Everyone with hereditary angioedema (HAE) experiences the condition differently. You’ll need to work with your doctor to develop a personalized treatment plan. Your treatment plan can help prevent and relieve symptoms earlier. It can also prevent symptoms from becoming life-threatening.
Your treatment plan will also help you:
- avoid situations that trigger attacks
- address attacks early
- manage life-threatening emergencies from airway swelling or shock
While there’s no cure for HAE, on-demand and preventive medications can help you control symptoms and how they impact your life. Since the severity of HAE can change over time, it’s important to regularly review your treatment with your doctor.
With early treatment, an HAE attack will be shorter and your swelling will decrease faster. It can take anywhere from 30 to 60 minutes before an on-demand treatment starts working.
The FDA has approved five medications to treat HAE attacks. These medications are delivered by injection or intravenously. They include:
- C1 inhibitors (Cinryze, Berinert, Ruconest)
- bradykinin receptors (Firazyr)
- enzyme inhibitors (Kalbitor)
Treatments work best when you get them early in the attack. For this reasons, you’ll likely be trained to provide yourself treatments at home or on the go. If you self-treat, your plan should include knowing where your medication is kept, how to use it, and who to contact if you need help.
Even if you self-treat, you should still seek medical care if:
- the attack involved your airway
- the treatment didn’t work as you expected
- the attack was unusual in any way
You should also have a backup plan in case your attack leads to throat swelling. This includes making sure you always have enough on-demand medication and knowing how to get to the nearest emergency room.
If you’re giving yourself on-demand treatment at home, you should have a backup plan in case your first line of treatment doesn’t work. This is especially important if the attack includes throat swelling. According to U.S. HAE Association recommendations, your backup plan should include:
- being ready access emergency care if you have problems with airway swelling
- discussing your HAE with your local hospital
- carrying a letter or electronic file summarizing your condition and treatment
- having your electronic medical records flagged so it’s clear that you have a rare condition with potentially fatal symptoms
Preventive treatments are taken at regular intervals over days, months, or years. These treatments may be given ahead of an event that could trigger an attack, such as dental surgery.
Preventive treatments include androgens or C1 inhibitors. Your choice of treatment depends on the severity of your HAE and your tolerance of the treatment’s side effects.
Women are known to have negative reactions to androgen therapy and shouldn’t use it before or during pregnancy. Attacks can still happen during preventive treatment, so it’s important to always be prepared for on-demand treatment.
Keeping track of your HAE
The U.S. HAE Association recommends keeping a paper or electronic log of every attack. By logging your attacks you can discover and avoid situations that trigger your HAE. It will also help in case you need to adjust your treatment plan. The log should contain:
- a description of your attack
- what you were doing before and during the attack
- how you treated the attack
- your response to the treatment
Communicating with your doctor
A doctor who is familiar with HAE and stays up-to-date on treatment options is an important partner in managing your condition. You should work with your HAE specialist to make sure your family, primary care doctor, and community emergency department or hospital are aware of your treatment plan.
Your treatment plan should include regular follow-ups with your HAE specialist. These visits are important to make sure your treatment is safe and effective. Since your HAE symptoms may change over time, your doctor can help make sure your treatment plan stays current.
Having a plan for treating and managing your HAE will help keep you safe from the most worrying symptoms of HAE while empowering you to maintain an active life.