<p>Self-Management Plans in Patients with Hereditary Angioedema: Strategies, Outcomes and Integration into Clinical Care</p>

Chronic conditions, whether genetic or acquired, impose a significant burden on health care systems with high utilisation of hospital and emergency department resources. Self-management is increasingly recognised as one of the pillars in models of care for those with long-term medical conditions. Hereditary angioedema (HAE) is a rare genetic disorder inherited in an autosomal dominant fashion. It is characterised by the occurrence of unpredictable attacks of swelling (angioedema) affecting many body parts including subcutaneous tissues, the gut mucosa and the upper airway. For those affected, it is associated with a high burden of illness and poor quality of life as a result of its unpredictability and the threat of asphyxiation from upper airway oedema or severe pain from abdominal involvement. Prompt recognition and appropriate treatment are necessary to avoid the pain and suffering associated with attacks and to manage life-threatening laryngeal swellings that around 50% of HAE patients will experience in their lifetime. Since the early 2000s, a number of very effective, albeit expensive, treatment options have become available, at least in some countries. Utilisation of these options within a written patient self-management plan provides the most satisfactory treatment outcomes and improves patient quality of life. Successful self-management depends on a productive partnership between patient and health care professional, with patient education the cornerstone of a successful outcome. This is a dynamic process, particularly in a condition such as HAE where frequency and severity of attacks may vary given different life circumstances.