6 Questions to Ask Your Doctor About HAE Attacks

Hereditary angioedema, also known as HAE, is a rare genetic disorder that affects between 6,000 and 10,000 people in the United States. People who have HAE experience recurring episodes or attacks of severe swelling. These attacks most commonly affect the hands, feet, face, genitals, the gastrointestinal tract and the throat. There is no cure for HAE, but there are treatments available that can help people with HAE manage the condition. The following slides look at a few questions related to HAE attacks that you may want to discuss with your healthcare provider, including how attacks are treated, what can trigger an attack and when to seek emergency treatment.

Medically reviewed in April 2018.

Most HAE attacks are not associated with an identifiable trigger. But some are. Mental stress, physical exertion, dental work, surgery and illnesses like the flu are all known to be potential triggers for an HAE attack.  About one-third of patient report experiencing a trauma or injury before an attack, especially trauma or injury caused by pressure. Women who are menstruating, pregnant and breastfeeding also report a greater frequency of HAE attacks. Additionally, medications that contain estrogen—such as hormone replacement therapies and oral contraceptives—are associated with an increase in frequency of HAE episodes as well as the severity of HAE symptoms. ACE inhibitors, a type of medication used to treat high blood pressure, are also associated with more frequent episodes and more severe symptoms.

There are two basic types of treatments available for HAE, treatments that reverse attacks and treatments that prevent attacks from occurring. Patients diagnosed with HAE will likely be prescribed a treatment to reverse an attack. These treatments are usually taken by an injection or infusion. Patients and loved ones may receive training by a healthcare provider on how to administer these treatments or may receive treatment at a doctor’s office or hospital when the treatment needs to be administered.

Treatments that prevent attacks are called prophylactic treatments. These may be prescribed short term, to prevent a potential attack brought on by dental work, surgery, pregnancy or a period of high stress. Prophylactic treatments may also be prescribed as a long-term treatment for patients who experience frequent HAE episodes.

You might. There is at least one published study of HAE patients where a large percentage of participants reported they could “feel” when an attack is in the near future. Also, some patients will experience symptoms during the 24 hours before an HAE episode. These symptoms may include fatigue, muscle aches, a tingling sensation in the area that will be affected and a tingling red rash that does not itch. Patients who have experienced HAE attacks that affect the intestinal tract have reported abdominal pain, nausea and other gastrointestinal symptoms within that same 24-hour time period. However, it’s important to understand that these symptoms are not reliable predictors of an impending HAE episode—they don’t always occur before an HAE episode, and they sometimes occur without being followed by an HAE episode.

Your healthcare provider should have prescribed a treatment to take to reverse an attack. If you experience an attack, take your treatment as directed, and take it as soon as possible. Though there are a number of HAE treatments available, and these treatments work in different ways, treatment is generally most effective when administered within the first few hours, while swelling is still increasing. Attacks that affect the airways, as well as attacks that cause severe gastrointestinal symptoms, require emergency medical care, and are discussed on the next slide.

Some HAE episodes can be life threatening and require emergency care. More than half of HAE patients will at some point have an episode that affects the airways (called a laryngeal attack), which can cause asphyxiation. Severe gastrointestinal attacks may also require emergency medical treatment to address dehydration, pain, cramping and other symptoms. Gastrointestinal symptoms may also require observation and evaluation by a healthcare provider, in case they are being caused by something other than HAE.

Work with your healthcare provider to come up with a plan on what to do in a medical emergency. This may involve carrying forms that identify you as an HAE patient. Because HAE is a rare condition, your healthcare provider may contact your local hospital or emergency services to make them aware of your diagnosis and ensure they are properly equipped to handle an emergency.

After an HAE attack subsides, you may feel tired and dehydrated—dehydration is especially common if symptoms included diarrhea and vomiting. Make sure to get plenty of rest and drink plenty of fluids in the days following an episode. If you administered a treatment at home, refill your prescription as soon as possible, since HAE attacks are unpredictable and you will need to be prepared for another.

Many healthcare experts recommend keeping a journal describing symptoms and attacks, which can be helpful in identifying potential triggers, as well as evaluating how well your treatment plan worked. Record any details about the episode you can recall, including symptoms, severity, what you ate and how the attack was treated.