Prophylaxis in hereditary angioedema: a United Kingdom Delphi consensus.

Hereditary angioedema (HAE) is a rare inherited disorder causing recurrent episodes of swelling that can be potentially life threatening. Treatment of HAE can be divided into on-demand treatment for swelling, and prophylaxis. The last UK consensus on HAE was in 2014 and since then, new medications for prophylaxis have been developed, with more drugs in the pipeline. International guidelines currently recommend the use of long-term prophylaxis (LTP) as the only way of achieving disease control and normalizing patient lives. Modern prophylactic medications are available in the UK, although access is restricted primarily by HAE attack frequency. To establish an updated view of UK clinicians and patients, a Delphi process was used to develop statements regarding LTP as well as other aspects of HAE management. There was consensus that UK access criteria for modern LTP agents based on numerical frequency of attacks alone are too simplistic and potentially disadvantage a cohort of patients who may benefit from LTP. Additionally, there was agreement that patients should be seen in expert centres, remote monitoring of patients is popular post-pandemic, and that the use of patient-reported outcome measures has the potential to improve patient care. Psychological health is an area in which patients may benefit, and recognition of this is important for future research and development.

Challenges of classifying and mapping perennial freshwater systems within highly variable climate zones: A case study in the Murray Darling Basin, Australia.

Perennial freshwater systems are valuable natural resources that provide important ecological services globally. However, in highly variable climates, such as Australia, water availability in rivers and streams can vary greatly from year to year and from decade to decade. Further, across Australia and many other regions, perennial river systems are projected to decrease because of anthropogenic climate change, placing the ecosystems they support under additional pressure. Quantifying the potential impacts of climate change on perennial freshwater systems requires robust databases of existing water features with accurate classifications. This is a challenge for rivers that display a high degree of interannual variability since the river classification can be dependent on the period of available data. In this study, we carry out a regional scale comparison of three different spatial databases commonly used in environmental and ecological assessments of perennial systems of Australia, namely Geodata, Geofabric and Water Observations from Space (WOfS). Focusing on the southern Murray Darling Basin (MDB), due to its national and international significance and its highly variable flow regimes, we show that no single spatial database is reliable by itself in terms of perennial water classification, with notable differences likely arising from variations in the periods analysed and methods used to classify the systems. Further, an analysis of high-quality gauged streamflow data (with approximately 40-year daily records) for four sub-catchments, and long-term simulation data (>100 years) for two sub-catchments in the lower MDB, confirm that flow persistence can be non-stationary through time, with some 'perennial' systems exhibiting sustained periods of cease to flow (i.e. becoming non-perennial) during prolonged droughts. This study demonstrates that due consideration is required in developing baseline classification of perennial freshwater systems for assessing future changes and measuring adaptive capacity.

Hereditary Angioedema patient experiences of medication use and emergency care.

BACKGROUND: Hereditary angioedema (HAE) is a rare inherited illness characterised by recurrent swellings. Four percent of HAE attacks are life threatening throat swellings requiring urgent medical intervention. The aim of this study was to gain an in depth understanding of patient experiences of use of treatment and emergency care. METHODS: 65 participants completed an online survey including open ended questions about their experiences and/or completed an in-depth interview. Interview participants were asked to share and talk about up to five images that they felt best represented their life experiences with HAE. Data were analysed using reflexive thematic analysis. RESULTS: Treatment experiences highlighted the life changing nature of new treatments and benefits for quality of life, but also illustrated common barriers to treatment administration. Emergency care experiences illustrated how throat attacks and fear of their future occurrence could be traumatic. DISCUSSION: Findings indicate that HAE patients need psychological support to process fears and negative experiences. In addition, psychological barriers to treatment administration must be addressed to ensure treatment is used effectively. Education for emergency practitioners is also needed to improve emergency treatment and reduce the psychological burden of delayed emergency care.