Background: Progressive ataxias are complex disorders that result in a wide variety of symptoms. Whilst we currently have a relatively good understanding of the symptom patterns associated with the various types of ataxia, and how these diseases progress over time, their impact on the person with ataxia is less well understood. In addition, little is known about how carers, friends and families are affected by them. This paper aims to provide preliminary information on the presence and impact of medical symptoms and day-to-day challenges on people with ataxia and their friends and relatives.
Method: Data were extracted from a survey by Ataxia UK for their members. The views of 366 people with ataxia and 52 friends and relatives are reported. Data were analysed for the entire groups, as well as for the three most common ataxia types represented in the sample, Friedreich's ataxia, inherited ataxia (excluding Friedreich's ataxia), and cerebellar ataxia of unknown cause.
Results: The survey confirmed the symptom patterns described in previous research, but further showed that the impact of these symptoms can vary across ataxia populations. Similar findings were observed for day-to-day challenges. Friends and relatives experienced similar challenges to people with ataxia, indicating that support provided has to consider those supporting people with ataxia as well as the patient. Respondents also highlighted limitations in terms of accessing support services, and not all services were able to cater fully to their specific needs.
Conclusion: This study begins to provide information that can be used in further research to explore the needs of people with ataxia and their carers, friends, and relatives. Such research will support treatment trial design, ensuring patients' needs are considered, help to tailor support services to their needs, and ensure health care professionals have the necessary skills to fully address them.
Keywords: Activities of daily living; Ataxia; Carers; Quality of life; Symptom burden; Symptoms.
Ataxias are movement disorders that result in poor coordination and can include a variety of other difficulties, including speech difficulties, heart conditions, and problems with eyesight. Every type of ataxia results in a different pattern of symptoms, and they also differ in the age at which symptoms first appear. In progressive ataxias, symptoms become more severe over time. The medical symptoms often impact daily living, such as moving about, eating and drinking, socialising, or the ability to work. They also affect those who live with or look after the person with ataxia. It is therefore important to collect people’s views about how the symptoms of their condition affect their daily lives, and what kind of support helps to alleviate these. This paper reports the results of a survey of people with ataxia and their friends and relatives to explore these questions. It was highlighted that people with ataxia deal with a wide variety of medical symptoms and that these have important effects on their lives. It also showed that those caring for them are similarly affected and also need support. The study has highlighted several areas where more research needs to be conducted to provide effective support for people with ataxia and their friends and relatives, and to inform the design of treatment trials to ensure the needs of patients are being addressed.
Copyright: © 2021 Lowit A et al.