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People with Rare Life-Threatening Condition to Walk Around the World for Global hae day :-) Awareness Day

May 16 marks International hae day 🙂 (HAE Day) – Hereditary Angioedema Day – to raise awareness of this debilitating disease.
According to Janice Strydom, Chairman of HAE SA, it is believed that many South Africans are living with this disease but remain undiagnosed – meaning they are suffering unnecessarily, and even at risk of death.

“There are only 104 patients diagnosed with HAE in South Africa. With a global incidence of 1 in 50 000, we know that there are many more people out there who remain undiagnosed. We are committed to raising awareness around HAE and locating those people, to provide them with the knowledge and care they need. We never want to see another South African with HAE lose their life because they are unable to access suitable treatment.”

Knowledge is essential to avoid misdiagnosis and even death
Sadly, due to a lack of awareness and misdiagnosis, it can take many years to recognize and diagnose HAE in a patient. Many doctors aren’t familiar with HAE symptoms, which may be similar to those of other common illnesses as well as allergies. This often leads to an incorrect diagnosis and consequently the wrong treatment. In some instances patients have died due to lack of correct treatment as doctors were unaware of the underlying cause.

What is HAE?
Professor Mike Levin, CEO of the Allergy Foundation of South Africa (AFSA) and head of the division of Paediatric Allergology at UCT, explains that HAE causes sufferers to experience recurrent, painful episodes of swelling of the skin or mucous membranes. This results in swelling of various body parts, including the hands, feet, face, gastrointestinal tract, and throat.

“Swelling in the throat is the most dangerous aspect of HAE because the airway can become constricted and, without treatment, can cause death by suffocation. Swelling in the abdomen and intestinal wall causes excruciating abdominal pain, nausea, vomiting, and/or diarrhoea.”

What causes HAE?
“Most people with HAE have a problem with a gene that produces a protein in the blood called C1-inhibitor.  Lack of C1 inhibitor triggers the uncontrolled release of bradykinin which causes fluid from small blood vessels to leak into, and under, the surrounding skin resulting in localized swelling and pain,” explains Prof Levin.

HAE symptoms, and why they can baffle doctors
According to Professor Johnny Peter, head of the Allergy and Immunology Unit at Groote Schuur and Head of Allergology and Clinical Immunology at UCT, HAE symptoms can differ from person to person. This means that one person with HAE could experience symptoms with greater severity and frequency, or in different locations, than someone else. As mentioned above, common symptoms include unexplained swelling in different parts of the body, most often in the abdomen, face, feet, genitals, hands or throat.

“HAE symptoms can also vary within the same person. For example, some women with HAE experience a shift in the frequency of their HAE attacks during various life stages, such as puberty, pregnancy or menopause. Patients with early-onset HAE tend to be more likely to suffer from a severe course of disease.”

Strydom, who lives with HAE herself, explains that people with HAE experience a significant disease burden and can have a reduced quality of life. “Due to pain and other debilitating symptoms of attacks, HAE can impact a person’s ability to carry out daily activities like going to work or school, or participating in leisure and social activities. Untreated HAE patients can lose 100 to 150 workdays per year, if not more”

“Because of this, people with HAE can also experience higher levels of depression and anxiety – exacerbated by the unpredictable nature of attacks, the potential for choking from a throat attack, and not being sure when one will strike.”

Triggers of an HAE attack
“In both children and adults, most HAE attacks occur without any warning,” says Prof Peter. “Yet some patients have been able to identify triggers that seem to set off their attacks. These triggers can vary from person to person and can be particularly difficult for children to identify or describe to their caregivers, such as:

  • Emotional stress
  • Injury
  • Infection
  • Dental or medical procedures
  • Hormonal influences, like menstruation
  • Mechanical pressure from physical activities, like typing or mowing the lawn

“It’s important to recognize what types of activities and situations may lead to your or your child’s attacks. Keeping a journal of these episodes may improve communication of symptoms and triggers for your doctor.”

HAE treatment
Thankfully, a simple blood test can confirm an HAE diagnosis and HAE can be successfully managed with effective preventative (prophylaxis) and on-demand (acute) treatment.

hae day 🙂 is organized by HAE International (HAEi), a global non-profit network of 93 patient associations dedicated to improving the lives of people with HAE. hae day 🙂 2022 will see people from across South Africa converting their activity time to walking around the world, raising awareness of the rare disease, hereditary angioedema (HAE).”

“We are very excited to be part of the global HAE activity for hae day :-). By coming together, we spotlight HAE and strengthen our voice to call for improved diagnosis, and consistent access to lifesaving therapies for everyone with HAE,” says Strydom.

HAE SA is urging South Africans to join them and take part in hae day :-). 2022 to help raise awareness that will enable timeous diagnosis and treatment.

“We hope everyone will be ‘Stepping Up for the Global HAE Movement’ and helping us to walk around the World as many times as possible in time for 16 May,” concludes Henrik Balle Boysen, Executive Vice President & Chief Operating Officer of HAEi.

You can learn more about HAE at https://haei.org/hae/faq/ or visit www.haei.org/southafrica. Follow HAE SA at www.facebook.com/haesouthafrica or on Instagram at @hae_southafrica. For further information and support contact Janice Strydom on haeadvocacysa@gmail.com or call 083 309 3569.


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