Rehabilitation and psychosocial aspects in DMD care

Transcription

1 Rehabilitation and psychosocial aspects in DMD care Budapest, Birgit F. Steffensen, Physiotherapist, PhD The National Danish Rehabilitation Centre for Neuromuscular Diseases

2 Denmark Demographics: Population 5.4 mill Childbirths pr year: New cases of DMD pr year: 6 Prevalence:160 2

3 The national rehabilitation centre for NMD All boys with DMD in Denmark are referred to the rehabilitation centre when diagnosed. They are followed in a life long course and assessed regularly DMD registered: 160 (3-42 yrs) Half of them is >18 years Steroid treatment since 2000 Assisted ventilation since

4 The national rehabilitation centre for NMD The staff is a multidisciplinary team of phys., occup., psychologists, social worker and physicians Assess the boys physical condition once or twice a year until the age of 18 and after that with longer intervals Suggest and supervise physical treatment plans to the patients local healthcare teams Deal with social, educational and psychological problems Organize family-courses for patients with the same diagnosis and kind of problems A standard of care programme for DMD in Scandinavia was developed in 2000 in cooperation with centres in Norway and Sweden ( 4

5 Rehabilitation as defined by the UN UN declaration, 1994 Standard Rules on the Equalization of Opportunities for Persons with Disabilities Rehabilitation The term "rehabilitation" refers to a process aimed at enabling persons with disabilities to reach and maintain their optimal physical, sensory, intellectual, psychiatric and/or social functional levels, thus providing them with the tools to change their lives towards a higher level of independence. 5

6 WHO: International Classification of Functioning, Disability and Health (ICF) Health Condition Disorder or disease Body Functions and Structures Impairment Activities Participation Environmental Factors Personal Factors 6

7 ICF Impairment Participation Activity 7

8 Rehabilitation as an ongoing process in DMD (physical aspects) 20 yrs 16 yrs 14 yrs 12 yrs 10 yrs 7 yrs 4 yrs Respiratory insufficiency Difficulty with using arms for eating Insufficient coughing Scoliosis Wheelchair Difficulty in walking Diagnosis 8

9 Rehabilitation as an ongoing process in DMD (psychosocial aspects) 20 yrs Starting an independent living 16 yrs Preparing further education 14 yrs Isolation as teen-ager? 12 yrs Learning problems? 10 yrs 7 yrs 4 yrs W/chair-practical problems in school School Diagnosis 9

10 Rehabilitation Covers a number of problems Physical Intellectual Emotional and social Environmental 10

11 Rehabilitation Covers a number of actors including the person with NMD and his relatives Physical physiotherapist, occupational therapist, orthopaedic surgeon, orthopaedic engineer, neurologist, paediatrician, GP, speech therapist, nutritionist Intellectual parents, preschool teachers, school teachers, psychologists Emotional and social parents, peers, sisters and brothers, class mates, school teachers, psychologists Environmental occupational therapists, social workers, legislation, politicians, 11

12 Aim of physical intervention In all stages of DMD: Maximise functional ability/ physical activity Maintain or improve muscle strength Minimise the development of contractures and scoliosis 12

13 Physiotherapy as a means to prevent loss of physical activity Physical problems: Loss of muscle strength Immobilisation Disuse atrophy Contractures Scoliosis Intervention to postpone and compensate for: Loss of muscle strength Fatigue, Difficulty in moving, breathing, coughing Contractures Difficulty standing, sitting, lying Scoliosis Deformities, pain 13

14 Exercises to prevent loss of muscle strength Submaximum, aerobic exercise/activity - Overexertion and overwork weakness should be avoided High-resistance strength training and eccentric exercise are inappropriate 14

15 Exercises to prevent loss of muscle strength 15

16 Exercises to prevent loss of respiratory muscle strength Studies show some improvement of muscle strength No functional improvement like: improved cough delay of the need of assisted ventilation decreased lung infections 16

17 Improve removal of secretions Use of PEP Use of CPAP 17

18 Improve coughing by frog breathing or air stacking 18

19 Air bagging via mask

20 Improving coughing by manual pressure 20

21 It helps to be two

22 Physiotherapy as a means to prevent contractures Studies on preventing contractures are sparse Stretching? Daily stretching combined with night splints is more effective than daily stretching alone in DMD 22

23 Contractures where?

24 Streching and bracing as means to prevent contractures 24

25 Standing as a means to prevent contractures/scoliosis 25

26 Scoliosis collapse of the spine insufficient breathing in sitting position difficulty in balancing in sitting 26

27 Spinal braces can load respiratory muscles and reduce lung volumes 27

28 Seating has to be considered 28

29 General principles Symmetry in the frontal, sagittal and horizontal plane Preserve lordosis Avoid pelvic obliquity and pelvic rotation Preserve the ability to move trunk Frequent change of position

30 Prevalence trends in relation to the introduction of assisted ventilation in the DMD population, absolute numbers Denmark Prevalence per years years Five processes forming a Creation of two Ventilator becomes DMD-ventilator policy respiratory centres a standard measure From: Jeppesen J et al. Neuromuscular Disorders 2003;10:

31 Men with DMD Interview of 68 young men with DMD age (18-40 yrs) How did they experience their lives as children, teenagers and as adults What is most important to get a good life 31

32 Body function Severe limitations in ability to move hands and fingers and to sit unsupported Still 98 % can operate his electric wheelchair And 87 % can write and play on computer 32

33 He can use 2 fingers... 33

34 Still contact to the whole world 34

35 Participation Live with parents 77 %, years Live alone 73 %, 24+ years 86 % of 24+ years live in a single-family house or a private apartment 1 lives with a spouse 1 lives in a nursing home/institution 35

36 Things that are ok My home suits me I have enough money I have mainly help/care enough 36

37 Things that are not ok Education Lonelyness No girlfriend 37

38 He has an independent living But no education, job or girlfriend 38

39 The most important things Min friend, the ventilator Freedom on four wheels My family Stable carers/helpers 39

40 The ventilator is my friend 40

41 Freedom on four wheels 41

42 Quality of life and worries about future Excellent quality of life 83 % Worried about the DMD-disease 23 % Worried about the future 19 % 42

43 This is my life Happy childhood Difficult youth Reasonable adult life 43

44 Rehabilitation in DMD is preparing for an adult life by supporting intellectual and social skills from early childhood the parents/paediatric teams have responsibility for the youth and old age Interventions: Family courses for parents and DMD child from early childhood Help to create broader and lasting social relations with other than family and personal assistants by means of courses Strengthen education and vocational training? Strengthen networking via internet (facebook) 44

45 References and Resources Bushby K. et al. The diagnosis and management of Duchenne Muscular dystrophy. Lancet Neurology 2010;9:77-93, Treat-NMD website: Care-NMD website: (Illustration of stretching exercises) (a home exercise book for parents) 45