What is Idiopathic Hypersomnia? (And my experience with disability claims)

What is idiopathic hypersomnia

Related: 

Back in 2014, I was under a lot of stress. If I remember them all, the major stressors were an incredibly toxic work environment with a boss actively trying (and wrongfully) to get me fired, our upcoming wedding (and nearly every wedding-related stressor you could imagine! My bridesmaids dresses fell through just 4 weeks prior), a car accident and having to move with less than a day’s notice. Saying I was stretched and stressed to the limit would be a severe understatement. All the stress made me incredibly anxious, causing severe insomnia (most days, I couldn’t fall asleep until at least 5 or 6 am) which only served to worsen my idiopathic hypersomnia. I had dropped over 20 pounds and was at 102 pounds at my lowest.

My doctor put me on a medical leave for a total of 3 weeks and filled in all the necessary paperwork for my short-term disability claim. It was denied. Both my doctor and I were surprised.

So my doctor wrote a detailed 2-page letter and we refiled the claim.

Again, it was denied. This second denial got me incredibly depressed and hopeless (but that’s a story for another time). All the denials just added to the stress (8 weeks before the wedding!) and now having 3 weeks’ of pay clawed back was just the icing on the cake. Our wedding was weeks away, we had just moved, our car was still in the shop and I was absolutely losing my mind!

I couldn’t understand how such a detailed physician’s note could get declined! The only thing I could think of was that the case manager just had absolutely no clue what she was dealing with. Before I had been diagnosed with idiopathic hypersomnia, I had never even heard of it, and I had spent so much time researching and self-diagnosing! (I thought I was narcoleptic.) How could the case manager approve my need to be off work when she had no understanding of what idiopathic hypersomnia was?

I was livid. My family doctor, who went through medical school, wasn’t even qualified enough to diagnose my sleep disorder. I had to go to a specialized sleep doctor to get the diagnosis. How could a case manager who wasn’t even a doctor be qualified to decide whether I was eligible for disability benefits when they didn’t even understand what my disorder was?! What resource was she consulting to even find out what IH was? Google? Wikipedia?!

After my weekend of feeling utterly hopeless and depressed in bed, I got up and decided it was ME that had to educate them. I had to tell them what idiopathic hypersomnia was. I had to paint them a picture of what I went through everyday. I had to make them understand.

And so with my third appeal, I included an 11-page paper, all of which was backed with medical research. The below is an excerpt of what I submitted (which, by the way, was finally approved.)


Idiopathic Hypersomnia

Idiopathic Hypersomnia is a sleep disorder even less common than narcolepsy, a rare sleep disease[1]. With narcolepsy prevalent in only 0.07% of the US population[2] and only ~0.8% of sleep centre patients being diagnosed with idiopathic hypersomnia[3], information and resources surrounding the condition are limited.

However, with scientific evidence suggesting substantial overlap between narcolepsy and idiopathic hypersomnia[4] and my condition being considered a variant of narcolepsy based on the presenting symptoms and the severity of my sleepiness, most of the narcolepsy findings can also be extended to idiopathic hypersomnia.

The biggest distinguishing characteristics include differences in REM (Rapid Eye Movement) sleep, the absence of cataplexy (a sudden weakness in the muscles while fully conscious, usually triggered by strong emotion like laughter or anger) and, perhaps most unfortunately (for me and fellow IHers), the lack of refreshment post-napping in idiopathic hypersomnia.

Idiopathic hypersomnia is characterized by a severe and pervasive excessive daytime sleepiness (EDS) despite adequate or longer-than-average nighttime sleep, as well as other symptoms including:

  • Sleep drunkenness and sleep inertia upon awakening
  • Sleep attacks
  • Automatic behaviour
  • Hypnopompic hallucinations
  • Sleep paralysis

Excessive Daytime Sleepiness

Excessive Daytime Sleepiness (EDS) is the severe “difficulty sustaining full alertness even during active and stimulating situations” and the high probability of falling asleep inadvertently during sedentary activities[6].

Although many have often claimed “I know how you feel” based on their experience of tiredness, it’s been estimated that the average person would have to remain awake for 48-72 hours straight to experience the degree of sleepiness I experience every day[7] and that it would take 7 days without sleep to experience some of the symptoms an unmedicated narcoleptic experiences daily[8].

The severity of the sleepiness and chronic fatigue has far-reaching implications; the associated impairment of attention and cognition impact all aspects of life including school, work, relationships and leisure activities including:

  • Irritability and mood swings
  • Impaired memory, attention, creativity, concentration, work performance and productivity and judgment
  • Lack of energy, extreme exhaustion
  • Reduced social and physical functioning and overall quality of life
  • Increased risk for developing depression, emotional lability
  • Visibly appearing “stuporous or encephalopathic” [9],[10]
  • Mental “fog” or cloudiness[11]
  • Depression
  • Severe forgetfulness
  • Slowed or clumsy movement (e.g. walking into furniture, dropping items or sustaining injuries from normal activities like deep cuts from cooking)
  • Frequent headaches, orthostatic disturbances, Raynaud-like symptoms[12]

Sleep Drunkenness and Sleep Inertia

Awakening every morning is a difficult and laborious task, followed by an extended period of sleep drunkenness and extreme sleep inertia[13]. Sleep inertia is the grogginess felt immediately following an abrupt awakening, resulting in impaired alertness which interferes with the ability to perform mental or physical tasks[14].

Sleep drunkenness is very similar to being intoxicated, with the level of “drunkenness” directly correlated with the level of fatigue. It occurs upon awakening as well as periods of heightened fatigue, manifesting similarly to Blood Alcohol Concentration levels of 0.10-0.125% with typical symptoms including headaches, significantly impaired motor coordination, loss of good judgment, slurred speech and impaired balance, vision and reaction times (driving with BAC over 0.08% is illegal in Ontario[15]). If the fatigue is extreme, it may appear closer to BAC levels of 0.25% (requiring assistance to walk, mental confusion, nausea and occasionally vomiting)[16].

Sleep Attacks

Despite intense urges to sleep throughout the day known as sleep attacks, daytime naps are long yet unrefreshing. The excessive daytime sleepiness (EDS) is so severe that it, by definition, “interferes with daytime activities, productivity or enjoyment” [17].

Sleep attacks are defined as the “recurrent, precipitous and unavoidable need to stop an activity and take a nap” [18] but many who experience it, including myself, feel that the definition doesn’t quite seem to adequately capture its essence.

Imagine swimming in open waters for an extended period of time. The exhaustion and fatigue you’d feel is like the chronic fatigue experienced all day, where your thoughts are primarily dominated by one goal: to keep swimming until you reach land and can collapse.

Then, suddenly and without warning, the waters get violently choppy AND weights have been attached to your arms and legs. You can’t let the waves overcome you or you’ll drown, but the struggle to keep your head above water is like trying to run under water. Despite channeling every ounce of strength and will you can muster into struggling to stay above water just for a gasp of air, the weights pull you back down. You’re still conscious – for now – but your mind is fully consumed by only one thought: don’t drown. The second you are too weak to thrash wildly to keep your head above water, the second the waves overpower you, you’ve lost the battle.

This extreme battle is one I fight every single time a sleep attack strikes. Trying to fight off a sleep attack is just as difficult as the above scenarios, and if I am somehow able to prolong succumbing to sleep, the only thing on my mind is how desperately exhausted I am and how badly I need to sleep.

Whether I’ve slept 4, 6 or even 10+ hours, I will never wake up feeling refreshed, and by the time I’ve finished brushing my teeth, I’m already sleepy enough to fall right back asleep. Despite adequate nighttime sleep, I will spend the rest of the day battling varying degrees of fatigue and sleep attacks.

When a sleep attack strikes, what little energy I have leaves my body and despite my fierce struggles to remain conscious, it always inevitably results in the same thing: eyelids drooping under what feels like the weight of a sack of bricks, head nodding and slurred speech. Whatever activity I’m in the middle of is interrupted as I weave in and out of reality/consciousness/dreaming and the dreams I try to fight off always make their way back into reality through my speech, what I’ve typed or written out or what I “remember” as reality.

If I do somehow manage to actually keeping my eyes open, I may not move or respond to external stimuli, not because I’m unaware of my surroundings, but because I am actually so tired that I cannot react to it.

Prior to starting Modafinil, sleep attacks occurred at least once daily but often multiple times a day. It didn’t matter if I was in the middle of a mundane activity or whether I was active in a stimulating environment; I’ve fallen asleep in important leadership meetings, in the middle of conference calls, in an interview with a principal, standing in line at Canada’s Wonderland and London’s Sealife Aquarium and midway through a song at choir practice. (I woke up still singing). The pull to sleep is always stronger than my ability to fight it off and always results in me nodding off, slurring my speech if I attempt to continue talking normally, or falling over backyards when standing, with only the jolt from tipping backwards waking me up.

Although Modafinil has given me the ability to fight off the vast majority of sleep attacks, it is important to note that it does not provide full relief. Even on the highest recommended dosage, I am still sleepy, physically fatigued and not fully alert for much of the day. However, in the weeks leading up to my medical leave and continuing until now, I have noticed that the fatigue is creeping back towards pre-medication levels and have found myself increasingly unable to fight off the sleep attacks (even now that I’m well-rested. For example, despite a full night’s rest and full Modafinil dosage, the sleepiness was so extreme this weekend that I unintentionally fell asleep in the cars at the Autoshow.)

The extent of my sleepiness may be better grasped if compared to a permanently insatiable hunger. You could eat for hours at a buffet only to feel peckish by the time you’ve paid and sharp hunger pangs by the time you reach your car.

Awakening “naturally” (i.e. without setting an alarm) means my body will typically wake up after 9-12 hours, but will leave me feeling as refreshed as the insatiable would feel satisfied after consuming a measly granola bar. Despite the desperate drive to sleep all day, giving in to sleep does not satisfy the need – it merely dulls the sharp hunger pangs for a fleeting moment before they restart. Even on medication, I may need two to three naps a day, sometimes even more.

Automatic Behaviour

This extreme fatigue often results in automatic behaviour, defined as episodes of “purposeful but sometimes inappropriate behaviour occurring during periods of sleepiness, usually with partial or absent recollection of the activity”. The spontaneous production of verbal or motor behaviour without conscious self-control or self-awareness is the reason behind the increasing occurrences of me misplacing items with no memory of doing so. The longer it’s been since a nap, the more frequently automatic behaviour will occur[19]. During periods of extreme fatigue such as when trying to fight a sleep attack, episodes of automatic behaviour always ensues, which have recently included:

  • Trying to fight a sleep attack while writing a list at work, struggling to remain conscious and continue writing but slipping in and out sleep, dreaming of my brother in between periods of consciousness and waking up to find his name written all over my paper
  • Trying to fight a sleep attack while in the middle of sending out emails and having to check my sent box at the end of the episode to determine what, if anything, I had sent out
  • Being fully consumed mentally in fighting off the fatigue while driving home from work and discovering I had no idea where I was when the “fog” lifted
  • Having a sleep attack in the middle of typing, fading in and out of dreams and waking up to see that I had typed out my dream interspersed with the original content of the message, full of typos
  • Conducting regular conversation while fatigued often results in a confusion upon “awakening” around whether the conversation was a dream, if it had actually occurred or if parts of my “memory” were actually dreams I had simultaneously. I constantly have to ask if events or conversations actually happened or if I had just dreamed that they had
  • Misplacing and losing many items (which has only begun within the last 6 months – I had previously been very prudent and not prone to misplacing items) including regularly being unable to find my keys, locking myself out without my keys, losing my security pass (which I’ve had since 2008), losing my credit card (which has never before happened), etc.

Hallucinations and Sleep Paralysis

Narcoleptics often have difficulty in keeping the various compartments of sleep separate (dreaming sleep vs. non-dreaming sleep vs. awake). However, these distinct boundaries are not maintained for narcoleptics, and sleep regularly encroaches on awake territory, which results in dreaming while awake, uncertainty of whether you were just awake or asleep, confusing reality with dreams and hypnopompic hallucinations[20].

Typically associated with narcolepsy, hypnopompic hallucinations and sleep paralysis occur exclusively during daytime naps for me. Since carpooling to work allows me to sleep ~1 hour each way, if I am unable to wake up upon arriving at work, I’ll continue napping in the work parking lot, where I’ve recently experienced several episodes.

Hypnopompic hallucinations are brief, “dreamlike episodes” occurring upon waking that are vivid and often distressing. This coincides with sleep paralysis, where muscle atonia occurs between sleep and wakefulness instead of the REM sleep cycle. Episodes are typically “extremely frightening” and have an underlying theme of danger and an impending sense of doom, but despite urgently needing to move, I am physically unable to do so25.

During these episodes, I’m fighting to regain and retain consciousness but fade in and out of vivid dreams. Every ounce of will and energy is concentrated on trying to force my eyes to open or my hand to move; the thinking always seems to be that if I could move just one part of my body, I could end the paralysis.

I may even dream I’ve succeeded in moving only to realize in frustration that was just a dream. Most recently, I’ve heard passersby making disturbing comments (two males walking by saying, “Oh look, she’s still sleeping!” indicating that they’ve been watching me) or seen frightening things (like a sign saying that I was being watched and a camera pointing right into the car) or heard approaching footsteps when I should be home alone.

Despite the urgency I feel to wake up, no matter how hard I will myself to move, I am unable to. There have been times that I’ve awoken to find that the frightening occurrences were distorted elements of reality (we had parked in front of a sign with a picture of a camera that said the parking lot was monitored but there were no cameras pointing directly into the car) but there are other times that I am unable to distinguish between what I actually heard while asleep and what was actually just a dream (were there actually two males walking by who commented on me still being asleep? Did I really hear footsteps or was I actually home alone?)

Effects of Narcolepsy

Narcolepsy is evidently and unarguably a “very disabling illness” with “devastating effects”[21]. Even treated individuals sustain severe emotional and social dysfunction in all areas of life including school, work, relationships and leisure activities.

Studies show that narcoleptics experience challenges with memory, thinking and attention and experience significantly higher rates of depression, ranging from 30-57% compared to 8% in the general population.

Many experts have even concluded that the psychological and social effects are even more severe than those caused by epilepsy[22].

62% of narcoleptics feel that their condition has reduced job performance, 53% attribute narcolepsy as the cause of worry or threat of losing a job and 38% have been impacted by a decreased earning capacity[23].

Related: 

Resources:

[1] Claudio Bassetti and Michael S. Aldrich, “Idiopathic hypersomnia: A series of 42 patients,” Brain 120 (1997): 1423 – 1435.

[2] Right Diagnosis, Prevalence and Incidence of Narcolepsy, http://www.rightdiagnosis.com.

[3] Bassetti and Aldrich, “Idiopathic hypersomnia,” 1429.

[4] Bassetti and Aldrich, “Idiopathic hypersomnia,” 1430.

[5] Dr. Adam Moscovitch, doctor’s note, November 30, 2012.

[6] Timothy F. Hoban and Ronald D. Chervin, “Hypersomnia and Narcolepsy,” in Handbook of Sleep Medicine, ed. Alon Y. Avidan and Phyllis C. Zee (Philadelphia: Lippincott Williams & Wilkins, 2006), 70.

[7] Julie Flygare, “Sleep’s Choice: Living with Narcolepsy”, in National Sleep Foundation, 2012.

[8] What is Narcolepsy, everythingnarcolepsy.com

[9] Timothy F. Hoban and Ronald D. Chervin, “Hypersomnia and Narcolepsy,” in Handbook of Sleep Medicine, ed. Alon Y. Avidan and Phyllis C. Zee (Philadelphia: Lippincott Williams & Wilkins, 2006), 70.

[10] Sudhansu Chokroverty, “100 Questions & Answers about Sleep and Sleep Disorders – Second Edition” (Massachussetts: Jones and Bartlett Publishers, 2007), 110.

[11] Extreme Daytime Sleepiness, National Heart, Lung and Blood Institute, www.nhlbi.nih.gov

[12] Claudio Bassetti and Michael S. Aldrich, “Idiopathic Hypersomnia”, 1525.

[13] Timothy F. Hoban and Ronald D. Chervin, “Hypersomnia and Narcolepsy,” in Handbook of Sleep Medicine, ed. Alon Y. Avidan and Phyllis C. Zee (Philadelphia: Lippincott Williams & Wilkins, 2006), 70.

[14] “Narcolepsy and Hypersomnias (Excessive Sleep)”, Stanford Hopistal & Clinics, http://stanfordhospital.org/clinicsmedServices/clinics/sleep/sleep_disorders/narcolepsy.html.

[15] Ontario Ministry of Transportation, http://www.mto.gov.on.ca/.

[16] Office of Alcohol and Drug Education, http://oade.nd.edu/educate-yourself-alcohol/what-is-intoxication/.

[17] Timothy F. Hoban and Ronald D. Chervin, “Hypersomnia and Narcolepsy,” in Handbook of Sleep Medicine, ed. Alon Y. Avidan and Phyllis C. Zee (Philadelphia: Lippincott Williams & Wilkins, 2006), 70.

[18] Timothy F. Hoban and Ronald D. Chervin, “Hypersomnia and Narcolepsy,” in Handbook of Sleep Medicine, ed. Alon Y. Avidan and Phyllis C. Zee (Philadelphia: Lippincott Williams & Wilkins, 2006), 70.

[19] What is Narcolepsy, http://everythingnarcolepsy.com/What_is_Narcolepsy_ZFQ9.html

[20] Common Sleep Disorders, Winthrop University Hospital, https://www.winthrop.org/.

[21] About Narcolepsy, Stanford School of Medicine, med.stanford.edu

[22] “Narcolepsy”, University of Maryland Medical Centre, http://umm.edu/.

[23] Education/employment, Narcolepsy UK, www.narcolepsy.org.uk

Mrs. Unchained 55

10 Comments

  1. That’s terrifying! And awful you had to endure even more stress just advocating for yourself to get your disability leave approved. I hope that this was a one-time thing for you and that it doesn’t recur!

    • Yeah, I’d have to say it was quite the ordeal! Thankfully now that I’m in a much better situation with much more tolerable levels of stress, the symptoms are far from being as extreme as I described above. And yes, hopefully that was a one time thing – that period of my life is an utter blur (despite my wedding being part of that time). I was so tired and stressed, I don’t remember much of it at all! Thankfully, I had documented that so I could go back and reread what had happened. It actually shocked me when I reread it because I had forgotten just how bad it had been, but am SO grateful the severity of my symptoms are now nowhere near it!

  2. Would it be ok to share this, ive recently been diagnosed and explaining this to someone who doesnt understa d is difficult..im si new to this but this is me to a t every single day for almost 2 years. ♡

  3. I will have to say, I have been trying to explain this feeling to friends and family for years! You have explained how I feel better than I ever could! Thank you. I was diagnosed with this disorder about 2 years ago, but I’ve had it as long as I can remember. The medication helps for the most part but I’ve had to up my dose 4 times in the last year and a half. Even on the high dose I am on now, I can lay down and go back to sleep. It sounds like you are feeling better now. (maybe not 100%) Can I ask how? What did you do?

  4. Thank you so much for sharing, my husband was recently diagnosed with IH and I’m trying to learn how to help him (and help protect his job) 🙂

  5. I hope he finds something that works for him. I don’t have a lot off issues with my employer, but my job keeps me extremely busy and that really helps. I don’t have time to think about being tired. But as soon. As I quit moving, I’m done for the day. Whether that’s 10am or 10pm. My meds have helped a lot, but not 100%. Good luck.

  6. You definitly explained the symptoms quite well. I also was diagnosed with IH and after being treated for a year by the doctor that diagnosed me, when I told him that the provigil was not working and my job wouldnt let me work because I kept falling asleep he told me I had depression. Iwas then refered to another physician who put me on disability and changed my meds to amphetamines they to helped for a short while. After about 6 months another sleep study, doctor tells me I have IH pretty bad . After loosing my job and being on long term disability about a year and a half my doctor decides I no longer am disabled and refuses to fill out paper work. Im sorry I just dont understand I havent had any changes in fact it has gotten worse even with meds. His office claims if I dont like his decission find another doctor. Im so frustrated and upset . Am I the only one dealing with such issues? I wish someone would tell me Im not the only one fighting this battle.

    • I’m so sorry you’re battling this. I wish IH was better understood at least by medical professionals! There are some Facebook support groups if you haven’t joined them already – lots of us on there!

Leave a Reply

Your email address will not be published. Required fields are marked *

CommentLuv badge