Further Information
The Prader-Willi Syndrome Association (USA) provides information with regards to ‘Sleep Problems in Prader-Willi Syndrome’ on their website.
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Sleep disorders and Respiratory issues are common in people with Prader-Willi Syndrome (PWS).
Sleep problems frequently occur in people with PWS. The most common problem individuals face is excessive daytime sleepiness (EDS). At times EDS can be related to sleep apnea which can disrupt the quality of sleep.
Obstructive Sleep Apnoea (OSA) is caused by the relaxation and narrowing of the throat, causing a physical obstruction and is associated with increasing body mass index.
Central Sleep Apnoea (CSA) is due to communication problems between the brain and the breathing-related muscles, failing to induce inhalation.
Many individuals with PWS experience oxygen desaturations as a result of both OSA and CSA, but some of this abnormally low concentration of oxygen in the blood, can occur independently of apnoeas; or, overly shallow breathing or an unusually low respiratory rate.
Interruption of the continuity of sleep can be caused by Sleep apnoea, and these disruptions result in a decrease in both the quality and quantity of sleep, which can result in Excessive daytime sleepiness (EDS).
Due to hypothalamic dysfunction, individuals with PWS have more sleep cycles per night and more REM periods (dream sleep), but the overall amount of REM sleep is decreased. Also, due to the occurrence of apnoeas, their sleep may be further compromised which reduces the amount of total sleep they have each day, putting them what the Prader-Willi Syndrome Association of USA refers to as ‘sleep debt’. Because excessive daytime sleepiness has been correlated with the intrusion of REM sleep into wakefulness, individuals with PWS may appear to have narcolepsy-like symptoms.
Treating a sleep disorder can help improve the quality of sleep. The same treatments that health care providers use with the general population can apply to individuals with PWS. Patients looking to begin Growth Hormone therapy will be required to have a Sleep Study performed to check for any Sleep abnormalities such as sleep apnoea, before an Endocrinologist prescribing treatment. Often the use of a CPAP machine is recommended.