In this thing we call #life - What's a EVM?

Hereditary Hemorrhagic Telangiectasia (HHT) is more than a genetic disorder affecting my family; it's a daily reality, especially for my wife. While HHT is often associated with abnormal blood vessel formations and significant bleeding, such as frequent nosebleeds, there's a less-discussed yet critical aspect of this condition: Extravascular Manifestations (EVMs). These EVMs have added layers of complexity and challenge to our lives. This article aims to illuminate the causes and impacts of EVMs and celebrate the power of community support in our journey.

EVMs are complications arising outside the vascular system, a perplexing twist given HHT's vascular origins. These manifestations stem from the genetic mutations at the heart of HHT, which, while leading to abnormal blood vessels, also indirectly affect other organs and tissues. For example, the liver might be overburdened by filtering an increased volume of blood due to abnormal artery-vein connections, resulting in various liver-related EVMs.

For my wife and many others living with HHT, EVMs have introduced a spectrum of challenges beyond abnormal bleeding. These include:

• Gastrointestinal Issues: Occult bleeding in the gastrointestinal tract can lead to iron deficiency anemia, a condition we've battled constantly, necessitating regular iron infusions and vigilant monitoring.
• Liver Complications: Liver involvement can manifest as fatigue, abdominal pain, or more severe issues like portal hypertension, significantly impacting daily life and well-being.
• Neurological Effects: Abnormal blood vessels in the brain and spine can increase the risk of seizures, headaches, and strokes, adding anxiety and uncertainty to our lives.

Managing these EVMs requires a multidisciplinary care approach, engaging specialists from gastroenterologists to hematologists and neurologists, each playing a vital role in addressing the diverse impacts of HHT

The journey through the multifaceted challenges of EVMs can feel isolating, but the HHT community has been a beacon of hope. This community has connected us with others who share similar experiences, offering insights into effective management strategies and treatments. Beyond practical advice, this support has provided emotional solace, making us feel part of a larger, understanding family. The Cure HHT community is also a crucial resource for learning about the latest research and potential therapies, empowering us to advocate for comprehensive care and access to treatments that address the full spectrum of HHT symptoms, including EVMs.

Our journey with HHT and its manifestations is ongoing, but we're bolstered by the knowledge and support of the HHT community. Sharing our story contributes to the collective understanding of EVMs, underscoring the importance of research, awareness, and a multidisciplinary care approach. To those on a similar path, remember, you're not alone. The strength of the HHT community lies in our shared experiences and collective wisdom. Together, we can face the challenges of EVMs with resilience and hope, striving for a future where the impacts of HHT are fully understood and effectively managed.