Experts share tips on how to provide comprehensive narcolepsy treatment for children using an interdisciplinary care approach.
By Risa Kerslake, RN, BSN
The psychosocial effects of pediatric narcolepsy can have far-reaching consequences that are sometimes overlooked by sleep specialists. When sleep physicians are focusing on the pentad symptoms of narcolepsy, it’s easy for them to underestimate the negative psychological and social issues that can arise in this patient population.
When narcolepsy is diagnosed, it’s usually after years of symptoms that haven’t been identified or treated. That’s time lost, says Jose Colon, MD, who is board certified in sleep medicine, child neurology, and lifestyle medicine. “We’re taught as physicians what medications we can use to treat, but the reality is that medications don’t reverse this disorder, so it’s important to take a look at all aspects,” says Colon.
Children who have narcolepsy can have more academic difficulty, trouble forming friendships, and challenges maintaining relationships with their peers, says Shalini Paruthi, MD, co-director of the Sleep Medicine and Research Center at St. Luke’s Hospital in Chesterfield, Mo. The sooner a child is diagnosed, the sooner they and their family can understand how and why narcolepsy symptoms happen and how to respond.
To increase awareness of the psychosocial challenges faced in the pediatric population, David Ingram, MD, a pediatric sleep physician at Children’s Mercy Kansas City, coauthored a paper about common challenges of living with narcolepsy as reported by children with the disorder, their parents, and sleep physicians.1
According to Ingram’s report, which featured surveyed responses from 116 parents and 35 youth, the top five most common psychosocial challenges reported by youth participants were difficulty focusing, worry about the future, schoolwork, getting easily upset, and diet/nutrition. However, when asked how often these challenges come up at patient appointments, less than a third of the 30 surveyed physicians rated these concerns as “frequently” encountered.
This could indicate a disconnect between the clinicians’ perception of their patients’ experiences and what their patients’ day-to-day struggles actually look like.
Psychosocial Challenges of Pediatric Narcolepsy
Mental health issues are also rampant in this patient population, including low self-esteem, feelings of isolation, anxiety, and depression, says Kevin Smith, PhD, a pediatric psychologist and colleague of Ingram at Children’s Mercy Kansas City.
Children who experience cataplexy, a distinct feature of narcolepsy characterized by a sudden loss of muscle control often triggered by strong emotion, might avoid situations where they could encounter a heightened emotional response.They might avoid going to a scary movie with their friends or playing sports due to concerns about having a cataplectic event, says Smith.
Around 33% of people with narcolepsy also have symptoms of ADHD.2 “The cause of the association is not clear, but recent evidence suggests that sleep disorders or fragmented sleep may predispose individuals to develop ADHD,” says Smith.
Directly impacting timely diagnosis and treatment is the level of education physicians receive in medical school and residency programs. “When I went to medical school, I received one lecture on sleep apnea by a pulmonologist and one lecture on sleep medications by a psychiatrist,” says Colon. In his pediatric residency, he never received one lecture about sleep.
It takes up to 15 years on average to get a narcolepsy diagnosis, and this delay can contribute to psychosocial challenges.3 “Children and families are experiencing struggles on a daily basis and are understandably wanting a clear diagnosis and treatment course as soon as possible. It’s a frustrating process, and it’s very easy to make a mistake, even for experienced sleep providers,” says Ingram. Physicians also want to provide relief from debilitating symptoms but need to balance that with a thorough evaluation before labeling someone with a chronic neurological condition.
Interdisciplinary Approach to Address Challenges
A child’s pediatrician is a key component to care, as they’re often the first stop for health concerns. Sleep specialists can work in conjunction with primary care clinicians and maintain open communication. Primary care physicians should also have an awareness of narcolepsy so they can make appropriate and timely referrals, says Colon.
Ideally, team members involved in care would include a sleep physician, psychologist, social worker, and dietician. “A psychologist knowledgeable in pediatric sleep disorders can evaluate for reasonable adjustment to a diagnosis of narcolepsy by the child and parents. They can also be helpful if the child is having difficulty adhering to treatment medications,” says Paruthi. Social workers can assist families with financial resources, crisis management, and help with basic needs.
Nutritional needs can also be overlooked in narcolepsy care management, but patients can benefit from a dietician referral. Patients are at risk for obesity in part from a sedentary lifestyle due to sleepiness. Given that medication alone doesn’t always work to effectively manage symptoms,1 many patients implement nonpharmacologic therapies to control symptoms. A study published in Neurology reported a low glycemic diet showed positive results in energy levels of those with narcolepsy.4 Colon says some of the adults he sees report better symptom management while on a mild ketogenic diet.
However, having these specialties all available together isn’t usually feasible. In Ingram’s narcolepsy clinic, he partners with Smith at Children’s Mercy to see patients together, along with a nurse and social worker available as needed.
It’s also important to communicate with the family when a diagnosis is made. In his practice, Colon requests that all family members attend the follow-up appointment. “I literally hold a family conference, and I discuss the sleep and REM cycles and how narcolepsy is not just a disorder of sleepiness but a disorder of REM intrusion,” he says.
Family members can have a difficult time with a narcolepsy diagnosis because they aren’t seeing any physical abnormalities. Giving a comprehensive explanation to those caring for the child is a first step of the treatment plan.
To help families think about and identify any significant challenges, whether medical or psychosocial, Ingram and his colleagues constructed a one-page pediatric narcolepsy pre-visit worksheet that families can complete before their appointment. The worksheet (figure 4 in the paper published in the Journal of Clinical Sleep Medicine1), based on Ingram’s research, allows clinicians to focus on the most distressing problems during their time with the patient and family.
Additionally, it’s crucial to involve the child’s school in understanding challenges that the child is likely to encounter in the classroom.
Advocacy groups such as Wake Up Narcolepsy and the Hypersomnia Foundation can provide a wealth of information and support, whether with resources presented through webinars, website information, or support groups. Gaining more knowledge about narcolepsy can help patients and their families know what to ask the care team.
“As with most chronic disorders, frequent follow-up is helpful, especially as the child and parent are learning more about narcolepsy and getting used to treatment,” says Paruthi. These visits might decrease once the child is doing well and has a good treatment plan with better daytime alertness. Prior to each visit, the child or caregiver can write down a few questions they really want to be answered. She says, “They may find it better at first to be seen more often until all of their questions are answered and they have a better understanding of their symptoms and treatment options.”
Risa Kerslake, RN, BSN, has a background in public health, case management, and mental health. This is her first article for Sleep Review.
1. Ingram DG, Jesteadt L, Crisp C, Simon SL. Perceived challenges in pediatric narcolepsy: a survey of parents, youth, and sleep physicians. J Clin Sleep Med. 2021 Jan 1;17(1):13-22.
2. Kim J, Lee GH, Sung SM, et al. Prevalence of attention deficit hyperactivity disorder symptoms in narcolepsy: a systematic review. Sleep Med. 2020 Jan;65:84-8.
3. Thorpy MJ, Krieger AC. Delayed diagnosis of narcolepsy: characterization and impact. Sleep Med. 2014 May;15(5):502-7.
4. Husain AM, Yancy WS, Carwile ST, et al. Diet therapy for narcolepsy. Neurology. 2004 Jun;62 (12):2300-02.