Considerations for Professionals
Working With People Who Have Hypersomnias
“Having idiopathic hypersomnia means my brain is almost always too foggy to function properly. My brain feels painfully broken from lack of sleep, no matter how much I sleep.”
In response to concerns voiced in the patient expert community over time, the Hypersomnia Foundation has prepared a series of guides informed by focus groups.* These guides are intended for professionals and their team members who interact or collaborate with person(s) with hypersomnias (PWH), particularly in the following communities: research, pharmaceutical, industry, sleep study centers, etc. These guides augment each other and provide information to assist in understanding, as well as communicating and working effectively with PWH. Including PWH and/or patient advocacy groups in discussions of these guides can also further this understanding.
It is important to be informed about the various hypersomnias and the evolving research in this arena. PWH and their supporters report that receiving an accurate diagnosis can be a years-long process within health care systems, often filled with misdiagnoses, and the seriousness and nature of their symptoms are often discounted. Symptoms may vary among PWH, especially between those diagnosed with narcolepsy type 1 (NT1) and those diagnosed with idiopathic hypersomnia (IH) or narcolepsy type 2 (NT2). Chronic sleepiness and the associated cognitive challenges (such as deficits in memory, attention, and concentration) are most common across all the hypersomnias. Cataplexy is also common, but it is specific only to NT1. Some PWH also experience severe sleep inertia (i.e., sleep drunkenness), sleep paralysis, hallucinations when going in and out of sleep (hypnagogic/hypnopompic), automatic behaviors, autonomic symptoms, etc. (see IH Summary, About IH, About Related Disorders, Classification of Hypersomnias, and the following patient videos).
PWH have unique needs. For example, their impaired wakefulness/cognition, which may be severe enough to substantially limit their ability to engage in routine activities, is often not apparent to others (i.e., “invisible illness”) and can also rapidly/suddenly change. As the need for sleep builds up (or during periods of sleep inertia), confusion, irritability, emotional lability, etc. can quickly increase dramatically, similar to delirium and necessitating support similar to that usually provided for this condition. Understanding the PWH’s sleep schedule/needs and additional symptoms can be extremely important for the accuracy of sleep and other testing. Given PWH symptoms, telehealth is often preferable when clinically feasible (although PWH may have additional struggles with technology due to their cognitive and sleep symptoms), and specific communication formats, such as succinct wording, can be very helpful.
Guide for Sleep Study Centers and Research Study Sites
The intent of this guide (which includes research study sites that don’t perform sleep studies) is to provide suggestions to address the unique needs of PWH and thereby improve site safety, quality of data collection, etc. For example, PWH may need discharge planning after sleep studies or even after long appointments, similar to that for a procedure under anesthesia. Given that hypersomnias are rarely diagnosed, teams at both sleep study centers and research study sites may be unfamiliar with the details of these diagnoses. Additionally, centers/sites may not be managed by sleep medicine specialists. For providers who are already familiar with hypersomnias, this content provides a helpful review and can also be used to help train team members.
Guide for Researchers
This guide’s purpose is to help researchers effectively recruit and retain participants for their studies, improve study design and validity of results, etc. A concomitant goal is to help reduce the burdens experienced by PWH when they choose to participate in research studies, by 1) ensuring researchers and all team members appreciate the full range of PWH hardships, including financial stressors, that may be encountered during these studies and 2) providing suggestions to address them. For example, open label extensions of clinical trials can be very helpful for PWH, since it can take many years for new medicines to get to market and even then access can be limited.
Guide to the Design of Survey Studies
Person(s) with IH (PWIH) have described facing challenges when involved in studies that include taking surveys. This guide was developed to provide considerations to inform the design of the survey tool based on information about the unique characteristics of PWIH. The goal is to improve patient recruitment, effective engagement, data quality, etc.
* The Considerations for Professionals Guides were developed by the Hypersomnia Foundation and approved by its Medical Advisory Board, Board of Directors, and Patient Advisory and Advocacy Council (PAAC). They are based on suggestions from focus groups of patient-experts; the focus groups were conducted in accordance with standard qualitative methods of research practice. It is important to note that the considerations presented do not necessarily apply equally to all PWH. Some focus groups consisted of both PWIH and people with NT2, while others consisted of PWIH alone (these details are noted within the individual guides). These considerations may also apply to people who have other hypersomnias, e.g., NT1 and Kleine-Levin syndrome (KLS), as well as those yet-to-be-diagnosed with a hypersomnia.