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Dylan at Xmas 2005

Information - Ullrich Congenital Muscular Dystrophy

 

This is a progressive, life limiting form of Muscular Dystrophy which is caused by a lack of the protein Collagen VI in the muscles.

There are classic features of Ullrich which effect all children with the illness. Every child has a individual level of mobility, and because of this the effects of the condition will become evident at different stages of development. Some children may not learn to walk at all, whereas others like Dylan may learn to walk, but all will lose this mobility eventually as the body becomes too heavy for the muscles to cope.

Dylan, sometimes struggles to walk independently, and can only manage climbing stairs/steps with adult support.  He also needs help to do most things, such as stand up, sit down, go up and down the stairs, get on and off chairs and toilets, get dressed/undressed, in fact most daily activities. Which is very frustrating for him. Dylan also has very poor muscle tone, especially in his hands and arms. This again causes frustration for Dylan, not being able to do simple tasks that we all take for granted, such as putting socks on or pulling a jumper over your head.

Even in children who do manage to walk, muscles are weak and mobility is limited. Muscles will begin to tighten, known as contractures, and limb and joint movement will gradually become more difficult. Dylan is getting "tighter" in his ankles, knee and ham strings, which has had an effect on his mobility and strength.  Daily physiotherapy exercises and orthotics can help to stretch tendons and attempt to slow down this process.

Most children will suffer form curvature of the spine known as scoliosis, for which surgery may eventually be needed to straighten the spine and relieve the pressure on the lungs.

The muscles used for breathing become affected. This causes breathing difficulties, which leave children feeling lethargic, loss of appetite and consequently weight loss. Chest infections become more frequent and a ventilator will be needed to aid breathing.

Weight loss needs to be closely monitored and food supplements may be introduced orally to ensure the correct intake of the correct nutrients.  Later, feeding may be administered direct to the stomach through a tube, this is enabled by a small surgical procedure called a gastrostamy.
 
As there is no cure for this form of Muscular Dystrophy and it is a potentially life limiting disease, we have to ensure that Dylan gets all that he needs to ensure a prolonged and happy life.
 
For further information or advice on Ullrich Congenital Muscular Dystrophy or any other form of Muscular Dystrophy please see the 'LINKS' page

 

Copyright Warren Kelsall 2006. All rights reserved.

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