One Woman’s Struggle to Survive With Lupus : Health: The condition has been called the great impersonator. It alerts the body’s defense systems to fight off enemies that aren’t there. Instead, the body turns on itself, attacking normal organs.

Joanna Baumer Permut had a pretty nice life. Her husband worked at Yale University; her young daughter was thriving, and she had a job she liked and a beautiful house in southern Connecticut. Permut and her family had just moved back to the States from Belgium, where they spent two years. It was 1976. She was 28 years old.

The next year she started feeling lousy. After four months of doctor’s visits she met the “wolf,” systemic lupus erythematosus, an autoimmune disease that can cause disfiguring rashes, debilitating fatigue and serious kidney damage--just three of a handful of afflictions known to lupus patients.

“My life as I’d known it changed forever,” says Permut, who has written “Embracing the Wolf,” which tells the story of a family struggling with the unpredictability of a chronic disease.

Lupus, as the disease is commonly known, has been called the great impersonator. It can damage any organ of the body. It alerts the body’s defense systems to fight off enemies that aren’t there. Instead, the body turns on itself, attacking normal organs.

Lupus, from the Latin word for “wolf,” was named because of the butterfly-shaped rash that often appears on the face of people with the disease. According to one story, the pattern resembles the markings on the face of a wolf. According to another, it looks like the marks left by a wolf bite. Erythematosus is a medical term for redness; it also refers to the rash.

The origins of the disease are unknown. It may have been described first by Hippocrates as early as 400 BC, but it was not until 1895 that Sir William Osler, chief of medicine at Johns Hopkins Hospital, realized it was more than a skin rash, but rather a series of systemwide afflictions. Lupus can strike repeatedly with different symptoms, and it can lie dormant for years.

Abnormal Life

“Fortunately we have better drugs today,” says Dr. Naomi F. Rothfield, a professor of medicine at the University of Connecticut Health Center in Farmington, who has been working with lupus patients for about 30 years. “We can often control this disease and help patients lead a fairly normal life.”

But not totally normal. In some cases, such as in Permut’s, normal becomes daily doses of medication, lots of rest, frequent moderate exercise, work at home and a constant awareness that the wolf is always with her.

“It puts pressure on you, your husband, your family,” she says. “It attacks your self-esteem.”

She writes: “The first day I spent at home after resigning my job, I awakened, remembered I had nowhere to go, and cried even before I got up.”

Permut spent much of her early lupus years taking care of her family’s needs: her dying mother, her grieving father, her 8-year-old daughter, her healthy husband. But the physical demands of her disease, her continuing struggle to make her husband aware of her limits and the thought of losing control of her life forced her, over years, to redefine how she would live.

It involved talks with her doctors, sessions with a psychiatrist and persuading her husband, Steven, to get therapy as well.

“Steven really comes off looking like a pretty nice guy by the end of the book,” Permut says. “But I felt it was important to show how things really were--the frustration I was feeling, the difficulties we had, the threats of divorce. I wanted to tell people that you have to be honest about a chronic illness and stop shielding the people you care about. That just backfires.”

She recalls when her daughter, Lisa, demanded to know more about lupus. Why was Mom home all the time, yet too tired to play with her or go horseback-riding with her?

Simple Answers

Simple Answers

Permut gave her a simple description of the disease, trying to reassure her. It went well for a few minutes, until Lisa asked her: “Am I going to get lupus too?”

It was the question Permut dreaded the most. She answered no. (The jury is still out on this question.)

“Are you going to die?” Lisa asked next. It was the question no one talked about.

Again, Permut said no, at least not for a very long time.

The survival rate of lupus patients has dramatically improved with medical management of the condition. About nine in 10 patients live for more than 10 years after diagnosis, and many can look forward to a normal life span.

Two forms of lupus are known. In “discoid lupus,” antibodies against the skin cause disc-shaped lesions, mainly on the face and other areas exposed to the sun. Other organ systems are not involved.

“Systemic lupus” is more dangerous. A rampage of antibodies can damage blood vessels and blood components, skin and joints, internal organs, the central nervous system. Brain and kidneys are vulnerable.

About 90% of lupus patients are women. It usually hits women between the ages of 15 and 35, although it can affect young children and elderly people. Blacks and Asians are more at risk than whites. The prevalence rate is one in 1,000, but some researchers, such as Rothfield, suspect the incidence rate is increasing. Although drugs are doing a better job of treating lupus outbursts and their inherent physical problems, there are still about 5,000 deaths a year, according to the Lupus Foundation of America, based in Washington.

“There’s always the feeling that we’re better at diagnosing, but it does appear that it’s getting more common,” Rothfield says. “We don’t know why.”

Many Theories

There are theories: There may be a genetic predisposition; the disease may be triggered by environmental factors such as the sun; it may be hormonal; it may be brought on by stress. It may be a combination of all of these things.

Treatment includes drugs and a change in life style. Permut takes Plaquenil, a trade name for hydroxychloroquine, used in many parts of the world to treat malaria. It combats the rash and eases joint pain, but it can do damage to the retinas. There’s also Prednisone, a steroid derived from cortisone, which reduces inflammation, the body’s normal response to tissue injury. Prednisone’s side effects include sharp weight gain and the moon-face characteristic of steroid treatment--the eyelids become puffed and swollen. Acetaminophens such as Tylenol can be used in place of aspirin for mild pain. Cytotoxic, or immunosuppressive, drugs are a new category of experimental drugs under study.

“When I travel I take a small suitcase full of drugs,” Permut says. These include a tranquilizer to calm her shaking leg muscles--the tremors were powerful enough to wake her husband in the middle of the night. The muscle tremors are a side effect from some of the medications.

Spontaneous abortions are often a sign of lupus. About 20% of lupus patients show a higher rate of miscarriage, but more women are carrying to term with improved medical treatment.

“We have progress here,” Rothfield says. “What’s gratifying is to see my younger lupus patients having children and seeing them all grow up.”

For Permut, life has become more serene.

“I learned to take control of my life,” she says. “I stopped being jealous and resentful when Steven was out having fun on a business trip. And he learned to respect my limits.”

The Permuts take an annual vacation to Aruba.

“Normally that would be off-limits for a lupus patient,” Permut says. “But I shield myself from the sun, wear gauzy clothes.”

Last May she was ready to take a trip to the Southwest when a flare-up started.

“I went anyway,” she says. “I rested as much as I could and vacationed when I could. I’m determined not to let this interfere with my life any more than it has to.”

Mental attitude is the key, Permut says.

“Of course you need good medical treatment, but how you handle it is up to you,” she says. “What helped me was learning all I could about wolves. Now I visualize the wolf as a protector who is always with me, not something to fight.”

Lupus Systems

In 1982 the American Rheumatism Association published a revised set of criteria for classifying systemic lupus erythematosus. The common symptoms are:

* Seizure convulsion or psychosos in the absence of drugs.

* Oral ulcers

* Rash, known as malar rash, which often occurs in a butterfly pattern across the cheeks.

* Discoid rash, with crusty red lesions or more severe lesions that can scar.

* Sun sensitivity. Skin rash due to exposure to the sun.

* Pleurisy, an inflammation of the lungs; or pericarditis, an inflammation of the lining around the heart.

* Renal disorder that involves excessive protein or other abnormal elements in the urine.

* Arthritis, involving two or more peripheral joints.

* Immunological disorder.

* Hematolytic anemia or leukopenia, lymphopenia or thrombocytopenia, all of which caused a drop in the white blood count.

* Antinuclear antibodies. The disease causes anemia by forming antibodies against red blood cells. A blood test determines the level of antibodies directed against the nucleus, or center of any cell. A high level of antibodies can be a sign of lupus.