famcare and physical therapy for people with pws: physical activity

Physical Activity for People with PWS

Georgina Loughnan Royal Prince Alfred Hospital Prader-Willi Syndrome Clinic

Sydney, Australia

PWSA Ireland Conference Dublin 2014

http://pwsai.ie/

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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• greater % fat greater fat storage

gain fat readily

increased cardiac risk

caloric requirements

sleep apnoea



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• hypothalamic hunger hunger satiety food focus

• reduced maturation risk of osteoporosis

• desire for sameness dislike “new” interventions like consistency

compliance with regularity



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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 “Burns” Energy


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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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Heart Rate Zones

(Cardio)


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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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Physical Therapy

Occupational Therapy


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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


Physical Therapy

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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


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

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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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famcare and physical therapy for people with pws: physical activity

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