P83: Quality of Life (qol) Assessments Following the Use of Prophylactic C1 Esterase Inhibitor Concentrate in Patients With Severe Hereditary Angioedema
Year: 2017
Type: conference-abstract
Abstract: Hereditary Angioedema (HAE) is a rare genetic condition resulting in a deficiency in C1 inhibitor protein and is characterised by both provoked and unprovoked episodes of angioedema. In a severe phenotype patients may experience repeated and frequent facial or limb angioedema, severe and recurrent abdominal pain and a risk of death by asphyxiation as a result of upper airway angioedema. The relentless nature of this condition with frequent and debilitating attacks along with previous ‘on-demand’ treatment requiring a hospital presentation for each attack has a profound impact on the quality of life for this patient group. In 2016 the National Blood Authority (NBA) allowed for the routine supply of C1 inhibitor concentrate to HAE patients experiencing eight or more acute attacks per month. With appropriate training patients are able to self administer treatment at home thus avoiding hospital presentations. This change in treatment is predicted to provide a dramatic change to the QOL for this patient group. HAE patients completed a HAE-QoL score pre and post the commencement of prophylactic use of C1 inhibitor concentrate, along with school/work attendance and documentation of disease activity. Following prophylactic use of C1 inhibitor concentrate, we have seen a marked reduction in disease activity and resultant improvement in quality of the life as measured by HAE-QoL score and school/work attendance. The availability of prophylactic use of C1 inhibitor concentrate in HAE patients with a severe phenotype has had a significant beneficial effect on the quality of life of this patient group.
Source: Internal Medicine Journal
DOI: 10.1111/imj.83_13578
Authors: A Merry, D Heyworth‐Smith, C Kennedy, S Robertson, L James
Institution: Greenslopes Private Hospital
Language: English
Subfield: Genetics
Field: Medicine
Domain: Health Sciences
Open Access status: bronze
OpenAlex ID: W2751323892