Barzilai University Medical Center

10 Research Day 2020 Barzilai University Medical Center 2 DESIGNING A NEW INSTRUMENT FOR THE EVALUATION OF PREMONITORY SIGNS AND SYMPTOMS (PRODROMES) OF HEREDITARY ANGIOEDEMA Iris Leibovich-Nassi 1,2 , Hava Golander 1 , Raz Somech 3 , Dov Har-Even 4 , Avner Reshef 2 1 Department of Nursing, Sackler school of Medicine, Tel Aviv University, 2 Barzilai University Medical Center, Ashkelon, 3 Safra Pediatric Medical Center, Sheba Medical Center, Ramat Gan, 4 Bar- Ilan University, Ramat Gan, Israel. Background Attacks of Hereditary Angioedema (HAE) are unpredictable and bear serious consequences. Many HAE patients report early premonitory signs and symptoms (Prodromes) which antedate the attacks. Hypothesis Systematic instruments for the evaluation of prodromes may contribute to early recognition of oncoming attacks, and possibly to better planning of therapeutic interventions. Objectives We sought to build a robust tool enabling to define attacks and prodromes of HAE. The study's main purpose was to investigate if patients could distinguish between the locations and dimensions of prodromes and subsequent attacks, and to analyze their associations. Results Seven questionnaires addressing HAE signs, symptoms, health- related quality-of-life were devised. Questions covered 6 predefined 'clusters' of body systems, known to be affected (i.e. limbs, face, larynx, abdomen, genitals). From 233 Israeli HAE patients 197 (84.5%) responded to the preliminary questionnaire, inquiring if they ever had a prodrome, and if they can predict an oncoming attack by having a prodrome. 165/197 (84%) reported ever having a prodrome, and 143/165 (87%) could predict an oncoming attack by experiencing a prodrome. Mean age was 36.7±20.3 years (range: 2-80, females 57.4%). Mean age of onset of HAE attacks was 10.74± 10.3Y and age of diagnosis was 15.54±15.1Y. Main triggers of attacks were physical trauma (69%) and stress (46%). Sixty-six (33.5%) completed another questionnaire, developed specifically to evaluate their experience with prodromes and attacks. The internal validity and reliability of this questionnaire was found to be very high (Cronbach's alfa .88-.99). There was a significant correlation between prodromes and attacks (p<0.01 ,r= .79). One-way ANOVA analysis shows that clinical dimensions of prodromes could be segregated from attacks [F (4, 56) = 45.7, P< .001, Eta 2 =.77]. Conclusions We designed a new prodrome evaluation instrument, which can assist in defining and comparing the clinical expressions of prodromes and attacks of HAE.