Where we’re going, we don’t need Roads

At the age of 4, Dylan has already been on 6 different types of prescription medications.

At 7, he will have spent more of his life medicated than unmedicated.

At his bar mitzvah, he will celebrate becoming a man and a decade of drug dependency.

At the age of 18, fifteen years post diagnosis, Dylan will finally be old enough to take Xyrem, the most potent and effective narcolepsy medication currently available.

At 23, Dylan will have been heavily medicated for two decades.

If a cure for narcolepsy is not discovered, Dylan will spend just three years out of his entire life unmedicated. Great Scott.

What are the long term effects of being on this cocktail of medications for 20 years? 40 years? 60 years? No one knows. How long will the medications be effective before Dylan acquires a drug tolerance? Another medical mystery. If only we had a flux capacitor and a DeLorean. There are certainly a number of unique challenges when a young child develops narcolepsy with cataplexy. Issues such as the life span of a drug’s effectiveness, the ability to swallow pills, adhering to a clean diet, and behaviour regulation are daily concerns because of Dylan’s short-lived existence. At four, it’s difficult for Dylan to fully comprehend the ramifications of his disorder and even more difficult for him to cope with the undesirable symptoms. Blaming the hardships on Dylan’s young age and operating under the perhaps misguided notion that life with a narcoleptic will be easier as he ages, I have been anxiously impatiently waiting for Dylan to grow up. “It will get easier when he’s older” has been my daily mantra. On particularly hard days, I remind myself that as Dylan matures, so too will his ability to cope with his disorder. But is that true? In my blinded haste to have Dylan grow up, I never stopped to think that what follows after childhood might be even more difficult.

When Dylan outgrows the challenges of dealing with narcolepsy as a child, he’ll entre the frightening world of narcolepsy as a teen. The combination of puberty and narcolepsy is strong evidence that either there is no god, or god has a twisted sense of humour. I tend to believe the former but if the latter is true, amen! “Acne, body odour, voice cracks, unwanted hair and general awkwardness isn’t enough” God declared, “let’s throw in an insatiable desire to sleep and random, ill-timed collapses to keep me amused“. Adolescence itself is arduous enough without having to manage a neurological disorder, a disorder that is inherently embarrassing and often leaves even confident adults self-conscious. Whether you’re a brain, an athlete, a basket case, a princess, a criminal or a narcoleptic, teenagers are bipolar atomic bombs of feelings. For teens fortunate enough to have an adequate amount of hypcoretin, these new found emotions, fueled by hormones and cannabis, can be overwhelming, exhilarating, scary, confusing, titillating, frustrating, and unsettling. For teens lacking hypocretin, these new found emotions can easily lead to cataplexy attacks because of the perplexing connection between strong emotions and loss of muscle tone. A large part of adolescence is spent navigating through the sea of novel emotions to make sense of them all. For narcoleptics who suffer from cataplexy, instead of experiencing and embracing all of these intense emotions they often spend their energy suppressing them to avoid a collapse. The thought of Dylan purposely trying not to laugh breaks my heart. At the moment, Dylan is too young to make the association between strong emotions and cataplexy attacks so his true feelings run free but what will happen when he’s older, possibly more self-conscious and hyper aware of the link? Will he choose a life void of emotions in an attempt to prevent any potential embarrassing falls or a life full of emotions with the constant threat of an impending, possibly dangerous, collapse? Both scenarios are tragic. Why do things have to be so heavy in the future?

At the age of four, Dylan is extremely limited in the medications he can safely use to treat his condition. Most of the drugs used in the management of narcolepsy and cataplexy are designed for adolescents and adults given the typical age of onset. In addition, even some of the approved medications are a non-option because they do not come in liquid form or cannot be crushed into a solution. As a result, Dylan’s symptoms probably aren’t being treated as best they could. When he’s older, there are a variety of options that will open up to him, including the infamous holy grail medication, Xyrem (a.k.a sodium oxybate, a.k.a. GHB, a.k.a the date rape drug). Previously, I waited with great anticipation for the day when he could finally enjoy a restful night’s sleep from this magical concoction. But while I was off daydreaming about all the positives of Xyrem, I overlooked the negative medical, emotional and social side effects of taking a Schedule III controlled substance. The list of side effects are long and scary ranging from shallow breathing to suicide. A particularly embarrassing side effect, is enuresis which is not uncommon amongst teenagers and adults. As a child, you can slap on some Pull-Ups and call it a night but as a young adult who may be camping with some friends or sharing a bed with his girlfriend enuresis may cause tremendous anxiety and embarrassment. He may even avoid these situations altogether, once again missing out on otherwise enjoyable activities because of his disorder.

The process of taking Xyrem also presents some challenges. Xyrem is taken at night to artificially induce deep sleep in narcoleptics who otherwise spend too much time in non-restorative REM sleep. Xyrem is a powerful drug that works almost immediately after it is ingested. In fact, it is recommended to take the first dose in bed because of how quickly it can take effect. Because Xyrem has a very short half-life, it only provides three to four hours of restorative sleep. In other words, three to four hours after the first dose has been taken, a second dose needs to follow. So assuming Dylan has to wake up at 6:30am in the morning to start his days, that means he will have to be in bed no later than 10:30pm to take his first dose of Xyrem. Can you imagine spending your teens and twenties with a 10:30pm curfew, let alone bedtime? What if he wants to take his girlfriend to dinner and a movie? What if he needs to pull an all nighter to prepare for a test or assignment? What if he has a late night hockey practice? At four, this simply isn’t a concern but as a young adult this may negatively impact his social life and academic success.

What really scares me when I think about Dylan growing older is the independence he is going to expect to receive. The thought of Dylan being unsupervised, even for a short amount of time, causes me great distress. As a child, watchful, caring, adult eyes are almost always on Dylan whether he’s at home, at school, at an extracurricular activity or at his grandparents’. If he falls asleep, someone is there to tuck him in. If he collapses, someone is there to catch him or pick him up. At four, his mercurial temperament and irascible behaviour are often excused, blamed on his age or dismissed entirely. But what will happen when I eventually have to unravel the protective Mommy bubble tape and let him out into the world by himself? What if he collapses on the walk to school? What if his friends mock him when he falls asleep in class? What if he doesn’t have any friends because of his anti-social behaviour that is no longer regarded as a cute personality quirk? I realize that letting go is difficult for every parent (more so now than ever before thanks to the awful soundtrack provided by Frozen), as it will be for me when I have to give Rylie her independence, but when it comes to Dylan, it’s as if there is this dark cloud of impending doom constantly hovering above him put there by narcolepsy.

When I was three months pregnant with Rylie, I remember telling my mom that since I made it through the first trimester I can finally stop worrying about her safety. My mom laughed at me and said “Lanna, you will worry about her for the rest of your life“. I suppose worrying about my cubs is my density.

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