The Southwest’s Orphan Disease Thrives on Ignorance

By Felicity Barringer

Valley Fever, a lung disease born of invasive fungal spores that are carried on clouds of swirling dust, is the best-known medical secret of the American Southwest. The parts of California and Arizona where the fungal spores flourish are once-rural places that are now population magnets, where new construction disturbs the earth and can send spores flying.

Tourists and new residents are unlikely to have the acquired immunity of those born in places the fungus calls home. Newcomers may not be warned, or warnings may not be taken very seriously, perhaps because most cases are mild. Yet of an estimated 150,000 infections annually, perhaps three percent become severe cases. A small fraction of these become incurable, leading to meningitis, amputations, or permanent lung damage.

At least 150 people a year die from the disease; the medical system spends hundreds of millions of dollars annually treating Valley Fever patients.

How can Valley Fever be a secret, when so much is known about it? Because there are two Valley Fever universes. In one: rural residents who live near it, doctors who treat and study it, and the victims, including those who have seen the worst it can do. In the other: the rest of the West’s population, and the tourists who visit areas like Phoenix or Tucson that harbor the fungus.

People who most need to know, like tourists and new residents of the Southwest who are unlikely to have immunity, usually haven’t heard about it, despite years of reports by specialized outlets like the University of Southern California’s Center for Health Journalism, regional newspapers, television broadcasts and articles in national magazines like The New Yorker and The Atlantic.

Who Should Know, and Who Should Tell Them?

Microscopic image of coccidiodomycosis infection.
Yale Rosen via Flickr

The two universes came face to face in a California courtroom a year ago. Five construction workers had contracted the disease while widening a culvert near Route 33 in Kern County, California for the state transportation department. They sued, claiming CalTrans should have told them the risks of working where high levels of the fungal spores are concentrated – the medical term is endemic. The state said the plaintiffs should have known.

“Most people in the state of California either have never heard of Valley Fever or believe it is caused by a mosquito,” said Peter Alfert, the lawyer for the plaintiffs. “Even some people living in the endemic area are unaware of what Valley Fever is.” He added there is “no justification” for state authorities not to warn people at risk. The jury awarded nearly $12 million to the plaintiffs.

Fewer people know about Valley Fever than know about other uncommon diseases like Zika, Avian flu, or Hantavirus. It was national news when mumps cases recently surged to 5,000 across the country. Now there are double that number of Valley Fever cases annually. John Galgiani, a University of Arizona professor who directs a Valley Fever research center in Tucson noted the irony that other, smaller risks get more attention.  "A recall of hundreds of millions of airbags is underway at a cost of billions of dollars,” he said, “even though the actual loss of life has been less than that caused by Valley Fever every year.”

Valley Fever, formally known as coccidioidomycosis, is not communicable but is one of Arizona’s most reported diseases. “Cocci” fungi are endemic in three fast-growing counties around Phoenix and Tucson.

A dust storm blowing through the outskirts of Phoenix in June 2012. Wind can distribute Cocci spores and lead to increased cases of Valley Fever. Alan Stark via Flickr
 

Urban Growth in Central Valley Stirs Fears

The company Tejon Ranch is building a large residential and commercial development in southern Kern County.

In California, another strain is endemic to the southern San Joaquin Valley, which gave the disease its name. This area around Bakersfield is largely rural. But it, too, is growing; last month, Kern County’s Board of Supervisors approved a proposal for 12,000 homes and 5 million feet of commercial space around Interstate 5.

Julie Solis, the wife of a disabled Valley Fever survivor, made an emotional plea at the December supervisors’ meeting. She urged that people be made aware of coccidioidomycosis before they build or buy the new homes. Still, the county Planning Department’s lengthy draft environmental impact report devotes one page to Valley Fever in one section and four paragraphs in another. The disease has also found its way into California prisons, resulting in severe cases and successful lawsuits.

For decades, centers like the Kern County Medical Center and Dr. Galgiani’s Valley Fever Center for Excellence have investigated coccidioidomycosis. Recently, thanks in part to attention from Kevin McCarthy, the Bakersfield congressman – who is the House majority leader – $5 million in federal funds has been awarded to study Valley Fever.

Dr. Galgiani has developed a vaccine he hopes to see certified by the federal Department of Agriculture for use in dogs. An approved human vaccine would require at least $50 million, maybe $100 million — the cost of trials required to win the Food and Drug Administration’s approval. Developing expensive drugs for conditions without large numbers of victims doesn’t pay. These are known as “orphan” diseases; that is how the FDA has classified Valley Fever.

Seeing the illness only through the lens of mild cases is “cruel and callous,” said David Filip, whose mother had a virulent case. He later wrote “Valley Fever Epidemic” and runs a website called Valley Fever Survivor. “Eventually,” he said, “if there is someone famous who gets really sick” publicity could make Valley Fever harder to ignore.