What is the management and treatment for Alpha-gal syndrome?

Management and Treatment of Alpha-Gal Syndrome

The cornerstone of managing alpha-gal syndrome is complete avoidance of all mammalian meat and related products, with careful attention to potential hidden sources of alpha-gal in processed foods, medications, and cross-contaminated restaurant meals. 1, 2

Diagnosis and Initial Management

• Alpha-gal syndrome is diagnosed based on consistent symptoms, elevated alpha-gal IgE titers, and symptom improvement on an alpha-gal avoidance diet 1, 3
• A positive IgE antibody test alone is insufficient for diagnosis, as many sensitized individuals remain asymptomatic 3
• Symptoms typically occur 3-6 hours after ingestion of mammalian products, often causing nighttime awakening with GI distress 3, 4
• Consider testing for alpha-gal syndrome in patients with unexplained abdominal pain, diarrhea, nausea, or vomiting, particularly in alpha-gal prevalent regions (Southeast, mid-Atlantic, Midwest, and East Central US) 1, 3

Dietary Management

• Eliminate all mammalian meat (beef, pork, venison, and other animals with hair) 1, 2
• Avoid mammalian-derived products such as lard, butter, and gelatin (found in marshmallows, gummy bears, and certain desserts) 1, 2
• Dairy products contain smaller amounts of alpha-gal, with high-fat dairy (ice cream, cream, cream cheese) being particularly problematic 1, 2
• Safe alternatives include fish, seafood, turkey, chicken, and other fowl 1, 2
• Avoid eating at restaurants due to high risk of cross-contamination 1, 2
• Be cautious with processed foods which may contain hidden animal-derived ingredients 1, 2

Prevention of Further Sensitization

• Take measures to prevent additional tick bites, as they can worsen the allergy 1, 2
• Perform tick checks and shower after outdoor activities in wooded areas 1, 2
• Create barriers at ankles by pulling up tight mesh socks over pant cuffs when hiking 1, 2
• Treat clothes and boots with permethrin to reduce tick bites 1, 2

Medication Considerations

• Some medications and medical products contain alpha-gal, which can trigger reactions in sensitive individuals 1, 2
• Patients with systemic symptoms (rash, facial swelling, difficulty breathing, hives) should be prescribed an epinephrine autoinjector 1
• Antihistamines may help manage mild reactions 5, 6
• Treatment protocols for hypersensitivity reactions depend on severity, ranging from antihistamines to epinephrine 5

Follow-Up and Monitoring

• Re-evaluate symptoms after at least one month of strict alpha-gal avoidance diet 1, 2
• Consider repeating alpha-gal IgE levels 6-12 months after diagnosis if tick bites are avoided 1, 2
• As IgE levels decrease or become negative, patients may gradually reintroduce first dairy products, then small portions of mammalian meat under medical supervision 1, 2
• Patients attempting reintroduction should:
  • Start with modest doses (quarter serving of meat) 1
  • Have antihistamines and autoinjectable epinephrine available 1
  • Have another person present to monitor for progressing symptoms 1

When to Refer to Specialists

• Refer to an allergist when the patient experiences systemic symptoms such as facial swelling, throat swelling, voice changes, difficulty breathing, hives, or fainting 1, 2
• Consider referral to a gastroenterologist if GI symptoms persist despite adherence to an alpha-gal avoidance diet 2, 3

Important Caveats

• Alpha-gal allergic reactions often do not happen after every exposure, so tolerance of meat on one occasion does not rule out the allergy 1, 3
• Co-factors such as NSAIDs, exercise, and alcohol can increase both risk and severity of reactions 1, 3
• Traditional oral food challenge testing is impractical for alpha-gal syndrome due to delayed reaction time 1, 3
• No definitive cure for AGS has been established, though experimental desensitization protocols are being investigated 5, 7
• Healthcare provider knowledge about alpha-gal syndrome is limited, potentially leading to underdiagnosis 8