

Then it happened. Following routine surgery, Claudia slipped into a coma. Nurses said her vital signs wouldn't stabilize. Days later, she was dead.

When I helped her family clean her apartment after her death, we found pill bottles in, on and under everything: table, counter, bed, floor, medicine cabinet, refrigerator, drawers. Labels reflected a myriad of doctors, hospitals and pharmacies spanning years. I no longer asked why Claudia had died but, instead, why she hadn't died sooner.

I never had much sympathy for Claudia back then, often wondering if the pain she claimed wasn't "all in her head" — a cry for attention. But 20 years later, Claudia has come back to haunt me with a vengeance.

Pain now controls my days, much as it once did Claudia's. Finally, I understand her.

Understanding Pain

"Does it hurt?"

When I was growing up with spinal muscular atrophy, people often asked me that question. Today, parents still ask, as they attempt to understand whether their son or daughter with a new SMA diagnosis feels discomfort.

I reassure them that SMA, as a disease, doesn't "hurt." Sure, growing up, I experienced the inevitable sprained ankles and muscle aches (my mom used to call these "growing pains"), but there's no pain per se with this disease.

So why, then, do I, and many of my peers, have so much pain now, in middle age? And where does this pain originate?

"Pain is one of the more enigmatic symptoms that all physicians confront, in one form or another, every day," explained Michael P. McQuillen, professor of neurology and medical humanities at the University of Rochester in New York. "Since its origin may be multiple and varied, one has to start with the context — character, origin, location, behavior, aggravating and ameliorating factors, and the physical findings — to determine why the pain is there."

McQuillen said a number of conditions common in middle age, such as degenerative joint disease or inflammatory conditions such as polymyalgia rheumatica, can contribute to increased pain in those with neuromuscular disease. The loss of limb movement over time can also contribute, he said.

I asked McQuillen, a former MDA clinic director in Wisconsin, about what I call "nerve pain," or phantom pain. Be it throbbing, shooting, dull or burning, almost every adult with neuromuscular disease I've spoken to seems to experience this type of pain at times. As an example, I might feel that my foot is pressed up against the bed sheets, accompanied by excruciating pain and elevated blood pressure. But my caregiver assures me that nothing is touching it, and after a short time the pain subsides.

McQuillen explained, "The neural basis of phantom pain is complex and poorly understood. It probably has something to do with spontaneously activated pathways at various levels of the central nervous system. It can be seen at almost any age."

Greg Carter, clinical associate professor of rehabilitation medicine at the University of Washington School of Medicine, offered some reasons for our pain.

"The first is simply biomechanical," he said. "People who have an imposed mobility problem will often develop secondary musculoskeletal conditions that can be painful. These include low back pain, frozen joints, stiff necks, arthritic joints from walking abnormally or sitting in a wheelchair all day, etc."

Mary E. Csuka, associate professor of medicine, Division of Rheumatology, at Froedtert Memorial Lutheran Hospital and the Medical College of Wisconsin, said arthritic pain may be common in middle-aged people with muscle diseases.

Csuka, a specialist in geriatrics, osteoporosis, rheumatology and scleroderma, pointed out that muscle is an important stabilizer to the joint. In people without muscle problems, "even a joint with severe degeneration can remain functional if the muscles can be strengthened."

However, she explained, "Patients with muscle-wasting diseases lose the stability factor of normal muscle and, hence, the joint capsules and ligaments are under greater strain even to maintain neutral position."

Immobility, by reducing blood supply to the cartilage of the bones, can also contribute to the symptoms of aching and stiffness, she added.

Acknowledging Pain

Knowing all of this, you'd think we'd all be complaining to doctors about our pain, yet this hardly seems the case. Wendy Peltier, assistant professor at the Medical College of Wisconsin in Milwaukee who serves as the MDA clinic director there, reported, "Pain is actually not a frequent complaint."

Carter, co-director of MDA's clinics in Olympia and Tacoma, Wash., suggested that the subject of pain may take a bit more prodding. Carter developed an interest in chronic pain and the way it affects physical and emotional functioning, so he started asking more neuromuscular disease patients if they have pain. He was "amazed at how many said yes."

On the basis of his conversations with patients, Carter began doing pain research on people with neuromuscular disease. He's working with psychologist Mark P. Jensen, an associate professor in the University of Washington's Department of Rehabilitation Medicine and a world-renowned expert on pain, and Ted Abresch, director of research at the University of California at Davis in the Neuromuscular Disease Research and Training Center.

I suggested to Carter that perhaps people with neuromuscular diseases are less apt to complain about pain, because they believe it comes with being disabled — just one more inconvenience or discomfort we must learn to accept.

"I absolutely agree," he responded, "and that is exactly why I think pain in NMD has been overlooked for so long. The good news is that there are great, new, effective physical and pharmaceutical ways to treat pain. Thus, I encourage patients to talk to their health care providers about their pain."

Talking About It

Establishing a dialogue with your physician, along with obtaining a thorough physical evaluation, will help determine the most effective treatment routes for pain associated with neuromuscular disease. The complaints can be many and varied: stiff joints from immobility, sore butt from sitting, pressure sores from orthotics and so on. Identifying elements of your discomfort, such as joint pain, is an essential start.

Sometimes pain diminishes when these problems are solved at the root — by correcting the fit of a brace, adjusting positions more frequently or being turned more often in the night. It's also important to treat pressure sores to prevent major skin breakdown and infection, and to be ex-amined for possible blood clots.

Your doctor may also recommend nonpharmaceutical treatments, including heat, cold or massage. These techniques can actually change blood flow and reduce inflammation. Some people have even found that psychological techniques, such as meditation and relaxation exercises, can help to alleviate pain, or at least relieve the tension that may be making pain worse.

But oftentimes, the "fix," that is, "Leave your brace off till your sores heal," or "Exercise more," isn't feasible. Psychological and mechanical treatments may only go so far, and your doctor may agree that you need medication for the pain (analgesics).

Nonsteroidal anti-inflammatory drugs (NSAIDs) such as aspirin are the most frequently prescribed for pain, but they can cause gastric problems in some people. Csuka said she generally prescribes acetaminophen (Tylenol) for stiff, inflamed joints. This is also the recommendation of the American College of Rheumatology as the first medication to try for the treatment of noninflammatory, or degenerative, arthritis pain. When compared to NSAIDs, acetaminophen has been found both effective and safe. (See "Understanding NSAIDs".)

She noted that Tylenol is available in a variety of forms, including syrup. "Arthritis Strength Tylenol is formulated so that patients only need dosing three times a day, rather than four times, when the medication is taken regularly," Csuka said.

If you take acetaminophen regularly, you should be monitored for possible liver toxicity, especially if you're also taking combination narcotic analgesics such as acetaminophen and codeine phosphate (Tylenol 3), propoxyphene and acetaminophen (Darvocet-N 100), hydrocodone bitartrate and acetaminophen (Vicodin), or oxycodone hydrochloride and acetaminophen (Percocet).

I grew up with aspirin being the strongest pain pills in our family medicine cabinet, so I asked Carter, "Is it just me, or do you find older neuromuscular disease patients are especially resistant to trying pain medication, especially narcotics?"

"Yes, indeed," he agreed. "That is why it is so important for the health care provider to spend time talking to the patient, providing education about the usefulness and side effects of pain medication.

"There is good evidence in the medical literature indicating that pain medications are hugely underprescribed in the United States and people suffer needlessly," Carter pointed out. "Plus, there are so many new, non-narcotic types of pain relievers available."