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Rash of pain: The anguish of shingles

With an increasingly large patient population at risk, researchers focus efforts on uncovering new ways to prevent and treat this perplexing ailment.

By Susan J. Landers, amednews staff. April 1, 2002.

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As baby boomers approach 60, one extraordinarily unpleasant and potentially brutally painful medical condition lies in wait: shingles.

The culprit, of course, is the varicella-zoster virus, nestled deep within the nerve cells lined up on either side of the spinal cord. It gained access to these cells probably decades ago, after a bout of the chicken pox, and has stayed there, dormant, ever since.

For people with both luck and a vibrant immune system, the virus remains blissfully asleep. But for those whose resistance flags, the virus can wake up, revealing itself through a chicken pox rash redux.

This rash -- shingles -- often forms a belt-like sweep along the strip of skin enervated by an affected sensory nerve. It is believed that this characteristic rash formation gives the disease its name, derived from cingulum, Latin for belt or girdle.

Each year 600,000 to 1 million Americans are diagnosed with shingles, and those numbers are expected to rise. "We know that the incidence of shingles is highly age-related, and as the population ages the absolute number of patients with shingles is going to increase," said Kenneth Schmader, MD, associate professor of medicine-geriatrics at Duke University Medical Center, Durham, N.C.

Also likely to contract the disease are those whose immune systems have been weakened.

Although shingles is not usually considered life-threatening, it is a serious condition. Shingles on the face is particularly serious, as it can sometimes lead to irreversible hearing and vision problems. For example, if the cornea is infected, it can cause temporary or permanent blindness.

But shingles is best known for the excruciating pain it can bring to the afflicted. Many physicians have horror stories to relate of patients who experience pain for many years and no treatment they administered seemed to help.

Many people experience such severe pain in the aftermath of the disease that even a breeze against the cheek or lightweight clothing against the skin are intolerable.

William J. Hall, MD, president of the American College of Physicians--American Society of Internal Medicine, treats one or two patients with shingles each month and has great sympathy for them. "These are some of the most miserable people on earth," he said. "Once you've had one, they'll stay with you for months."

If the pain persists for weeks, months or even years after the infection ends, the condition is known as postherpetic neuralgia. PHN affects about 20% of all people with shingles, but increases to 40% in patients over age 60.

The pain is neuropathic, caused by damage wreaked by the virus on the nervous system.

Preventive measures

Michael Oxman, MD, professor of medicine and pathology at the University of California, San Diego, is working with other researchers to try to put an end to some of this suffering.

Dr. Oxman serves as the national chair of the large, multicenter Shingles Prevention Study, a joint five-year effort of the U.S. Dept. of Veterans Affairs and the National Institute of Allergy and Infectious Diseases to test the effectiveness of a vaccine developed by Merck & Co.

The vaccine, although 10 times stronger, is similar to the chicken pox vaccine, which has proven to be very effective at preventing that disease.

There are 38,546 participants enrolled in the trial, all of whom are over 60 and, at least before the start of the trial, had never had shingles. The hope is that the vaccine will prevent shingles or at least reduce the pain of PHN.

"Over the past 10 years I have talked to hundreds of people who have had their retirement years totally destroyed by persistent pain. And that's what we hope to prevent," Dr. Oxman said.

Half of the participants in the double-blind study received the vaccine and half got a placebo. Researchers are now recording each case of shingles and measuring its severity. It is hoped that, when the code is cracked, "most of the misery will be in the placebo-administered group," Dr. Oxman said. "If it isn't, then we'll all cry for a long time," he said.

Results of the study are expected by the middle of 2004.

Meanwhile, the success of the chicken pox vaccine is raising questions in researchers' minds about its possible impact on the development of shingles. Before the introduction of that vaccine in 1995, there were about four million cases of chicken pox each year. Now some pediatricians are reporting that they hardly see a single case.

How the elimination of chicken pox will affect future cases of shingles is an area ripe for speculation. So far, although the evidence is scant, the chicken pox vaccine seems to afford some protection from severe cases of shingles. "What we've seen so far is that shingles has been less after vaccination than after the natural infection," said Anne Gershon, MD, professor of pediatrics at Columbia University in New York.

Dr. Gershon theorizes that the chicken pox vaccine renders the varicella-zoster virus less likely to become latent and re-emerge as shingles. This favorable outcome has been witnessed among vaccinated children whose immune systems were undermined by disease.

"It's going to take a long time," Dr. Gershon acknowledged. "But all of the data are in the right direction."

Dr. Schmader is also optimistic about the future. The drop in the number of chicken pox cases is terrific news, he said. "Hopefully 50 or 60 years from now we'll be saying 'Remember chicken pox and shingles ..."

Treatment options

While the future may be promising, patients here and now are turning to their physicians for help, and Dr. Hall for one finds that "all of the treatment modalities are equally bad."

Nonetheless, there are treatments that could work, although none is likely to work for all patients 100% of the time. If a physician can begin treatment early, at the first signs of shingles, the use of antivirals may help.

"Don't wait until Monday if the rash appears on Friday," Dr. Oxman said. Antiviral drugs don't make dead cells come back to life, they only prevent further virus multiplication, he added.

If PHN develops, physicians can start patients on another line of treatment. But slow and steady may win this race. For, as Dr. Schmader points out, "Neuropathic pain is sometimes kind of a tough nut to crack."

One first line defense against PHN is the use of tricyclic antidepressants, including amitriptyline, desipramine and clomipramine.

"What's important is that you have to start at a very low dose and tell people that it won't make the pain go away, but hopefully it will move it from something that is severe enough so they can't sleep to something that lets them sleep," Dr. Oxman said.

One mistake is to prescribe too large a dose too quickly, he said. "You should slowly change the dose on a weekly basis, escalating it until you have relief of pain or an unacceptable rise in the possible side effects."

Another medication that seems to help is Neurontin, or gabapentin, said Robert Dworkin, PhD, executive director of the University of Rochester Medical Center's Anesthesiology Clinical Research Center, Rochester, N.Y. The drug was introduced in the United States in 1994 as an anticonvulsant for patients with epilepsy. How it works as a pain reliever is still a puzzle.

A third approach to pain relief is topical: the lidocaine patch, which is the only drug approved by the Food and Drug Administration specifically for PHN, Dr. Dworkin said.

After some early controversy, narcotics are also now recognized as a fourth option for treating the pain of PHN, Dr. Dworkin said.

"It's up to the doctor to determine which to try first," he noted. "Most aren't going to try an opioid first, while they might try the lidocaine patch or a tricyclic or gabapentin."

As for efficacy, the treatments generally produce about the same results. "What we say is about half of the patients get about half of their pain relieved. But that still leaves a lot of pain," Dr. Dworkin said.

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 ADDITIONAL INFORMATION: 

Treatment tips

• Start on antivirals early.
• Provide pain relief both for humane reasons and also for the theoretical reason that if pain during shingles is decreased, the probability of persistent and residual pain may be reduced.
• Provide good psychological support.
• Avoid steroids.
• Consider anything else that is not harmful and not expensive, such as biofeedback, acupuncture or tai chi.

Source: Michael Oxman, MD, chair of the Shingles Prevention Study

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No easy answers

Below are questions patients are likely to ask when diagnosed with the painful and persistent ailment.

Is shingles contagious? You can't catch shingles from someone who has it. But anyone who has not had chicken pox and is exposed to shingles may develop chicken pox, because the same virus causes both diseases.

If my parents or siblings have had shingles, am I more likely to develop it? Having relatives who have had shingles does not mean you are at greater risk for inheriting or developing the disease.

Can stress bring onshingles? There is no direct evidence that stress alone can trigger the development of shingles.

How serious an illness is shingles? Most people experience a prolonged, severe pain that interferes with daily activities. The pain and discomfort may be severe enough to interfere with sleep or be irritated by clothing. Pain that persists for weeks, months or even years after the rash has healed is postherpetic neuralgia, which affects 20% of all people with shingles, but increases to 40% in shingles patients over age 60. If shingles appears on the face, it can lead to serious and sometimes irreversible hearing and vision problems. It can also be especially severe in people with impaired immunity caused by diseases such as cancer or HIV or by drugs used to treat cancer.

Can I get shingles more than once? Most people do not get shingles more than once.

Can shingles be confused with other diseases? Before the characteristic rash appears, it may be difficult to diagnose. Also, other rashes, such as those caused by herpes simplex or a contact dermatitis, may be similar in appearance to the shingles rash.

Source: National Institute of Allergy and Infectious Diseases

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Weblink

Shingles Prevention Study (http://www.niaid.nih.gov/shingles/)

Shingles Information Page from National Institute of Neurological Disorders and Stroke (http://www.ninds.nih.gov/health_and_medical/disorders/shingles_doc.htm)

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