The unsolved mystery of CFS and its AIDS-like misery

By Llewellyn King, June 2, 2011:

What's in a name? A great deal, if you suffer from awful, long-term diseases that have no cure.

Chronic fatigue syndrome (CFS) is a name that infuriates patients, who number perhaps 1 million to 4 million in the United States and 17 million worldwide. It also frustrates the small but dedicated cadre of doctors and researchers who have made the disease and its casualties their concern.

The Centers for Disease Control in Atlanta picked the CFS moniker in 1988, although the term myalgic encephalomyelitis (ME) is still in use in Europe and elsewhere and is favored by patients.

The new name quickly became despised because “it trivializes the disease and misleads people,” in the words of Leonard Jason, professor of psychology at DePaul University in Chicago. Certainly it brings to mind chronic whiners and everyone's everyday fatigue.

Part of the misleading, Jason and numerous medical professionals have noticed, is that the name has allowed governments and psychiatrists, especially in Britain, to sweep a plethora of psychological diagnoses into the tent. This obscures the central unsolved mystery of CFS and its AIDS-like misery, while diverting government funding away from serious biomedical research. Jason and some of his colleagues believe that the most promising lines of investigation, pathogens, are being under-researched in the process.

Two years ago, the journal Science published the results of joint research by three teams of investigators pointing to the retrovirus XMRV as a likely culprit. But in a disappointing setback to CFS sufferers, the journal published two online articles Tuesday backing away from this conclusion. Although disheartening to the CFS community, this does not put everything back to square one. The most current thinking is that although CFS may be caused by a combination of factors, including genetic predisposition, the most likely trigger is one or more pathogens and the immune system’s hyper-response to infection.

“The immune system pours out its toxins to stop this agent,” Dr. Jay Levy, a University of California professor who co-authored the new studies told The New York Times. “And then the immune system doesn’t calm down.”

Although it has been around for centuries, and variously labeled, modern concern with the disease dates to a major outbreak at London's Royal Free Hospital in 1955. That outbreak was big enough -- nearly 300 sufferers -- to worry public health officials.

Its appearance in a cluster at the hospital suggested that it was contagious. Then, as now, there was no real treatment and no clue as to the path of the potential contagion: Was it airborne or foodborne? How about contaminated surfaces? Were bodily fluids involved? Was there a genetic link?

None of those questions have been answered. What is known is that the disease can appear in clusters, but it is more often found in isolated cases. It has spread in families, making it frightening; but the spread is rare, and seemingly random.

The next major event to get the attention of health professionals was in Nevada at Incline Village, a resort on Lake Tahoe, in 1985. At over 300 cases, it proved too big to ignore, finally attracting attention from the CDC as well as state public health authorities.

The CDC sent two young epidemiologists to investigate the outbreak, Gary Holmes and Jon Kaplan. They estimated the number of sufferers at perhaps 20,000 throughout the United States, a majority of whom were women. The same year, a second outbreak occurred in Lyndonville, a farming and manufacturing village in the northwest corner of New York state, with 216 cases out of a population of 900. Lyndonville only had one doctor, David Bell. He has followed the disease's progress tirelessly, and he has become something of a trailblazer in the field.

Over the years, the disease popped up around the country, attracting distinguished researchers in its wake. In 1987, Harvard Medical School professor Anthony Komaroff published a report about increasingly significant numbers in his Boston practice. Dr. Nancy Klimas, an immunologist and AIDS expert at the University of Miami, found her clinic flooded with sufferers from the new disease and soon found their immune systems showed strange characteristics.

The numbers were clearly overflowing the CDC's estimate, but no one yet realized the extent.

Then entered Jason and his team of researchers at DePaul University. They studied the disease in society from a psychological point of view and found in 1990 about 1 million sufferers in the United States.

They also believe the disease was caused by an unknown pathogen, was not psychological in nature, and that the cure rate was extremely low. Additionally, they and other researchers found that one of the prevailing symptoms was immune system suppression.

For most patients, CFS is a one-way ticket to hell. The affliction is acute and mostly incurable. Horrifically, it takes away even life's littlest pleasures.

According to many interviews and hundreds of e-mails I have received since first covering the disease, sufferers are hit first with symptoms of what seems to be flu. Sometimes there is a short, deceptive remission -- sometimes two or three. Then the pattern emerges of collapse after every exertion, especially exercise. Finally, full onset occurs: There are no more normal days, only different degrees of weakness, pain and other symptoms. Doctors term the disease relapsing and remitting. That means you might have weeks, months or years of slightly better days, and then stretches -- often years, sometimes decades -- of almost total helplessness. It is goodbye to the life you have known; goodbye to work, to hobbies, to lovers and spouses, to everything short of hope.

Deborah Waroff, a gifted New York author and securities analyst, is typical in the devastation of her life. Before, Waroff was a skier, a sailor, a passionate squash and tennis player. Now the aloneness of the disease weighs her down. Very old friends -- some from her days at Harvard, a few from childhood, a handful from work -- sustain her with telephone calls, when she can answer the phone, and some drop by. Nonetheless, the brutal loneliness is always there.

Waroff was first felled at the end of July in 1989. Her engagement calendar grew full of forlorn cancellations for dinners, parties and meetings. One day in 1991, a bad headache arrived that lasted three days; after that, it came again and again.

Gradually, with help from a tireless and creative doctor, Waroff began to find medications and methods that would allow her to work a few hours a day. Pushing herself with sheer willpower to complete a chore would exaggerate her symptoms -- more mixed-up speech, stumbles, near-falls, dizziness, rising fevers. Afterward, she would be immobilized for days.

Then things got worse.

In September 2003, Waroff woke up to find that she was too weak to fill out a simple form -- just to renew library books -- and fax it. That was the beginning of month after month of near-death incapacity. “I was as weak as you can imagine. I lay on the couch, its high back and sides making me think how much this was like being in a coffin, inert, my consciousness flattened by illness. I was too weak to read and often too weak to watch television. I would turn my back to the screen and let the sound wash over me, not taking it in.”

CFS, like AIDS, suppresses the immune system. Typical symptoms include tremendous fatigue that is unrelieved by sleep, as well as flare-ups of herpes- family diseases (such as HHV-6 and Epstein-Barr). Other typical symptoms include swelling of the lymph nodes, muscle aches and other pain, dysphasia (the inability to use the right words), general cognitive failure, nausea and faintness.

Elisabeth Tova Bailey, once a professional gardener in Maine, was felled by CFS. Unable to leave her bed for more than a year, she filled her days by watching a single snail in a terrarium make its fascinating way through life.

When she was feeling somewhat better, Bailey studied the snail through the wonderful work of the 19th-century naturalists -- that special breed of romantics who studied by watching, rather than by dissecting in the lab. The result is the sweet, well-reviewed book, The Sound of a Wild Snail Eating.

The most famous person to have CFS, and to have managed in great adversity to be productive, is Laura Hillenbrand who has over time written two incontrovertible bestsellers, Seabiscuit: An American Legend and Unbroken: A World War II Story of Survival.

Hillenbrand’s achievement is Herculean. She seldom is able to leave her home in Washington, D.C. In a recent interview, she told the story of how she had to leave her own wedding because she was so sick.

Statistically, two-thirds more women are afflicted than men. But I have heard from a lot of men, including a medical doctor and a young man, who was thrown out by his father who accused him of malingering. His plight is terrible, as is the plight of other people who do not have the intellectual capital or financial resources to do anything but suffer in isolation. Insurance companies drop coverage routinely, and many doctors misdiagnose or are influenced by psychiatric arguments.

Recovery, like that of DePaul's Leonard Jason, is rare. If it does not occur within the first two years, it is unlikely to occur at all. Usually only the young and well-supported socially are able to regain a good part of the health they once had. The beacon of hope in this wasteland of human wreckage is a private institute in Reno, Nev. Affiliated with the University of Nevada, it is called The Whittemore Peterson Institute for Neuro-Immune Disease (WPI). It was founded and funded by Harvey Whittemore and his wife, Annette. Their 33-year-old daughter, Andrea Whittemore Goad, has been a CFS sufferer since she was 11.

The medical establishment has been cool to WPI, and the NIH turned down all six research grant applications it made last year. But at least 1 million very sick Americans are cheering for this frontal attack on CFS, which they prefer to call ME/CFS in deference to the older, less trivializing name.

While these things are argued, the life in limbo that so many endure is described by Waroff this way: “You know the trouble with this disease? All this time goes by with nothing in it. You don't get a chance to put anything in it. It's just empty time.”

Llewellyn King is executive producer and host of “White House Chronicle” on PBS. His e-mail is lking@kingpublishing.com.