Hereditary angioedema (HAE) is an autosomal dominant disorder characterized by deficient or dysfunctional C1 esterase inhibitor. Clinically, it is characterized by paroxysmal attacks of swelling of subcutaneous tissues and mucous membranes that may be life threatening. Current long-term treatment is achieved with the attenuated androgens danazol and stanozolol, drugs that are known to have minimal virilizing side effects. We report a teenager with hereditary angioedema whose nonadherence with the prescribed medications and clinic visits, as-well as her incomplete understanding of the life-threatening severity of the disease, led to acute airway obstruction requiring tracheostomy. Following appropriate patient education measures, she was adherent to the prescribed danazol. Her disease was controlled at a minimal dose (200 mg/day) with no significant side effects. This case report highlights the importance of adherence and the value of education in patients with this disorder or any other chronic disease.
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health
- Immunology and Allergy
- Pulmonary and Respiratory Medicine