Occupational Therapy in Epidermolysis bullosa (eBook)

1 Epidermolysis bullosa (EB) – the condition.- 1.1 Definition.- 1.2 Pathology of EB.- 1.2.1 EB simplex (EBS).- 1.2.2 Junctional EB (JEB).- 1.2.3 Dystrophic EB (DEB).- 1.3 The most common sub-types and their signs and symptoms.- 1.4 The most common cutaneous and extra-cutaneous complications.- 1.5 Diagnosis.- 1.6 Therapy.- 2 Motor development in early childhood.- 2.1 Motor development screening.- 2.1.1 Typical development of motor ability.- 2.1.2 Assessment of children with EB.- 2.1.3 Parental questionnaire focusing on the early motor development.- 2.2 Results from motor development screening.- 2.3 Occupational therapy.- 2.3.1 Advising parents.- 2.3.2 OT intervention focusing on the early motor development.- 3 Treatment of the tactile, vestibular and proprioceptive perception.- 3.1 Hypotheses concerning perception.- 3.1.1 The relevance of tactile, vestibular and proprioceptive perception to child development.- 3.2 Assessment of perception.- 3.2.1 Clinical observation of children with EB – considerations.- 3.2.2 Useful additions for children with EB.- 3.3 Assessment results.- 3.3.1 Discussion of the results.- 3.4 Occupational therapy.- 3.4.1 OT intervention focusing on the development of perception.- 4 Development of hand functions.- 4.1 Development of prehension (grasp).- 4.2 Possibilities for assessment of children with EB.- 4.3 Assessment results.- 4.4 Occupational therapy.- 5 Graphomotor skills.- 5.1 Development of pencil grasp and of drawing skills.- 5.2 Assessment of children with EB.- 5.2.1 'Graphomotorische Testbatterie'.- 5.2.2 Observation of drawing and writing.- 5.2.3 The 'Mann-Zeichen-Test'.- 5.3 Assessment results.- 5.4 Occupational therapy.- 5.4.1 OT intervention focusing on drawing and graphomotor skills.- 6 Independence in everyday life and provision of assistive devices.- 6.1 Assessment of children with EB.- 6.1.1 Parental questionnaire focusing on the coping skills of children with EB in everyday life.- 6.2 OT intervention focusing on everyday life and use of assistive devices.- 6.2.1 Self-care.- 6.2.2 Mobility / Locomotion.- 6.2.3 Leisure / Hobbies.- 6.2.4 Kindergarten (nursery) / School / Work.- 6.2.5 Barrier-free living.- 7 Client-directed therapy using the COSA.- 7.1 Client directed practice.- 7.2 What is COSA?.- 7.2.1 Contents and structure of COSA.- 7.3 Client-directed assessment and therapy for children with EB using the COSA.- 7.3.1 Additions to the ‘Intended Meaning Reference Guide’ for children with EB.- 7.3.2 Additional items for children with EB.- 7.3.3 Formulating goals.- 8 Rehabilitation of the hand.- 8.1 Pathophysiology of the hand.- 8.2 Contractures and deformities.- 8.2.1 Fingers.- 8.2.2 Thumb.- 8.2.3 Palm and arches of the hand.- 8.2.4 Wrist.- 8.2.5 Interdigital spaces.- 8.2.6 Causes of contractures and their relevance to daily life.- 8.2.7 Pathological changes to the joints due to contractures.- 8.3 Radiological changes.- 8.4 Surgical procedures.- 8.5 Occupational therapy process.- 8.6 Occupational therapy intervention.- 8.7 Occupational therapy assessment.- 8.7.1 Assessment form for the hand – using the AO neutral O-method.- 8.7.2 Assessment form for the hand – range of motion / sensibility / pain / grip forms / dexterity.- 8.7.3 Assessment form for the hand – longitudinal growth und pseudosyndactyly-  8.7.4 Assessment form for the hand – abduction of the digits.- 8.8 Occupational therapy.- 8.8.1 Aims of intervention.- 8.8.2 Bandaging and compression gloves.- 8.8.3 Splinting for contracture prophylaxis.- 8.8.4 Types of splint for contracture prophylaxis.- 8.8.5 Exercises to prevent deformities.- 8.9 Post-operative occupational therapy after digital separation.- 8.9.1 Aims of treatment.- 8.9.2 Post-operative splinting.- 8.9.3 Post-operative problems.- 8.9.4 Assessment form for the hand – sensibility.- 8.9.5 Training of sensibility.- 8.9.6 Treatment of scars.- 8.9.7 Treatment of oedema.- 8.9.8 Manual therapy.- 8.9.9 Functional exercises after operative digital separation.- 8.9.10 Bandaging to prevent MCP flexion and ulnar deviation.- 9 Rehabilitation of the foot.- 9.1 The foot in people with DEB.- 9.2 Comparison of hand and foot.- 9.3 Anatomy and functional principles of the foot.- 9.3.1 The cuneiform principle.- 9.3.2 The spiral principle.- 9.3.3 The transverse arch.- 9.3.4 How DEB affects these principles.- 9.4 The pelvis and the axes of the legs.- 9.4.1 The pelvis.- 9.4.2 The axes of the legs.- 9.5 The development of the foot and the axes of the legs during growth.- 9.5.1 The new-born baby.- 9.5.2 The infant.- 9.5.3 The toddler.- 9.5.4 The school child.- 9.5.5 Considerations relating to the foot positions and leg axes in people with EB.- 9.6 Malpositions of the foot.- 9.6.1 Pes (talipes) valgus/varus.- 9.6.2 Fallen arches, pes planus (flat-foot), pes planovalgus.- 9.6.3 Pes excavatus (high-arched foot).- 9.6.4 Pes transversoplanus (splay foot).- 9.6.5 Considerations relating to the foot in people with DEB.- 9.7 Assessment.- 9.8 Exercises.- 9.8.1 Exercises for the perception of the feet.- 9.8.2 General functional exercises.- 9.8.3 Mobilisation.- 9.8.4 Functional exercises for the malpositions of the foot.- 9.8.5 Bandaging.- 10 Conclusion.- Literature.