gilberto - pw zalau 4 definitivo

8/8/2019 Gilberto - PW Zalau 4 Definitivo

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DISEASE-SPECIFIC REHABILITATION

CYCLES IN PRADER-WILLI SYNDROME

We report our strategy and our experience

in P.W. management

2nd Eastern European Conference on PWS

Gilberto Andrighetto, M.D.

Mauro Baschirotto Institute for Rare Diseases

B.I.R.D. Foundation (Vicenza Italy)

Zalau October 2010

Baschirotto Institute for Rare Diseases BIRD Foundation Europe Onlus


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Length of stay: 19-26 days

Average attendance: 6 patients/cycle

64 patients:

33 boys (5 - 37 years; average age: 23 years)31 girls (5 43 years; average age: 23 years)

50% has attended more than 2 cycles

PWS rehabilitation activity(Sept 2002 - May 2010)


Average of 4 rehab. cycles/year


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A TYPICAL DAY-SCHEDULE07.30 - 08.00 Wake-up, toilette and weight control

08.00 - 09.00 Breakfast

09.00 - 10.30 Rehabilitative activity and/or medical examination

10.30 - 10.45 Snack

10.45 - 12.30 Nature trail, recreational activity

12.30 - 13.30 Lunch

13.30 - 14.30 Relax, recreational activity

14.30 - 16.30 Rehabilitative activity and/or medical examination

16.30 - 16.45 Snack

16.45 - 19.30 Walk, recreational activity

19.30 - 20.30 DinnerDinner

20.30 - 21.30 Relax, recreational activity

21.30 - 22.00 Prepare for bedPrepare for bed



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1.500 kcal/ die ;

carbohydrates 60 %, fats 20% or less , protein 20%

5 meals:

breakfast: 15-20% of total Kcal

2 snacks: fruit/low-fat yogurt ,occasionally a slice of cake

lunch and dinner:

2-3 portions of different types of raw or cooked

vegetables, with balsamic vinegar used as dressing,

without oil

only 1 main course : grains ( pasta, rice ), meat, fish oreggs

Meal time: 1 hour


Mediterranean Diet


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Food dosage weight (g) calories(Kcal)

Milk glass 125 75

wine glass 125 95

Rice spoon 9 29

Oil- butter spoon 9 80

Balsamic vinegar spoon 9 2,5

Parmisan spoon 9 35

Sugar small spoon 5 20

Honey spoon 9 27



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PWS rehabilitation

Aims towards patients:

Continuous research about the rehabilitative, genetic and

therapeutic aspects of treatment for Prader Willi Syndrome

Behavioral self-education

Weight loss and fitness program

Self-awareness and

psychological fitness


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Family relief

General patients health check-up

Customized guidelines to patients care


PWS rehabilitation

Aims towards

the family:


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WeightWeight lossloss..

Improvement of muscle tone, trophism, balance, and

coordination.

Prevention and stabilization of orthopedic problems.

Improvement of language, pronunciation, phonetics.

Enhancement of respiratory skills.

Correct and positive body perception.

Physiotherapy

AIMS


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General physiotherapy exercises

Postural reeducation exercises


Physiotherapy

ACTIVITIES

Respiratory educational exercises

Fitness tools exercises


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Improvement of body image and sense

Improvement of self-esteem, (having sense of ability and limits)

Improvement of spatial and temporal organization, attention to

time (especially to common tasks), memory and logic challenging

tasks

To foresee actions, reactions and effects of challenging tasks

and to stimulate the ability to reflect upon actions

To stimulate verbal comprehension and production

Adaptation to rules, turns and rhythms

Psychomotricity

AIMS


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Perceptive and symbolic

Musical and rhythmic

Pictorial, plastic and graphic

Team Games focused

on motor activities

Psychomotricity

ACTIVITIES



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Self-management

Autonomy improvement.

Emotions management (enforcing strategies of

excesses prevention and flexibility).

Behavioral Rehabilitation

AIMS


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Music therapy

Occupational therapy

Pet-therapy

Relaxation

Educational activities

Behavioral Rehabilitation

ACTIVITIES


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Between 2002 2010

We analyzed 64 p.w. Patients

70 % adults 30% adolescents

10 % with growth hormone

15 % with sexual hormones


The Study


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4-week Rehabilitation

Cycles

4 times a year


Cycles Timing


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0

1

2

3

4

5

6

7

Days 5 10 15 20

Mean weight loss is related to the number of days

of therapy for the PWS cycles 2002-2010



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0

1

2

3

4

5

6

7

1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26

Duration training (days)

Weight

loss(Kg)

Figure 1


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0

0.5

1

1.5

2

2.5

3

3.5

4

4.5

5

5.5

6

6.5

7

kgl

o

4-5 cicli 2-3 cicli 1 ciclo

numb rofcycles attended in a year

weight loss/ cycles peryear

BaschirottoInstituteforRareDiseases BIRDFoundationEuropeOnlus

4-5 cycles 2 -3 cycles 1 cycle


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BMI average decrease: 2.1 points in one4-week training cycle

BMI average decrease: 8.9 points for patients

who attended 4-week training 4 times peryear for 6 years

Males lost weight 1.5 more than females

Males gain weight faster than females after

dismissal ( different behaviour ?)

P.w. that attended rehabilitation cyclesregularly mantained their weight loss until 6

months laterBaschirotto Institute for Rare Diseases BIRD Foundation Europe Onlus

Results


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Correct diet

Physical exercise Specific rehabilitation cycles :

correct level of alternation between

family life and institutional life


Weight control in P.W.


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The Prader-Willi Syndrome requires amultidisciplinary approach:

on one side is the diagnosis of the

disease, clinical and molecular; on the

other side is the daily battle with the

disease through hormonal therapies, diet

or education of all people that interactwith a patient.


Conclusion


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Rehabilitation team:

Psychologist

Neuropsychomotricist

Musical therapist

Psychomotricist

Physical therapist

Educators

Medical team:

Pediatrician

physiatrist

Neurologist

Neuropsychiatrist

Others: Endocrinologist, Dentist,

Opthalmologist, Dietician,

Dermatologist


PWS Team


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Diagnosis Free Project

Prader-Willi Sindrome

MESSICO

GUATEMALA

COSTARICA

NICARAGUA

ROMANIA

LITUANIAPOLONIA

SPAGNA

PARAGUAY

COLOMBIA

REPUBBLICA

DOMENICANA

PAKISTAN

MAROCCO

BELGIO

ITALIA

ALGERIA

ARMENIA

CINA

CROAZIA

GEORGIA

GIORDANIA

INDIA

KENIA

MALESIA

CUBASLOVENIA

VENEZUELA

PERU

VIETNAM

USA

SERBIA

SERBIA

IRAN

BULGARIA

FILIPPINE


In collaboration with IPWSO


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Project Diagnosis Free

APECED(au o mmune o en ocr no a -can as s-ec o er ma s ro )


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Project Diagnosis Free

Lesch-Nyhan Sindrome


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Together for raredesease !

thanks


gilberto - pw zalau 4 definitivo

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