When Juan Solis shuffles out of his dark bedroom, he’s careful not to get too close to the windows.
He makes sure he only walks his dogs at night.
If he must go out during the day, he lathers on sunscreen, makes sure his legs and arms are covered, even during the peak of summer in Bakersfield’s blistering heat. And he never forgets his sunhat.
Juan has extreme light sensitivity, caused by valley fever. He contracted the respiratory disease in 2008, but he was misdiagnosed for so long that he developed complications. The coccidioidal fungus that causes valley fever spread to his bloodstream, causing cocci meningitis, a lethal illness. If left untreated, it kills about 95 percent of patients in the first two years. The only drug that worked to help control his symptoms was Voriconazole, or VFend, an antifungal drug. There are two problems, though. The drug can cost more than $900 a month. And it has severe side effects, including an outbreak of cancerous lesions on Juan’s skin.
“We can’t do anything except keep taking these pills that are in turn killing you, but it comes down to this: do you want to die really fast or die slower with these pills? You get to pick,” Juan’s wife, Julie, said.
Juan chose to die a little slower. Julie has been hoping for a cure. Valley fever, which is endemic to much of the southwest United States and contracted by the simple act of breathing, has no effective treatment, no vaccine, no antifungal medication that comes without side effects. The extreme light sensitivity experienced by Juan, which in turn has led to his skin cancer, is a very severe side effect for a valley fever patient, and yet, with so few options, Juan knows he likely will be taking those drugs for the rest of his life.
***
Juan had a fever that would not abate, but nobody knew why.
Extreme fatigue took its toll, so Juan stopped going to his job designing trusses for residential homes. His bosses told him to stay home and to come back when his health was in order.
He lost 50 pounds in about two months. But none of the doctors he saw could give him a diagnosis that explained what he was feeling. One recommended going to the dentist for a root canal. Another one suggested an optometrist, wondering if the pressure Juan felt in his head was from eye strain. When Julie asked her own doctor about Juan’s symptoms, she was told he probably had pneumonia.
Meanwhile, the cocci fungus was disseminating into Juan’s bloodstream. Juan’s fever was rising so high he became delirious. He would stumble down the hallway to his easy chair in the mornings and lay there until his wife and kids came home in the afternoons.
Marisol, Juan’s then-6-year-old daughter, would come home and see her once-energetic father, who had coached her softball team, resting in his recliner, pale, sick.
“Is daddy dead yet?” Marisol would ask.
For six months, nobody thought to diagnose Juan with valley fever.
Then Julie found her husband passed out and slumped over in his chair. It was Father’s Day.
When they got him to San Joaquin Community Hospital (now called Adventist Health Bakersfield), the doctor drew a vial of spinal fluid, diagnosed Juan with coccidioidal meningitis and said he probably wouldn’t make it through the night.
That’s a night Juan’s kids and wife still refer to as “the end.” It’s the night Juan was supposed to die.
He pulled through, but the disease progressed to the point where Diflucan, the standard antifungal used to treat valley fever, would not work.
“The majority of people don’t get it in the spinal column, and unfortunately, we don’t have a cure or medication,” said Claudia Jonah, health officer at the Kern County Department of Public Health Services. “Just a small percentage of patients get something that severe, but they have it for the rest of their lives.”
Juan’s doctor prescribed Voriconazole, a drug that saved Juan’s life but has been robbing him of his health.
Figuring out a drug’s value when it becomes unaffordable
The costs were staggering, both financially and emotionally. Julie and Juan’s insurance initially didn’t cover the full cost of the drugs, and they were paying $900 for Juan’s 800 mg daily dose of Voriconazole. They drained $10,000 they had in a certified deposit and sold ATVs, a boat, furniture and, eventually, their home to make bills.
Juan, unable to return to work, signed up for social security benefits and got on Medi-Cal, the state insurance plan.
“Obama’s my homie,” Juan says. Without the Affordable Care Act, he would not be able to afford his medication, which now costs than less than $100 a month.
The medication keeps valley fever at bay, but Juan now faces a new challenge: skin cancer.
***
Juan’s dermatologist warned him to avoid the sun.
That’s tough for a guy who used to coach his three kids’ volleyball, softball and baseball teams. He doesn’t like to project himself as a sick man. At a recent valley fever fundraiser, he leaned back in his chair, ate pizza and laughed and played with his 4-month-old nephew. You wouldn’t know he was sick.
But that’s not always the case, his 15-year-old daughter, Daisy, said.
“Some days, he’s off. When he won’t get out of bed, I think about those days and I wonder if he’ll make it,” Daisy said.
Her family hasn’t had a real conversation about that possibility, though, she said.
When it does come up, Juan tells his kids the same thing his doctor tells him: “as long as you keep taking these pills, you’re good.”
This theory was put to the test last spring. There was a problem with Juan’s social security benefits. It took two months to work out the confusion, and money was getting thin. Juan started splitting his pills in half.
“Maybe it’s dormant?” Juan said he thought at the time.
It took about three weeks of splitting pills before Juan relapsed and was back in an emergency room. That visit cost $27,000.
Julie had heard of West Nile virus, which in 2008 infected two people in Kern County. She had never heard of valley fever, which that year infected more than 1,100 and left six dead — her husband nearly became the seventh.
Why were doctors misdiagnosing valley fever? she wondered. And why hasn’t there been a broad public awareness campaign?
She calls it a “dirty little secret.”
Juan, soft-spoken, is a little more understanding.
“I don’t blame it on the doctors because they didn’t know,” he said of his misdiagnosis.
But Julie knows that if her husband had been diagnosed sooner, he wouldn’t be in the situation he’s in. His daughter, Marisol, tested positive for valley fever shortly after Juan got sick. Her symptoms subsided after being treated for one year with an antifungal drug.
***
Burning off lesions
When the electric needle hit Juan’s skin, it smoldered. A plume of smoke lingered in the air. He shut his eyes and gripped his wife’s hand. It was all Julie could do to keep from crying.
A little orange flame from the needle flickered across Juan’s ear, turning his skin black.
These doctor’s visits, which range in cost from $100 to $150, have become routine, along with visits to the neurologist, infectious disease specialist and general practitioner.
But the dermatologist Juan visited on this particular day — the day before Thanksgiving — is his favorite. David Elbaum burns and scrapes the lesions off Juan’s face and body.
That day, a spot of squamous cell carcinoma that looked like a wart on his ear was bothering him. It kept him from sleeping on his right side. The lesion on his upper cheek bothered Julie because of how close it was to his eye. Elbaum zapped that one with liquid nitrogen.
It’s all a result of the medication, which was never developed for valley fever but has been used off-label for years to treat the disease. Clinical trials indicated just 2 percent of patients would develop photosensitivity, but those trials were conducted in controlled settings. In the real world, experts in Kern County say it could be as high as 30 percent.
Vfend’s side effects have distinguishing features, such as the freckles that develop up and down the arms of patients like Juan, or the chronic ulcer on the lower lip he sees on every valley fever patient who takes the medication. Juan had lip reconstruction a few years ago to get rid of his.
“This is the first drug I’ve ever seen do this. Some drugs cause photosensitivity, but because this is a lifesaving one, we can’t stop it,” Elbaum said. “People on Vfend in Bakersfield are on it for life. This disease is in their brains, their bones.”
As Juan recovered after his procedure, Elbaum answered a question Julie never thought to ask: if there’s a breakthrough in research and an effective medication comes to market, will Juan’s skin cancer subside? Will Julie get her husband back?
Elbaum was direct.
“He’s so far past it,” he said. “If he immediately did get cured, he’s had so much sun damage, it will not go away because of medication.”
Walking out of the office, Julie wiped tears away, still in a state of shock.
“I really thought that if they found a cure, the skin cancer would subside,” she said.
Juan tried to console her. Tried to convince her that it would be better than nothing. They could still make the valley fever go dormant, he told her. They could make it go to sleep. Maybe then, he would have just one disease to battle instead of two.
“It’s just my life now,” Juan said.
We continue our reporting this week on the fungal disease known as valley fever with a story about a potential route to prevention. One of the first lines of defense against any disease is determining who’s at risk. It’s possible to develop immunity to valley fever, and a new skin test could be used to screen for that immunity—but that’s only if the test overcomes some major hurdles.
Faith Herrod wants to be a veterinarian when she grows up. The 11-year-old lives in the small Central California town of Lemoore with her family, three dogs and three cats. Someday, she’ll get a rabbit, too—as soon as her mother lets her.
In her free time, Faith should be out playing with her dogs. But for almost a year, she was not able to do so. She would come home from school at 4 p.m. and go right to bed. That’s because last October the sixth-grader was diagnosed with valley fever, a fungal infection that kept her out of school for months and left her with regular headaches and chronic pain.
“Sometimes, when you get super tired, it'll feel like your ribs will go in,” she said, wrapping her arms around her stomach. “It'll feel like your ribs go in and hurt really bad.”
Faith contracted valley fever by breathing in fungal spores carried by the wind. That’s how the disease is contracted, and it can happen at any time. Faith’s mother, Caren Herrod, isn’t entirely sure when it happened, but her best guess is while they were doing yard work one day—something they had done dozens of times before. Herrod never imaged that, after so much time, Faith would not have built up natural immunity and that she could still be at risk.
“If I had known that she was susceptible, it would've been different,” Herrod said. “We would've done things differently.”
As it turns out, Faith and her mother could have known. A new skin test called Spherusol can detect whether a person has developed natural immunity, meaning they’ve overcome valley fever before. Because most valley fever cases are asymptomatic, many people whose immune systems have battled the disease may never know it.
Advocates are excited about the test. So are doctors — like Dr. John Galgiani, director of the University of Arizona’s Valley Fever Center for Excellence. He dreams of seeing Spherusol being used as a tool to screen for past infections.
“I think that Spherusol's best use will be in primary care doctors' offices, to test their patients on a routine basis to find out if they've indeed previously had valley fever,” Galgiani said. If patients knew they had never conquered valley fever, they could better prepare themselves against it; and doctors might be more likely to diagnose the disease if patients showed unusual symptoms.
Experts call for a change in FDA rules on promising test
But despite its promise, the test isn’t in wide use.
“Frankly, I don't use it very often myself,” Galgiani said. “Even specialists don't use it very often.”
That’s because the Food and Drug Administration hasn’t approved Spherusol for testing immunity. Instead, the test is supposed to be used by clinicians only after a person has been diagnosed with the disease. Galgiani and others would like to see the FDA change the rules to allow its use whenever a clinician thinks a test is warranted.
“If the labeling is changed to allow the test to be used to test for prior infection, then it opens up a whole different value of the test to the clinical community,” Galgiani said.
Valley fever lurks in dirt and dust in the desert throughout the Southwest. Most people who inhale the spores fight off the disease without ever knowing they had it. Some develop flu- or pneumonia-like symptoms. In rare cases, it can cause severe lung infection or disseminate throughout the body, requiring lifelong treatment or leading to fatal meningitis.
But there is some good news.
“Once you've had valley fever and gotten over it, you are for all practical purposes immune from a second infection,” Galgiani said.
Revealing a disease exposure with a skin test
That’s why Spherusol could have such an impact: It could reveal a person’s history of exposure. Before, that was something most people could only wonder about.
Spherusol works similarly to a tuberculosis skin test. A clinician injects a small amount of spores under the skin, and the reaction indicates whether immunity has developed. Similar skin tests for valley fever were first developed in the 1930s, but the most recent iteration was discontinued in the 1990s after the company that produced it was unable to turn a profit.
Spherusol was released in 2014 and costs about $62. No studies have directly compared its efficacy to previous tests.
Right now, Spherusol is only indicated for use after a patient has already been diagnosed with the disease. So, instead of being used as a way to determine valley fever exposure, it’s considered a sort of immune system checkup for those recovering from the disease.
“It’s indicated for understanding how their body is reacting to the disease,” said Tom Carpenter, president and CEO of Nielsen Biosciences, the San Diego-based company that developed Spherusol. “Is their immune system engaged? Or is their immune system overwhelmed? Or are they potentially immune-compromised and not even able to respond to the infection?”
Carpenter says that screening patients could be a great way to use the test, but getting the FDA to approve a change in the labeling could take years. It would involve new clinical trials and potentially millions of dollars of investment. He says his company is looking into it.
In the meantime, however, he points out that doctors are already allowed to use Spherusol for off-label uses.
“Health care providers right now have the ability to make a medical judgment on how best to use the skin test,” Carpenter said. “So, it’s certainly not preventing them from making that use, but we can’t speak to that use.”
But using Spherusol off-label has challenges —like its price tag. A single test may not break a budget, but regular valley fever screening would mean periodic trips to the doctor to pay $62 for a prick under the skin. Most health insurers in California, including Medi-Cal, say they cover it — but only for its prescribed use. Even then, some insurers could reimburse as little as $4. By contrast, a patient getting a flu shot would likely pay nothing, and the clinic would be reimbursed up to around $35.
And then there’s the fact that the test is packaged in bulk. Spherusol can only be bought in vials of 10 doses; once the vial is open, the countdown to expiration begins. Dr. Royce Johnson, chief of infectious disease at Kern Medical in Bakersfield, says the test is “extraordinarily stable.” Even so, he said, many pharmacies have policies against storing products more than 30 days after they’ve been opened. “If you don't use it all, it costs a lot of money,” Johnson said. “So there’s some resistance to stocking it.”
Another problem? Spherusol is only approved for 18- to 64-year-olds, even though children can be hit hard by valley fever and the CDC says people over 60 are most at risk.
Prisoners benefit from new test
Despite all of its obstacles, the test has been used to screen one very large patient group: California state prisoners, where it appears to be helping to prevent the disease. Prisoners who test negative to Spherusol, which indicates they haven’t built up an immunity to valley fever, are not sent to serve their terms in the two Central Valley prisons hit hardest by the disease.
Caren Herrod may wish that her daughter Faith had access to Spherusol a year ago, but she admits that still wouldn’t have solved the root of the problem: that she didn’t take valley fever seriously enough because so little information about it is available.
“It’s so, so limited,” Herrod said. “With a disease that is so prevalent, for there to be so little information, it’s sad. It's very sad.”
Meanwhile, Faith is back at school full-time, and hopes she can soon take her dogs Moses and Jasmine out for a walk.
Correction:
An earlier version of this story stated that valley fever kills about 95 percent of patients in the first two years. That is true only if valley fever is left untreated. With treatment, the fatality rate drops to 30 percent.
This project results from a new venture – the Center for Health Journalism Collaborative – which currently involves the Bakersfield Californian, Radio Bilingüe in Fresno, Valley Public Radio in Fresno and Bakersfield, Vida en el Valle in Fresno, the Voice of OC in Santa Ana, the Arizona Daily Star in Tucson, La Estrella de Tucsón and CenterforHealthJournalism.org. The collaborative is an initiative of the Center for Health Journalism at the University of Southern California’s Annenberg School for Communication and Journalism.
