Opinion | Are You Ready to Diagnose Valley Fever?

Last month, on a pleasant spring day, my neighbor Matt* was about to teach his 3-year-old granddaughter how to somersault. But just as the wiry, fun-loving man crouched on his lawn, his wife frantically shook her head, waved her hands, then shouted: “Your shunt!”

Darn! thought Matt. She’s right. He then did a shoulder roll to better protect the precious tube in his neck. Little Charlotte was none the wiser of course, but for Matt, painful memories of the worst-ever illness of his life came flashing back once again. This illness not only led to months of the worst-ever headaches of his life, it also bought him a ventriculoperitoneal (VP) shunt plus a forever course of anti-fungal pills.

“You’d better stay on those pills,” Matt’s neurosurgeon warned after he finally felt well, albeit 25 lb lighter than his pre-fungal self.

The denouement? Some people reading this column, especially those in the arid Southwest, already know Matt’s perp. Others know that — even without classic risk factors for dissemination to multiple organs (e.g., dark-skinned ethnicity, immunosuppression, or diabetes) — Matt could still suffer a dire, extra-pulmonary complication of the infection commonly called Valley Fever (VF).

But here’s what otherwise savvy doctors may not know. According to climate modeling experts, Coccidioides immitis, the soil-borne fungus that causes VF, may soon expand its domestic habitat well beyond California, Arizona, New Mexico, Nevada, Texas, Utah, and southwestern Washington state to as far north as northern Montana and as far east as western Minnesota.

So, readers, be forewarned. Perhaps C. immitis, often nicknamed “coxy,” may one day waft its barrel-shaped arthroconidia into patients near you.

Coxy Most Murderous

Now for a fast clinical primer. Acute VF has many hallmarks from virtually nothing to erythema nodosum to extended fatigue, dyspnea, arthralgias, and a persistent, hacking cough. But disseminated coxy in skin, bones, brain, you name it, is far worse.

Worst of all is meningitis, a dreaded complication that once required ongoing intra-cisternal instillations of amphotericin B. In fact, when I first moved back to Los Angeles in the 1980s to serve as the sole infectious diseases specialist at a public hospital in the San Fernando Valley, I inherited a patient of Hispanic descent who was regularly receiving this tricky treatment to the base of his skull via blind, cervical taps. What can I say? He was happy to be alive, and my predecessor was one gutsy doctor. I arranged for the patient to continue his taps with a neuroradiologist.

Glenn Mathisen, MD, my long-time successor at UCLA-Olive View Medical Center, also bravely did a few cisternal taps, then switched to Ommaya reservoirs for the delicate injections. But he has now treated scores of patients with coxy meningitis with newer, oral drugs. His experience led to a seminal monograph that compared clinical features in 60 coxy meningitis patients treated both before and after oral imidazoles like fluconazole (Diflucan), voriconazole (Vfend), and posaconazole (Noxafil) became available. But no matter what they received, 30% of patients in both cohorts required VP shunts for coxy-induced hydrocephalus.

The new drugs plus shunts are life-savers, Mathisen recently said, but they can also prove challenging, especially in county hospital patients. Several in his clinic “get regularly admitted,” he added, recalling one man in particular with recurrent mental health issues. “We always have to determine: is it a breakdown of the shunt? Does he need a shunt revision? Or is it just a problem with some of his meds?”

Most recently, a psychotic break followed the patient’s switch to voriconazole. Fortunately, his shunt was fine, and he is now tolerating a different drug.

Geography Is Destiny, But So Are High-Risk Settings and Hosts

It was my neighbor Matt who reminded me of “Death Dust,” a 2014 New Yorker article that details many factors fueling California’s ongoing surge of coxy. Author Dana Goodyear first cites The Tempest from Tehachapi, a dust storm that “spread dirt over an area the size of Maine,” eventually infecting residents of Sacramento, 400 miles north of where the storm hit. Disrupted soil due to vast tracts of newly-constructed housing both in Southwest desert cities and places like Lancaster and Antelope Valley — communities near Mathisen’s hospital — are other factors fueling the steady uptick in cases Mathisen and other experts have personally witnessed over three-plus decades.

In addition, every California infectious diseases doctor knows the hazards posed by burgeoning prisons in the San Joaquin Valley, for which VF was originally named. Because many inmates are people of color who, according to certain historical observations and studies, could be genetically predisposed to disseminated infection, they often face double jeopardy. “If you get sent to one of those prisons in the Central Valley,” Mathisen ruefully shared, “it’s like you serve two sentences…not just your [incarcerated] time, but the risk of lifelong disseminated coxy.”

Of course, the list of modern risk factors goes on. Organ transplantation can awaken old, dormant infection, as can HIV/AIDS, treatment with steroids, or simply advanced age leading to less robust immunity. Screening blood tests for prior exposure to C. immitis makes sense for many at-risk individuals, but this approach is often woefully under-used, as shown by a recent study of Medicare patients in Arizona on immunosuppressive, anti-rheumatic drugs.

Personal Coda

I barely knew Matt when he first fell ill after his annual trip to a heavenly, central California grassland called the Carrizo Plain where the soil is heavily freighted with C. immitis. But over the years, we’ve bonded, sometimes chatting about our dogs or something equally mundane; all the while, I couldn’t help noticing how thin my neighbor was.

Then one day, he generously shared details of his saga, and I realized anew what a life-changing illness he’d endured. Not that he was complaining. Despite his chronically diminished appetite and his pivot from basketball and tennis to pickleball (“lighter racket, lighter ball, less impactful on your body,” he said), Matt remains upbeat, wise, and most of all, thankful.

“Until 2017, I was always a very fit, athletic guy. I’m in my 50s, I’m Superman! I don’t get problems like this,” he exclaimed with a certain irony.

“But I do sort of count my blessings,” he continued. “While it’s not great, I’m on the right medication. Like my neurosurgeon said: ‘You don’t take it, you’re going to get sick and die.’ And so that makes me feel like, ‘Okay, I’m good.'”

*Person’s name has been changed for privacy.

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