You are your own advocate! AGS introduced!

It’s funny how people who don’t deal with Alpha Gal syndrome or Chronic Lyme disease, come out and minimize it.

Let’s dive into AGS:

Introduction:

Alpha-gal Syndrome (AGS) is a unique allergy to non-primate mammalian meat (and derived-products) that is associated with tick bites and is due to a specific IgE antibody to the oligosaccharide galactose-α-1,3-galactose (alpha-gal). AGS has many novel features that broaden the paradigm of food allergy, including that reactions are delayed 3-6 hours after exposure and patients have frequently tolerated red meat for many years prior to the development of allergic reactions. Due to the ubiquitous inclusion of mammal-derived materials in foods, medications, personal products and stabilizing compounds, full avoidance is difficult to achieve.

Expert opinion:

The number of patients with AGS is rising and may have exceeded awareness of the diagnosis amongst healthcare providers. In summarizing experience gained to thus far, we hope to create a resource for identifying and managing this unique allergic syndrome.

Keywords: Alpha gal allergy, food allergy, anaphylaxis, red meat, IgE, tick, galactose.

In 2009 Alpha-gal Syndrome (AGS) was described in 24 patients who reported delayed allergic reactions to mammalian meat [1]. This clinical observation was supported by prior identification of immunoglobulin E (IgE) specific for the oligosaccharide galactose-α-1,3-galactose (alpha-gal) that was associated with hypersensitivity reactions (HSRs) to cetuximab [2]. In >90% of cases the diagnosis of AGS can be made based on a history of delayed allergic reactions after eating non-primate mammalian meat ^* (e.g., “red meat” such as beef, pork, or lamb) and a positive blood test (>0.1 IU/mL) for IgE to alpha-gal [3]. The combination of both an appropriate clinical history and supporting blood test is necessary to establish an accurate AGS diagnosis [1]. Unlike more traditional food allergies where consumption of an allergen produces symptoms within minutes, AGS reactions typically occur 3-8 hours after eating [4]. Thus, many patients fail to consider food as a possible trigger and many healthcare providers do not routinely recognize the characteristic delay – both issues can prolong time to reach a diagnosis [5].

Blood levels of alpha-gal IgE often decrease in patients who avoid recurrent tick bites but the rate of decline varies from patient to patient [7].

The titer of alpha-gal specific IgE does not predict reaction severity; rather dose (amount consumed) and presence of co-factors (alcohol, activity) affect the delay before reaction and resulting clinical manifestations [4].

Table 1 –

Clinical pearls for diagnosis and management of patients with AGSConsistently Inconsistent

• Whether a reaction occurs to an individual exposure is inconsistent and often appears to follow no identifiable pattern for patients.
• The lack of consistent reactions is, in itself, almost a diagnostic hallmark.
• Over time, patients may experience a “progression” to more consistent reactivity and this likely reflects a new tick bite.

Isolated Gastrointestinal

• Reactions can be GI only.
• Many patients carried a diagnosis of chronic diarrhea, IBS or GI food allergy syndrome prior to AGS diagnosis.
• Overwhelmingly, their symptoms are resolved following diagnosis; oral cromolyn solution can be useful in those patients with lingering GI symptoms.

Co-factor Dependence

• Activity, alcohol consumption, and exercise can have profound influence on reactivity.
• Some patients appear to have reactions that requireco-factors such that they can tolerate exposures in isolation; consistent with a diagnosis of co-factor dependent-AGS

Bedtime Routines

• Many episodes occur after 10pm, some even later in the night
• Do not underestimate how many patients take diphenhydramine nightly for sleep and could be influencing the reactions in both occurance and severity.

Although AGS is no different from other food allergies in this regard, avoidance of mammalian-derived products is more challenging due to a lack of adequate labeling and the inclusion of common components, such ‘natural’ flavorings, in numerous foods. For <10% of patients, the avoidance diet also includes removing dairy and derivatives as well as gelatin [11].

In addition, multiple medications are derived from mammals and specific mammalian tissues are used as medical devices. Products such as heart valves, gelatin-based plasma expanders, and pancreatic enzymes, are sources of alpha-gal exposure yet may trigger a reaction in more select patient groups [12,13].

In keeping with this, a minority of patients may benefit from avoiding a wide range of products that are prepared with mammalian-derived constituents [14]. Owing to the ubiquitous inclusion of mammal-derived products within both food and healthcare settings, allergen avoidance for patients with AGS can present unique challenges for management and we routinely include a dietician as part of our care team (or suggest referral) [11].

Diagnosis of AGS

The following characteristics occur in 85% of patients with AGS:

1. Onset in adult life after eating mammalian meat without problems for many years
2. Reactions range from pruritus, localized hives or angioedema to anaphylaxis
3. Patients can report strictly gastrointestinal symptoms (diarrhea, abdominal cramping, emesis) almost to the exclusion of cutaneous, cardiovascular or respiratory manifestations
4. Reactions start 3-8 hours after eating non-primate mammalian meat (or consumption of dairy, gelatin, or other mammalian-derived products)
5. Positive testing for alpha-gal IgE (>0.1 IU/mL)
6. Improvement of symptoms when adhering to an appropriate avoidance diet
7. Description of large local reactions to tick or other arthropod bites, often including report of an ‘index’ bite that behaved differently than prior bites

In many settings, the critical point is for the astute clinician to consider AGS as a possible cause of the patient’s symptoms and is included as a diagnostic algorithm.

However, it is important to realize that some patients with AGS may not present with these characteristics. Despite point i) above, a significant number of pediatric cases of AGS has been reported [15] and children represent approximately 12% of subjects in one published cohort [16].

Although published data suggest that the condition in children has similar features to those of adult patients, it is not unusual for children with AGS to report a specific association with limited number of foods, predominance of GI symptoms and particular relevance of activity (e.g., sports practice).

I found out I had Lyme disease by symptoms. There is no PCR tests!!

I found out I had AGS when I ended up in the ER and all doctors had no idea what was going on. I finally made the discovery by accident, and found a naturalist doctor to confirm. I went to Natural Resources and became a licensed Holistic Health Practitioner to save my own life.

Moreover, a study of over 200 patients with AGS found that 16% reported subjective symptom onset in less than 2 hours after consuming mammalian meat [16]. History of a tick bite, larval tick bites (e.g., seed tick bites) or ‘chigger’ bites can be supportive of AGS as a diagnosis but the absence of such a history is not uncommon. Tick bites can be painless, and about half of the people who develop a tick-borne infection may not even remember being bitten by a tick. ‘Chiggers’ is a term often mentioned in the South that has become synonymous with ‘very itchy, small, red bites’ that can occur in isolation or in large numbers. We often inquire about ‘chigger’ bites with patients to gain a history of bug bites in general and less in direct questioning of their exposure to members of the Trombiculidae family of insects. Whether Trombiculidae bites can lead to the development of alpha-gal IgE is unclear but is a research question under our investigation.

Research and resources found here https://pmc.ncbi.nlm.nih.gov/articles/PMC8344025/