Fabry disease in focus

Management of Fabry disease

The information on this website is intended only to provide knowledge of Fabry disease health topics. This information should not be used in place of advice from your GP or other healthcare professional.

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Lifestages

Because the progression of Fabry disease varies from person to person it also means that symptoms appear at different ages and with differing severity (see also onset of symptoms). It is therefore important to consider the different symptoms and to understand the impact of them at different stages of life. To find out about people’s experiences with Fabry disease, go to the personal stories section.

Infants

Of the many symptoms of Fabry disease, it is often pain and heat-related discomfort that appear first in young children with Fabry disease.1 Parents should therefore be careful not to expose young children with Fabry disease to extremes of temperature.

Children and adolescents

As well as episodes of pain and burning sensations in the hands and feet, children and young people with Fabry disease often develop a spotted, dark red skin rash (angiokeratomas) seen most densely between the belly button and the knees; changes in the appearance of the cornea may also develop and be found by an optician during a routine eye examination when using a special piece of equipment. Parents and teachers should consider the effects of physical exertion, exercise and extremes of temperature on children with Fabry disease. There can also be social-related issues to do with school or employment and it may be useful to consult with specialists about these (see who can help and self-management).

Adults

Fabry disease is slowly progressive and symptoms resulting from damage to the kidneys, heart and central nervous system usually appear between the ages of 30 to 45.2 In some individuals with Fabry disease it is only when tests are carried out to find the cause of problems with the heart or kidneys that the accumulation of Gb3 is identified and a diagnosis of Fabry disease made. Symptom management can help reduce the effect of symptoms, and lifestyle considerations such as type of employment, choice of leisure activities and diet can all play their part.

References:
  • Ramaswami U, et al. Acta Paediatrica 2006; 95: 86-92.
  • Beck M. In: Mehta A, et al (eds). Fabry disease: perspectives from five years of FOS, 2006.
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Page last updated: 10-Feb-2010