NARCOLEPSY

NARCOLEPSY – Etiology, Signs and Symptoms, Diagnostic Evaluation and Management (Medical and Nursing)

Narcolepsy is a neurological disorder that affects the control of sleep and wakefulness. People with narcolepsy experience excessive daytime sleepiness and intermittent, uncontrollable episodes of falling asleep during the daytime. These sudden sleep attacks may occur during any type of activity at any time of the day

ETIOLOGY

• The cause of narcolepsy is not known; however, scientists have made progress toward identifying genes strongly associated with the disorder. These genes control the production of chemicals in the brain that may signal sleep and awake cycles
• Some experts think narcolepsy maybe due to a deficiency in the production of a chemical called hypocretin by the brain

SIGNS AND SYMPTOMS

• Excessive daytime sleepiness (EDS): in general, EDS interferes with normal activities on a daily basis, whether or not a person with narcolepsy has sufficient sleep at night. People with EDS report mental cloudiness, a lack of energy and concentration, memory lapses, a depressed mood, and extreme exhaustion
• Cataplexy: this symptom consists of a sudden loss of muscle tone that leads to feelings of weakness and a loss of voluntary muscle control. It can cause symptoms ranging from slurred speech to total body collapse, depending on the muscles involved, and is often triggered by intense emotions such as surprise, laughter, or anger
• Usually, these delusional experiences are vivid and frequently frightening. The content is primarily visual, but any of the other senses can be involved. These are called hypnagogic hallucinations when accompanying sleep onset and hypnopompic hallucinations when they occur during awakening
• This symptom involves the temporary inability to move or speak while falling asleep or waking up. These episodes are generally brief, lasting a few seconds to several minutes. After episodes end, people rapidly recover their full capacity to move and speak
• Microsleep is a very brief sleep episode during which the patient continue to function (talk, put things away, etc.) and then awaken with no memory of the activities

DIAGNOSTIC EVALUATION

A physical exam and exhaustive medical history are essential for proper diagnosis of narcolepsy. Two tests that are considered essential in confirming a diagnosis of narcolepsy are the polysomnogram (PSG) and the multiple sleep latency test (MSLT).

• Noctural polysomnogram: this overnight test measures the electrical activity of brain and heart, and the movement of muscles and eyes
• Multiple sleep latency test (MSLT): this test measures how long it takes to fall asleep during the day
• Spinal fluid analysis: the lack of hypocretin in the cerebrospinal fluid may be a marker for narcolepsy. Examining spinal fluid is a new diagnostic test for narcolepsy

The Epworth Sleepiness Scale measures daytime sleepiness. Use the following scale to choose the most appropriate number for each situation:

0 = would never sleep

1 = slight chance of dozing or sleeping

2 = moderate chance of dozing or sleeping

3 = high chance of dozing or sleeping

Situation            Chance of dozing or sleeping

Sitting and reading                —

Watching TV                           —

Sitting inactive in a public place   —

Being a passenger in a motor vehicle for an hour or more —

Lying down in the afternoon    —

Sitting and talking to someone    —

Sitting quietly after lunch (no alcohol)     —

Stopped for a few minutes in traffic while driving   —

Total score (add the scores up)    —

A total score of 10 or more is considered sleepy. A score of 18 or more is very sleepy

MANAGEMENT

• Schedule sleep periods: take a few brief, scheduled naps during the daytime (10-15 minutes each). Try to get a good night’s sleep during the same hours each night. Planned naps can prevent lapses into sleep
• Avoid caffeine, alcohol, and nicotine: these substances interfere with sleep
• Avoid over-the-counter drugs that cause drowsiness: some allergy and cold medications can cause drowsiness, so should be avoided
• Involve employers, coworkers and friends: alert others so that they can help when needed
• Carry a tape recorder: record important conservations and meetings, in case you fall asleep
• Break up larger tasks into small pieces: focus on one small thing at a time
• Exercise on a regular basis: exercise can make you feel more awake during the day and stimulate sleep at night. For example, take severe short walks during the day
• Avoid activities that would be dangerous if you had a sudden sleep attack: if possible, do not drive, climb ladders, or use dangerous machinery. Taking a nap before driving may help you to manage any possible sleepiness
• Wear a medical alert bracelet or necklace: a bracelet or necklace will alert others if you suddenly fall asleep or become unable to move or sleep
• Eat a healthy diet: aim for a diet rich in whole grains, vegetables, fruits, low fat dairy, and lean sources of protein. Eat light or vegetarian meals during the day and avoid heavy meals before important activities
• Relax and manage emotions: narcolepsy symptoms can be triggered by intense emotions, so you may benefit from practicing relaxation techniques, such as breathing exercises, yoga or massage

Medical Management

Common medications used to treat narcolepsy symptoms include:

• Stimulants: stimulants are the mainstay of drug treatment for narcolepsy. These include modafinil, a stimulant used during the day to promote wakefulness and alertness
• Sodium oxybate: this strong drug maybe prescribed if one has severe cataplexy. Sodium oxybate is also known as GHB, or the ‘date rape drug’,  but is considered safe for treating narcolepsy when used responsibly to promote sound sleep, diminish daytime sleepiness, and reduce incidences of cataplexy
• Antidepressants: selective serotonin reuptake inhibitors (SSRIs) used to treat depression may also be used to help suppress REM sleep, and alleviate symptoms of cataplexy, hallucinations and sleep paralysis

NURSING MANAGEMENT

• Instruct patient to follow as consistent a daily schedule for retiring and arising as possible
• This promotes regulation of the circadian rhythm, and reduces the energy required for adaptation to changes
• Instruct to avoid heavy meals, alcohol, caffeine, or smoking before retiring
• Though hunger can also keep one awake, gastric digestion and stimulation from caffeine and nicotine can disturb sleep
• Instruct to avoid large fluid intake before bedtime. For patients may need to void during the night
• Increase daytime physical activities as indicated, to reduce stress and promote sleep
• Instruct to avoid strenuous activity before bedtime. Over fatigue may cause insomnia
• Discourage pattern of daytime naps deemed necessary to meet sleep requirements or if part of one’s usual pattern. Napping can disrupt normal sleep patterns. However, the elderly do better with frequent naps during the day to counter their shorter night-time sleep schedule
• Suggest use of soporifics such as milk. Which contains L-tryptophan that facilitates sleep
• Recommend an environment conductive to sleep or rest (e.g. quiet, comfortable temperature, ventilation, darkness, closed door). Suggest use of earplugs or eye shades as appropriate
• Suggest engaging in a relaxing activity before retiring, such as warm bath, calm music, reading an enjoyable book, relaxation exercises
• Explain the need to avoid concentrating on the next day’s activities or on one’s problems at bedtime
• Suggest using hypnotics or sedatives as ordered
• If unable to fall asleep after about 30 to 45 minutes, suggest getting out of bed and engaging in a relaxing activity. Provide nursing aids (e.g. back rub, bedtime care, pain relief, comfortable position, relaxation techniques)
• Organize nursing care: eliminate nonessential nursing activities. Prepare patient for necessary anticipated interruptions/disruptions
• Attempt to allow for sleep cycles of at least 90 minutes
• Move patient to room farther from the nursing station if noise is a contributing factor.
• Post a “do not disturb” sign on the door.