Table of ContentsView AllTable of ContentsSymptomsTiming and FrequencySigns in Someone ElseSpecialistsMonitoring
Table of ContentsView All
View All
Table of Contents
Symptoms
Timing and Frequency
Signs in Someone Else
Specialists
Monitoring
Hereditaryangioedema(HAE) is a rare inherited condition that causes episodes of swelling inside and outside the body. Its effects can include visible symptoms, such as hand swelling, as well as symptoms due to swelling inside the body, such as abdominal discomfort.
The effects and symptoms of HAE can occur unexpectedly and with an unpredictable pattern. Certaintriggers, such as getting a cold or experiencing stress, can also cause the symptoms to develop.
HAE treatmentscan help prevent episodes from occurring, and some medications can help decrease the impact of an episode that’s occurring.
Recognizing Hereditary Angioedema Symptoms
The key effects of HAE are swelling of the hands, feet, tongue, throat, and intestines. The effects of HAE can cause you to feel tired and sick overall, in addition to your specific symptoms. Often, the first symptoms of HAE begin during childhood, may worsen during adolescence, and continue into adulthood.
Some people may notice warning signs before an episode, which may involve tingling, nausea, or fatigue.
What Is Angioedema?
Emergency Symptoms
Breathing problems due to tongue or throat swelling are the most serious symptoms of HAE.If you or anyone you know (who has or has not been diagnosed with HAE) appear to be choking or having trouble breathing, it’s important to get emergency medical attention.
Gastrointestinal (GI) symptoms of HAE can become serious and even potentially life-threatening.If nausea, abdominal discomfort, pain, or vomiting become severe or persistent, get medical attention.
This photo contains content that some people may find graphic or disturbing.See PhotoReproduced with permission from © DermNetdermnetnz.org2023.
This photo contains content that some people may find graphic or disturbing.See Photo
This photo contains content that some people may find graphic or disturbing.
Reproduced with permission from © DermNetdermnetnz.org2023.
Symptoms of Angioedema
Skin Symptoms
The most visible symptoms of HAE are angioedema of the hands and angioedema of the feet. The swelling of the hands and feet looks like puffiness, can cause pain and discomfort due to stretching of the skin, and can affect one or both sides of the body. Sometimes HAE is associated with a skin rash.
This photo contains content that some people may find graphic or disturbing.See PhotoSaevichMikalai / Getty Images
SaevichMikalai / Getty Images
GI Symptoms
Swelling of the intestines can cause a variety of symptoms that are vague and not necessarily specific to HAE.
GI symptoms of HAE include:
If you or your child has already been diagnosed with HAE, these symptoms could indicate a flare-up.
Does Not Respond to Medications for Allergic ReactionsSymptoms of HAE do not improve with treatments typically used for allergic reactions, such asantihistaminesand steroids.
Does Not Respond to Medications for Allergic Reactions
Symptoms of HAE do not improve with treatments typically used for allergic reactions, such asantihistaminesand steroids.
Symptom Timing and Frequency of Episodes
Symptoms of HAE episodes begin suddenly and typically develop over a few hours. If not treated, they may last up to a week.
Some people may experience more frequent and severe effects. Others with the condition may not have as many effects. Noticing the timing, triggers, or any factors that may be associated with your symptoms can help reduce the likelihood of having them.
Therapies used to treat ongoing symptoms and certain medications used to prevent symptoms can help reduce the frequency, severity, and impact of your symptoms.
Unpredictability of HAE symptoms
How to Identify HAE Signs in Someone Else
If your child is at risk of this condition because it runs in the family, you may already know the symptoms and might bewatching for its effects on your child.
When you know someone of any age who has HAE, it’s important that you are aware of the danger of breathing problems with this condition. This can start with symptoms such as difficulty swallowing and struggling to speak.
Gasping for air is a sign that a person’s airway is blocked. This is an emergency situation requiring immediate medical attention.
How Angioedema Is Diagnosed
Specialist Who Oversees Treatments
If you or your child has HAE, it’s important to have medical care from a team of specialists who are familiar with this rare condition.
Providers you may need to see if you have HAE include:
Many different medications can trigger or worsen HAE, includingangiotensin converting enzyme (ACE) inhibitors(a type of blood pressure–lowering medication) and estrogen-based therapies.Your pharmacist, as well as any healthcare providers who prescribe medication for you, should be made aware that you have HAE.
How to Monitor HAE Symptoms
After diagnosis of HAE, an individual management plan will be developed.This will include educating the person and their caregivers on symptoms, triggers, andwhen and how to use medications to prevent or treat an episode.
Keeping a symptom diary is often part of the management plan. Information to record about an attack includes:
Beyond the immediate and distressing symptoms, HAE has been associated with a higher-than-average risk of blood clots.
Blood clots can develop anywhere in the body and may cause various symptoms, including pain, swelling, and weakness. Getting medical attention for any new symptoms you experience is important, even if they are not your usual HAE symptoms.
Summary
Hereditary angioedema causes episodes of swelling of the hands and feet, abdominal symptoms, and difficulty speaking, swallowing, and breathing. All of the symptoms occur when HAE causes edema (swelling) in these areas of the body.
Symptoms can be unpredictable and distressing, and triggers such as stress, illness, and medical procedures can increase the likelihood of symptoms.
If you have a family history of HAE, you must be on the lookout for these symptoms in your child. Swelling of the throat can make it hard to breathe and can be life-threatening.
If you’ve already been diagnosed with HAE, you need to be aware of the symptoms and get prompt medical attention if you experience serious symptoms. Medical therapies can help her reduce the frequency and severity of symptoms of HAE and improve your quality of life.
9 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Busse PJ, Christiansen SC, Riedl MA. et al.US HAEA Medical Advisory Board 2020 guidelines for the management of hereditary angioedema.J Allergy Clin Immunol Pract.2021;9(1):132-150.e3. doi:10.1016/j.jaip.2020.08.046Gil-Serrano J, Labrador-Horrillo M, Galvan-Blasco P, et al.Systemic inflammation biomarkers during angioedema attacks in hereditary angioedema.Front Immunol.2024;15:1400526. doi:10.3389/fimmu.2024.1400526Sarkar A, Nwagwu C, Craig T.An overview of hereditary angioedema for the primary care physician.Med Clin North Am.2024 Jul;108(4):747-755. doi:10.1016/j.mcna.2023.08.005Do T, Riedl MA.Current and emerging therapeutics in hereditary angioedema.Immunol Allergy Clin North Am.2024;44(3):561-576. doi:10.1016/j.iac.2024.03.009Perego F, Zingale LC, Cesoni Marcelli A, et al.A human centred innovative approach based on persona in hereditary angioedema.Orphanet J Rare Dis. 2024;19(1):291. doi:10.1186/s13023-024-03302-xGenetic and Rare Diseases Information Center.Hereditary angioedema.Caballero T, Canabal J, Rivero-Paparoni D, Cabañas R.Management of hereditary angioedema in pregnant women: a review.Int J Womens Health. 2014;6:839-48. doi:10.2147/IJWH.S46460Valerieva A, Nedeva D, Yordanova V, Petkova E, Staevska M.Therapeutic management of hereditary angioedema: past, present, and future.Balkan Med J. 2021;38(2):89-103. doi:10.5152/balkanmedj.2021.21094Kyrle PA, Eichinger S.Hereditary angioedema: beyond swelling.Blood. 2024 Jul 25;144(4):354-355. doi:10.1182/blood.2024024958
9 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Busse PJ, Christiansen SC, Riedl MA. et al.US HAEA Medical Advisory Board 2020 guidelines for the management of hereditary angioedema.J Allergy Clin Immunol Pract.2021;9(1):132-150.e3. doi:10.1016/j.jaip.2020.08.046Gil-Serrano J, Labrador-Horrillo M, Galvan-Blasco P, et al.Systemic inflammation biomarkers during angioedema attacks in hereditary angioedema.Front Immunol.2024;15:1400526. doi:10.3389/fimmu.2024.1400526Sarkar A, Nwagwu C, Craig T.An overview of hereditary angioedema for the primary care physician.Med Clin North Am.2024 Jul;108(4):747-755. doi:10.1016/j.mcna.2023.08.005Do T, Riedl MA.Current and emerging therapeutics in hereditary angioedema.Immunol Allergy Clin North Am.2024;44(3):561-576. doi:10.1016/j.iac.2024.03.009Perego F, Zingale LC, Cesoni Marcelli A, et al.A human centred innovative approach based on persona in hereditary angioedema.Orphanet J Rare Dis. 2024;19(1):291. doi:10.1186/s13023-024-03302-xGenetic and Rare Diseases Information Center.Hereditary angioedema.Caballero T, Canabal J, Rivero-Paparoni D, Cabañas R.Management of hereditary angioedema in pregnant women: a review.Int J Womens Health. 2014;6:839-48. doi:10.2147/IJWH.S46460Valerieva A, Nedeva D, Yordanova V, Petkova E, Staevska M.Therapeutic management of hereditary angioedema: past, present, and future.Balkan Med J. 2021;38(2):89-103. doi:10.5152/balkanmedj.2021.21094Kyrle PA, Eichinger S.Hereditary angioedema: beyond swelling.Blood. 2024 Jul 25;144(4):354-355. doi:10.1182/blood.2024024958
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
Busse PJ, Christiansen SC, Riedl MA. et al.US HAEA Medical Advisory Board 2020 guidelines for the management of hereditary angioedema.J Allergy Clin Immunol Pract.2021;9(1):132-150.e3. doi:10.1016/j.jaip.2020.08.046Gil-Serrano J, Labrador-Horrillo M, Galvan-Blasco P, et al.Systemic inflammation biomarkers during angioedema attacks in hereditary angioedema.Front Immunol.2024;15:1400526. doi:10.3389/fimmu.2024.1400526Sarkar A, Nwagwu C, Craig T.An overview of hereditary angioedema for the primary care physician.Med Clin North Am.2024 Jul;108(4):747-755. doi:10.1016/j.mcna.2023.08.005Do T, Riedl MA.Current and emerging therapeutics in hereditary angioedema.Immunol Allergy Clin North Am.2024;44(3):561-576. doi:10.1016/j.iac.2024.03.009Perego F, Zingale LC, Cesoni Marcelli A, et al.A human centred innovative approach based on persona in hereditary angioedema.Orphanet J Rare Dis. 2024;19(1):291. doi:10.1186/s13023-024-03302-xGenetic and Rare Diseases Information Center.Hereditary angioedema.Caballero T, Canabal J, Rivero-Paparoni D, Cabañas R.Management of hereditary angioedema in pregnant women: a review.Int J Womens Health. 2014;6:839-48. doi:10.2147/IJWH.S46460Valerieva A, Nedeva D, Yordanova V, Petkova E, Staevska M.Therapeutic management of hereditary angioedema: past, present, and future.Balkan Med J. 2021;38(2):89-103. doi:10.5152/balkanmedj.2021.21094Kyrle PA, Eichinger S.Hereditary angioedema: beyond swelling.Blood. 2024 Jul 25;144(4):354-355. doi:10.1182/blood.2024024958
Busse PJ, Christiansen SC, Riedl MA. et al.US HAEA Medical Advisory Board 2020 guidelines for the management of hereditary angioedema.J Allergy Clin Immunol Pract.2021;9(1):132-150.e3. doi:10.1016/j.jaip.2020.08.046
Gil-Serrano J, Labrador-Horrillo M, Galvan-Blasco P, et al.Systemic inflammation biomarkers during angioedema attacks in hereditary angioedema.Front Immunol.2024;15:1400526. doi:10.3389/fimmu.2024.1400526
Sarkar A, Nwagwu C, Craig T.An overview of hereditary angioedema for the primary care physician.Med Clin North Am.2024 Jul;108(4):747-755. doi:10.1016/j.mcna.2023.08.005
Do T, Riedl MA.Current and emerging therapeutics in hereditary angioedema.Immunol Allergy Clin North Am.2024;44(3):561-576. doi:10.1016/j.iac.2024.03.009
Perego F, Zingale LC, Cesoni Marcelli A, et al.A human centred innovative approach based on persona in hereditary angioedema.Orphanet J Rare Dis. 2024;19(1):291. doi:10.1186/s13023-024-03302-x
Genetic and Rare Diseases Information Center.Hereditary angioedema.
Caballero T, Canabal J, Rivero-Paparoni D, Cabañas R.Management of hereditary angioedema in pregnant women: a review.Int J Womens Health. 2014;6:839-48. doi:10.2147/IJWH.S46460
Valerieva A, Nedeva D, Yordanova V, Petkova E, Staevska M.Therapeutic management of hereditary angioedema: past, present, and future.Balkan Med J. 2021;38(2):89-103. doi:10.5152/balkanmedj.2021.21094
Kyrle PA, Eichinger S.Hereditary angioedema: beyond swelling.Blood. 2024 Jul 25;144(4):354-355. doi:10.1182/blood.2024024958
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