I. THE OUTBREAK
On October 2, 2008, the California Department of Public Health (CDPH) issued a report linking an outbreak of Campylobacter illnesses to unpasteurized milk from Alexandre Eco Farms Dairy. The report was the result of an investigation commenced on July 14, 2008, when Dr. Thomas Martinelli, the County Health Officer for Del Norte County, California reported four cases of laboratory confirmed Campylobacter infections and five additional cases of diarrhea in Del Norte County residents. Eight of the original nine sick individuals were members of the Alexandre Eco Farms “cow-leasing” program. Eight of these individuals had consumed milk produced on the farm. The ninth sick individual worked with cattle on the Alexandre Eco Farms Dairy. One of the eight individuals who were sick, Mari Tardiff, had already been hospitalized with GBS, following the onset of acute gastroenteritis after consumption of the milk.
As part of the investigation, health department officials retrieved a refrigerated carton of partially consumed Alexandre Eco Farms milk from Mari Tardiff’s home. Mari had consumed a portion of the milk before her illness. The specimen tested positive for Campylobacter jejuni DNA using a test called polymerase chain reaction (PCR). Testing indicated that multiple strains of Campylobacter jejuni were present in the milk. Del Norte County officials eventually identified 16 cases of Campylobacter jejuni associated with the outbreak. Fifteen of those were persons who consumed milk from Alexandre Eco Farms Dairy. The 16th case was the farm employee. CDPH and Del Norte county officials concluded that “the available epidemiologic and laboratory data support the conclusion that this cluster of acute diarrheal illness in Del Norte County was an outbreak of C. jejuni infections caused by consumption of unpasteurized milk from [Alexandre Eco Farms Dairy.]”
The causal link between Alexandre Eco Farms Dairy and Mari’s illness was so clear, and her injuries so remarkable, that the physicians that treated her published a report on her case in the medical journal. “Investigation of the First Case of Guillain-Barre Syndrome Associated with Consumption of Unpasteurized Milk – California, 2008.” Amy K. Earon, T. Martinelli, W. Miller, C. Parker, R. Mandrell, D. Vugia. The authors explained the laboratory methods used in investigating Mari’s illness:
We reviewed the patient’s medical record and interviewed her husband to assess her symptoms and exposures. We used polymerase chain reaction (PCR) and multilocus sequence typing (MLST) to test a six-week old unpasteurized milk sample, obtained from the cow leasing-program and partially consumed by the patient, for genes encoding the bacterial membrane component lipooligosaccharide (LOS) in GBS-associated Campylobacter jenuni.
In addition to the DNA testing, the authors also tested Mari’s blood for anti-bodies to GBS. The authors then explained that the PCR and MLST testing of the milk detected Campylobacter jejuni gene. In addition, the blood test was positive for anti-bodies that indicated the presence of GBS. The authors concluded, “Combined laboratory and epidemiologic evidence established the first reported association between GBS and unpasteurized milk consumption.”
II. MARI TARDIFF’S ILLNESS
On the weekend after Mari drank raw milk, she developed flu-like symptoms, including diarrhea and vomiting. By Thursday, June 12, the food poisoning was overwhelming her body with an amazingly swift force. First her vision blurred. Then her hands went numb. Mari went to an emergency room, and there lab work was done and abdominal X-rays were taken. But doctors could not determine what was wrong. On Friday, Peter took Mari to a neurologist. An MRI was normal but the doctor and radiologist mentioned a frightening possibility – Guillain-Barré syndrome, or GBS, a potentially fatal inflammatory disorder.
Hours later Mari’s legs were on fire, searing with pain that, ironically, only hot water helped to soothe. Her legs hurt so much that she soon retreated to bed, wrapping her legs in warm towels and a heating blanket. During that night, Mari awoke and realized she could not move. Peter bear-hugged her to lift her to the toilet and then carried her back to bed. In the early hours of the morning, he called for help, which led to an ambulance ride to the small Sutter Coast Hospital, and then a medivac flight to the Intensive Care Unit at the larger, better-equipped Rogue Valley Medical Center (RVMC) in Medford, Oregon. She remained hospitalized for two and one-half months.
Mari was moved to Redding Rehabilitation Hospital and was finally able to come home on November 1, 2008. Today, Mari lives in her family room, which now is equipped with a hospital bed, portable toilet, a Hoyer lift and a stand-up frame, all purchased by the Tardiffs. Using their own resources, they also renovated a downstairs half-bath and laundry room into a handicapped-accessible bathroom and shower. The Tardiffs pay two nurses $10.50 an hour to care for Mari from 7:30 A.M. until 7:00 P.M. five days a week while Peter is at work. Home health physical and occupational therapists also come to the house five days a week.
Mari works very hard at therapy but it is a slow, painful process. Peter has found it so upsetting that he no longer can watch. Every improvement is celebrated, but he knows how much discomfort and frustration goes into each minute, regained movement. Mari may never walk again. She lost her job, she lost her dreams and plans that she held dear. The illness has been a long, arduous journey for Mari, her family and friends, and while she has made progress, there remains a long way to go.
READ MORE ON CAMPYLOBACTER AND GULLAIN BARRE SYNDROME
Campylobacter jejuni (pronounced “camp-e-low-back-ter j-june-eye”) is a bacterium that was first recognized as a cause of human gastrointestinal illness in 1975. Since that time, the bacterium has been identified as the most common cause of bacterial foodborne illness in the U.S., ahead of Salmonella – the second most common cause (MMWR Weekly, 2000, March 17; Tauxe, 1992).
Over 10,000 cases are reported to the Centers for Disease Control and Prevention (CDC) each year; however, many more cases go undiagnosed or unreported and estimates are that Campylobacter causes 2 to 4 million cases per year in the United States. Active surveillance for cases indicates that over 20 cases for each 100,000 persons in the population are diagnosed yearly, and 124 deaths are attributed to C. jejuni annually (CDC, 2005, October 6). Current estimates are that each case of campylobacteriosis costs $920 on average due to medical and productivity (lost wages) expenses with an annual total cost of approximately $1 billion (CAST, 1994).
The CDC reported that the incidence of Campylobacter infection decreased by 30 percent in the ten-year period between 1996 and 2006 (MMWR, 2007, April 13).
Campylobacter jejuni is a gram-negative, microaerophilic, thermophilic rod that grows best at 42°C (107°F) and low oxygen concentrations. These characteristics are adaptations for growth in its normal habitat – the intestines of warm-blooded birds and mammals. Several closely related species with similar characteristics, C. coli, C. fetus, and C. upsalienis, may also cause disease in man but are responsible for less than one percent of human infections annually (CDC, 2005, October 6).
Food is the most common vehicle for the spread of Campylobacter, and chicken is the most common food implicated. Contamination occurs during animal slaughter and processing when it comes into contact with animal feces. Ingestion of as few as 500 organisms – an amount that can be found in one drop of chicken juice – has been proven to cause human illness (FSIS, 1996; Tauxe, 1992). Despite this low infectious dose and the prevalence of Campylobacter jejuni in the environment, most cases of Campylobacter infection occur as isolated, sporadic events, and are not usually a part of large outbreaks. But, very large outbreaks (>1,000 illnesses) of campylobacteriosis have been documented, most often from consumption of contaminated milk or unchlorinated water supplies.
Identified common food vehicles for Campylobacter, in addition to poultry include unpasteurized milk, undercooked meats such as beef, pork, lamb, and livestock offal, and occasionally shellfish, fresh produce, and eggs.
IV. GULLAIN BARRE SYNDROME
Guillain-Barré (ghee-yan bah-ray) syndrome (GBS) is a disorder in which the body’s immune system attacks part of the peripheral nervous system. The peripheral nervous system includes the cranial nerves (except the optic [eye] nerve), the spinal nerves, and the autonomic nervous system that governs involuntary actions. The central nervous system is the spinal cord and brain.
GBS includes several subtypes, the most common of which is a multifocal demyelinating disorder of the peripheral nerves referred to as an acute inflammatory demyelinating polyneuropathy (AIDP). Some cases of GBS are associated with a primarily motor axonal process (acute motor axonal neuropathy; AMAN) with axonal degeneration (axons are long, thin extensions of the nerve cells and carry nerve signals) and sparing of the myelin (the myelin is an electrically insulating phospholipid layer that surrounds the axons of many neurons). Other cases appear to involve both sensory and motor axons and such cases are termed acute motor and sensory axonal neuropathy (AMSAN). More than 90% of patients with GBS in Europe and North America have AIDP. AMAN occurs in less than 10% of persons with GBS in the western hemisphere but in more than 40% of those affected in China and Japan. The incidence of AMSAN is very low (less than 10% of that of AMAN). Miller Fisher syndrome (MFS) is another GBS variant that occurs in about 5% of people affected by GBS. It is characterized by opthalmoplegia (eye muscle weakness), areflexia (absence of reflexes), ataxia (the inability to coordinate voluntary muscular movements such as walking), and, in some cases, facial and bulbar palsy (affecting vital functions, like breathing, and swallowing or speech).
GBS can affect anybody. It can strike at any age; men may be more likely to develop GBS than women. Although this syndrome is rare (affecting about one to two persons in 100,000) it is the most common cause of acute neuromuscular paralysis in the world.
GBS often occurs a few days or weeks after a person has had symptoms of a respiratory or gastrointestinal viral or bacterial infection; in fact, two-thirds of affected individuals have had a preceding infection. Campylobacter jejuni, cytomegalovirus, Epstein-Barr virus, and Mycoplasma pneumoniae are commonly identified antecedent pathogens. Campylobacter jejuni is the most common pathogen that elicits GBS. Occasionally surgery or vaccinations will trigger the syndrome. GBS is not contagious. It has been reported that GBS occurs more in men than in women and more often in the elderly. Seasonality has not been reported in developed countries like the United States (Church Potter & Kaneene, 2003).
No one yet knows why GBS strikes some people and not others. Nor does anyone know exactly what sets the disease in motion. What scientists do know is that the body’s immune system begins to attack the body itself, causing what is known as an autoimmune disease. Usually the cells of the immune system attack only foreign material and invading organisms.
In GBS, the immune system starts to destroy the myelin sheath that surrounds the axons of many peripheral nerves, or even the axons themselves. The myelin sheath surrounding the axon speeds up the transmission of nerve signals and allows the transmission of signals over long distances. In diseases in which these myelin sheaths are injured or degraded, the nerves cannot transmit signals efficiently. That is why the muscles begin to lose their ability to respond to commands from the brain that must be carried through the nerve network. The brain also receives fewer sensory signals from the rest of the body, resulting in an inability to feel textures, heat, pain, and other sensations.
Alternately, the brain may receive inappropriate signals that result in tingling, “crawling-skin,” or painful sensations. Because the signals to and from the arms and legs must travel the longest distances, they are most vulnerable to interruption. Therefore, muscle weakness and tingling sensations usually first appear in the hands and feet and progress upwards.
Guillain-Barré syndrome can be a devastating disorder because of its sudden and unexpected onset. Most people reach the stage of greatest weakness within the first 2 weeks after symptoms appear, and by the third week of the illness 90 percent of all patients are at their weakest. The recovery period may be as little as a few weeks or as long as a few years. About 30 percent of those with Guillain-Barré still have a residual weakness after 3 years. About 3 percent may suffer a relapse of muscle weakness and tingling sensations many years after the initial attack.