What is Narcolepsy?

fall drive on Skyline Drive in Shenandoah NP earlier this year

The following is courtesy of Kiara Miller, who posted this in one of my narcolepsy Facebook groups, and gave us all permission to share it. She wrote this to help us explain to those around us how narcolepsy impacts our lives. I added the subheadings.

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Dear family or friends of someone with narcolepsy,

I am a narcoleptic writing on behalf of my fellow sleepy people, in hopes that these words might help even one or two people to understand a little better what their loved one is dealing with. This may seem very long, and you may think it is an inconvenience to take the time to read it, but I deeply encourage you to do so. I have put a great deal of care into writing this.

What is narcolepsy?

Narcolepsy is a disorder of the brain, but it is not to be confused with a mental disorder. This disorder is very real and very physical in origin. It is brain damage. For now, this brain damage is irreversible. Luckily, new research is being done all the time on narcolepsy, and we are learning more and more about the cause and the best ways to treat it, even if we can't cure it. 

Narcolepsy is, at least in most cases, an autoimmune disorder. What this means is that at some point in our lives our bodies got some type of infection, like the flu. While fighting this infection, our bodies made a mistake, and thought that an important part of our brain was actually part of the virus, and therefore needed to be killed off. 

This part of the brain that got attacked has around 70,000 neurons (nerve cells) that produce something called orexin (also referred to as hypocretin). In the case of narcolepsy, almost *all* of these neurons are destroyed. 

Orexin is best known for regulating appetite and energy expenditure (how much energy is needed to do what), and stimulating or encouraging wakefulness. It is responsible for taking in a ton of information and using that information to tell the body when it should be awake, and when it should be asleep. 

This information includes things like circadian rhythms (we should sleep at night, be awake during the day) and sleep debt. Sleep debt means you didn't sleep enough at night (or didn't get good quality sleep) so your body stores up a need for extra sleep. You might need to take a nap, or sleep in late on the weekends, and in a healthy body, getting enough extra rest will make up the sleep debt and you will feel better. Many people have sleep debt these days because of the demands of modern society or from being a parent, which is why caffeine is so popular. However, if they were able to get that extra sleep they needed, they would feel refreshed. 

For a narcoleptic, all of this is messed up. We have no regulation of sleepiness and wakefulness. We want to be awake during the day, but we physically can't because the part of our brain that says we should be awake is gone. This is exacerbated by the fact that we almost never get truly restorative sleep, even if we were to sleep for days. We are constantly increasing our sleep debt, and unable to ever make it up.

What narcolepsy looks like

Even though narcoleptics are thought to sleep all the time, that's not always true. At night, many of us struggle with insomnia and fractured sleep because now our brains aren't telling us it's time to sleep. But even when narcoleptics do sleep (whether at night or during the day), we usually spend a significant portion of our sleep in the lightest sleep stages, which are not restful, and in REM, where the brain activity is closest to how it is when awake. REM stage is when dreaming happens. Although this stage is very important, it is supposed to be in balance with deep sleep, when brain activity becomes very quiet and allows our brains to rest. The two stages work in harmony to keep people healthy and functional. Narcoleptics get far too much REM sleep, and not enough deep sleep, so our brains are very active most of the night. During the day, when our brains are constantly pulling us into sleep, even if we sleep briefly we often go directly into REM sleep. So even if we nap, our brains are working hard, they are active, they aren't resting. 

So during the day, not only are we missing the part of our body that tells us to stay awake, we are also chronically sleep deprived whether we sleep or not. Like a battery that only ever charges to 30 percent and instantly starts draining as soon as it is unplugged, sometimes hitting empty within a couple hours, sometimes within a few minutes. 

The disregulation and sleep deprivation causes a constant overlap of wakefulness and sleep. We can actually think we are awake, and be asleep. You might even think we are awake, we might even be talking to you, but some part of our brain could still be asleep (and dreaming). Yeah, it's really freaky! When narcoleptics take the mslt, or the daytime sleep study, many of us don't even think we fell asleep during the naps. But not only did we sleep, we were dreaming and hitting the REM sleep stage super fast. A healthy, well rested brain should never hit REM sleep during a 20 minute nap. 

We are prone to having very vivid, life-like dreams, which can cause great confusion about what is reality, and what was a dream. It can also cause us to think we have done something we said we would do or believe we had conversations we never had, because we dreamt it in perfect clarity. We can also wake up from a dream, and fall back asleep, picking up exactly where we left off in the dream, or immediately entering a new dream. Many of us also have severe nightmares, and because the nightmares are so vivid, they can cause increases in our stress hormones, our adrenaline, and our anxiety. They can also make us scared to sleep and contribute to the insomnia. 

Another symptom of narcolepsy is that many of us enter REM before we are even fully asleep. This can cause hallucinations. These hallucinations are dreams overlapping our actual life as we are falling asleep or as we are waking up. Since we easily fall asleep during the day, some of us experience hallucinations throughout the day as well, which is disorienting and can be incredibly scary. 

Related to this is another symptom called sleep paralysis. During REM sleep, the body is supposed to produce a chemical that causes temporary loss of muscle tone and paralyzes us so we don't injure ourselves or others by acting out our dreams. This is called REM atonia. For many narcoleptics, they wake up while still paralyzed and usually while still partially dreaming. This can cause feelings of helplessness as well as fear because sleep paralysis is often accompanied by hallucinations. 

Finally, a very significant symptom of narcolepsy is called cataplexy. Cataplexy is only present in type 1 Narcolepsy, but some people may have it and not realize it because it's mild or they thought it was normal. Cataplexy is regularly misdiagnosed and unrecognized by medical professionals. Remember REM atonia, the muscle paralysis that should happen when you dream? In a person with cataplexy, this muscle paralysis gets triggered during the day, while a person is awake and conscious, because of emotional responses. The brain senses emotion and thinks, quick, produce this chemical, and loss of muscle tone follows. This can be mild and brief, like sudden drooping of the face. It can affect the tongue causing slurring. It can be more moderate, such as an arm giving out and dropping something, the head suddenly falling down, buckling knees, or the torso slumping. In severe cases, the body can completely collapse. A person with cataplexy might get angry or stressed, or they might laugh at a joke, or they might feel very happy or very sad or excited, and then they just collapse like a puppet that had its strings cut. They might have their eyes closed, and be completely unresponsive. But they are aware of everything, and aware of their inability to move or respond. Undiagnosed, this is terrifying. It also makes us very vulnerable if it happens in public or with people who are untrustworthy. 

Some narcoleptics also suffer from something called sleep inertia. This means that it can be nearly impossible to wake up on demand, even if someone is shaking our body or if we have set 20 alarms. Imagine that only seconds after you wake up, you feel drunk or drugged or like you've been hit by a truck, and then you are pulled back into sleep. Imagine this wave of drugged, painful consciousness happening over and over again every time you try to wake, and if you manage to force yourself out of bed you might get a horrible migraine or feel like throwing up, or you actually throw up. In any case, you are completely and utterly miserable and it can last for hours. Imagine feeling this way even after a 20 minute nap. This is often debilitating and can make it so hard to wake up in the morning to go to school or go to work.

What narcolepsy really means on a day-to-day basis

So please, think about all of this for a moment. Think about the most tired you have ever felt, so tired that all you could think about was sleep, that all you could do was sleep, and imagine that never going away. It's like being given heavy doses of sleeping pills throughout the day, against your will, and then trying to work, or study, or enjoy life. That's just one symptom, the excessive daytime sleepiness, and most of us have at least one of the other symptoms, sometimes all of them. 

Imagine finally learning that this horrible, lonely, disabling disorder is actually brain damage, and then the people who should support you still see you as lazy even though you desperately want to be functional. Imagine that you find a medication that actually gives you any degree of functionality, but then you get judged for taking it. And that is if you are lucky enough to respond well to treatment. Many of us, unfortunately, do not tolerate the available medications or we can sleep right through them. It is discouraging, frustrating, and isolating. 

If your loved one has narcolepsy, it may feel like a shock. It may cause feelings of hopelessness, anger, denial. But it is in fact a blessing. This would have been a lifelong issue with or without a diagnosis, but without a diagnosis, we wouldn't have the hope of finding a treatment that works. We wouldn't have support groups that makes us feel less alone. We would be constantly berating ourselves and wondering why even when we sleep, it feels like we haven't. We wouldn't understand why we are struggling, and neither would our doctors, and neither would our families and friends. But now we can understand it, and we can look for doctors who understand it, and if you try to, and open your hearts and minds to new information, you can understand it as well. And that kind of understanding is life changing for us.

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Me again, with some more thoughts.

When I read this, it struck me that this is what I've been trying to articulate, but haven't been able to do so. And I'm not the only one- so many people in the group who read this had the same reaction.

As I've mentioned before, I'm very lucky to have a mild case of narcolepsy. I do have disordered sleep, insomnia,  and excessive daytime sleepiness and I believe I've had sleep inertia in the past, but I've been able to regulate it through a mindful diet. I don't have hallucinations, sleep paralysis, or cataplexy, but I know this could change, and I'm praying it doesn't. So far, the one stimulant I tried didn't work. I don't know if other stimulants will work. I haven't tried the nighttime meds to help me sleep better, and I'm not sure I want to. All these meds come with side effects, may not work, or may lose their efficacy after being taken for awhile. And lets not even talk about how much they all cost.

That is one part she didn't mention: the financial aspect of narcolepsy. Many people go undiagnosed or misdiagnosed for years, despite many doctor visits (raises hand- it took almost 20 years to get my diagnosis). Doctor visits and testing are expensive. Medications are expensive, and insurance companies frequently decide that people don't need this more expensive one, they should take the cheaper one, even though it didn't work for them.

Schoolwork suffers when you can't stay awake long enough to study or to make it to class. Comprehending new material becomes a huge challenge. Once bright futures fade away with each sleepless night.

And what about jobs? Those of us with milder cases of narcolepsy are able to work (though I don't think I'd be able to hold down a desk job at this point, so I am very grateful for the flexible schedule that comes with being self-employed), but many with worse symptoms simply cannot hold a job. They are doomed to live out the rest of their lives at or below subsistence level, relying on disability and government handouts and praying that their medication will be covered. What kind of life is that?

Despite all this, I am glad to finally have a diagnosis. As she said in the last paragraph, it's such a blessing to know why I never seem to sleep well, why I'm always tired, why I'm so non-functional in the afternoons, why I used to wake up feeling like I had a hangover, despite never touching a drop of alcohol in my life (that would be the sleep inertia).

Knowing there's a reason for all this and that certain things won't ever change for the better has made it easier to re-evaluate what I expect to be able to do on any given day. I'm moving tasks around and changing things up, and it feels more productive. I'll write more about this when I've had a chance to try things out a bit more, but in the meantime, I do feel like things are looking up a bit.