Living With Hereditary Angioedema
If you or someone you love has
hereditary angioedema (HAE), you may be concerned about you or that person
being able to live a full and active life. Rest assured that most people with HAE
successfully manage the condition. Remember that knowledge is power, and that understanding
how HAE can impact your life at various stages can help empower you to live a full
HAE is a
hereditary disease that occurs equally in men and women. An inherited
disorder, it is a lifelong disease. However, the severity of the disease as well
as the frequency, type, and timing of HAE attacks can vary widely between individuals.
Furthermore, the pattern of attacks can be inconsistent within any given person,
such as having greater or fewer episodes during one life stage compared to another.
Questions? See if they are answered in
Questions on Living With HAE.
In families affected by HAE, all children should be tested for C4 (another protein
that regulates the immune system that is almost always low in patients with type I or II HAE)
and C1 levels after age 1. (Before this age, tests aren’t very accurate.) Even if
a child has no symptoms, an advance diagnosis may help you prepare for a possible
future attack. 6
Physicians report that their youngest patients who have HAE attacks tend to be preschoolers,
rather than infants or toddlers. However, many people do not experience attacks
until their teen years or later.
If your young child does have attacks, keep in mind that children usually have more
difficulty than adults in conveying feelings of discomfort, pain, and other medical
symptoms. Be alert to the changes in your child’s mood or behavior that may herald
the onset of an attack. As your child grows, help him or her identify and name (prodromal) symptoms and personal triggers.
If your child has HAE, inform healthcare professionals and first responders at day
care, school, summer camp, and after-school programs. It is a good idea to create
a patient information card, that includes key family and medical
contact numbers and instructions describing steps to be taken in the event of an
HAE emergency. In addition, a letter (Click
here to see sample letters) from your child’s physician to other healthcare
professionals should be available wherever your child plays or works.
Hormonal and emotional changes increase during the teenage years. Many people report
the onset of their first HAE attacks during puberty. Moreover, numerous people report
changes in the location, severity, and frequency of attacks as the teenage years
As your child develops more independence and spends more time away from home, it
becomes increasingly important for him or her to carry personal medical information.
An ID bracelet or chain, a card in a wallet or purse, and an understanding of HAE
among your child’s inner circle can discreetly help keep your child safe at a time
when a desire to fit in becomes important.
During the teen years, the ultimate responsibility for a child’s health should shift
gradually from parent to child. Your child’s firm grasp of personal triggers, warning
symptoms, and treatment options will help him or her transition to the college scene
or the working world.
Living with HAE means understanding how your specific symptoms trigger attacks and
how you and your physician can best manage HAE.
Although no definitive causes for HAE attacks have been established, attacks may
be triggered by emotional or physical stress. Surgical procedures, even mild dental
work, can trigger attacks in some people. 6
Changes in hormonal levels have been associated with increased HAE attacks in women.
Many young women report changes in HAE attack frequency and severity when using
contraceptives that contain estrogen. Doctors typically recommend that women with
HAE use only contraceptives that do not include estrogen. 6
Other medications may also impact HAE attacks. Before starting a new prescription,
be sure to discuss your options with a specialist in HAE. If you have trouble locating
a specialist, you may want to contact the U.S. Hereditary Angioedema Association (HAEA). This organization maintains
a robust list of doctors across the country who treat people with hereditary
Natural hormonal changes can also cause changes to patterns of HAE attacks. Some
pregnant women notice an increase in frequency of attacks during pregnancy. For
others, the number and severity of attacks fall during pregnancy. 8
Menopause is a time of physiological change in which hormonal levels are altered.
If you are considering estrogen replacement therapy during this change of life,
discuss your treatment options with your HAE specialist. 8