This week the European Respiratory Society (ERS) published its Statement on the multidisciplinary respiratory management of ataxia-telangiectasia. The document reviews all the published data on the care of the lungs in A-T and makes a series of recommendations for approaches to treatment.
The statement was produced by a multi-disciplinary ERS task force, made up of international A-T specialists, as well as a parent in the form of our trustee and former chair, Lian Yarlett. It was set up at the initiative of Dr Jayesh Bhatt of the A-T specialist centre in Nottingham, with the support of the A-T Society. Dr Bhatt chaired the task force together with Dr Peter Merkus of the Dutch A-T centre in Nijmegen.
The ERS is an important body that helps professionals working with lung disease network and learn from each other. It requires its task forces to have an organised and systematic approach and members from a range of disciplines and different countries. The report of such a group therefore carries a lot of weight.
This statement sets out our current state of knowledge of lung problems in A-T and how to treat them. It is based on research and it has been put together by clinicians who have the most experience of looking after people with A-T. It also highlights the gaps in our knowledge and needs for research.”
It is less than 10 years since lung-care became a regular part of the Nottingham and Papworth clinics, but it has rapidly become one of the most important areas of treatment. As the statement sets out, there are many different factors that play a role in causing lung problems in A-T, and unlike the neurological symptoms, lung disease can develop and extend without the person realising and eventually lead to extremely serious problems.
However, on the positive side, experience with other conditions, such as cystic fibrosis, suggest that with systematic and proactive care, we can do a lot to prevent and treat the lungs and if we do this we can keep people healthy much longer.
This is certainly Dr Bhatt’s belief: “We are extrapolating a lot from other conditions but what we are doing is common sense, basic things. As treatments and outcomes have improved in other conditions the hope is that it will be similar in A-T.”
William Davis, the Society’s chief executive welcomed the publication of the report. “This is a very important publication for people with A-T across Europe and the world. The A-T Society believes very strongly that regular lung checks, backed up with proactive and aggressive treatment based on this document and our own clinical guidance, will significantly improve quality and length of life for people with A-T. People with A-T need to make sure that the doctors treating them have read these documents and base their treatment on their recommendations.”
The ERS statement is aimed at doctors and therapists and those without a medical background may not find it easy reading. However, the advice on treatment is very much in line with the Clinical Guidance document published by the A-T Society at the end of last year. So if you are a parent or carer of a child with A-T and you want to know about the treatment your child should be getting, please refer to this.