Living with hereditary haemorrhagic telangiectasia: Coping and psychological distress – A cross-sectional study

Abstract

Purpose: The purpose of this study was to examine the relationship between coping strategies measured by Coping Orientation to Problems Experienced Scale (COPE) and psychological distress measured by Hospital Anxiety and Depression Scale (HADS) and Becks Hopelessness Scale (BHS) in individuals living with Hereditary hemorrhagic telangiectasia (HHT) and to examine if coping strategies might have a mediating role between experienced illness and psychological distress. HHT is mainly caused by mutations in the ENG- or ALK1-genes and associated with a shorter life span. 90% of patients have recurrent nosebleeds. Method: 66 individuals affected of HHT participated in this cross-sectional study, completing questions due to demographic variables, Experience of illness, COPE, BHS and HADS. X² test, bivariate correlations with Pearson r and hierarchical multiple regression were used using PASW 18. Results: Experience of illness made the highest variance in anxiety, depression and hopelessness and the coping strategy “behavioral disengagement” seems to have a mediating role between nose bleedings, being afraid of complications, satisfied with life and psychological distress. Conclusions: Experience of illness is of big importance in psychological distress in individuals affected of HHT, and behavioral disengagement explained the actual relationship between experience of illness and psychological distress.

Implications for Rehabilitation

• Hereditary haemorrhagic telangiectasia (HHT) is an inherited dominant disease, with recurrent and severe epistaxis as the most common presentation.

• Other characteristics are telangiectasias and visceral lesions. Without relevant treatment the disease is associated with a shorter life span.

• Experience of illness is of big importance in psychological distress in individuals with HHT.

• The coping strategy behavioral disengagement seems to explain the actual relationship between experience of illness and psychological distress

Keywords::

• Coping
• experience of illness
• hereditary haemorrhagic telangiectasia
• psychological distress

Declaration of Interest: The authors declare no conflicts of interest.