Hereditary angioedema can cause episodes of swelling that are called attacks. The swelling is most likely to affect your body parts.
It can affect your airway, gastrointestinal tract, and other body parts, which can be life threatening.
Take a moment to learn more about this condition.
Hereditary angioedema (HAE) is a rare genetic condition that runs in families. It affects an estimated 1 in 50,000 to 150,000 people worldwide.
It leads to severe, painful swelling, sometimes due to stress. Attacks can happen spontaneously.
There are three main types of HAE. Different genetic changes cause each type.
The three genetic variations cause your body to produce too much bradykinin, a fragment that helps drive inflammation.
Changes in your blood vessel walls are caused by Bradykinin.
Excess fluid can leak from your blood vessels into other tissues, which can cause swelling.
Type 1 HAE makes up about 85 percent of all cases of HAE.
It’s caused by mutations in the SERPING1 gene. This gene tells your body how to produce C1 inhibitor, a protein that limits bradykinin production.
“If you have a genetic abnormality that causes type 1 HAE, your body doesn’t produce enough C1 inhibitor.”
Type 2 HAE accounts for about 15 percent of all cases of HAE.
Similar to type 1, it’s caused by mutations in the SERPING1 gene.
“If you have a genetic abnormality that causes type 2 HAE, your body will make a C1 inhibitor that doesn’t work.”
Type 3 HAE is very rare. The exact cause isn’t always known, but some cases are caused by mutations in the F12 gene.
The instructions for making coagulation factor XII are given by this gene.
Your body produces a factor XII if you have a genetic abnormality that causes type 3 HAE.
The main goals for HAE are to alleviate the symptoms of an attack and reduce the number of attacks.
The doctor can prescribe one of the following drugs to treat the HAE symptoms.
- Berinert or Ruconest are C1 inhibitors.
- Ecallantide is a word.
- Icatibant is a drug.
These medications are called on-demand treatments. Your doctor can help you learn how to use them.
If you have trouble breathing during an attack, you should use on-demand medication. Follow-up care can be888-607-3166 or888-607-3166, then contact either the emergency department or the hotline.
Intubation or a tracheotomy are procedures that your doctor may need to perform to treat breathing difficulties.
One of the following medications can be prescribed by your doctor to help reduce the number of attacks.
- C1 drugs include Cinryze or Haegarda.
- lanadelumab-flyo is a drug.
- The androgen hormone therapy is danazol.
If you have HAE, limiting your exposure totriggers may help. Changes in lifestyle can help you manage the condition.
It might help to:
- Prepare for dental work or surgery. If you have dental work or other medical procedures scheduled, let your doctor know. They may prescribe medication to lower your risk of a symptom attack.
- Plan travel carefully. Before you travel for work or pleasure, learn where and how you can get help in case of an emergency. Consider avoiding trips to areas where medical care is hard to access.
- Take steps to limit and relieve emotional stress. Try not to take on more obligations at work or home than you can comfortably manage. Strive to make time for stress-relieving activities, such as yoga, reading, or quality time with loved ones.
- Meet with a mental health professional if needed. Living with a rare condition like HAE can affect your mental health. A mental health professional can help you develop coping strategies and a treatment plan if needed.
- Join a support group. Connecting with other people who live with HAE may give you a network of social and emotional support. You may also be able to share practical tips with each other for managing HAE.
If you are having difficulty with the physical, emotional, or social effects of HAE, please let your doctor or other members of your healthcare team know.
They can offer tips for managing the condition, adjust your treatment plan, or refer you to other supportive resources.
The first symptoms of HAE are usually in childhood or adolescence.
You may experience some symptoms during an attack.
- There is swelling of your face, hands, feet, genitals, or other body parts.
- Swelling of the gastrointestinal tract can cause abdominal pain, nausea, vomiting, or diarrhea.
- If swelling affects your airway, you may have difficulty swallowing, speaking or breathing.
About one-third of people with HAE develop a rash that’s not itchy or painful.
If you have swelling in your airway, it can cause breathing problems. It is important to get treatment immediately.
Triggers that can cause symptoms to develop or worse are found in HAE.
- There are physical injuries.
- There are dental procedures.
- There are viral infections.
- emotional stress
You may experience symptoms without a known cause.
The HAE attacks are more or less the same for everyone. Attacks can be very rare for some people. They can happen on a weekly basis for others.
HAE is likely to cause frequent symptom attacks without treatment. It is possible to relieve acute symptoms and reduce the number of attacks.
On average, people with untreated HAE will experience an attack roughly
Over time, the Frequency of Attacks can change in an unpredictable manner.
Some of the symptoms of HAE are similar to other conditions. Swelling, gastrointestinal symptoms, and breathing difficulties can be caused by allergic reactions.
Your doctor will conduct a clinical evaluation and ask you about your family history to find the cause of your symptoms.
If your doctor suspects that HAE is causing your symptoms, they will order blood tests to check the level of certain proteins in your blood. They may order genetic testing to check for certain genetic defects.
The genetic changes that cause HAE may be passed on from parent to child. The condition tends to run in families.
People with no family history of the disease can be affected by the disease.
HAE is a rare and unpredictable genetic condition that causes swelling around the body.
If you experience swelling in your airway, it can cause life threatening breathing difficulties. It is essential to get treatment immediately.
There is no cure for HAE, but current treatment options help many with the condition manage attacks.
The average life expectancy for a person with HAE is the same as the general population, according to recent research.
Talk to your doctor or other member of your healthcare team about the treatment options for HAE.