Prader-Willi Syndrome and Poor Sleep

Prader-Willi syndrome (PWS) is a genetic disorder estimated to affect between 1 in 10,000 and 1 in 30,000 births. The most common cause of PWS is by loss of imprinted gene expression from the paternal copy of chromosome 15. PWS is a complex disorder with many life challenges, such as constant feeling of being hungry, mental illness and other behavior issues, learning disabilities, scoliosis and short stature. These are just the major issues often faced by a person with PWS, there are many more unfortately.

An under appreciated condition that has a major impact in the quality of life is people with PWS are sleep deprived. The most common issue is excessive daytime sleep (EDS). It is rare you will find a person with PWS who is not sleepy through out the day. This makes doing school tasks, work and interaction with others more difficult. In the PWS community it is well understood that they have sleep issues, but what is just starting to be highligted is how much these sleep issues impact them.

It’s only been over the last three or so years where we as a society are realizing the importance of sleep. So often it you slept a lot you were considered lazy. We embraced the leaders who only slept four hours a night. Science is now highlighting the fact that not getting enough sleep is not only impacting cogntive funcstions but our other all health. Slowly but surely society is realizing that sleep is important to a quality of life.

Here is an example of how sleep deprivation impacts a person with PWS. EDS is correlated with a reduction in REM sleep. REM is often throught as the time when creativity reveals itself. This is true but an even bigger function of REM is the processing all the the previous days events and dealing them from an emotional perspective. A lack of REM sleep does not allow the person to properly deal with life’s troubles. Issues become bigger than they should be and situations that are no big deal become the flame for temper tamtrums. In short getting enough REM sleep allows you to better regulate your emotions. People with PWS often have trouble dealing with their emotions. Getting better sleep will help improve their ability to deal with life’s challenges.

More focus and research needs to be applied on how sleep can be improved in the PWS population. Better sleep may not completely resolve all the challenges PWS brings but it can help reduce them. Any reduction is an improvement in quality of life and their ability to contribute to society.