Batten disease is a condition characterized by progressive loss of vision, a decline in motor skills, cognitive impairment, and difficulty speaking. The loss of these important skills requires adaptations in everyday life, at home and school. Specialized equipment also can help overcome challenges patients may face in their daily routines.
Adaptations for the loss of motor skills
Physiotherapy can help overcome the problems that Batten disease patients experience as a result of the loss of motor skills. Physiotherapists also can recommend useful equipment. Splints and braces can help support posture. Standing and walking devices can improve patients’ ability to move around. Orthoses and bandages can be used to provide additional stability. Eventually, many patients will require a wheelchair to stay mobile.
Home adaptations such as ramps and hoists facilitate free movement at home and may help prevent accidents.
Velcro and zippers, instead of shoelaces and buttons, can help patients get dressed.
Nutrition and feeding management
Cutlery with bigger handles makes eating more comfortable. With disease progression, swallowing difficulties occur and the risk of aspiration increases. Fortified foods and supplements can help meet nutritional needs. Eventually, tube feeding might be required.
Adaptations for language impairment
Speech impairment occurs in most Batten disease patients. Alternative communication methods such as gestures and symbols can help improve communication, and so can speech therapy. These strategies should be implemented as early as possible because it becomes more difficult to acquire these communication skills as cognitive function declines.
Management of sleep disturbances
In patients with sleep disturbances, behavioral and environmental strategies can help improve the quality of sleep when they are followed routinely. These may include shading the bedroom, avoiding heavy meals before bedtime, listening to calming music, and using weighted blankets.
Adaptations for vision loss
The parents of a child with Batten disease should pay close attention to their child’s progressive vision loss. Developing alternative ways of doing things that rely less on vision and more on hearing can help the child adjust to gradual loss of vision.
In addition to playing a role in communication, stimulating the sense of hearing can be of therapeutic value. As many other skills are lost, music can be a source of comfort, and can help decrease anxiety, pain, and boredom.
Seizures occur frequently in all types of Batten disease and cannot be foreseen. Family and caregivers should be provided with emergency seizure management plans.
The age of onset of Batten disease mainly determines whether the child can continue to attend a mainstream school or has to switch to a special school.
Because of visual impairment, problems articulating themselves, and learning, children with Batten disease usually require an individual education plan. This plan should be developed with the child’s teacher and should define learning targets. The plan also should contain teaching strategies and methods. With progressing visual impairment, children rely more and more on their sense of hearing, so teaching resources and methods should be adapted accordingly.
Batten Disease News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.