berinde - !!!!prader-willy
TRANSCRIPT
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NURSING ASPECTS
FOR PWS PATIENTS
Asistent: Berinde Livia
Goron Claudia
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PWS is a lifelong, life-
threatening, non-
inheritedgenetic disorder that
results from a defect on
Chromosome 15
PWS equally affects all
races and both sexes.
PWS is the most common
genetic cause of
obesity
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Clinical Features
PWS can be thought of as a Two-stage Disorder
Stage 1 Infancy Stage
Low birth weight and subsequent failure to thrive
Severe muscle weakness (hypotonia), excessivesleepiness
Suck/swallow problems, reflux, respiratory problems
Subtle dysmorphic facial features such as triangleshaped mouth, narrow forehead, almond-shaped
eyes Underdeveloped sexual organs
Delayed motor/physical milestones
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Clinical Features
Stage 2 Hunger Stage
Between ages 2-6, hyperphagia symptomsbegin with a preoccupation with food and/or
compulsion to eat Voracious appetite begins as brain does not
receive/process signals of feeling full
Slow metabolic rate causes rapid weight gain
Low energy level makes it difficult to exercise
Emotional labiality and behavioralmanifestations
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Physiological Characteristics
Hypotonia - Weak Muscle Tone
Abnormal Growth (short stature, small hands & feet)
Problems with Strength, Balance, Coordination, Motor Planning
Hyperphagia - Dysfunctional Appetite Regulating System
Respiratory Issues Gastrointerological IssuesGastroparesis, Slow Bowel Motility,
Inability to Vomit
Hyper- & Hypothermia - Irregularities in Body TemperatureRegulating Systems
Incomplete Sexual Development Hypopigmentation
High Pain Threshold, Bruise Easily
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Physiological Characteristics
Disordered Sleep
Cognitive Limitations
Speech and Language Issues (Dyspraxia & Apraxia)
Dental Problems (decreased saliva production cancause severe problems)
Skin Scratching and Picking
Temperament and Behavior Issues with OlderChildren Through Adulthood
Social / Psychological / Psychiatric Problems
Other common characteristics may include: scoliosis,eye abnormalities (strabismus), medicationsensitivity, orthopedic problems
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Cognitive Characteristics
Most individuals with PWS have decreased intellectual functioning.
Average IQ typically 55-70.
Decreased abilities in:
Picture recognition
Mathematics
Short-term memory
Daily living skills despite IQ
Areas of Strength:
Friendliness, affectionate, desire to please, desire to nurture Long-term memory
Recognize and evaluate shapes and figures
Integrate stimuli in spatial relationship
Puzzle solving
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Feeding and pursuit of body weight
They are incapable to feel the full sensation
They need food all the time;
They have lower caloric needs in comparison with the
children with the same age and sex; Infants can ask for nipples (ex. Feeding advices -FeederHabermann);
Other foods will not be served instead of thepersonalized menu;
The patients will get only skim milk; The milk is served without sugar;
The bread will not be served if it is not specified;
The food is served on tray in the carring room;
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The patients can drink tea without any restrictions butit has to be without sugar
It is not allowed to offer or consumed food close toPWS patients;
The regime has to be followed by the parents,patients, and the nursing as well;
The body weight will be check out every day;
The weight will be written down on the observation
paper; The feeding of patients will be put under observation;
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Treatment
Growing hormone
injection; Genotropin will be
injected one/day to a fixhour, following thedoctor directions;
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The problems which appear in PWS
They can have unusual reactions to the normal doze ofmedicine and to the anaesthesias;
Using under attention of the medicine which producelifelessness;
Intoxication with water;
Sensitivity to the pain;
Breathing prepblems;
Seldom vomitings can drow attention on a deadly disease;
Sever gastric disease;
Fluctuations of the body temperature ( hypertermia-
hypotermina); Hyperphagia;
Wounds of the skin and scratches(because of the commoncustoms of the patients-scratching and pinching;
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Behaviour problems
The patients can be:
Nervous
Stubborn
The behaviour can have positive aspects : Caring
Happy
Large minded
Developed sense of humour Conservative
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The quality of life
The teenagers and the
adults with PWS can workgreat in groups if the
dietetic control and
environment condition are
assured.
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Thank for your attention!