How Should You Explain HHT to Emergency Room Doctors?

When you arrive at the emergency room, the most critical step is ensuring the medical staff immediately understands your condition and its life-threatening risks. Because Hereditary Hemorrhagic Telangiectasia (HHT) is rare, many ER doctors and nurses may not be familiar with its complications ^[1]. Your “elevator pitch” should be concise, authoritative, and focused on the highest risks. You should tell the triage nurse and the doctor: “I have HHT, a rare bleeding disorder with internal vascular malformations. I am at high risk for stroke and internal bleeding. If I need an IV, you MUST use an IV air filter to prevent a stroke.” Immediately hand them an official HHT emergency card or clinical guidelines ^[2].

(Note: While you should use the term “bleeding disorder” in your pitch to convey urgency, HHT is technically a vascular dysplasia—an abnormal blood vessel disorder—not a coagulation issue like hemophilia ^[3]. Ensure the attending doctor understands that your bleeding comes from fragile vessels, not an inability to clot, so they do not incorrectly order coagulation therapies.)

Why This Specific Phrasing Matters

Emergency room staff are trained to triage patients rapidly based on perceived risk. Unless a severe, uncontrollable nosebleed is the specific reason for your ER visit, leading with a history of frequent nosebleeds might cause staff to underestimate your systemic risks and treat your visit as a minor issue ^[4]. Using terms like internal vascular malformations signals that your situation involves serious internal risks that require immediate, specialized attention and a multidisciplinary approach ^[1].

The Critical Need for IV Air Filters

The most urgent safety intervention for an HHT patient in the ER is the use of an IV air filter ^[2]. This rule applies to all patients who have known Pulmonary Arteriovenous Malformations (PAVMs) or whose PAVM status is currently unknown.

In a person without HHT, the lungs act as a natural filter, catching tiny air bubbles or blood clots before they can circulate to the rest of the body. However, PAVMs are abnormal connections between arteries and veins in the lungs that create a direct “shunt” or shortcut, bypassing the lung’s natural capillary filter ^[5].

If an IV is placed without an air filter, microscopic air bubbles from the tubing can travel directly through these shunts and into your brain. This can cause a cerebral air embolism or a stroke ^[6][5]. Furthermore, because these lung shunts also fail to filter out bacteria or tiny blood clots, HHT patients are generally at a high risk for brain abscesses and ischemic strokes ^[6]. You must insist on an air-in-line filter (typically 0.22 micron) for any IV fluids or medications ^[2].

What if they refuse or don’t have a filter?
If a nurse pushes back, does not know what an air filter is, or claims they do not have one in the department, calmly but firmly escalate the situation. Ask to speak to the Charge Nurse or the Attending ER Physician immediately, and present your physical HHT emergency card as clinical proof of the requirement. Do not allow them to start the IV until the filter is secured.

How to Prepare for an ER Visit

Navigating a medical emergency is stressful and overwhelming. Preparing in advance ensures that you can advocate for yourself safely, even if you are in pain or frightened.

• Carry an Official HHT Emergency Card: Organizations like Cure HHT provide printable emergency cards and checklists specifically for ER staff on their website. Keep one in your wallet and another in your car or with your medical records. Handing this physical document to the triage nurse establishes instant clinical credibility ^[2].
• Bring Your Specialist’s Letter: Keep a physical copy of the International HHT Guidelines or a summary letter from your specialist detailing your specific vascular malformations (such as known brain, liver, or lung AVMs) ^[7].
• Bring a Current Medication List: ER staff need to know if you are receiving frequent iron infusions for severe anemia or taking anti-angiogenic therapies (like bevacizumab) for your HHT ^[8].
• Bring an Advocate: If possible, bring a family member or friend who knows your elevator pitch. They can insist on the IV filter, escalate to the charge nurse if needed, and hand over your emergency card if you are too ill to speak for yourself.