What is Prader-Willi Syndrome?

Prader-Willi Syndrome (PWS), a genetic disorder affecting chromosome 15, is the most common cause of genetic obesity and is synonymous with hypotonia (low muscle tone). Diagnosis is often delayed until early childhood as the clinical findings during infancy are often subtle or non specific.

There are certain typical features which distinguish a child with PWS. Their body composition is characterised by excess body fat, especially around the central portion of the body, and they are often short in stature. A child with PWS will also often have a narrow face, almond-shaped eyes, small-appearing mouth, a thin upper lip with down turned corners of the mouth, and full cheeks. The child's eyes may also cross.

PWS occurs in one of every 12,00 to 15,000 births in both boys and girls, and in people of all ethnic backgrounds.

The benefits of The Bambach for Prader-Willi Syndrome

• The hip joint is its position of maximal joint surface contact (closed pack position).
• Focus on pelvic control maintains an upright stable pelvis in its neutral position. In this position the spine, shoulder girdle, trunk, head and neck can function in their most stable position.
• Limbs are in a mid-range position which can minimise abnormal tone.
• A clear midline assists balance and symmetry.
• The individual sitting upright is in an active dynamic posture rather than a passive collapsed position.
• The Individual has greater control of sensory inputs which can trigger unwanted motor responses.
• Partial weight bearing can be encouraged through to standing from a half-sitting position.
• Dynamic sitting facilitates muscle activity which ensures the stimulation of normal and symmetrical growth of bone and soft tissue.
• Trunk and head control are easier in the natural position so that functional activities, such as swallowing and speech are improved.
• Thoracic, abdominal and pelvic spaces are maximized resulting in improved lung and internal organ function and circulation.
• With the upper body stable and free to move in this upright posture, eye contact, communication and social interaction are a great deal easier.
• Self care activities, such as meals, washing and dressing, writing, computer work, hobbies and playing games, are also more easily performed.

Bambach understands the problems associated with Prader-Willi Syndrome

• Early feeding problems/failure to thrive during infancy
• Central hypotonia (low muscle tone) and uneven growth and development
• Mild to moderate mental retardation from early childhood
• Obesity and food seeking behaviour from mid childhood leading to rapid weight gain
• Behavioural disorders and hypogonadism during late childhood and adolescence
• Sleep apnoea and cor pulmonale in adulthood
• Individuals may also develop Scoliosis (lateral curvature of the spine)

The Bambach design and Prader-Willi Syndrome

The Bambach saddle seat was designed by an Australian Occupational Therapist as a result of her observation following years spent working with 'Riding for the Disabled'.

She found that disabled individuals suffering from hypotnia (low muscle tone) in conditions such as PWS often had great difficulty in maintaining a healthy posture and assumed a 'collapsed' or 'slouched' posture when seated in a conventional wheelchair and required considerable support (or strapping) to remain sitting.

These same individuals when sat on horseback were however capable of sitting independently and unsupported and showed remarkably improved functional abilities as a result of being put on a horse on a saddle.