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Published:March 17th, 2015 11:04 EST

Moshe Turner's Thoughts on Narcolepsy

By SOP newswire3

This letter is in response to Robert Paul Reyes` article:

Regarding your recent article on World Sleep Day, I thought you should be in possession of the following information:

In the hypothalamus of our brain there is a small population of a few hundred thousand specialized cells called hypocretin neurons which produce several neurotransmitters.  Due to their having been identified and named almost simultaneously by separate teams in the US and Japan they are also known as orexin neurons, as each team named their discovery independently.  For simplicity we will call them orexin neurons, and the peptide they primarily produce, orexin.


When these orexin neurons are either completely or partially killed off by a misdirected autoimmune response, orexin becomes unavailable or its availability becomes severely reduced.  The result is that orexinergic signaling either stops or becomes drastically limited.  This causes much damage to the nervous system.  The most visible and recognizable physical manifestation of this damage is a cluster of symptoms collectively known for the last 100 years as narcolepsy.  The wake/sleep cycles of narcoleptics are dysregulated due to diminished or absent orexinergic signaling; their nighttime sleep is interrupted and their daytime wakefulness is punctuated by bouts of Excessive Daytime Sleepiness (EDS).  Other obvious symptoms of narcolepsy include sleep paralysis,

hypnagogic and hypnopompic hallucinations, and cataplexy.


Narcolepsy used to be known as a sleep disorder of psychological origin as scientists had not been able to find a physiological cause.  That changed with the discovery of orexin in 1998.  In recent years narcolepsy has been reclassified as neurological disorder.  Type 1 narcolepsy (with cataplexy) occurs in 65-70% of patients, with most of the remaining sufferers falling into the category of Type 2 narcolepsy (without cataplexy).


Orexin is a critical part of nervous system function.  It regulates or is involved in the regulation of almost all of the other neurotransmitters, and plays such a prominent role that it has been called "the conductor of the neural symphony".  Apart from regulating the sleep/wake cycle, orexin functions as a sensor and integrator of internal and external environmental conditions and in response to changing conditions such as hunger, fear, cold, etc., orexin regulates food seeking, homeostasis, thermogenesis, respiration, executive function and cognition, motivation, sex drive, mood, circulatory and cardiac function, intestinal motility, olfactory perception and a host of other processes. Also, because orexin directly regulates other neurotransmitters, when orexin is not available the result is a series of cascading failures of those other systems in ways that that appear to be unrelated. 


Very few clinicians are knowledgeable about narcolepsy and are not able to understand the connections between these apparently unrelated symptoms.  They often dismiss their patients as having psychological problems or simply lump them all under the catch-all diagnosis of dysautonomia.  Accordingly, patients are often denied treatment for the very real symptoms of what is essentially an invisible illness.  


Even fewer medical people have the knowledge and skills to accurately diagnose narcolepsy.  Although modern internet communication has gone a long way towards improving diagnosis times, it is still not unusual for a patient to go 6-10 years before getting a diagnosis.  Older narcoleptics will often tell you that they went without diagnosis for as long 30 or 40 years.


Undiagnosed, narcolepsy slowly destroys the lives of those who are afflicted with it.  This stealthy and silent condition affects every aspect of living.  Often, by the time a diagnosis is obtained it is too late to rescue what has been lost; marriages are ruined, careers lost, family becomes estranged, friendships ended.  Further, it is so difficult for other people to understand what it`s like to be a narcoleptic that even after diagnosis it`s effects continue to wreak havoc in a person`s life. It is not unusual for narcoleptics to end up poor, divorced and unemployed.


Ask any person who is narcoleptic what they want more than anything else and almost every time the answer you`ll get is "I want other people to understand what it`s like to be me."


While narcolepsy is essentially a stable condition, over time the effect of a lifetime without restful sleep and a dysregulated nervous system eventually take their toll on a body and symptoms will worsen and new ones may arise.   In particular, while the arrival of middle age often signals a slowing down for most people, the effect is amplified many times over in narcoleptics, whose bodies` ability to regulate homeostasis is already impaired.


There is no cure for narcolepsy.  Most narcoleptics are treated with stimulant medications for the excessive daytime sleepiness.  Some of those with type 1 narcolepsy make use of antidepressants, especially SNRI`s to help with cataplexy.  Those who can tolerate the often serious physical and psychological side effects use Xyrem, a sleep consolidator and anti-catiplexy drug.  However, many narcoleptics are denied the medicines they need by their insurance carriers, many have bad reactions to various medications, and others are uninsured or can`t afford the staggering costs of some of these medicines.  For example, a month`s worth of treatment with Xyrem costs upwards of $10,000, and many insurers balk at making such payments.


Moshe Turner

Baltimore, MD USA