10-year-old carries scars of valley fever

Delayed diagnosis gave disease time to spread

Reporting on Health CollaborativeNovember 10, 2012 

Jayden Lugo has had 56 surgeries in her short life.

The 10-year-old from Wasco in the Central Valley has brain damage, uses a walker to get around, undergoes therapy once a week and takes three pills every night before she goes to bed.

Jayden was born healthy but she contracted coccidioidomycosis, or valley fever, becoming one of an estimated 150,000 adults and children a year who get the disease, prevalent in California and Arizona.

What happened next illustrates the impact valley fever can have when it is not diagnosed early enough.

"A simple blood test would have prevented all of these. That's what makes us so mad," said her mother, Jillian Lugo. "She would have been a totally different kid."

That blood test is just part of the problem. Public awareness of the signs and symptoms of valley fever is low nationally, even in the Southwest where it is common.

Doctors too often diagnose influenza or a bacterial infection. They don't think of valley fever until the disease has progressed and the patient is very sick. Although a blood test would have caught Jayden's valley fever earlier, even the available tests are inadequate because they take too long to produce results.

Public doesn't recognize disease

Jayden was 2 months old in October 2002 when her breathing became labored, she developed a bad cough and her temperature started to rise. Her parents thought she was coming down with the flu or a cold.

"Being at that age, I thought that this was basically her first time getting sick," her mother said. So the family didn't worry.

Jillian Lugo's response is common. When valley fever strikes, most people fight it off without even developing symptoms. So many people are exposed to the fungal spores in California that even in the coastal areas where there are relatively few cases, experts estimate that millions of people are infected.

Dr. Steven Larson, a Riverside infectious disease specialist and member of the California Medical Association's executive committee, said widespread exposure has been documented using skin tests, even in areas outside of the Central Valley, the valley fever hot spot.

This means, for example, "probably more than one-half of the population of Orange County that's lived there more than 10 years will have acquired antibodies to this infection," Larson said.

If people do get sick, the symptoms often mimic those of other common illnesses such as a cold or the flu.

Public health departments in California do not include valley fever as part of regular awareness campaigns, like that of the influenza virus. The disease doesn't generate intense media coverage either, unlike the recent outbreak of fungal meningitis. People don't think to ask their doctors to test for the disease.

When Jayden came down with flulike symptoms, the state was in the middle of a spike in valley fever cases unlike anything seen in a decade. In 2000, there were 860 people officially diagnosed with the disease statewide. By 2002, the number had more than doubled -- to 1,727.

But state and federal public health officials did not issue any warnings about the disease.

The rise in cases wasn't even mentioned in the news media, according to media databases. Jayden's parents say they had heard of valley fever when doctors finally diagnosed it in their daughter, but they didn't know much about it.

If someone lives in an area where valley fever is common or has recently traveled there and he or she suffers from cold or flu symptoms that persist for more than a week, the person should visit a doctor and ask about the possibility of a fungal infection, said Dr. David Stevens, a Stanford scientist who has worked on valley fever-related studies since the early 1970s.

Stevens, president of the California Institute for Medical Research in San Jose, said delays in seeking medical treatment are one of the main reasons for the most serious cocci cases.

"There's an education need that's out there," he said.

Valley fever often misdiagnosed

When doctors diagnosed Jayden with pneumonia in October 2002, her mother was worried but hopeful. Pneumonia seemed manageable. Short term. Jayden would get better, she thought.

But doctors never tested for valley fever, and so the disease continued to grow and spread throughout her body.

Despite the rise in cases since 2000 -- growing to 6,146 in California in 2011 -- many doctors aren't looking for valley fever. The symptoms can present themselves in so many ways that doctors can suspect rheumatic fever, tuberculosis, even cancer.

And that is the key reason that physicians can miss the signs, according to public health experts and scientists.

Public health officials said doctors need more training in how to identify whether a supposed flu or pneumonia may actually be valley fever. They point to the gap between the number of people suspected of having valley fever -- 150,000 nationwide -- and the number diagnosed with the disease, about 20,000, according to the Centers for Disease Control and Prevention.

The disease, once confined to the Southwest, now has cases reported in every state due to increased travel and for reasons that researchers are trying to understand.

Physician groups and hospital systems are starting to wake up to the problem. Kaiser Permanente announced it will hold a Kern County physician education session focused entirely on valley fever. Its guest speaker is Dr. Navin Amin, a Kern Medical Center physician and valley fever expert.

"If (patients) are sweating ... or they develop a very high fever and headache, of course that should give some clue to think about valley fever," Amin said. "Valley fever can present in a lot of different ways and in a lot of different manifestations. For every patient who comes in, valley fever should always be on the back of their minds."

Amin said the key signs are:

High fever

Shortness of breath

Fatigue

Skin problems, such as rashes and skin ulcers

Headache and sweats.

A study in the November issue of the CDC's Emerging Infectious Diseases journal shows 3,089 documented deaths nationwide between 1990 and 2008 in which valley fever was an underlying or contributing cause. The new research nearly doubles the previous CDC estimates and researchers said they believe many more cases go undiagnosed.

Jayden's condition didn't improve for five months after her diagnosis, but her doctors didn't consider valley fever as a possible culprit.

Her condition was worsening week after week. Fluid was building up around her brain, and after doctors put in a shunt to drain the fluid Jayden developed an infection.

Doctors should have rethought the pneumonia diagnosis and looked for what was causing Jayden's health to fail, valley fever experts say.

"In an area where the disease is common, a case of pneumonia should always awaken the suspicion of coccidioidomycosis and prompt the doctor to request the adequate tests," Dr. Rafael Laniado-Laborin, a valley fever specialist in Tijuana, Mexico, said in Spanish.

"If it's not suspected and the patient isn't getting better with conventional antibiotics, the possibility of a fungal infection should be considered and studied," he said.

Screening tests too slow

It wasn't until July 2003, nine months after her first symptoms, that doctors determined Jayden had valley fever. By this time, Jayden had developed multiple infections, according to her medical records.

Tests using her spinal fluid showed signs of a bacterial infection, and doctors found the coccidioidomycosis fungus growing in her abdomen, records show.

"(Doctors) told us that she probably wouldn't make it because she was so sick," Jillian Lugo said.

They could have found out many months earlier with a blood test.

If doctors suspect valley fever, they can use blood tests, microscopic examinations of body fluids or tissue, or cultures grown in labs to determine its presence in a patient. Those tests generally are accurate. The trouble is time.

There are very few labs nationwide that perform these tests, and it can take from a few days to several weeks to produce results.

Even a few days of going undiagnosed or being misdiagnosed can have a significant impact on the final outcome of the patient, said Dr. Arash Heidari, assistant clinical professor at UCLA and infectious disease specialist for Kern County and Kern Medical Center.

Patients and doctors may think a patient has pneumonia, but, because no tests have been done to identify valley fever, the fungus can spread and cause meningitis, a dangerous swelling of the brain.

"A simple pneumonia is now a meningitis," Heidari said.

Just a small delay could make the difference between a patient being treated for a few months to having to take antifungal medications for the rest of his or her life, Heidari added.

Delay was the enemy in Jayden's case. She wasn't even tested for the disease initially. Then, when she was tested, her family had to wait for the results. By the time she was diagnosed with valley fever it had spread to her brain, her mother said.

Doctors could be helped in other cases if a quick test was available to show whether valley fever was a possible cause of illness.

Larson said new tests are becoming available that can identify within hours whether a patient has valley fever. And unlike the slow, older lab tests, which could expose lab workers to the disease, the new tests don't carry such a risk. They also tell doctors exactly what type of fungus is causing the disease, Larson said.

The new tests, when widely available, will "revolutionize" the ability of doctors to make diagnoses, he said.

Better tests are needed to "ensure that people get that right treatment, so we are not inappropriately treating patients, not putting them on a whole bunch of drugs they don't need to be on," said David Engelthaler, director of programs and operations for TGen North, a company in Arizona hoping to license a new rapid test.

Over the past nine years, Jayden has suffered many complications and infections, resulting in 56 surgeries by her mother's count.

For example, when she was 3 years old, doctors had to put her on an intravenous medication when the antifungal drugs she took proved ineffective. But there were no pediatric nurses able to go to the Lugo's house to administer the IV. Instead, Jayden had to undergo surgery to place a central line in her chest so her mother could do it.

The central line then caused a blood clot in Jayden's heart, which required another operation.

Also, when she has complications from the disease or from the procedures to treat the disease, she has to have a tube placed in her head to drain the fluid from her brain, Jillian Lugo said.

Jayden will have to take antifungal drugs for the rest of her life.

Kellie Schmitt and Rachel Cook contributed to this report.

JUST ONE BREATH

Another in an occasional series about valley fever.

ON THE WEB

www.reportingonhealth.org/valley fever

ABOUT THE SERIES

Why are people still dying from valley fever and tens of thousands getting sick? Misdiagnosis. A lack of public awareness. And a long history of inaction by government agencies. In this series, we will explore the startling rise of cases, the science of studying the disease, the high costs to patients and taxpayers, the weak federal and private interest in funding treatments and vaccines, and the public health response.

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