Medical professionals can contact PWSA (USA) to obtain more information and put you in touch with a specialist as needed.
Medical Alert General Information
Anesthesia, medication reactions
People with PWS may have unusual reactions to standard dosages of medications and anesthetic agents. Use extreme caution in giving medications that may cause sedation: prolonged and exaggerated responses have been reported.
Anesthesia
Adverse reactions to some medications
People with PWS may have unusual reactions to standard dosages of medications. Use extreme caution in giving medications that may cause sedation: prolonged and exaggerated responses have been reported. Water intoxication has occurred in relation to use of certain medications with antidiuretic effects, as well as from excess fluid intake alone.
High pain threshold
Lack of typical pain signals is common and may mask the presence of infection or injury. Someone with PWS may not complain of pain until infection is severe or may have difficulty localizing pain. Parent/caregiver reports of subtle changes in condition or behavior should be investigated for medical cause.
Respiratory concerns
Individuals with PWS may be at increased risk for respiratory difficulties. Hypotonia, weak chest muscles, and sleep apnea are among possible complicating factors. Anyone with significant snoring, regardless of age, should have a medical evaluation to look for obstructive sleep apnea.
Lack of vomiting
Vomiting rarely occurs in those with PWS. Emetics may be ineffective, and repeated doses may cause toxicity. This characteristic is of particular concern in light of hyperphagia and the possible ingestion of uncooked, spoiled, or otherwise unhealthful food items. The presence of vomiting may signal a life-threatening illness.
Severe gastric illness
Abdominal distention or bloating, pain, and/or vomiting may be signs of life-threatening gastric inflammation or necrosis, more common in PWS than in the general population. Rather than localized pain, there may be a general feeling of unwellness. If an individual with PWS has these symptoms, close observation is needed.
An X-ray and an endoscopy with biopsy may be necessary to determine degree of the problem and possible need for emergency surgery.
Another consideration is gastroparesis, a weakness of the stomach. This is a condition that is common with PWS and can be more life threatening then in a typical situation. A child with Prader-Willi syndrome when diagnosed with Gastroparesis may need hospitalization. Eating while the stomach is distended with gastroparesis can be very dangerous.
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Body temperature abnormalities
Idiopathic hyper- and hypothermia have been reported. Hyperthermia may occur during minor illness and in procedures requiring anesthesia. Fever may be absent despite serious infection.
Skin lesions and bruises
Because of a habit that is common in PWS, open sores caused by skin picking may be apparent. Individuals with PWS also tend to bruise easily. Appearance of such wounds and bruises may wrongly lead to suspicion of physical abuse.
Sunday, November 25, 2007
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