When Jennifer Burton staggered into the bathroom at her home on Beaver Lake early one morning in February 2017 and began throwing up, she didn't realize just how sick she was.
Her husband followed her into the bathroom and turned on the light. Burton began to realize how serious the situation was when her husband gasped and asked, "Have you seen yourself?"
Her face looked like she had been beaten with a baseball bat, and her eyes and ears were nearly swollen shut from a life-threatening anaphylactic reaction. Anaphylactic shock can cause airways to swell shut. The reaction was even more dangerous for Burton because she lives nearly 45 minutes from the nearest hospital.
Burton had been suffering from hives and other allergy symptoms since 2011, but after the incident in 2017, she and her husband were determined to find out what was causing the problem, afraid it would kill her.
They connected with Dr. Tina Merritt Meinholz, a Bentonville-based allergy specialist, who used a blood test to diagnose Burton with alpha-gal syndrome, named for the galactose-alpha-1,3-galactose carbohydrate that causes the allergic reaction.
Alpha-gal syndrome is a beef, pork and mammal allergy that usually develops after being bitten by an Amblyomma americanum tick, commonly known as the lone star tick, as well as other tick species, according to Meinholz. People can develop reactions that range from mild hives to life-threatening anaphylactic shock, she said.
Most allergic reactions happen within 15 minutes of exposure, but reactions to alpha-gal can be delayed up to six hours after the person is exposed to a mammal product, making it difficult to pinpoint the problem, Meinholz said.
It's not enough to avoid eating pork and beef; those with the allergy also have to be careful around a host of mammal products, ranging from common personal and home care products to medicines with binders that contain mammal ingredients and even certain vaccines grown in mammal cells, Meinholz said.
Some people are allergic to dairy, while others are sensitive to cross contamination from food prepared with the same cooking utensils used for mammal ingredients. Some even have reactions caused by the smell of meat being cooked, she said.
Alpha-gal is a carbohydrate present in all mammals except primates, according to Dr. Laura Rothfeldt, state public health veterinarian for the Arkansas Department of Health. It's also in the saliva of lone star ticks, she said. The allergen enters a person's blood stream when a tick bites the person, which can sensitize them to alpha-gal, according to the Department of Health.
Whether a person becomes allergic to alpha-gal after exposure depends on the person, Rothfeldt said. She compared the alpha-gal allergen to peanuts -- many people are exposed, but only some become allergic.
DISCOVERY
Meinholz was on the team of doctors who developed the test for alpha-gal and discovered its association with tick bites.
In 2004, biopharmaceutical company ImClone asked Dr. Thomas Platts-Mills at the University of Virginia to develop a test for severe reactions to the cancer drug Cetuximab, she said. In 2006, a Bentonville man died from his first dose of the drug, she said.
Meinholz worked with Platts-Mills to develop a blood test for alpha-gal, she said. An article about the allergy by Meinholz and her team appeared in the New England Journal of Medicine in 2008.
Then the researchers noticed significant overlap between the location of known alpha-gal allergy cases and cases of Rocky Mountain spotted fever and ehrlichiosis, both of which are transmitted by the lone star tick. They discovered the association between alpha-gal, meat allergies and tick bites, she said.
Meinholz would later learn she is positive for alpha-gal, a condition she believes stems from a tick bite at Girl Scout camp in third grade.
There is no treatment for alpha-gal syndrome other than avoidance. If people avoid additional tick bites and allergens for a number of years, they might go into remission, Meinholz said.
It's unclear how many people have alpha-gal syndrome, Meinholz said. Health care providers aren't required to report cases of alpha-gal syndrome to the Arkansas Department of Health, so there isn't a tally of how many people in the state have the allergy, Rothfeldt said.
Meinholz said she had seen more than 1,000 alpha-gal patients when she stopped counting a few years ago. Not all of her patients are from Northwest Arkansas because some are treated via telemedicine, she said.
Between 2010 and 2018, at least 105,000 specimens were tested for alpha-gal nationwide, and about a third -- or 34,000 -- were positive, Meinholz said. There likely are many more undiagnosed cases, she said.
Burton said it took her six years to get a diagnosis. If she had known earlier, she may have been able to avoid additional tick bites that caused her to go out of remission several times, Burton said.
Anna Ahlman, a retired nurse from Bella Vista, also had a difficult journey to a diagnosis. She was treated for allergy problems, and her doctor wanted to do surgery to reduce the swelling in her nasal passages.
Ahlman finally resorted to sleeping in a recliner because the swelling made it difficult to breathe at night. Two years later, she went to Meinholz as a last resort before surgery and was tested for the allergy, she said.
"I ate bacon and sausage every single day, not knowing it was killing me," she said.
HEAVILY INFESTED
The distribution of alpha-gal cases correlates with the lone star tick's range, Rothfeldt said. The tick is found in southeast parts of the United States, from Texas up to Kansas, then east toward the Atlantic seaboard, but because of climate change its range has spread more into the Midwest and northeast, she said.
Arkansas is heavily infested with lone star ticks, and they represent the majority of ticks scientists find when collecting ticks in the field, Rothfeldt said.
Female lone star ticks have a white dot on their back, hence the name. Males are slightly smaller and don't have the white spot, according to the federal Centers for Disease Control and Prevention. Both adult and nymph stage ticks, commonly known as seed ticks, can transmit pathogens to humans, according to the Department of Health.
Black legged tick saliva also contains alpha-gal, Meinholz said, and there are reports of people being sensitized to alpha gal by wasp and chigger bites, she said.
SUPPORT GROUP
Jennifer Burton founded a nonprofit support group, Alpha-Gal Encouragers, in 2017 to encourage, empower and educate people in Northwest Arkansas about alpha-gal syndrome. Meinholz also serves on the nonprofit's board.
"We stress prevention -- don't get bitten, don't get sick," Burton said.
Between 15 and 20 people attend the monthly in-person meetings that alternate between Bella Vista and Springdale, and more than 1,500 people are members on Facebook.
During the Feb. 12 meeting at the Highlands United Methodist Church in Bella Vista, Burton lined up food and household items on a row of tables to show group members which ones can cause problems, along with safe substitutes. But even that can vary from person to person because different people are sensitive to different things, she said.
The group swapped tips on recipes and restaurants, grocery stores that cater to alpha-gal patients and methods of tick bite prevention, as they ate an alpha-gal-safe catered meal.
It's helpful to come together with others who have a common experience, said Karen and Steve McAuley of Bella Vista. Karen McAuley was diagnosed with alpha-gal syndrome in July. Hearing other people's experiences has opened their eyes to many aspects of the syndrome they didn't know about, she said.
It's also helpful for spouses, Steve McAuley said.
"I am her first line of defense if she has a reaction. I have to know all the procedures and where the EpiPens are," he said.
The syndrome makes social eating difficult, Karen McAuley said. The couple enjoys travel and eating out, which is still possible if she is diligent and talks to the kitchen manager or chef ahead of time.
Fish and poultry are safe protein sources for people with alpha gal syndrome, Burton said. Many people use emu and ostrich meat as a substitute for beef, she said.
Darryl Descourouez, a group member, believes he was exposed 10 years ago on the first day he moved from Chicago to Monkey Island, Okla. He picked up a lone star tick while crossing a grassy area in a parking lot in Grove, Okla., he said.
It took Descourouez six more years to get a diagnosis. Countless people go undiagnosed because of the delayed reaction, he said.
Descourouez, an avid fisherman, now makes his own sausages from fish he catches and sells them to his fellow Alpha-Gal Encouragers members. Group members said the sausage doesn't taste like fish and is especially good on pizza with vegan cheese.
Rob and Misty Ruckle of Prairie Grove, who are both diagnosed with alpha-gal, attended the support group for the first time on Feb. 12. It took them more than six months to learn which products were safe for them to use, especially concerning medicine and health care products, they said. Like many people with alpha-gal syndrome, they even have to feed their pets an alpha-gal friendly diet to minimize exposure to the allergen, they said.
PREVENTION
People can prevent alpha-gal syndrome by avoiding tick bites, Meinholz said. While some of her patients claim they have never been bitten by a tick, they likely were bitten by a larval or nymph tick that was so small it was mistaken for a chigger or not noticed at all, she said.
Rothfeldt encourages people to still enjoy the outdoors, but cautions the more tick bites a person has, the higher the potential for an allergic response. People can avoid ticks by staying on cleared paths when they are outdoors, wearing U.S. Environmental Protection Agency-approved insect repellent such as DEET and permethrin on clothing, and tucking pant legs into shoes, she said.
Rothfeldt also suggested people perform tick checks on themselves if they are outdoors for an extended period. The sooner a tick is removed, the less likely the person will have a response, she said.
Anna Ahlman talks about her experience dealing with Alpha Gal syndrome. (NWA Democrat-Gazette/Flip Putthoff)
Jennifer Burton (right) sets out materials on Saturday Feb. 12 2022 at a meeting of Alpha Gal Encouragers. (NWA Democrat-Gazette/Flip Putthoff)
Samples of food that may be suitable for people with Alpha Gal syndrome. (NWA Democrat-Gazette/Flip Putthoff)