Valley Fever: The Mysterious Fungus Infecting the Southwest without a Vaccine or Cure

Valley Fever or Coccidioidomycosis, also known as cocci, is a fungal disease endemic to the soils of the Southwest. Around 60% of those exposed never have symptoms. The majority of the other 40% have flu-like symptoms. About 5 to 10% of those exposed develop serious and long-term problems with their lungs. In about 1% of those exposed, the fungus spreads throughout the entire body, infecting areas such as the brain and bones (1).

Although the first diagnosis was in 1892, Valley Fever continues to infect people without a vaccine or cure. In fact, the number of cases per year is steadily increasing (2). The cocci fungus lives in the dry soil of southwestern United States as well as in parts of Central and South America. Strong winds create dust-storms in these areas that lift spores into the air, exposing people to the infection. The severe forms of the disease last a lifetime, necessitating the continued use of expensive anti-fungals with unpleasant side-effects to prevent growth of the infection. If the cocci becomes disseminated, it can infect the heart, liver, bones, skin, and central nervous system, potentially causing fungal meningitis (4).

People at risk for developing severe forms of Valley Fever include those with weakened immune systems, such as people who have HIV/AIDS or who have had an organ transplant. Additionally, pregnant women, diabetics, and people who are of Black or Filipino descent also have a greater risk (3).

Research on the fungus has been ongoing since the 1930s. In the 1950s, both the U.S. and Russia had biowarfare programs using cocci, programs that were later discontinued because of the difficulty in controlling the spread of the disease (5). However, the pace of the research for a cure or vaccine has been extremely slow.

The slow pace of research for Valley Fever in Big Pharma can be attributed to its low economic incentive due to its status as an orphan disease, which is a rare disease that only affects a small portion of the population.

As an orphan disease localized in only one region of the U.S., Valley Fever has received very little national recognition and funding. As cocci requires the use of Bio Safety Level 3 labs and specialized equipment, the research is also more costly.  The only research is scattered among universities in the Southwest. The research can be dangerous, as lab work is considered to be the second greatest occupational risk for the disease behind farmwork (6).

Some question if the lack of funding is due to the fact that mostly lower class people are affected by the disease. Besides researchers, the highest rate of exposure occurs to farm workers, oil workers, and prisoners, with added likelihood of symptoms in people of Black and Filipino descent. Many in these fields are uneducated and speak little English, making it harder for them to receive the help they need (7).

Orphan diseases received little attention from big pharmaceutical companies until the Orphan Drug Act of 1983, which gave economic incentives to creating drugs to treat orphan diseases, known as orphan drugs. The incentives include clinical research subsidies, enhanced patent protection, and tax incentives (8). Since this act, the FDA has continued to incentivise research for orphan drugs. Currently, the FDA is looking into giving expedited access for approval for drugs and medical devices that target unmet medical needs (9).

Incidentally, these incentives have helped the viability of commercialization of orphan drugs. Orphan drugs benefit from a lack of competition, allowing drug companies to charge higher prices. Due to the enhanced patent protection, these drugs get an extra two years before generic versions become available, allowing the pharmaceutical company to profit on the drug for longer. In 2010, Soliris, the industry’s most expensive drug, cost $409,000 per year of treatment, allowing Alexion Pharmaceuticals to make $541 million in sales on a disease that only four to six thousand people suffer from in the United States (10). Although now more economically viable, orphan drugs still present a risk to pharmaceutical companies and lower returns than conventional drugs.

Perhaps with the advancement in economic viability of orphan drugs and the increase in cases of the disease, the pace of research on Valley Fever will soon increase, allowing for this disease to have a cure or vaccine for the first time since its discovery in 1892.

Endnotes

1. “Valley Fever Symptoms.” cdc.gov. Centers for DIsease Control and Prevention, 2014. Web. 16 Nov. 2014. Retrieved from  http://www.cdc.gov/fungal/diseases/coccidioidomycosis/symptoms.html
2. “Valley Fever Statistics.” cdc.gov. Centers for DIsease Control and Prevention, 2014. Web. 16 Nov. 2014. Retrieved from http://www.cdc.gov/fungal/diseases/coccidioidomycosis/statistics.html
3. “Valley Fever Prevention.” cdc.gov. Centers for DIsease Control and Prevention, 2014. Web. 16 Nov. 2014. Retrieved from http://www.cdc.gov/fungal/diseases/coccidioidomycosis/risk-prevention.html
4. Mayo Clinic Staff. “Valley Fever Symptoms.” mayoclinic.org. Mayo Clinic, 2014. Web. 16 Nov. 2014. Retrieved from http://www.mayoclinic.org/diseases-conditions/valley-fever/basics/symptoms/con-20027390
5. Goodyear, Dana. “Death Dust: The valley-fever menace.” The New Yorker 20 Jan 2014. Web. 16 Nov 2014. Retrieved from http://www.newyorker.com/magazine/2014/01/20/death-dust
6. Goodyear
7. Goodyear
8. “Orphan Drug Act.” fda.gov. U.S. Food and Drug Administration, 2013. Web. 16 Nov. 2014. Retrieved from http://www.fda.gov/regulatoryinformation/legislation/federalfooddrugandcosmeticactfdcact/significantamendmentstothefdcact/orphandrugact/default.htm
9. “Expedited Access for Premarket Approval Medical Devices Intended for Unmet Medical Need for Life Threatening or Irreversibly Debilitating Diseases or Conditions – Draft Guidance for Industry and Food and Drug Administration Staff.” fda.gov. U.S. Food and Drug Administration, 2013. Web. 16 Nov. 2014. Retrieved from  http://www.fda.gov/MedicalDevices/DeviceRegulationandGuidance/GuidanceDocuments/ucm393879.htm
10. “The Economic Power of Orphan Drugs.” thomsonreuters.com. Thompson Reuters, 2012. Web. 16 Nov. 2014. Retrieved from http://thomsonreuters.com/business-unit/science/subsector/pdf/the-economic-power-of-orphan-drugs.pdf