What is the treatment for a female patient with Mast Cell Activation Syndrome (MCAS) and Alpha-Gal allergy, experiencing symptoms of hypotension, brain fog, fatigue, and dizziness after eating?

Treatment for MCAS and Alpha-Gal Syndrome with Hypotension and Neurological Symptoms

The primary treatment for a female patient with both MCAS and Alpha-Gal Syndrome experiencing hypotension, brain fog, fatigue, and dizziness after eating is strict elimination of all mammalian meat and products, combined with mast cell stabilizing medications such as cromolyn sodium and antihistamines. 1

Alpha-Gal Syndrome Management

Dietary Elimination

• Complete avoidance of all mammalian meat (beef, pork, venison) and related products (lard, butter, milk) is the cornerstone of managing alpha-gal syndrome 1
• Particularly avoid high-fat dairy products like ice cream, cream, and cream cheese which contain higher amounts of alpha-gal 1
• Safe alternatives include fish, seafood, turkey, chicken, and other fowl 1
• Avoid gelatin-containing products (marshmallows, gummy bears, gelatin desserts) as they are derived from mammalian collagen 1
• Avoid eating at restaurants due to high risk of cross-contamination 1
• Be cautious with processed foods which may contain hidden animal-derived ingredients 1

Environmental Management

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

MCAS Management

Pharmacological Treatment

• H1 and H2 antihistamines should be used to block mediator receptors 2
• Mast cell stabilizers like cromolyn sodium are indicated to prevent mediator release from mast cells 3, 2
• Cromolyn sodium works by indirectly blocking calcium ions from entering mast cells, thereby preventing mediator release 3
• Leukotriene receptor antagonists may help manage symptoms 2
• For acute severe reactions, intramuscular epinephrine is essential 4, 2
• For prolonged episodes, short courses of corticosteroids may be considered 2

Addressing Specific Symptoms

For Hypotension

• Ensure patient has an epinephrine auto-injector available at all times for emergency treatment of severe hypotensive episodes 4
• Consider referral to an allergist for patients with hypotension, as they are at risk for anaphylaxis 1
• Monitor blood pressure during and after meals to identify patterns and triggers 2

For Neurological Symptoms (Brain Fog, Fatigue, Dizziness)

• Document symptom patterns in relation to food intake to identify potential hidden triggers 5
• Consider a food diary to track reactions and identify patterns 6
• Ensure adequate hydration, particularly during symptomatic periods 2

Follow-Up Management

• Re-evaluate symptoms after at least one month of strict alpha-gal avoidance diet 1
• Consider repeating alpha-gal IgE levels 6-12 months after diagnosis if tick bites are avoided 1
• As IgE levels decrease, patients may gradually reintroduce first dairy products, then small portions of mammalian meat under medical supervision 1

Common Pitfalls and Caveats

• Delayed reaction onset (typically 3-5 hours after exposure) often leads to misdiagnosis or confusion with other conditions 6
• Many healthcare providers have limited knowledge of alpha-gal syndrome, which can lead to underdiagnosis 7
• Patients with both MCAS and alpha-gal syndrome may have more severe and complex reactions due to multiple factors triggering mast cell activation 8
• Some medications and medical products contain alpha-gal, which can trigger reactions in sensitive individuals 1
• Alpha-gal sensitization may be associated with increased risk of coronary artery disease, making management even more important 1

When to Refer to Specialists

• Refer to an allergist when the patient experiences systemic symptoms such as facial swelling, urticaria, or respiratory difficulty 1
• Consider referral to a gastroenterologist if GI symptoms persist despite adherence to an alpha-gal avoidance diet 1