Important Considerations For Routine Or Emergency Treatment
New publications not listed in the general information below:
- New Medical Alert
- Central Adrenal Insufficiency in Individuals with Prader-Willi
Syndrome Click here
- Guidelines for Postoperative Monitoring of Pediatric Patients with
Prader-Willi Syndrome Click here
- Medical Alert for Inpatient Care
Medical professionals can contact
PWSA (USA) to obtain more information and put you in touch with a
specialist as needed.
Medical Alert General Information
Order this information in an easy to use booklet format
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
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
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.
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
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
that delays emptying and may lead to dilation of the stomach size.
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. For more
information go to:
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
Hyperphagia (excessive appetite)
Insatiable appetite may lead to life-threatening
weight gain, which can be very rapid and occur even on a low-calorie diet. Individuals
with PWS must be supervised at all times in all settings where food is accessible. Those
who have normal weight have achieved this because of strict external control of their diet
and food intake.
Surgical and Orthopedic Concerns
In view of the increasing number of infants and children with PWS
undergoing sleep assessments prior to growth hormone treatment and
the potential rise in surgical procedures (e.g., tonsillectomy)
requiring intubation and anesthesia, it will be important to alert
the medical team about complications. These complications may
include trauma to the airway, oropharynx, or lungs due to possible
anatomic and physiologic differences seen in PWS such as a narrow
airway, underdevelopment of the larynx and trachea, hypotonia,
edema, and scoliosis.í
Musculoskeletal manifestations, including scoliosis, hip
dysplasia, fractured bones and lower limb alignment abnormalities,
are described in the orthopedic literature. However, care of this
patient population from the orthopedic surgeonís perspective is
complicated by other clinical manifestations of PWS.
Clinical Concerns for the Orthopedic Surgeon
Growth Hormone Consensus Statement
IN THE EVENT OF DEATH
Organ Donation for Research
When a child or adult with PWS dies, the family
may wish to consider donation of organs for research. PWSA (USA) has established a
procedure for such donations to the Brain and Tissue Banks.
Click here for more information.
Reporting of Deaths
The Prader-Willi Syndrome Association (USA)
has created a research database of reported deaths of individuals with PWS. Although most
premature deaths are attributable to morbid obesity, cases unrelated to obesity have
recently been noted, leading PWSA to begin a formal investigation of causes of death.
PWSA also provides bereavement support to families who have lost
children with PWS. In the event of death of someone with PWS, please contact PWSA (USA):
1-800-926-4797 or after hours click here.
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