My son’s greatest challenge every day is waking up. Kleine Levin Syndrome (KLS) changed my family’s life. Kleine Levin Syndrome is a poorly understood disease that’s marked by recurring episodic symptoms of hypersomnia, derealization and mood changes that are not yet fully explained. What that really means for our family is that I’ve been speaking to doctors about the possibility of KLS, progressively moving towards a diagnosis for my son since late 2016 and speaking to everyone I could, starting with the psychologist who diagnosed my son’s ADHD and Asperger’s syndrome and my son’s former pediatrician. Sometimes I spoke to complete strangers if I discover they have a link to treatment or information. Battling KLS for the past six years looks like this.
My wife, Barbara and I took our son to an ER where they had never seen the disorder or even heard of it. From the local hospital, we went to Shands, the teaching hospital for the University of Florida, where we finally received the diagnosis but no help. Five months of searching and intensive research of my own after the diagnosis, finally brought our family to the amazing Dr. Bobbi Hopkins at Johns Hopkins All Children’s Hospital, where we finally got referred to Emory University Sleep Center where we spent two days speaking with Dr David Rye, one of the top researchers looking at hypersomnia and Kleine Levin Syndrome. Once our son was seen at the research and teaching hospitals, everyone agreed that KLS was something real and we were really dealing with it, but no one knows what causes it or how to stop it.
Instead, we walked away with advice (some markedly better than others), metaphors and sometimes medications which may show some level of promise. All the supposed insight builds upon supposition and educated guesses.
KLS affects only 1 or 2 of every million people. It often gets misdiagnosed as mental illness or dismissed as adolescent behavior. Fewer than 1000 patients (only about 600 according to one study I read) worldwide have this diagnosis. The practical result is that there isn’t a large enough sample for any substantive science or drug testing. About the only thing doctors who know KLS agree on is what it looks like. So, it looks like this:
When I woke on November 7, 2014, nothing tipped me off that my life had changed forever. The day presented a mounting dilemma. Avner couldn’t wake up. It had taken some months, but my son and I finally found our niche in Boy Scout Troop 115 and this weekend promised some remarkable memories. The troop was camping at Pensacola Naval Air Station for the Blue Angels homecoming show and I couldn’t wake my son. Did he catch an ill timed infection on a camping weekend? I didn’t know but he didn’t seem warm, he just didn’t wake up. I called the pediatrician and the only advice they gave was that if I was worried, I should take my son to the ER. In hindsight, it was advice worth taking but after all, he was just sleeping. He wasn’t running a fever at the time, he was just peacefully sleeping the day away.
We used to joke that Avner was pathologically healthy. One time, every kid in his preschool except for Avner and one other kid had developed strep, even all the teachers. Nieces and nephews seemed to constantly have late night trips to the ER, but not our kid. So, why should I worry about some extra sleep. Maybe, as he had done before, he was reading into the late hours and never slept during the night. We’d have to talk about that.
By about 2:00 pm, I called Crill, our scoutmaster to tell him that Avner can’t wake up, but we’ll try to find the troop on Saturday morning at the air show. Thankfully, some new mobile technology made that possible with the large crowd that was expected.
I called my friend Barbara, a pediatrician who now practices law. She raised the issue of hydration. To our knowledge, Avner hadn’t had anything to drink or eat since the previous night’s dinner. She also suggested the hospital if I was worried, but since he seemed to be sleeping “normally” except for the duration and refusal to wake, I held off for a couple years.
About 4:30 that afternoon, he woke up and seemed perfectly fine. I had some words with him about staying up too late reading which received the expected “but I wasn’t.” Sure!
The next morning we drove the three hours to Pensacola NAS. Thankfully we gained an hour and we managed an early start while Avner slept in the car. We had a nice breakfast at Waffle House, grabbed some earplugs and with the miracle of mobile technology, located our scout troop among hundreds or thousands of Boy Scouts. The air show was wonderful. The headliners and hometown heroes, The Blue Angels really were amazing to watch. By the time we got to the air station museum, we were all exhausted from being on our feet all day cheering and being wowed with aerobatic feat after aerobatic feat.
We hiked back to our vehicles and set out for CiCi’s pizza buffet, because that’s how you feed a troop of teenage boys who don’t have the space at their campsite to cook. Halfway to the restaurant, I notice my missing wedding band. I had recently taken up triathlon as a hobby and lost over 30 pounds. In the course of the preceding months before the air show, the wedding band became progressively looser. I was in a panic. I was driving my own car because I had to find the troop, on account of our late start, and when I turned the wheel to make a u-turn, I first noticed the band was missing. I caught up with the church bus that carried our the troop and while they looked at an engine problem, I dug around every corner of car, hoping to find the ring.
My wife and I have matching custom mokume gane bands cut from the same billet and losing that band seemed to be my biggest problem. It was nowhere to be found. I called my wife and told her the bad news. I promised to retrace my steps in the morning. Fortunately, I had my own car and didn’t need to go with the bus. The talk among the adult leaders was respectful but I took some well meaning ribbing about how wedding bands generally get tighter, not looser with age. I suggested they take up my training program to see if the same wouldn’t happen to them. Erica, who runs half marathons gave me no pass on the weight loss.
My son and I both have ADHD. It’s not unusual to be a little groggy in the morning before the usual stimulant medications. Since the medication affected Avner’s appetite, I let him sleep in the car as we retraced our steps from the previous day. The ring was nowhere in the field where I had parked my car, or on the way back to the museum. I knew I had it in the museum because the ring had nearly slipped off my hand when I left the restroom after I washed my hands.
A crowd was gathered near the entrance that morning, before they opened and I pressed myself against the glass door to see if I could flag the attention of someone inside. At one point I opened a drawing app on my phone and wrote in very large letters “HELP ME!!” I was ignored until the doors opened and then a guard walked up to me.
“Did you lose a wedding ring?” I hadn’t been ignored. I just wasn’t getting any special treatment, even if I was wearing a Boy Scout uniform. I was directed to the lost and found office in the back of the museum. I described my wedding band and was told that it was found just after we left on Saturday, next to the chair I had been sitting in. Apparently, it slipped off my hand as I sat there tired from the day’s activity.
Wedding band returned to my hand, I drove to the nearest Waffle House with Avner for a filling breakfast. I gave Avner his Concerta, a longer acting form of the same medication in Ritalin, and looked forward to a nice ride back to Tallahassee, talking about the weekend’s events. I fully expected Avner to be fully awake and engaged within a few minutes. Instead, he slept for the entire ride home. When we got home, he immediately went upstairs to his room and crawled back in bed sleeping again until about 4:30 in the afternoon.
As the smallest scout in the troop, Avner’s height worried us. Enough media buzz surrounds ADHD medications and their side effects, including sleeplessness, weight loss and growth inhibition that we had been trying new medications, to address appetite and insomnia. Intuniv was supposed to encourage appetite and help with sleep. It could cause daytime sleepiness. Avner had stepped up from the minimum dose to the maximum without incident. After tolerating the medication so well, the sleep was unexpected but not alarming. So these episodes of hypersomnia were brushed off a medication side effect. We stopped giving Avner Intuniv and within a couple weeks the sleep seemed to return to normal for about a year.
Some mornings were challenging, but adolescents sleep more, right? In the mean time, Avner turned into a really amazing young man. The painful elementary school choral performances finally rewarded us with genuinely enjoyable middle school concerts. In spite of an autism spectrum diagnosis, Avner had a really cool cluster of friends in chorus and scouts. He volunteered for projects that made my wife, Barbara and I roll our eyes, because of his procrastination, but somehow, he usually managed great results, sometimes requiring divine intervention or unplanned parental support, but the work was always his. He was pushing the limits of his International Baccalaureate Prep program and keeping his head above water, thanks to his Individualized Education Plan (IEP) which provided for both his ADHD/Autism Spectrum and gifted status.
As we expected, he had some trouble with writing assignments, but his math and science scores usually made up for any written shortcomings. On the county Algebra assessment, he scored a five of a possible five. He argued with the science teacher about chemistry and physics concepts that textbooks glossed over. He had to live with simplified answers until he could get to advanced high school classes.
We visited Kennedy Space Center for the Grail probe launch to Jupiter and Avner became a topic of Internet chatter when someone tweeted a picture of him taking Dr Neil DeGrasse Tyson to task about the number of stars in the observable universe. Why? Because Dr Tyson gave a rough estimate of the number of stars and Avner disagreed. “I do this for a living” said Dr Tyson, “why do you think I’m wrong?” Without hesitation, Avner answered “because new stars are being born all the time.” Dr. Tyson signed Avner’s VIP pass for the launch, “Please excuse Avner from class today. He needed his space. Neil DeGrasse Tyson.”
In eighth grade something changed. In retrospect, the change seems sudden, but like a frog in a slowly warming pan, we barely noticed at the time. Mornings became more fraught with fights about waking on time. I had resorted to any means possible to get him going, some became extreme. He became rude and secretive. Teen years were becoming a challenge beyond expectation. Our very bright student started to fall behind in school. Grades slipped and he didn’t seem to care. A major assignment didn’t get done. Instead of taking time to spend with his friends during spring break, he stayed all day in a corner pretending to work on a the missing history project.
When we discovered the subterfuge, Barbara and I became livid. Privileges were suspended, he had no access to games or tv for months and nothing improved. We had discovered that he had found his way around parental controls. He openly defied us. It became nearly impossible to wake him for school.
Somehow, he finished middle school with passing grades. He earned the highest possible end of course score for high school algebra and near top scores for his other academic courses.
It’s a well written piece on what must be a difficult subject to write about. Maybe the writing is somehow therapeutic, getting it all out into the open and thought organized. I have hypersomnia and without my medication, I will sleep all day, waking up only to eat my meals. I remember the time before my diagnosis, when I couldn’t wait for the weekend so I could sleep the weekend away. I can only hope that you find a treatment that works. I know the frustration a life of sleep brings.
Thank you for your kind words. I’ve recently started putting some approximate dates on more of the posts. This is a memoir and it’s written in hindsight. Not too much of a spoiler here, but my son started college a few months ago. I’m so proud of him. He’s still struggling with sleep issues, especially with the coronavirus isolation measures, but he has a medication which has helped a lot. The worst of the KLS symptoms haven’t been seen for a long while, and we hope they are mitigated with the medication. My goal here is to raise awareness of KLS/hypersomnia. Until I happened in our own family, we never even imagined that these issues existed.
This format allows me to draw in a larger audience because it will allow me to address topics that are more broadly shared, including FAPE, IEP, raising a special needs child, and of course, hypersomnia related issues. I’m hoping that I can build an audience with this broader appeal and educate the public about these sleep issues.
I probably don’t need to tell you, that when I mention that my son has a sleep disorder, most people automatically assume that he has insomnia or sleep apnea of some kind. When they learn about our reality, they are frequently shocked and don’t even know what to ask next. This is my attempt to stem that reaction.
I’ve been working in media since the late 1980s, so writing has been a big part of my life. It’s certainly rewarding to write on this subject, but a writing instructor told me a long time ago that good writing is not therapy, and I hope to adhere to that wisdom.
Again, thank you for taking the time to read what I’ve written. Please invite others you know to read along as I tell our story. IF you can, support my Patreon. Most importantly, stay safe and be well. I understand the havoc that hypersomnia can have on a person’s life. If this blog/memoir helps one more person, then my efforts have been successful.
How do they diagnose KLS? My children and I have been diagnosed with Narcolepsy without cataplexy. Unfortunately medication for the Narcolepsy isn’t working very well for any of us and we are at a loss. My 16 son sounds just like your son as does my 19 year old daughter. We are curious if it could possibly be more than just narcolepsy. Thanks for writing about this and it gives me more to look into. We know how rare this is because we also have an x linked genetic disorder called Hyper IgM and it’s also a 1 in a million occurrence.
Firstly, I’m so happy that you found this resource helpful. As you know, comorbidities are common in people with rare and chronic disorders. Only a specialist can say if that’s the case with your children, but I know of another young man, finishing his Eagle Scout project who has both the KLS and narcolepsy with cataplexy diagnoses. According to Dr. David Rye of Emory University, there may be more in common between different hypersomnia classifications than differences. There’s a great video of a speech he gave on this topic at the hypersomnia foundation website.https://www.hypersomniafoundation.org/whats-in-a-name-understanding-the-origins-of-the-terminologies-for-the-family-of-hypersomnias/
Now to directly address your question, KLS is diagnosed by the process of elimination. If you pursue this avenue, be prepared for more tests. If you already have a Narcolepsy with catatplexy diagnosis, it may be challenging to have another sleep study to look for another sleep disorder. Historically, patients with KLS have normal or at least mostly normal sleep studies. If follow this blog in the next few weeks, I will be starting that part of the story. I have a few more personal adventures, but not many. I promise.
In my son’s case, he was scanned by CT and MRI, had various blood tests, a sleep study and finally a psychiatric evaluation between February and April of 2017. I know, as a parent, all we want are answers about why our kids struggle with these issues. I can say as someone who got an answer, that it was like getting off a roller coaster and being left at the edge of an abyss.
Let me know if you would like contact with the family who has a child with both KLS and narcolepsy. I may be able to arrange that.