Physical Activity for People with PWS
Georgina Loughnan Royal Prince Alfred Hospital Prader-Willi Syndrome Clinic
Sydney, Australia
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Prader-Willi Syndrome – the facts 1
People with PWS have:
• lower muscle tone and bulk
resistance to movement
mm strength loose muscles less stable joints
lower total daily energy expenditure
less fat burning potential
poor posture
lowered cardio-respiratory capacity
decreased capacity for physical work
motivation to exercise
caloric restriction mm loss
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People with PWS have:
• greater % fat greater fat storage
gain fat readily
increased cardiac risk
caloric requirements
sleep apnoea
Prader-Willi Syndrome – the facts 2
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People with PWS have:
• hypothalamic hunger hunger satiety food focus
• reduced maturation risk of osteoporosis
• desire for sameness dislike “new” interventions like consistency
compliance with regularity
Prader-Willi Syndrome – the facts 3
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• “Nature makes no provision for digesting more than her proper wants demand”
- John Harvey Kellogg (1852-1943)
• “In obesity, the genes will load the gun but the environment pulls the trigger”
- George A Bray (1931- )
The “Basics” of Obesity
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Relevance of Exercise for PWS
• Weight management
• Cardio-vascular fitness
• Respiratory fitness
• Muscle strength
• Body composition
• General skill training
• Mood
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EXERCISE Increases Metabolic Rate
-1 0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18
0.0
0.4
0.8
1.2
1.6
2.0
2.4
2.8
3.2
Time (min)
Oxygen Uptake
(L/min)Exercise Recovery
Oxygen consumed
at rest
Ref: Adapted from Lamb,D.R. Exercise Physiology. 1984.
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EXERCISE Maintains “Diet Effect”
0.0
0.5
1.0
1.5
Water
Protein
Fat
Weight loss
Diet only Diet & Exercise
(kg)
Ref: Adapted from Lamb,D.R. Exercise Physiology. 1984.
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EXERCISE Builds Muscle
• Uses stored energy
• Increases metabolic rate
• Maintains “diet effect”
• Strengthens muscle
• Improves insulin sensitivity
• Improves circulation
• Decreases stress & reduces boredom
• Protects bone
• Aids oedema
Known to occur in PWS (U Eiholzer 2003)
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Exercise
• Uses stored energy
• Increases metabolic rate
• Maintains “diet effect”
• Strengthens muscle
• Improves insulin sensitivity
• Improves circulation
• Increases HDL
• Decreases stress & reduces boredom
• Protects bone
• Slows ageing
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EXERCISE
Improves Circulation
Improves concentration high blood pressure
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Figure 18a. Obesity with distal lymphatic dysfunction in a 34-year-old woman.
Witte C L et al. Radiographics 2000;20:1697-1719
©2000 by Radiological Society of North America
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EXERCISE Reduces Oedema
• Chronic venous insufficiency
• Calf muscle exercise
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EXERCISE Improves Bone Density
Bone tissue responds to dynamic loading
Small increase in bone mineral density can result in large increase in bone strength
Osteogenic potential is greater with separated sessions
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EXERCISE Increases Wellbeing
• Release of endorphins can be increased by up to 5 times resting levels
– improves mood & level of relaxation
– reduces anxiety
– reduces tension
– reduces anger
– reduces confusion
– reduces boredom
– alleviates food focus
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Effect of Exercise on Mood
Annesi, J.J. (2003). Sex differences in relations of cardio-respiratory and mood changes associated with self-selected amounts of cardiovascular exercise. Psychological Reports, 93, 1339-1346.
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Types of Exercise
• Aerobic-type - large muscle groups / whole body
- fat burning
• Toning - specific muscle groups
- strengthening
- resisted
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Where to Start
• Childhood:
– muscle tone
– muscle strength joint stability
– gross motor skills / coordination
– achievement of motor milestones
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Swimming
8th International PWS Conference Cambridge 2013
•Fun •Low impact •Whole body •Family •Competitive
Facilitated Play
Family and / or carer involvement
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Physical Therapy
8th International PWS Conference Cambridge 2013
8th International PWS Conference Cambridge 2013
Part of Life
• Adolescence / adulthood
– weight management
– bone strength
– co-morbidities
– fitness
– mood
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Initial Exercise
• 10 -15 mins
• 1-2 per day (x 6 days per week)
• Add to “work out” time
• Extra incidental activity
• – park the car further away from destination
• stairs rather than lift
• the long way around
• to the next bus stop
• household chores
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Exercise Prescription
• 30-60 minutes supervised • 5-6 days per week • Before meals • Effective exercise - walking /treadmill
cycling swimming
dancing
• In home equipment
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Intensity of Exercise
• PER
Hot 1 2 3 4 5 Puffed 1 2 3 4 5 Sweaty 1 2 3 4 5 Exhausted 1 2 3 4 5
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Warm-up and Cool down
• Slow rhythmic movement / stretches
• 3-5 mins slow start/finish of exercise
• To prevent soft tissue injury
• To prevent sudden change in BP
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Strengthening Exercise
• Specific muscle groups
• Weighted arm exercises
• Trunk exercises
• Incidental activities - tidying room
cleaning bathroom
hand clothes washing
hanging out washing
*Bulks and firms muscles
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Posture
Remind person to:
• Lift head up when talking/walking
• Hold a straight back / walk tall
• Sit up straight in the chair
• Elbows at table height
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Benefits of Fitness in PWS
• Decreased fat and improved fitness
• Ease of movement
• Improved mood & self esteem
• Enhances opportunity for employment/day programme attendance
• Reduced co-morbidities
• Healthy longevity
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Mean Weight Change
over 12 months
N Age (yrs)
X + SD
Weight (kg)
X + SD
Regular exercise
12
8M:4F
31.64
8.59
-9.42
6.09
No regular exercise
12
8M:4F
31.42
8.83
*2.00
4.35
* p = .0001 PWS Clinic RPAH Sydney – retrospective data
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70
80
90
100
110
120
130
140
150
160
Apr
-98
No
v-9
8
Jun-
99
Jan-
00
Au
g-0
0
Mar
-01
Oct
-01
May
-02
Dec
-02
Jul-
03
Feb
-04
Sep
-04
Apr
-05
No
v-0
5
Jun-
06
Jan-
07
Au
g-0
7
Mar
-08
Oct
-08
May
-09
Dec
-09
Jul-
10
Feb
-11
Sep
-11
Ap
r-1
2
Nov
-12
Jun-
13
Jan-
14
Waist 142BMI 65
Waist 132BMI 61.7
Waist 106.5BMI 35.2
Waist 98BMI 31.8
Case Examples SB(-66kg) & DB(-77.1kg)
kg
Waist cm BMI kg/m2
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Case Example - AS
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60
65
70
75
80
85
90
95
100
105
110
Waist 80
BMI 21.1
Waist
116.2
BMI 35.6
Kg
Waist cm BMI kg/m2
Weight Maintenance Kade 20 yrs
Day Activity Duration
Monday Maui Thai Special Olympics – swimming or Dog walking
60 mins 60 mins 60 mins
Tuesday Go to the gym - Xtrain 60 mins
Wednesday Walk on treadmill at home 40 mins
Thursday Go to the gym or treadmill 40 mins 45 mins
Friday Dog walking, washing and feeding 90 mins
Saturday Walk on treadmill at home 40 mins
Sunday Go to the gym – Tough Class 45 mins
February 2012: weight 83.7 kg June 2014: weight 76.1 kg BMI 27.3kg/m2 BMI 24.8 kg/m2
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Exercise is the vital link to good health and fitness
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