Treating Acute Attacks

As a person with HAE, it is important for you to become your own advocate. Recognize the symptoms of HAE attacks and learn as much as you can about treatment. Monitor symptoms and work with your doctor to choose the best treatment plan for you. The following information about disease treatment is important for you to know:

• Acute Attacks: For several years in Canada and Europe, the treatment of choice for acute attacks has been replacement with complement-1 esterase inhibitor protein (C1-INH) concentrate. In 2009, the first C1-INH treatment for acute attacks was approved for use in the US.
  
  Prior to C1-INH, fresh-frozen plasma (FFP) was administered for acute HAE attacks. Unfortunately, the therapy contains proteins that may make the condition worse.

Because people who have abdominal attacks may release 1-2 liters of fluid into their abdomen and develop low blood pressure, they may require intravenous fluid replacement therapy.

Moreover, the preventive treatments mentioned on the Potential Attack Triggers page--danazol, stanazolol, oxandrin, tranexamic acid (TA), and epsilon-aminocaproic acid (EACA)--are not usually effective for acute attacks.

• Acute Laryngeal edema: Depending on the symptoms and the sites of the angioedema, intensive support may be necessary, including tintravenous fluids. This is often the case during a laryngeal edema (swelling) attack, which must be controlled to protect the person’s airways. Sometimes a tracheotomy (TRAKE ee OTT o mee) must be performed to allow oxygen to flow into the lungs. A tracheotomy is an incision through the neck into the windpipe that is made when the upper airway is blocked.
• Pregnancy: Short-term use of androgens may be a treatment for HAE attacks in pregnant women during the third trimester of pregnancy. However, the risk of  potentially masculizing effects—particularly for a female fetus—are of concern, and many physicians view pregnancy as an absolute contraindication for the use of attenuated androgens. Some physicians view the judicious use of tranexamic acid as an option during pregnancy. In most countries, however, C1-INH is viewed as the treatment of choice during pregnancy.

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WARNING! : Call your local emergency number if you notice the following: Hoarse voice or laryngitis Whistling or wheezing when breathing Shortness of breath Swollen tongue Lip or facial swelling Throat swelling These can be the first indications of potentially life-threatening atacks.

Record information about your attacks and treatment in a diary/journal. This will help you and your doctor monitor and manage your disease more effectively.