Hereditary Angioedema (HAE) is a rare genetic disorder characterized by recurrent episodes of swelling in various parts of the body, including the skin, gastrointestinal tract, and airways. It is caused by a deficiency or dysfunction of a protein called C1 esterase inhibitor (C1-INH), which plays a crucial role in regulating inflammation and preventing excessive swelling.
Unfortunately, there is currently no known cure for Hereditary Angioedema. However, there are several treatment options available to manage the symptoms and prevent or reduce the frequency and severity of attacks.
Acute attacks of angioedema can be treated with medications such as antihistamines, corticosteroids, and medications that block the effects of certain inflammatory substances. These medications help to relieve the swelling and associated symptoms, such as pain and discomfort.
Long-term management of Hereditary Angioedema involves two main approaches: preventive therapy and on-demand therapy.
Preventive therapy aims to reduce the frequency and severity of attacks by increasing the levels of functional C1-INH in the body. This can be achieved through regular infusions of C1-INH concentrate or medications that stimulate the production of C1-INH. These preventive treatments can help to minimize the impact of HAE on daily life and reduce the risk of life-threatening attacks.
On-demand therapy involves the prompt treatment of acute attacks as they occur. This typically includes the use of medications that help to relieve symptoms and reduce swelling. It is important for individuals with HAE to have access to these medications and to be aware of the early signs of an attack to initiate treatment promptly.
In addition to medical interventions, lifestyle modifications can also play a role in managing Hereditary Angioedema. These may include avoiding triggers that can precipitate attacks, such as certain medications, stress, trauma, or infections. It is important for individuals with HAE to work closely with their healthcare team to develop a personalized management plan that suits their specific needs.
Research is ongoing to better understand the underlying mechanisms of Hereditary Angioedema and develop new treatment options. While a cure for HAE remains elusive, advancements in medical science continue to improve the quality of life for individuals living with this condition.