- Hoarse voice or laryngitis
- Whistling or wheezing when breathing
- Shortness of breath
- Swollen tongue
- Lip or facial swelling
- Throat swelling
Attacks of HAE can occur suddenly and without warning. However, many people with HAE notice early stage symptoms that warn them that an attack is about to happen. These are called the prodrome, or prodromal symptoms.
Some people notice these symptoms minutes or hours before an attack, while others can feel them a whole day or two before a full attack begins. Learning to recognise early stage symptoms of an attack will help you to stay in better control of your condition, as well as being aware of signs that it may be developing into a more serious attack.
Should your symptoms prevent you from completing everyday activities such as tasks for work, socialising or house chores then you should have a treatment plan agreed with your immunology team (see Working with your healthcare team).
Some HAE attacks can be extremely serious.
Even though HAE attacks are often unpredictable, some are triggered by stress, hormones, infections and even medications. It’s important to become familiar with your HAE triggers so you can be prepared should an attack occur.
Be aware of events, changes in medications, or situations that may precede or trigger an HAE attack
Avoid situations that may trigger an attack. Talk to your doctor before exposure to any known triggers, such as dental work, surgery or giving birth.
Pay careful attention to patterns of attacks and identify any medications (such as oestrogen contraceptives, hormone replacement therapy, or ACE inhibitors (for high blood pressure)) that may contribute to the onset of your HAE attacks.
For some people with HAE, an attack cannot be avoided. In that case, the best policy is to be prepared (see below for further advice).
Many people living with HAE find that it helps to work with their healthcare team to manage the condition. One of the strategies to help you and your healthcare team manage your HAE effectively is to make sure you understand what you can expect from each other.
Maintain a detailed history of HAE attacks:
Be informed and mindful of health conditions.
Know about other family members with HAE.
Have an agreement on pain medication.
Provide a letter of diagnosis to be shown to the hospital A&E department if required.
Keep a detailed record of HAE attacks:
Tell your doctor about any changes in medication or any planned surgeries or dental procedures.
Make healthy lifestyle and food choices.
Be aware of mood changes.
Know your prodromal symptoms (early HAE attack warning signs).
Get to a hospital when necessary.
Have someone who will watch out for you.
Remain hydrated to replace fluids lost to swelling.
During painful attacks, take pain control medications early.
Notify your doctors, dentists and your child’s school, e.g. by providing a letter of diagnosis.
If you believe you might have HAE, the first step is to visit your GP who will be able to assess your symptoms, conduct some investigations and decide whether you need to be referred to an immune system specialist, called an immunologist, who can further help with diagnosis and treatment.
HAE impacts everyone differently depending on the severity of the disease as well as the frequency, type, and timing of attacks.
If you are responsible for a child with HAE, it’s recommended that you get them tested after age one for levels of two proteins that regulate the immune system:
Before the age of one, tests can often be inaccurate. Even if a child has no symptoms, an advance diagnosis may help you prepare and put measures in place for a possible future attack.
Doctors report that their youngest patients who have HAE attacks tend to be pre-schoolers rather than infants or toddlers. However, some people do not experience attacks until their teen years or later.
If your young child does have attacks, keep in mind that they are likely to have far more difficulty than adults in conveying feelings of discomfort, pain and other medical symptoms. So try and be alert to the changes in your child’s mood or behaviour that may herald the onset of an attack and as your child grows, help him or her identify and name prodromal symptoms (e.g. a rash or irritability), as well as any personal triggers.
If your child has HAE it is also important to inform healthcare professionals, nursery/preschool or school teachers, holiday clubs and after-school programmes.
It’s recommended that you provide a letter from your child’s doctor wherever your child plays or goes to school, to help anyone supervising your child understand his or her condition.
As teenagers develop more independence and spend more time away from home, it becomes increasingly important for them to carry personal medical information. An ID bracelet or chain, a card in a wallet or purse, and an understanding of HAE among friends can discreetly help ensure safety, without having to miss out.
When approaching adulthood, the ultimate responsibility for a young adult’s health should shift gradually from parent/guardian to the individual. This includes having a firm grasp of personal triggers, warning symptoms and treatment options, which will help when moving on to university or into the working world.
Although no definitive causes for HAE attacks have been established, attacks may be triggered by emotional or physical stress, surgical procedures and even mild dental work.
Changes in hormonal levels have been associated with increased HAE attacks in women, and many young women report changes in attack frequency and severity when using contraceptives that contain oestrogen.
Other medications may also impact HAE attacks, so before starting a new prescription be sure to discuss possible options with your healthcare team.
Unfortunately, there’s no definitive way to establish how pregnancy will affect HAE in different women; some notice an increase in the frequency of attacks during pregnancy, while others see a decline in number and/or severity. Similarly, many women find that their attacks return to their previous frequency after they give birth, while others experience a permanent change.
If you are considering hormone replacement therapy during this stage of your life, make sure you discuss safe treatment options with your healthcare team.