This special report is a project of the Reporting On Health Collaborative
Valley fever starts with the simple act of breathing.
The fungal spores, lifted from the dry dirt by the wind, pass through your nostrils or down your throat, so tiny they don’t even trigger a cough. They lodge in your lungs. If you’re fortunate – and most people are – they go no further.
But if you are one of the more than 150,000 people stricken with coccidioidomycosis every year nationwide, it’s because the spores have sent roots into the moist tissue of your lungs.
They start to feed, and, over time, they can rob you of your health. In serious cases, your muscles waste away. Your bones become brittle. Pustules appear on your arms, neck and face and then erupt.
Once the fungus takes root, it never leaves you. In about 100 cases every year nationally the fever kills. That’s more deaths than those caused by hantavirus, whooping cough, and salmonella poisoning combined, yet all of these conditions receive far more attention from public health officials and are more widely known.
As horrible as the disease can be, people in Bakersfield, Fresno, Merced, Stockton and other parts of California's San Joaquín Valley have come to accept it as a way of life. Everyone knows somebody who has had valley fever, and most have survived.
But talk to the people who deal with the disease every day – epidemiologists, clinicians, and the patients themselves – and you’ll hear a sense of urgency. Valley fever numbers are soaring so high that health experts call it an epidemic.
"Only few diseases have increased like this in my career," Gregg Pullen, the infection control manager for Children's Hospital Central California in Madera, said at a recent public health gathering in Hanford. "I can't remember ever seeing cocci like we have in the past two years."
The cocci fungus is common in much of the southwest and in northwestern Mexico, especially in the dry earth of California’s Central Valley and in the areas around Phoenix and Tucson in Arizona. It can be found, however, in soils of the beach haven of San Diego, the wine country of Sonoma County and inland in the Sierra foothills.
But you don’t have to live in one of those areas to catch the disease. Millions of people drive through the heart of valley fever country every year on Interstate 5 and can inhale the fungus with just one unlucky breath.
Efforts to stem the tide and to prevent new infections are hampered by widespread misdiagnosis, a lack of research funding, and decades of neglect by state and federal policymakers. Misdiagnosis is so pervasive, experts say, that some suffer and even die from valley fever without knowing they had the disease.
Just this summer, the small pool of funding for a potential vaccine dried up, leaving little hope of conquering the disease. Higher profile illnesses like West Nile virus command 20 times as much in federal funding. Yet valley fever harms far more people and is far more costly, according to National Institutes of Health data and a series of academic studies.
“Valley fever is not occurring in D.C., it’s not occurring in Atlanta, and it’s not occurring in Bethesda, Maryland. It’s invisible to the most important policymakers when it comes to health funding,” said valley fever researcher Dr. George Rutherford at the University of California San Francisco. “And that won’t change unless the need for a vaccine is elevated in some way.”
A hidden menace
By many measures, valley fever should be a disease that prompts an aggressive public health response. For nearly two decades, statistics from the U.S. Centers for Disease Control and Prevention show coccidioidomycosis doing more and more harm every year, with the rate of infection rising faster than most diseases reported to the CDC.
Some of that rise is due to an uptick in diagnosis and better disease reporting to state and federal agencies. But the trend can’t be explained by better reporting alone.
In 2000, cocci wasn’t even among the nation’s top diseases, as ranked by the rate of infection. There were fewer than four cases for every 100,000 people. By 2009, though, the rate of cocci had more than quadrupled to 13.24 for every 100,000 people, meaning that people in states that report the disease to the CDC had a higher likelihood of catching cocci than AIDS, hepatitis, or chickenpox.
While cases of tuberculosis and AIDS fell by half between 1995 and 2009, cocci rose 12-fold, according to CDC data. And the numbers continue to climb. In 2011, more than 13,200 people were diagnosed. But most cases are not diagnosed, and researchers estimate that the fungus infects more than 150,000 people every year who either suffer serious ailments without knowing the cause or who have mild cases that escape detection.
Most of the cases are concentrated in two states: Arizona and California. Arizona’s rate of valley fever jumped from 31 cases for every 100,000 people in 1999 to 157 cases in 2011. California officials did not provide similar data.
California cases jump annually
Alarmed by the rise in cases along the state’s main north-south corridor, Interstate 5, California county health officials called a meeting this past June in Hanford. Every participating county – except Tulare — reported an increase in valley fever cases.
"If you're driving up and down I-5, hold your breath,” Kern County epidemiologist Kirt Emery said, half-jokingly.
Kern County is a good case study for the rest of the state. The county’s history of strong health department reporting shows a relatively constant trend in cases over the past three decades with two exceptions: a dramatic spike in the mid-1990s, which observers have dubbed the “Great Epidemic,” and another significant climb now, which some call “The Second Epidemic.”
An epidemic occurs when growth in cases far exceeds what has become the baseline rate over time.
Kern’s numbers more than tripled from 2009 to 2010, for a total of 2,051 that year. In 2011, they jumped to 2,734. So far, this year is following a similar trend.
Emery, the Kern County epidemiologist, was concerned that the uptick could be the result of more labs being required to report the disease because of a 2010 change in state law. So he removed the data from those labs from his analysis. Even with that new data excluded, there was still enough of a leap to call the rise an epidemic, Emery said.
“We’ve been reporting the exact same way since the 1980s, so it doesn’t across the board explain the increase we’re seeing,” Emery said. “In most counties, they’re pretty much doing the same thing they’ve always done.”
A little understood disease
Even though the first known case of valley fever was discovered more than a century ago, scientists continue to puzzle over exactly how the disease spreads and how to prevent it.
No one knows how much cocci needs to be breathed in to contract the disease. Computerized air screening systems can detect a wide range of spores for diseases such as anthrax, one of the most worrisome bioterrorism threats. But no one knows how to screen the air adequately for cocci during windy days. No one knows why some people die and others never know they are walking around with spores in their lungs.
People who work outside – farm workers and construction workers – are most likely to get it. The disease hits blacks and Filipinos harder than the rest of the population, and people with compromised immune systems and older people are more likely to suffer complications.
For Bakersfield physician Dr. Yakdan Al Qaisi, the fight with the fungus began with a fever that drenched his pillow at night. No medications would reduce it. He lost his appetite. By the time his health improved, he had spent time in the hospital with pneumonia, lost 30 pounds and missed work for nearly six weeks.
“If I had known I would get this, I wouldn’t have come here and exposed my family to this,” Al Qaisi said. “It was a very scary time.”
In a small percent of cases, the fungus spreads beyond the lungs. This is called disseminated valley fever, and the effects can be devastating. Doctors compare the illness to tuberculosis because of its ability to damage so many distinct areas of the body. The fungus can enter the bones, skin and other organs, leading to brain swelling, lung failure, and, eventually, death.
“Disseminated cocci can be terrible,” said Dr. Navin Amin, the chair of the family practice department at Kern Medical Center. “We need to catch it earlier and start treating it aggressively.”
Treatments usually include several rounds of antifungal medications and, at times, surgeries to remove infected bone or skin. If it is caught early, physicians have a better chance of keeping the disease at bay. Without treatment, a person who develops a brain infection from the disease is much more likely to die. But physicians don’t often catch it early. Researchers say that most cases are misdiagnosed or missed entirely, in part because of a lack of training and attention in the medical community, in part because the symptoms are so varied.
When the disease causes unsightly abscesses on the skin, doctors think first of bacterial infections like staphylococcus. When the fungus invades the joints and leads to swelling and excruciating pain, doctors think of arthritis or cancer.
In January 2011, 12-year-old Tyler Bridgewater of Oildale died after doctors in Bakersfield treated him for viral meningitis instead of valley fever, his mother said.
Even if the disease is treated, some patients with cocci could be on anti-fungal treatments for the rest of their lives, often with troublesome side effects. Most patients with healthy immune systems who beat the disease are able to wall away the valley fever fungus and never be sickened by it again. In some cases, though, it resurfaces when the immune system is weakened by another disease, or when a patient takes medications that lower the body’s defenses.
Valley fever a low priority
Cocci has never had the high profile of diseases that affect far fewer people. The CDC didn’t even track valley fever until 1995. In 2007, it stopped gathering data about deaths from the disease.
In 2009, there were 720 cases of West Nile virus and 12,926 cases of cocci nationwide, meaning fewer people were stricken by West Nile virus over the entire year than by cocci every month. Yet, the agency has a whole campaign dedicated to West Nile and it publishes information about it in 11 languages. It provides an online map that shows data as current as three days earlier.
Federal agencies also have paid little attention to valley fever on the research side. The National Institutes of Health has neglected research funding on valley fever for decades.
Since 2000, 1,287 projects received a total of $585 million from the National Institutes of Health for work involving West Nile. Valley fever projects have received about 4 percent of that amount – $25 million over the past 12 years. The impact on human health is nearly the reverse. Valley fever has stricken about four times more people than West Nile virus, with thousands more going undiagnosed. It has killed many more people, too.
States have had very different responses to the disease.
In 1997, Arizona required all physicians and testing laboratories to report any cases of valley fever to the state so it could accurately track it. After repeated studies showed that most valley fever cases were not being diagnosed, Arizona policymakers changed the guidelines to encourage better diagnosis and tracking of the disease in 2008.
California did not make the change in reporting requirements until 2010, and the state has yet to change the diagnosis guidelines or develop a surveillance system that allows it to follow trends in the disease easily. Dr. Gil Chavez, the state’s chief epidemiologist, explained that the state only has access to data on valley fever as far back as 1990. And those are just paper records. From 2000 forward, the state started making annual compilations of electronic data, but none of the databases are linked.
“I don't have a single dataset that I can query and give me everything for the last 20 years,” Chavez said. “I would have to go through every year, and every one of those queries requires a lot of staff time. It’s just something that we don’t do very often.”
Reporters tried to interview state researchers who apparently are working on a valley fever project funded by the CDC but were sent a form email from the California Department of Public Health that said: “CDPH does not discuss studies that are ongoing.”
Perhaps it’s not surprising that the state health department has not issued any warnings or press releases about valley fever in the previous five years. No major political figure in California has sounded the alarm about the rise in cases, either.
In August, the mayor of Dallas, Mike Rawlings, declared a state of emergency in Texas because of West Nile virus. There have been 43 deaths in the state, the most on record. “I cannot have any more deaths on my conscience because we did not take action,” he said.
Unlike West Nile, though, no one is keeping a careful count of valley fever cases.
“For the areas affected, the impact of this disease is every bit as important as polio before the vaccine or chicken pox before the vaccine,” said John Galgiani, the director of the Valley Fever Center for Excellence in Arizona. “The numbers are going up and no one’s talking about it. Those communities hurting the most should be pushing the hardest for action to be taken.”
Yesenia Amaro and Joe Goldeen contributed to this report.