Hereditary angioedema (HAE) is a rare genetic disorder that causes swelling in different areas of the body for several days. Ways to care for a child with HAE include helping prevent and manage attacks and getting the right support.

HAE symptoms can resemble those of other syndromes and disorders. This can lead to a delay in diagnosis. In the meantime, a child may be living with fear, pain and discomfort, and unusual stress related to attacks of swelling.

While getting a proper diagnosis is an important first step, parents and caregivers have other considerations when caring for a child with HAE.

This article reviews tips and strategies to help care for a child with HAE.

HAE is a rare condition that typically starts in young children and worsens during and after puberty.

Due to its rarity, doctors are often unaware of the condition before working with a child who has it. Doctors may mistake symptoms, such as stomach pain or upset, as resulting from other conditions.

Most cases of angioedema — swelling of deep layers of the skin and other tissues — are not the result of HAE, so it is reasonable that a doctor may misdiagnose the condition.

However, it can take up to a decade from the first symptoms to get an accurate diagnosis. The only way to confirm a diagnosis is via blood and genetic testing. Doctors will look for:

  • C1-inhibitor quantitative (antigenic)
  • C1-inhibitor functional
  • C4 levels

Parents or guardians who suspect their child may have HAE may need to advocate for their child and look for doctors who may be familiar with the condition.

A parent or guardian can also use the US Hereditary Angioedema Association’s physician finder, which helps connect people with doctors familiar with the condition who may be able to help diagnose HAE sooner.

Studies show that living with HAE has a profound effect on young children and teens. This can include:

  • painful attacks
  • unnecessary procedures looking for answers about symptoms
  • emotional distress
  • hiding symptoms due to fears of social isolation or anxiety
  • risk of death

Parents and caregivers may be able to help reduce stress and anxiety by talking with their child about the condition. Giving a child a safe space to communicate what they are going through, their symptoms, and a chance to ask questions may help them navigate the disorder.

Connecting a child with a psychologist or counselor may also be helpful. They do not necessarily need to be familiar with HAE, but counselors familiar with helping children who live with chronic diseases may be beneficial.

Counselors can help a child express their emotions and cope with the stresses related to living with HAE. This may include learning strategies, such as stress reduction or mindfulness.

HAE causes a person to experience attacks. Attacks are recurring episodes of swelling in one or more areas of the body, including the:

  • hands
  • feet
  • genitals
  • stomach
  • face
  • throat

These periods can last for about 2–5 days.

HAE attacks may not be fully preventable as some are spontaneous, but children and caregivers can take steps to help reduce the chances of an attack by understanding and then managing triggers. Triggers are anything that can cause an attack to occur.

Some common triggers include:

  • stress
  • typing for long periods
  • anxiety
  • surgery
  • minor trauma
  • hammering
  • illnesses, such as colds, flu, or other viral infections
  • shoveling
  • engaging in physical activities
  • hormonal changes
  • taking certain medications such as ACE inhibitors
  • dental procedures

Understanding what can trigger a child’s attack can be challenging. Keeping a journal of activities and when attacks occur may help determine them.

HAE requires a different treatment approach than allergic angioedema. Allergy-driven swelling will typically respond to antihistamines, epinephrine, and corticosteroids. HAE will not.

Treatment for HAE typically includes the use of medications such as:

  • C1 inhibitor concentrate
  • icatibant, a bradykinin-receptor antagonist
  • ecallantide, a kallikrein inhibitor

These medications may help treat an attack and help prevent a new one from occurring.

As many as 50% of people with HAE may experience swelling that closes their airways, with as many as 30% of these cases being fatal. In an emergency situation where swelling blocks the airways, doctors may need to use intubation or surgical airway intervention to restore breathing.

Once a child receives a diagnosis of HAE, parents and caregivers may wish to discuss treatment strategies and emergency plans.

The right care team may make a difference in the care a child receives for HAE. Few pediatricians are familiar with HAE due to the rarity of the condition. This can make both diagnosis and treatment more challenging.

To locate a doctor, a person can use the US Hereditary Angioedema Association’s physician finder. This tool highlights doctors familiar with HAE that may be nearby.

Often, an allergist-immunologist will provide the primary care for a child or adolescent with HAE. They can also make the initial diagnosis.

In addition, other specialists that can play a part in care and management include:

  • pulmonologist
  • primary care physicians
  • pediatric doctors
  • emergency room doctors

It may be helpful to create a binder or other way of organizing information with key or important information that doctors and the care team should know. A parent or caregiver can then bring the binder to help explain the condition to doctors who may not be familiar with it.

Supportive services and groups are available and may help with several aspects surrounding HAE. Some possible services and benefits include:

  • patient registries
  • ways to connect with others
  • up-to-date treatment and research information
  • areas to share personal stories
  • easy-to-read information
  • financial aid and travel resources
  • lists of specialists or specialty centers

The US Hereditary Angioedema Association offers many services for family members and children with HAE. A person can donate time or money, find support groups, look for doctors in their area, and other care-related services.

A parent or caregiver with a child with HAE can start by exploring the different resources.

HAE can be stressful, uncomfortable, and potentially life threatening to children. Parents and guardians can take steps to help their child understand the disease, monitor and prevent attacks, find the right doctors, and make sure that their treatment works for them.

Organizations, such as the US Hereditary Angioedema Association, can help provide additional resources, including general information, ways to find local doctors, and connections with support groups that can help parents and caregivers as they support their child.