Treatment

At this time, there is no U.S. FDA-approved treatment for IH. However, there are several treatments approved by the FDA for use in narcolepsy, and it is common practice to use wake-promoting medications that are known to be effective in patients with narcolepsy to treat the sleepiness associated with IH (this is called “off-label” use).

Most of these treatments for narcolepsy have not been studied to nearly the same extent in patients with idiopathic hypersomnia, and some patients with IH do not achieve adequate control of symptoms with these medications. These medications, in some cases, also may stop working over time and/or have bothersome side effects. These medications may sometimes be used in combination, especially in people who are treatment-resistant.

Researchers continue to test medications approved for other disorders, as well as novel treatments, for IH. In addition, some people with IH have found that behavioral approaches, including cognitive behavioral therapy (CBT), can be helpful for learning skills to cope with IH.

More information about treatments, including some Australian-specific information, can be found HERE.

More information about prescription medication affordability in the U.S. can be found HERE.

Medications FDA-Approved for Sleepiness

There are three major classes of medications approved for the treatment of sleepiness associated with narcolepsy: stimulant medications (i.e., derivatives of amphetamines), non-stimulant wake-promoting medications (e.g., modafinil, armodafinil, solriamfetol, and pitolisant), and sodium oxybate.

Stimulants approved for the treatment of sleepiness in narcolepsy include dextroamphetamine (e.g., Dexedrine) and methylphenidate (e.g., Ritalin). While stimulants may be effective, potential side effects include dependence, aggressive behavior, dental problems, and heart problems.

Non-stimulant wake-promoting medications include modafinil and armodafinil. While it is not completely known how these medications work, they appear to influence the brain chemistry that increases wakefulness, particularly the neurotransmitter dopamine. Modafinil has been studied in two placebo-controlled trials that included patients with IH and has been shown to help with sleepiness in patients with this disorder. However, modafinil and armodafinil can interact with hormonal birth control to make it less effective (learn more about “Women and Hypersomnia” HERE) and can result in a life-threatening rash. In addition, modafinil and armodafinil can sometimes result in headaches, which, if severe enough, can cause a user to reduce the dosage, thereby limiting the effectiveness of the medications.

A new non-stimulant wake-promoting medication – solriamfetol – was approved in March 2019 by the U.S. FDA. This new medication was approved for the treatment of sleepiness caused by narcolepsy and obstructive sleep apnea, and is available under the brand name Sunosi. It has different pharmacology than either the stimulants or modafinil/armodafinil (it is the first dual-acting dopamine and norepinephrine reuptake inhibitor approved to treat excessive daytime sleepiness in adults).

Another new non-stimulant wake-promoting medication – pitolisant – was approved in August 2019 by the U.S. FDA (anticipated to be available several months after approval) and approved in Europe in 2016. This new medication is a selective histamine 3 (H3) receptor antagonist/inverse agonist that works through a novel mechanism of action to increase the synthesis and release of histamine, a wake-promoting neurotransmitter in the brain. Pitolisant improved sleepiness in approximately ⅓ of IH patients whose symptoms did not respond well to other medications.

Sodium oxybate  (Xyrem) is a medication taken at bedtime (and again during the night) that promotes deep sleep and improves daytime sleepiness in people with narcolepsy; however, its effects in those with idiopathic hypersomnia are not as well characterized. A 2016 study found that sodium oxybate improved daytime sleepiness in people with IH to the same degree as in people with narcolepsy type 1. The drug also improved severe sleep inertia in 71% of people with IH. Of the medications listed above, sodium oxybate is the only medication that treats both sleepiness and cataplexy.

Medications FDA-Approved for Cataplexy

Sodium oxybate is the only FDA-approved medication for cataplexy. Antidepressants, especially serotonin and norepinephrine reuptake inhibitors, are frequently used “off-label” to treat cataplexy.

Novel and Emerging Treatments for Sleepiness in Primary Hypersomnias

As the brain systems regulating sleepiness and wakefulness are better understood, scientists will be in a better position to design treatments that target key portions of this system. For example, based on the role of histamine in keeping people awake (and hence the common side effect of antihistamines such as diphenhydramine causing sleepiness), medications that act on histamine are under development for the treatment of excessive sleepiness (see pitolisant, as the first in this class, above).

In the case of the primary hypersomnias related to excess activity of the GABA system, medications that could counteract this activity have the potential to improve sleepiness. The antibiotic clarithromycin has been shown in a small, randomized trial to improve sleepiness and quality of life more than placebo. Another medication which acts on the GABA system, flumazenil, has also been used to treat sleepiness in IH patients whose sleepiness is not controlled with other medications.

Information about clinical trials for new treatments and how to access treatments that aren’t yet available in your home country can be found HERE.

Behavioral Treatments

In addition to medications, behavioral approaches are sometimes recommended. Short, planned naps can be helpful for sleepiness in people with narcolepsy. Unfortunately, for many people with IH, planned naps are not as helpful, because they tend to be long and unrefreshing, and it can be difficult to awaken from naps.

Although behavioral approaches have not been shown to improve EDS in people with IH, different types of therapy may help people learn to cope with this chronic disease. For example, in CBT (cognitive behavioral therapy), one goal of therapy is to help patients learn to reduce their negative emotional responses (e.g. frustration, anger, depression) to their disease symptoms. This and other types of therapy can help people learn coping skills and adjust to the sometimes large lifestyle changes brought on by IH symptoms. (Read more HERE.)

Finally, because symptoms of IH can affect school, work, and relationships, it is important to educate everyone who might regularly interact with people with IH about this sleep disorder. Since some people with IH require educational and/or work accommodations so they can be successful in reaching their career goals, this may require a conversation between healthcare providers and school and/or employer about what IH is and how the symptoms affect people. Similarly, spouses, partners, parents, and close friends can also benefit from learning more about IH, so they can better understand IH symptoms and how these symptoms may affect their loved one.


Further Information

1) 2018 HF Conference Video featuring Dr. Lynn Marie Trotti: Current Treatments for Idiopathic Hypersomnia.

2) SleepHub Australia – Wake-Promoting Medication.

3) SomnusNooze articles about treatments, including novel and emerging treatments.

4) Free full text journal articles:

 

Revised 8/2019 by Lynn Marie Trotti, MD, MSc, Chairperson, HF Medical Advisory Board

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