What is rigid spine syndrome?
Rigid spine syndrome is a rare genetic disorder in which the back muscles and the muscles of the trunk are severely cramped or stiff. Affected children often have difficulty standing up, sitting or walking, which significantly restricts their mobility. This can be accompanied by breathing problems, scoliosis and general muscle weakness. The disease usually progresses slowly, so that the restrictions can worsen over time.
Aids that are frequently used for rigid spine syndrome:
Aids and support for everyday life
The provision of aids for rigid spine syndrome has two main objectives: Maintaining an upright position while relieving pressure on the airways.
- Positioning & trunk support: As sitting is often painful or unstable due to stiffness and scoliosis, individually adapted seat shells or therapy chairs are necessary. These must support the spine without causing pressure points.
- Respiratory support: Due to diaphragmatic weakness, many patients require a ventilator (non-invasive ventilation, NIV) for the night. Cough assist devices (CoughAssist) can also help to keep the lung passages clear.
- Mobility aids: Depending on the stage of the disease, active wheelchairs or power wheelchairs support mobility when walking becomes too strenuous.
- Orthopaedic corsets: Custom-made corsets are often used to slow down scoliosis. However, these must be precisely tailored to the breathing situation so as not to constrict the chest too much.
- Positioning aids: Special positioning cushions help at night to find a position that relieves pressure on the spine and at the same time allows optimal ventilation of the lungs.
Learn more
You can find more helpful information about rigid spine syndrome here:
www.orpha.net