Treating Acute Attacks
As a person with
hereditary angioedema (HAE) , you should become your own advocate. Recognize
the symptoms of HAE attacks. Learn as much as you can about treatment. Monitor your
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
Prior to 2009, there were no specific treatments available in the United States
for acute attacks of HAE. However, there are now two treatment options:
-
C1-INH (Complement 1 esterase inhibitor protein) concentrate for acute abdominal
and facial attacks in adults and adolescents
- Plasma
kallikrein inhibitor for acute HAE attacks in individuals 16 years of age
and older
C1-INH product with indication for acute attacks
C1-INH (complement 1 esterase inhibitor) therapy works by replacing the missing
or malfunctioning C1-INH protein in patients with a C1-INH deficiency. C1-INH for
acute attacks is given when an attack occurs, optimally at the first sign of the
attack (prodrome.) The most frequent side effects from the clinical trial included
nausea, diarrhea, abdominal pain, and muscle spasms.
Kallikrein inhibitor
A kallikrein inhibitor works by blocking the generation of kallikrein and its byproduct,
bradykinin, which is thought to cause HAE attacks. The most frequent side effects
are dizziness, fatigue, headache, nausea, and vomiting.
Other treatment options
Before C1-INH and kallikrein inhibitor were available, fresh-frozen plasma ( FFP)
was administered for acute HAE attacks. Additional treatment options include epinephrine,
which only provides temporary relief, and androgens and antifibrinolytics, which
don’t take effect until several days have passed and have undesirable side effects.
Neither corticosteroids nor antihistamines have been shown to have any real effect
on HAE attacks.
Pain medications may be helpful during an acute attack. Ask your doctor about their
use.
Acute laryngeal edema
Depending on the symptoms and the sites of the
angioedema, intensive support may be necessary, including intravenous
fluids. This is often the case during a
laryngeal
edema (swelling) attack, which must be controlled to protect the
airways. C1-INH may be administered for this type of attack. But if the laryngeal
edema has progressed rapidly, a
tracheotomy (TRAKE-ee-OTT-o-mee) may have to 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.
Emergencies that require immediate medical attention
These can be the first indications of a potentially life-threatening attack:
- Hoarse voice or laryngitis
- Difficulty in swallowing
- Feeling of tightness
- Voice changes
Recording information
Record information about your HAE attacks and treatment in a diary/journal. This
will help you and your doctor monitor and manage your condition more effectively.