Understanding Hereditary Alpha-Tryptasemia

Did you know? Variations in human genetics can create a distinct story within the complex fabric of our body, influencing how it reacts to its surroundings. One such unique chapter that’s often uncharted in the genetic storybook is Hereditary Alpha-Tryptasemia (HαT), a genetic condition characterized by elevated levels of alpha-tryptase. 

WHAT IS HEREDITARY ALPHA-TRYPTASEMIA?

Hereditary Alpha Tryptasemia (HαT) is a rare genetic condition characterized by elevated levels of alpha tryptase in the blood.  According to the American Academy of Allergy Asthma & Immunology, HaT is an inherited genetic trait where an individual has one or more extra copies of the alpha tryptase gene.

"Tryptase is a chemical produced by immune cells called mast cells during allergic reactions and is known to be higher in patients with HaT," it reported. 

"HaT is found in up to 8% of the general population and has been linked in earlier reports to a variety of seemingly unrelated medical conditions including flushing, itching, gastric reflux, increased joint flexibility, blood pressure changes with standing, and allergy to bee stings."

The Mast Cell Disease Society, on the other hand, listed some facts about the condition. According to them, HαT is caused by extra inherited copies of the gene TPSAB1, which makes extra α-tryptase and leads to high tryptase in peripheral blood at baseline.

"HαT is common and has been found to be present in approximately 5% of people in Western Europe and the United States," it said. "If you have HαT, there is a 50% chance your children will inherit HαT."

HOW COMMON IS IT? 

According to the National Institute of Allergy and Infectious Diseases (NIAID), elevated serum tryptase is present in perhaps up to 6 percent of the general population. 

"While large studies of multiple different ethnicities need to be done, the estimate is that hereditary alpha tryptasemia may be present in a similar percentage of the general population," they said. 

"Because that means that there could be millions of people carrying multiple copies of the alpha tryptase gene, it should come as no surprise that some people will have more than one explanation for their symptoms," they added, stressing there is a "great variability from person to person in terms of what symptoms the duplications or triplications do or do not cause.

WHAT ARE THE SYMPTOMS?

The NIAID emphasized that individuals presenting with a blood tryptase level surpassing 10 ng/mL, especially when there is a similar elevation observed in another close relative, face an elevated likelihood of hereditary alpha-tryptasemia. 

"Despite the prevalence of a diverse array of symptoms among individuals with the associated syndrome, a substantial portion of these manifestations is characterized by commonality," they explained. 

Hereditary alpha tryptasemia syndrome may be characterized by a number of symptoms that impact various systems, such as (but not limited to) the following:

  • Chronic skin flushing, itching, or hives
  • Bee sting allergy
  • Dizziness and/or difficulty maintaining a normal pulse and blood pressure
  • Chronic head, back, and joint pain
  • Skeletal abnormalities
  • GI disturbances including heartburn, IBS, and numerous food and drug reactions and intolerances
  • Sleep disturbances

It is difficult to determine whether hereditary alpha-tryptasemia is present based only on observed symptoms, though, because many of these symptoms are similar to one another.

WHAT ARE THE REMEDIES? 

The NIAID noted that until therapies directly addressing the genetic change associated with hereditary alpha-tryptasemia are identified, symptomatic treatment remains the primary approach. Given that mast cells produce tryptase, and many symptoms are linked to the release of mast cell-derived mediators like histamine, clinical strategies targeting mast cell activation, such as antihistamines, are frequently employed and proven to be beneficial. 

While there is no cure for HαT, managing symptoms is crucial for improving the quality of life. Some approaches include:

  • Medication: Depending on the severity of symptoms, healthcare providers may prescribe antihistamines, mast cell stabilizers, or other medications to manage specific symptoms.
  • Avoiding Triggers: Identifying and avoiding triggers, such as certain foods, stress, or environmental factors, can help minimize symptom flare-ups.
  • Lifestyle Modifications: Incorporating stress-management techniques, maintaining a balanced diet, and getting regular exercise can contribute to overall well-being.
  • Regular Monitoring: Regular check-ups with healthcare professionals are essential to monitor symptoms and adjust treatment plans accordingly.

Individuals are advised to engage in discussions with their healthcare providers to explore and tailor these approaches for their specific needs.

HOW DOES SELECTING THE APPROPRIATE CLOTHES AFFECT HαT?

Since individuals with HαT may experience chronic hives, which are raised, red, and itchy welts on the skin, it's imperative to consider wearing certain clothing to minimize skin irritation and reduce the risk of triggering symptoms. 

The way people with HαT choose to dress can have a big impact on their comfort and general well-being. Here are some suggestions:

  • Choose Loose-Fitting Clothing: Opt for loose-fitting, breathable clothing to minimize friction and allow air circulation. Tight clothing can potentially exacerbate mast cell activation and trigger symptoms.
  • Select Soft Fabrics: Choose soft, non-abrasive fabrics to reduce the risk of skin irritation. Natural fibers like cotton may be a good choice, as they are gentle on the skin and allow for better breathability.
  • Avoid Rough or Scratchy Materials: Steer clear of fabrics that are rough or scratchy, as these can irritate sensitive skin. Instead, look for clothing with smooth textures that won't cause unnecessary discomfort.
  • Consider Allergen-Free Fabrics: Some individuals with HAT may have sensitivities to specific fabrics or dyes. Consider clothing made from hypoallergenic materials to minimize the risk of allergic reactions.
  • Layer Clothing for Temperature Control: Dress in layers so you can easily adjust to changes in temperature. This allows you to add or remove layers as needed without compromising comfort.
  • Wear Long Sleeves and Pants: If you're prone to hives or other skin reactions, wearing long sleeves and pants can provide an additional layer of protection against environmental triggers. This can be particularly important in situations where exposure to potential allergens is likely.
  • Use Sun Protection: Sun exposure can trigger symptoms in some individuals. Wear clothing that provides good coverage or use sunscreen to protect your skin from harmful UV rays.
  • Choose Comfortable Undergarments: Select comfortable undergarments made from soft materials. Avoid materials that may cause irritation or pressure on the skin.
  • Avoid Tight Elastic: Clothing with tight elastic bands, such as waistbands or cuffs, may contribute to discomfort. Choose items with gentle, non-constrictive elastic, or consider clothing with adjustable features.
  • Consider Moisture-Wicking Fabrics: For individuals who experience sweating as a trigger, moisture-wicking fabrics can help keep the skin dry by drawing moisture away from the body.

We, at Cottonique, believe that nature has the answer to the health concerns of millions of people all over the world. For this reason, our research and development team is primarily focused on natural materials and innovative technology to develop hygienic allergy-free products to benefit dermatological, gynecological, and environmental concerns.

If you're looking for safer clothing pieces that allow people with skin allergies and sensitivities to live easily without the use of synthetics, check out our allergy-free clothing made with 100% organic cotton. You may also read our previous blog: 5 Reasons Why You Should Make the Switch to Organic Clothing.

TAKEAWAY: Managing hereditary alpha tryptasemia requires a multifaceted approach due to its challenges. Though a universally applicable solution may not exist yet, it is important to recognize that attire plays a significant role in improving comfort and reducing symptoms. 

Those with HϱT can actively enhance their quality of life by choosing fabrics carefully, dressing in appropriate ways, and taking environmental considerations into account. Navigating this uncommon genetic condition also requires teamwork with healthcare providers for customized treatment plans and ongoing monitoring. 

If you want to know more about the condition, visit this helpful resource from the NIAID. 

DISCLAIMER: The information presented on Cottonique is not, and will never be, intended to be a substitute for professional medical advice, diagnosis, or treatment. All content materials found on this site, including text, treatments, outcomes, charts, graphics, photographs, and study findings, are created and published for general informational purposes only. It should not, in any way, be construed as a standard of care to be followed by a user of the website. 

Thus, readers are encouraged to verify any information obtained from this website with other accurate references and review all information regarding any medical condition or treatment with their physician. As Cottonique strives to help those with allergies live better days, the hypoallergenic apparel brand encourages everyone to always seek the advice of their physician or other qualified health providers with any questions they may have regarding a medical condition.