Medical Alerts / A&E Resources
Medical Alert: ANAESTHESIA
Possibility of unusual reactions to anaesthetics. Close monitoring of general anaesthesia with risks specific to PWS carefully evaluated means that the majority of complications come from less well supervised conscious sedation. Prolonged and exaggerated response to narcotics/ sedatives can occur. Perioperative concerns of PWS include OSA/CSA, decreased pulmonary reserve secondary to chest wall deformity (i.e. scoliosis), hypotonia, obesity and primary myocardial involvement. Plus, ….
Plus, an abnormal response to hypercapnia and hypoxia, risk of PE, a narrowed oropharyngeal space, hypoplastic dental enamel, thick saliva, an increased risk of gastric aspiration, convulsions, disturbances in thermoregulation, difficult IV access, and the possibilities of CAI, hypothyroidism, diabetes mellitus and glucose intolerance. Due to hyperphagia, it should be assumed a patient with PWS has eaten food unless confirmed otherwise by a caregiver. Risk for complications increases with young age.
Gradual return to drinking and eating recommended due to high choking risk and possibility of slow GI function recovery. Outpatient procedures may require overnight observation due to prolonged effect of anaesthesia and effects of pain medications.
Please see Medical A-Z section for more detailed information on anaesthesia, post-operative monitoring and medicine contraindications.
Article: Anaesthesia and PWS – James Loker, MD, Laurence Rosenfield, MD
Case Reports: Special Anesthesia Concerns for Patients with PWS: The Winthrop University Hospital Center Experience
Case Reports: Anesthesia and PWS: preliminary experience with regional anesthesia
Medical Alert: MEDICATIONS, WATER INTOXICATION
Use caution with medications that may cause sedation, suppress breathing or slow digestion further (pain meds, anaesthetics and psychotropic meds.) Combinations of medications can fatally impair breathing in PWS, particularly after surgery. Standard dosages of medications may produce unusual reactions and lower dosages may be required. Side effects more likely in some medications at standard dose (SSRI’s and neuroleptics.)
The increased risk for disrupted temperature regulation or hyperglycaemia /diabetes mellitus in PWS should be considered with some medications.
Water intoxication has occurred in relation to some medications with antidiuretic effects (including some neurotropics) and antidiarrhoeal medications may cause severe colonic distension. Water intoxication can also occur as a result of excessive fluid intake when compulsive water drinking related to hyperphagia becomes a problem. Concerns include: possibility of hormone imbalances in PWS affecting electrolytes, speculation about SIADH risk and medications frequently used in PWS include neurotropics and antidepressants. Check electrolytes and monitor for hyponatremia.
Please see Medical A-Z section for more detailed information on medicine contraindications.
Medical Alert: Medication combinations may fatally impair breathing in PWS
Medical Alert: Antidiuretic Hormone
Article: Water Intoxication Alert – PWSA(USA)
Medical Alert: DIABETES MELLITUS
Prevalence of type 2 diabetes in PWS exceeds that of the general population. The risk of developing T2DM is complex and appears to differ to non-PWS patients, possibly due to an altered glucose metabolism in some patients (impaired fasting glucose, impaired glucose tolerance). Risk increases after pubertal age and if obese (average age of onset = 20 years). Some common medications used by PWS patients may increase risk of hyperglycaemia / T2DM.