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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!

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