Tuesday, 24 November 2015

Non-24-Hour Sleep-Wake Disorder Awareness Day - The World Spins Madly On

"I just got lost and slept right through the dawn, and the world spins madly on"

Today is November 24th. It is also the ‘Awareness Day’ for Non-24-Hour Sleep-Wake Disorder, a rare and "extremely debilitating" circadian rhythm disorder. We have no idea how many (or how few?) people are affected by it, or how to even treat it.

There has been a fair bit written on why 'Awareness Days' are, in general, a bit rubbishself-indulgent and excessively corporate and fundraising-y. I generally agree, but I'm going to defend this one (and a few others) - N24, like many things, is rare enough that not even doctors have heard of it if they don't specialise in the area and our suffering could be so significantly alleviated by understanding and accommodations - and this can only happen once people are 'aware'.

I'm also not raising money and, unlike a lot of 'Awareness Day' stuff, I'm someone with the condition under discussion. This is us trying to make our (and other people's) lives better, and not get Good Samaritan Browniepoints while ignoring the issue for the rest of the year. This is a daily struggle, and one without end.

I'm also only really dealing with N24 as it affects sighted people, because Sighted N24 and No-Light-Perception N24 should be considered different conditions. 

N24 Awareness Day feels particularly important to me this year, due to a (hopefully innocent) campaign of misinformation which is also why I'm focussing only on sighted people. When I published a photo of my latest research submission, I got a comment of surprise that N24 could affect us. This is because of a pharmaceutical company which has a license for a drug to treat N24 in NLP people only. Their adverts describe N24 as something that only happens to blind people, and it went so far as getting regulatory agencies (eg FDA, EMA) to define N24 as "occurring in blind people". This is patently not the case (the first reported case of sighted N24 was in 1971) but this myth is getting traction.

This culminated in that drug company donating money to America's National Sleep Foundation which then produced an absolutely atrocious website  with the gem "N24HSWS is transient, reversible, and may be associated with a psychiatric disorder in sighted subjects".

Awareness is the opposite of ignorance. Hopefully some awareness will fight against that ignorance.


1: What is N24?

N24 is a chronic disorder characterised by a circadian rhythm that differs from 24 hours - the vast majority are over 24 hours but cases of less than 24 hours exist. Mine, for example, is 26 hours.

2: but what does that mean?

The circadian rhythm is your 'body clock'. It controls every time-related function of your body - it controls your blood pressure, appetite, and more subtle things like cortisol secretion. Most obviously, it affects sleep. Your circadian rhythm is the mechanism by which you fall asleep every night and wake up every morning, and it reacts to light and darkness to make sure this happens.

For whatever reason the circadian rhythm isn't 24 hours, that causes the time-linked biological functions to vary by how far off 24 hours it is. My circadian rhythm is 26 hours so, relative to the 24 hour clock, my biological functions get two hours later each day. I fall asleep at midnight one day, then 2am the next. Then 4am, 6am, 8am.. ad infinitum. Below is a copy of my 'actigraphy' (the standard diagnostic test for N24, a wrist-worn accelerometer that measures movements. Fewer movements = sleep) showing this pattern.


3: Okay, so why is this bad?

Being forced into functioning in the typical 24 hour pattern causes us a great deal of (physical and psychological) distress. Following our own pattern causes us a great deal of psychological distress, and severe difficulties with employment, interactions with society, education, relationships.

There is a harrowing case study (n24 is so rare that case studies are often all that is possible) of someone who developed N24 symptoms as a child - he had been misdiagnosed with multiple conditions, including "depression, schizotypal personality disorder, and learning disabilities". He was described by a psychologist as:

"being extremely introverted with severe narcissistic traits, poverty of thought, and disturbed thinking, including thoughts with persecutory content and self-destruction that led to a paralyzing anxiety, anhedonia, social isolation, and withdrawal"

When he had been successfully treated (a rarity in itself, unfortunately), there was no evidence of psychopathology. Hopefully this gets across the horrific effects of the long-term sleep deprivation we are often forced to endure, but there are physical effects too. Shift workers are very useful for studying the health effects of inflicting circadian disruptions on people - and shift work has been linked to various cancersendocrine disorders and cardiovascular disease.

I've got my own research, currently under peer review at the journal Qualitative Health Research, which shows the harmful psychological and 'quality of life' effects the nocturnal phases of N24 have, but others have noted that, despite almost universal and severe difficulties with education and employment, depression symptoms alleviate when the person is in their 'dirunal' (ie awake during the day) phase.

4: How is n24 treated?

Treatment is more frequently successful in N24s without light perception, because their condition is due to a disruption in the light-suprachiasmatic nucleus-pineal gland system and this can often be helped with melatonin or other similar drugs (eg the recently licensed tasimelteon - it hasn't been tested, but I'm willing to go on record as predicting it to not be significantly different from melatonin. It only entrained ~30% of blind N24s, if memory serves. I can't find the FDA documents again).

Few treatments are studied in sighted N24s and those that are have very weak, if any, evidence for their usefulness. The standard approach is melatonin and bright (10k) lux, but the evidence for their effectiveness is case studies, and for every supportive case study there is a negative one. Basically, 'it'll work sometimes' but as my own neurologist said, "[n24] is very difficult to treat and keep under control, with short-term relief usually only possible with short courses of strong hypnotics and stimulants" [his full letter is at the bottom, it's a good brief description of n24].

How is N24 treated? It's a crap-shoot, and it usually doesn't work anyway.

5: What would make your life better?

Precluding a wonder-drug that allows entrainment in the absence of debilitating symptoms that sometimes accompany it (eg extreme tiredness), there are two things that would be most helpful, and these stem from awareness.

The first is compassion. Our lives are a struggle and more often than not we are blamed for it, by everyone. We are blamed by our parents and teachers, our doctors and employers. We're "lazy", "inconsiderate" "scroungers" who "just don't try hard enough".

This is partly based on the way society misunderstands sleep - people believe they are in control of their sleep. They think they can chose when stay awake and when to sleep, but they can't. They don't see that because, as a general rule, their sleep falls within the boundaries society is built to accept. They'll usually complain about "waking up too early on their day off" or "not being able to get to sleep" but never seem to put two and two together. You can put yourself in a situation where you are more likely to sleep, but you can't make yourself fall asleep.

But because people think they are in control of their sleep, they think we are too and thus we are to blame for missing work, or sleeping through an appointment, or spending the day in bed instead of going clubbing with you. We internalise this blame, and we hate ourselves.

The second is societal change and accommodations. In a lot of areas, this seems to be going in he right direction, albeit to a small degree. 

Work hours are becoming more flexible, but it's not enough. Employers still demand work be done "during the 9 to 5 (or 7 to 3)" when it could be done just as easily and well at any time, even if the person is allowed flexible working hours or to work from home.

We're allowed to book doctor appointments on-line during the night, but we need to do it days and weeks in advance when we won't have a clue what state we'll be in (and this consequently affects the appointment and our test results, eg blood pressure, hormones, etc).

24 hour shopping is becoming more commonplace, but is meaningless when public transport stops for 10 hours at 9pm, as many N24s cannot drive due to safety risks.

Life is much less lonely now we're able to socialise on the Internet at any hour of the day but this is no substitute for face-to-face interaction for those that need it.

There are so many little difficulties and they all add up. We'll often ask you if you can make a change to your procedures at work and so on, and they'll usually be little things like "don't discharge me from my sleep disorders hospital clinic for sleeping through an appointment".

In general though, ask yourself this: could someone nocturnal and with little ability to plan in advance use my shop/work for me/access, as easily as others, the service I provide? If the answer is no, you'll spot ways you can make the life of sighted N24s* a little easier.

*and people with Delayed and Irregular CRDs, and NLP N24s, and countless other sleep disorders and things that cause severe tiredness and fatigue.

Edit: Doctor's letter!

2 comments:

  1. Circadian Sleep Disorders Network successfully campaigned to have the American National Sleep Foundation correct that "atrocious website". The corrected page now properly acknowledges sighted Non-24:
    https://sleepfoundation.org/non-24/content/sighted-vs-blind

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    1. I know. The link was to CSDNs page on it.

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