In the month of August 2019 a very interesting and important research study on the life strategies of people with Usher Syndrome type 2A was published in Sweden. There are no previous studies of this type.
On the one hand this shows greater attention to this disease, on the other it certifies its rarity. Not least shows how patients with this disease have been left alone, without knowing exactly how to cope with the consequences of a double sensory limitation.
These strategies, therefore, are totally individual, and the result of personal determination and strength.
The conclusions are nothing short of exciting.
It is shown that patients with Usher Syndrome type 2A want to take control of their lives, engage in a process of striving to live an active life in accordance with their own values, in other words, to be at the helm of their own lives.
Authors:
Mattias Ehn,Agneta Anderzén-Carlsson,Claes Möller &Moa Wahlqvist
ABSTRACT
Purpose: To explore life strategies in people with Usher syndrome type 2a.
Background: There are no studies on life strategies in people with Usher syndrome. People with deafblindness are often described in terms of poor health and low quality of life, or as being vulnerable. From a clinical point of view, it is of importance to balance this picture, with an increased knowledge of life strategies.
Methods: The study had a qualitative explorative design. Fourteen people aged 20–64 years (4 women, 10 men) with USH2a in Sweden participated in focus group interviews, which were transcribed and analysed by qualitative content analysis.
Results: The content analysis resulted in seven categories; remaining active, using devices, using support, sharing knowledge, appreciating the present, maintaining a positive image and alleviating emotional pain. Two sub-themes: resolve or prevent challenges and comforting oneself was abstracted forming a theme “being at the helm”.
Conclusion: The findings show that people with USH2a have a variety of life strategies that can be interpreted as highlighting different aspects of psychological flexibility in a life adjustment process. The study demonstrates that people with USH2a manage in many ways, and metaphorically, by “taking the helm”, they strive to actively navigate towards their own chosen values.
KEYWORDS: Deafblindness, focus groups, life strategies, psychological flexibility, qualitative content analysis, Usher syndrome type 2
Read or Listen the full study here
Our results show that people with USH2a have a variety of life strategies to handle deafblindness-related challenges with a high degree of psychological flexibility. This contradicts the common description of people with deafblindness as vulnerable. Our results show that by Being at the helm the participants are committed agents in a process of striving to live an active life in accordance with their own values.
The experiences shared by our participants could be used by professionals as an inspiration or starting point to draw attention to important life areas that might need to be addressed in counselling. Furthermore, the findings could be used to identify areas of psychological inflexibility that need attention, but just as important, they could also help people with USH2 to recognize their own personal resources and to emphasize psychological flexibility. The findings underline the importance of being offered early intervention as well as psychological, medical and technical support.
The findings could also form a base for designing research exploring life strategies in other clinical or genetic sub-groups of USH to attain a more comprehensive picture of the experiences of living with USH. Future studies concerning how to support people with USH2 to continue striving towards important values in life are also of significance.