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UCL EyeTherapy Blog


A blog by the Gene and Cell Therapy Group at the UCL Institute of Ophthalmology Department of Genetics


After 50 years, Inherited Retinal Disorders are Now the Leading Cause of Blindness in People of Working Age!

By Andi M Skilton, on 8 March 2014

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Introduction to the paper

Data published in BMJ Open, from Dr Michel Michaelides, UCL Institute of Ophthalmology, and colleagues show that ‘for the first time in at least five decades, diabetic retinopathy/maculopathy (DRM) is no longer the leading cause of certified blindness among working age adults in England and Wales, having been overtaken by inherited retinal disorders (IRDs)’.

The authors, from the National Institute of Health Research Biomedical Research Centre at Moorfields Eye Hospital NHS Trust and UCL Institute of Ophthalmology, analysed the national database of blindness certificates of vision impairment (CVIs) in England and the Welsh equivalent (CVI-Ws), to determine the number and causes of blindness in people of working age (16 to 64 years, inclusive).

Findings and speculations from the paper

The report, A comparison of the causes of blindness certification in England and Wales in working age adults (16-64 year), 1999-2000 with 2009-2010’, reveals that CVIs for IRDs have risen by 4.4% (from 15.8% to 20.2%) moving from second to first position. During the same period CVIs for DRM have decreased, dropping 3.3% (from 17.7% to 14.4%), whilst optic atrophy remains the third leading cause.

“What is clear from the findings is that inherited retinal disease should no longer be thought of as rare and not relevant,” says Dr Michaelides. “These conditions have long been, and will continue to be, an important avenue of our research. But in the future the provision of care and resources in the NHS and the allocation of research funding must be addressed if we are to tackle these conditions which now represent the commonest cause of certification in the working age population.”

The authors speculate that it is perhaps the allocation of resources and funding seen in recent years for diabetes that has led to a subsequent decrease in CVIs for DRM. This decline is not an indication that incidence of diabetes (and subsequently incidence of DRM) are decreasing as latest data shows an increase. Likewise, incidences of IRD are not necessarily rising. Instead these findings could be attributed to the increased focus of the Government and Health Services in recent years on the treatment and management of diabetes and the effectiveness of DRM screening programmes and strategies to improve glycaemic control, among others.

Implications of the findings from the paper

This analysis takes into consideration only those cases which have been certified. Certification in England and Wales is by no means compulsory and many cases of vision loss go undiagnosed, misdiagnosed or unreported. However, these findings formalise the diagnostic trends that RP Fighting Blindness, a national charity funding pioneering research and support services for people with Retinitis Pigmentosa and other related conditions, have been noticing for some years.

Whilst in terms of absolute numbers other conditions that cause severe vision loss such as age-related macular degeneration affect more people, the national impact of IRDs on the productivity of this otherwise fit and able group of working age people, as well as additional health and social costs, is huge. This is why a new focus on IRDs supported by the learnings from other diseases such as diabetes will be so important for the future.

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