Download History of Narcolepsy

1
Goals of this Presentation
1. Learn how to prepare for a successful
pediatric sleep study
2. Learn what to look for and how to respond
during the study
3. Learn about pediatric sleep disorders and
their treatments
2
Children:
Not just short adults
3
Pediatric Polysomnography
Requires Patience and Preparation
• Polysomnographic procedures may be fear
provoking to children
• Children require more time to set up for a
polysomnogram than do adults
• Crying and removing electrodes may extend set
up time past the child’s usual bedtime,
resulting in an overtired child
4
A Family Centered Care Approach
• Parents are the experts on their child and a constant in
their child’s life
• Procedures should be conducted to create the least
amount of trauma for the child
• The test environment should be inviting and childfriendly
• Psychological preparation of the child and parent are
fundamental to the procedure
• Coping-skill development enhances a child’s sense of
mastery and control over a potentially stressful
experience
Zaremba et al, JCSM, 2005
5
Important “Mind-Set” Changes by
the Polysomnography Staff
FROM
TO
needs of the staff
needs of the child, parent
“Good Guy – Bad Guy”
parent, child and tech on
the same team
a child lying down
performing the procedure
with the child sitting
6
Zaremba et al, JCSM, 2005
Preparing the Family for a
Polysomnogram
• Provide detailed information about the test
• Schedule testing for the child’s usual bedtime
• Communications: Confirmation letter sent with:
– Logistics of reaching the center
– What to bring (food, transitional objects)
– No caffeine, no naps, no hair oils
• Answer questions as they come up
7
What the Parent Should Know
• No acute or very recent medical issues
– Parents should call to cancel if child is ill
• Recommend shampoo night before
– Avoid scalp oils
– Avoid new braids
•
•
•
•
Avoid caffeinated beverages
Comfortable, loose two piece pajamas
Bring a favorite book, video
Bring usual medications
8
Creating a Calm Environment
• Take time to establish rapport
• Explore the child’s past experiences and coping
strategies
• Create a good first impression
– Have books or toys on the bed
– Cover set up supplies, equipment if possible
• Use a calm and soothing tone of voice
9
Child and Family Preparation
• On the study night…
–
–
–
–
–
Allow the child to explore room and sensors
Define each person’s job
Develop a plan for coping
Maintain patience, flexibility, positive attitude
Lavish the child with praise
• Focusing on the desired behavior
10
Engaging the Parent
• Make the parent part of the team
• Encourage the parent to interact in a
reassuring way with the child
• Respond positively to parents questions
and concerns
• Provide parents with explanations of the
procedures
11
Optimizing the Environment
for Sleep and Safety
• Quiet – away from doors, overhead paging
• Dark – shades over windows
• Can you see, hear, communicate with child?
– Call button, two-way communication for calibrations
– Need for infrared lighting
• Safety
– Outlet plugs, no sharp corners, bed rails up
– Hypoallergenic, latex free supplies, no sharp corners
• Access: emergency equipment, personnel
12
Ground Rules for Bedroom
Electronics
• No active phones or pagers in sleep room
– Arrange local phone access for parent
• Cell phones must be muted
– No calls in the room after lights out
• Plan video or TV to end before lights out
– Avoid electronic games immediately before bed
13
Explanations
• Short, objective and concrete explanations are
appropriate for younger children
• Children may regress when upset
– May need to aim explanations at a developmental
level less than child’s age
• Be honest and careful in your word choice
• Sarcasm and teasing may be misinterpreted and
should be avoided
14
Tips for Improving Cooperation
• Younger children may want to sit in their
parent’s lap during set-up
• Distractions are often useful (stickers, bubbles,
toys, favorite video)
• Medical play may reduce anxiety (put the
electrodes on a doll)
• Older children can help by holding electrodes
or sensors
15
Positions for Comfort
16
Zaremba et al, JCSM, 2005
Pediatric Polysomnography
EEG
EOG
Nasal EtCO2
Nasal Oral Airflow
Chin EMG (2)
Microphone
SaO2
EKG
Tech Observer
Video Camera
Respiratory Effort
Documents arousals,
parasomnias, abnormal
sleeping position, and attends
to any technical problem
Leg EMG (2)
Records behavior
17
Courtesy of Dr. Carol Rosen
During the Night
• Children need more frequent adjustment of
sensors during the night than adults
• Nearly all studies of children require that the
sensors be replaced at some point during the
night
• Technologists should warn the patient and the
parent that they will be entering the room
during the night
18
Documentation
• Due to the prevalence of parasomnias,
children’s studies need frequent documentation
• Children may have significant sleep disorders
without dramatic polysomnographic findings
• Recordings may be ambiguous at times (i.e.,
when breathing sensors have been displaced);
technologist observations become crucial to
interpretation
– For example: “discovered nasal pressure transducer
pushed to side of face – restored to proper position”
19
Describe What You See
• Helpful
– Sat up abruptly--staring
and mumbling
– Patient breathing quietly
– Mom moving, wakes child
– Went into room, snoring
from mother, not patient
• Not Helpful
– Possible seizure
– Can’t hear patient
– Patient moving in
bed
– Artifact
– Sounds from room
20
The Spectrum of Pediatric Sleep
Disorders
Prevalent in
Children Using
Different Criteria
Than in Adults
More Prevalent in
Children Than
Adults
Delayed sleep phase
syndrome
Obstructive sleep
apnea
Sleepwalking, sleep
talking
Periodic limb
movement disorder
Restless legs
syndrome
Sleep terrors
Narcolepsy
Nightmares
Prevalent in
Children and
Adults
Unique to
Children by
Definition
Behavioral insomnia
of childhood
21
Estimated Prevalence of
Sleep Disorders in Children
• Insufficient sleep – 10% (higher in teens – up to 33%)
– Behaviorally based - 25%
•
•
•
•
Sleep related breathing disorders - 2%
Narcolepsy – 0.05%
Sleep/wake timing (delayed sleep phase) - 7% teens
Partial arousals (parasomnias)
– Night terrors 2 - 3%
– Sleep walking 5%
• Rhythmic movement disorder 3 -15%
• Restless legs syndrome – 2%
22
Who Should Have a Polysomnogram?
Guidelines for Investigation of
Sleep Related Breathing Disorders in Children
• All children should be screened for snoring
–
–
–
–
Habitual snoring with labored breathing
Witnessed apnea
Restless sleep
Evidence of daytime sleepiness
• And be sent for a polysomnogram if they show physical
signs of sleep apnea
– Growth abnormalities
– Signs of upper airway obstruction
– Evidence of pulmonary hypertension
American Academy of Pediatrics, 2002
23
Prevalence of Sleep Related
Breathing Disorders in Children
• Habitual snoring – 10%
• Sleep disordered breathing – 2%
• Risk factors
–
–
–
–
African-American heritage
Family history of OSA
History of prematurity
Chronic conditions - cerebral palsy, trisomy 21, achondroplasia
and other genetic syndromes
– Obesity (less risky than in adults)
– No gender difference in prepubertal children
Rosen et al 2003
24
Many Pediatric Diagnoses Do Not
Require a Polysomnogram
Usually requires polysomnography:
• Obstructive Sleep Apnea, Pediatric
• Narcolepsy
Usually diagnosed by tests other
than polysomnography (i.e., ICU
monitoring)
• Primary Sleep Apnea of Infancy
(formerly Primary Sleep Apnea of Newborn)
• Congenital Central Hypoventilation
Syndrome
Usually does not require
polysomnography:
• Behavioral Insomnia of
Childhood (Sleep Onset Type)
• Behavioral Insomnia of
Childhood (Limit-Setting Type)
• Sleepwalking, Night Terrors
• Sleep Enuresis
• Restless Legs Syndrome
• Sleep Related Rhythmic
Movement Disorder
May require polysomnography with
extended EEG montage:
• Complicated or atypical parasomnia
25
Evaluating Breathing during Sleep
in Children
• Children experience less desaturation with apnea
• Carbon dioxide monitoring is recommended (< 12 years)
• Monitoring behavior, body position, snoring is important
• Additional measures of effort such as esophageal
pressure monitoring may be helpful in special cases
26
Scoring Rules
• Apnea is recurrent partial or complete airway
obstruction despite continued effort
– Adult -- respiratory event is 10 seconds or longer
– Child – “two missed breath” duration
• ETCO2 levels above 50 mm Hg for more than
10% of sleep time may be abnormal
27
Types of Sleep Related
Breathing Disorders in Children
• Upper airway resistance syndrome is common
– Repetitive respiratory effort related arousals without
discrete apnea or hypopnea
– No changes in oxygen saturation or ETCO2
• Obstructive hypoventilation is common
– Upper airway narrowing with gas exchange
abnormalities, but without clear apnea or hypopnea
• Most prominent in REM
28
The Spectrum of Obstructive Sleep
Related Breathing Disorders in Children
APNEA
HYPOPNEA
OBSTRUCTIVE
HYPOVENTILATION
RESPIRATORY EFFORT
RELATED AROUSAL
SNORING
LOW
Degree of Obstruction
HIGH
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Normal Breathing – NREM Sleep
Note time scale
Delta
activity, K
complexes,
spindles in
EEG
Very
regular
breathing
No oxygen
desaturation
or CO2
elevation
8 y/o with daytime sleepiness
30
Normal Breathing –REM Sleep
Rapid eye
movements,
low voltage
fast EEG
pattern
Breathing,
heart rate
somewhat
irregular
8 y/o with daytime sleepiness
31
RERA
Arousal
(alpha
activity
at arrow)
Recurrent
episodes of
flattened
nasal air
pressure and
minimal
oxygen
desaturation
10 y/o with restless sleep
32
Apnea and Hypopnea
Hypopnea –
between
30 and 70%
air flow
Apnea –
less than
30% air
flow
9 y/o with snoring and gasping at night
and poor school performance
33
ICSD-2 Diagnostic Criteria:
Obstructive Sleep Apnea, Pediatric
•
•
The caregiver reports snoring, and/or labored or obstructed
breathing, during the child’s sleep.
The caregiver reports observing at least one of the following:
i.
Paradoxical inward rib-cage motion during inspiration
ii. Movement arousals
iii. Diaphoresis
iv. Neck hyperextension during sleep
v. Excessive daytime sleepiness, hyperactivity, or aggressive
behavior
vi. A slow rate of growth
vii. Morning headaches
viii. Secondary enuresis
34
Obstructive Sleep Apnea, Pediatric
ICSD-2 Diagnostic Criteria (cont.)
• Polysomnographic recording demonstrates one or more
scoreable obstructive respiratory events per hour (i.e.,
apnea or hypopnea of at least two respiratory cycles in
duration)
– Note: Very few normative data are available for hypopneas,
and the data that are available have been obtained using a
variety of methodologies. These criteria may be modified in
the future once more comprehensive data become available.
35
Obstructive Sleep Apnea, Pediatric
ICSD-2 Diagnostic Criteria (cont.)
• Polysomnographic recording demonstrates either i or ii.
i. At least one of the following is observed:
•
•
•
•
a. Frequent arousals from sleep associated with increased respiratory effort
b. Arterial oxygen desaturation in association with the apneic episodes
c. Hypercapnia during sleep
d. Markedly negative esophageal pressure swings
ii. Periods of hypercapnia, desaturation, or hypercapnia and
desaturation during sleep associated with snoring, paradoxical
inward rib-cage motion during inspiration, and at least one of the
following:
• a. Frequent arousals from sleep
• b. Markedly negative esophageal pressure swings
36
Obstructive Sleep Apnea, Pediatric
• Many children have associated cognitive problems and
difficulty at school
• Pediatric obstructive sleep apnea is frequently
associated with adenotonsillar hypertrophy
• Adenotonsillectomy is effective in most children
• When applied to pediatric recordings, adult
polysomnographic measures alone (i.e., AHI) may
underestimate the number of patients who would
benefit from adenotonsillectomy
37
CPAP Therapy for Children
• Continuous positive airway pressure is an effective
second-line treatment in pediatric patients
• A desensitization program is an extremely important part
of treatment
• Successful trials reported in 74% of patients, with 86% of
those able to use the therapy long-term
38
Primary Sleep Apnea of Infancy
(formerly Primary Sleep Apnea of Newborn)
ICSD-2 Diagnostic Criteria
• Apnea of Prematurity. Prolonged central respiratory pauses of 20
seconds or more in duration (or shorter-duration events that include
obstructive or mixed respiratory patterns and are associated with a
significant physiologic compromise, including decrease in heart rate,
hypoxemia, clinical symptoms, or need for nursing intervention), are
recorded in an infant younger than 37 weeks conceptional age.
• Apnea of Infancy. Prolonged central respiratory pauses of 20
seconds or more in duration (or shorter-duration events that include
obstructive or mixed respiratory patterns and are associated with
bradycardia, cyanosis, pallor, or marked hypotonia), are recorded in
an infant with a conceptional age of 37 weeks or older.
39
Primary Sleep Apnea of Infancy
• Should be distinguished from Acute Life Threatening
Events (ALTE), an ill-defined disorder based on parental
complaints and Sudden Infant Death Syndrome (SIDS), a
post-mortem diagnosis
• A polysomnogram is the best way to evaluate breathing
during sleep
• Prognosis is excellent with infrequent events
– Prognosis guarded when frequent resuscitation is required and
events persist over time
40
Congenital Central Alveolar
Hypoventilation Syndrome
ICSD-2 Diagnostic Criteria
• The patient exhibits shallow breathing, or cyanosis and apnea,
of perinatal onset during sleep.
– Note: In severely affected infants, consequences of hypoxia, including
pulmonary hypertension and cor pulmonale, may also be present.
• Hypoventilation is worse during sleep than during wakefulness.
• The rebreathing ventilatory response to hypoxia and
hypercapnia is absent or diminished.
• Polysomnographic monitoring during sleep demonstrates severe
hypercapnia and hypoxia, predominantly without apnea.
41
Congenital Central Alveolar
Hypoventilation Syndrome
• Present from birth
• Requires lifelong treatment
– Mechanical ventilation or pacing
– Most patients do not need treatment when awake
• Associated with abnormality of the PHOX2B gene
• Associated with Hirschsprung's disease
42
Narcolepsy in Children
• Narcolepsy with cataplexy is rare in children younger
than four years old
• Daytime sleepiness frequently presents as
reappearance of napping in a child that has stopped
napping
• Sleepiness at school may be manifest by symptoms
similar to attention deficit disorder
• Diagnosis may be clinical or supported by findings from
overnight polysomnography with multiple sleep latency
testing. Alternatively, measurement of levels of
hypocretin in cerebrospinal fluid may be appropriate
for certain patients.
43
Recognizing Sleepiness in Children
• Sleepy children do not always “act sleepy”
– Parent may endorse other terms like seems “overtired”
• Children with insufficient or disrupted sleep can show:
–
–
–
–
Inattention
Hyperactivity
Behavioral disturbances
Poor school performance
• Persistent, overt sleepiness is uncommon in
preadolescent children unless the disorder is severe
44
Pediatric MSLT
• Use standard MSLT protocol from AASM Practice
Parameter
– Review procedure with child and parent and answer
any questions
– It is recommended that parents leave the testing
room during naps
– Ask if child needs to go to the bathroom
– Put up side rails if necessary
– Remind the child, “I will come back in to the room
when the nap test is over.”
45
SOREMP in a Child
Nap #1
lights out
Alpha
activity
Reduced
tone
Nap #1
00:30
Rapid eye
movement
12 y/o referred for excessive daytime
sleepiness and cataplexy symptoms
46
Minutes of sleep
Nocturnal Sleep Decreases with Age
Ohayon et al SLEEP 2004;27(7):1255-73.
47
Napping is Normal
in Very Young Children
Age (months)
Acebo et al. SLEEP 2005; 28(12): 1568-1577.
48
Sleep Latency during MSLT Naps Decreases
in Adolescents with Increasing Tanner Stage
NOTE: Mean sleep latency is longer in children compared with adults
MSLT Latency
20
18
Latency (min)
16
14
12
10
8
6
4
2
0
I
II
III
IV
Tanner Stage
V
Older
Teens
Data from Carskadon MA. The second decade. In Guilleminault C, ed, Sleeping and waking disorders:
indications and techniques. Menlo Park: Addison Wesley, 1982: 99-125
49
Sleep Latency Increases with
Age after Adolescence
From Arand et al, SLEEP 2005;28(1):123-144.
50
Interpreting Pediatric MSLT Results
• Two or more sleep onset REM periods are necessary
to support a diagnosis of narcolepsy
• Age has a complicated and profound impact on MSLT
mean sleep latency
• Limited normative data is available
• Mean sleep latencies that might be considered
normal for adults are often abnormal for children
• The ICSD-2 states, “The MSLT has not been validated
as a diagnostic test in children younger than eight
years of age.”
51
Parasomnias
• Children are often referred to the sleep center
because of unusual behaviors during the night
–
–
–
–
Sleepwalking
Sleep terrors
Nightmares
Seizures
52
Abnormal Breathing and EEG
Activity in Sleep
9 y/o with known epilepsy and snoring
53
Sleepwalking and Sleep Terrors:
Partial Arousal Parasomnias
Partial arousal parasomnias
– Occur during first half of night
– Arise from slow wave sleep
– Child is not awake
• Sleepwalking
– Child moves around room or house
– May be quiet or agitated
– May engage in purposeful activities, like unlocking door
• Sleep terrors
– Child abruptly sits up screaming
– Appears frightened and agitated
54
Night Terrors
Nightmares
•
•
•
•
•
• REM sleep
• Last half of night
• Child alert; describes dream
content
• Comforted by parent
• Difficulty going back to
sleep
• Recall the following day
Deep NREM sleep
First third of night
Child confused or agitated
Difficult to reassure
Intense arousal lasting 210 min
• Abrupt return to sleep
• No recall in the morning
55
Technologist Response
to Unusual Behaviors
• Parasomnias can lead to injury
– Be sure patient is safe
• Parasomnias sometimes resemble seizures
– Seizures (especially frontal lobe) can resemble parasomnias
• During study describe what you see
• Note event on record when it is happening
–
–
–
–
–
Sitting up yelling
Patient mumbling – can’t understand words
Patient’s left arm and leg twitching
Mother trying to comfort, patient keeps yelling “mommy”
Patient trying to get out of bed
56
Confusional Arousal
5 y/o with witnessed apnea and restlessness
57
Restless Legs Syndrome
ICSD-2 Diagnosis in Adult Patients
• The patient reports an urge to move the legs, usually
accompanied or caused by uncomfortable and unpleasant
sensations in the legs.
• The urge to move or the unpleasant sensations
– begin or worsen during periods of rest or inactivity (lying or
sitting)
– are partially or totally relieved by movement, such as walking or
stretching, at least as long as the activity continues
– are worse, or only occur, in the evening or night
58
Restless Legs Syndrome
ICSD-2 Diagnostic Criteria
• The child meets all four essential adult criteria for RLS listed above and
relates a description, in his or her own words, that is consistent with leg
discomfort.
OR
• The child meets all four essential adult criteria for RLS listed above but
does not relate a description in his or her own words that is consistent
with leg discomfort.
AND
• The child has at least two of the following three findings:
i. A sleep disturbance for age
ii. A biological parent or sibling with definite RLS
iii. A polysomnographically documented periodic limb movement index of
five or more movements per hour of sleep
Note: Criteria for probable and possible childhood RLS have been
developed for research purposes and are included in a National
Institutes of Health diagnostic workshop report.
59
Restless Legs Syndrome (RLS) &
Periodic Limb Movement Disorder (PLMD)
• Prevalence in children 0.5-2%, familial link
– RLS - “growing pains”
– PLMD – leg jerks - what’s normal
• Relationship with hyperactivity?
• Can be associated with:
– Iron deficiency/low ferritin
– Chronic renal disease
• Diagnostic controversies in adults
– Scant data in children
– May present as insomnia or sleepiness
60
Criteria for Sleep Related Rhythmic
Movement Disorder
ICSD-2 Diagnostic Criteria
• The patient exhibits repetitive, stereotyped, and rhythmic
motor behaviors.
• The movements involve large muscle groups.
• The movements are predominantly sleep related, occurring
near nap or bedtime, or when the individual appears drowsy
or asleep.
• The behaviors result in a significant complaint as manifest by
at least one of the following:
i. Interference with normal sleep
ii. Significant impairment in daytime function
iii. Self-inflicted bodily injury that requires medical treatment (or
would result in injury if preventable measures were not used)
61
Sleep Related Rhythmic Movements
• Repetitive movements
– Head banging or head rolling
– Body rocking
• Before sleep, light sleep, or even awake
• Prevalence of rhythmic movements decreases with age
– At nine months = 59%
– At eighteen months = 33%
– At five years = 5%
• No gender difference
• Polysomnogram or treatment rarely indicated
62
Sleep Enuresis
ICSD-2 Diagnostic Criteria
Primary
Secondary
• The patient is older than five
years of age
• The patient exhibits recurrent
involuntary voiding during
sleep, occurring at least twice
a week.
• The patient has never been
consistently dry during sleep.
• The patient is older than five
years of age
• The patient exhibits recurrent
involuntary voiding during
sleep, occurring at least twice
a week.
• The patient has previously
been consistently dry during
sleep for at least six months.
63
Prevalence of Enuresis
Children (%)
40.0
30.0
20.0
10.0
0.0
4
5
6
7
8
10
18
Age (years)
64
Developmental Overview of
Common Non-respiratory Sleep Problems
Newborn/
Young Infant
Usually normal
Developmental
Self limited
Older Infant
and Toddler
Preschooler
School Age
Night wakings
Difficulty
settling
Night terrors
Night wakings
Bedtime
resistance
Night terrors
Sleep walking
Insufficient
sleep
Bedtime
resistance
Sleep walking
Rhythmic
movements
Bedtime fears
Rhythmic
movements
Bedtime fears
Nightmares
Enuresis
Bruxism
Teenager
Insufficient sleep
Delayed sleep
phase
Narcolepsy
65
Behavioral or Life Style Sleep Problems
• Sleep onset association disorder
• Limit setting disorder
• Poor “sleep hygiene”
• Caffeine
• Irregular schedule
• TV/computer/cell phone/electronics in bedroom
• Overlap with delayed sleep phase
– Perpetuated by weekend sleep-in and late day naps
• Management – change behaviors
66
Behavioral Insomnia of Childhood
(Sleep-onset Type)
ICSD-2 Diagnostic Criteria
• Falling asleep is an extended process that requires
special conditions
• Sleep-onset associations are highly problematic or
demanding
• In the absence of the associated conditions, sleep onset
is significantly delayed or sleep is otherwise disrupted
• Awakenings require caregiver intervention for the child
to return to sleep.
67
Sleep Onset Type
Typical Presentations
• Child falls asleep during rocking or patting, needs to be
rocked or patted after night waking
• Child falls asleep feeding, needs to be fed to fall asleep
• Child falls asleep with parent singing, reading or lying
next to child, but cannot fall sleep alone
• Child falls asleep in car seat, needs to be driven around
to fall asleep
68
Behavioral Insomnia of Childhood
(Limit-setting Type)
ICSD-2 Diagnostic Criteria
• The child has difficulty initiating or maintaining sleep
• The child stalls or refuses to go to bed at an
appropriate time or refuses to return to bed following a
nighttime awakening
• The caregiver demonstrates insufficient or
inappropriate limit setting to establish appropriate
sleeping behavior in the child
69
Limit-setting Type
Typical Presentations
• Child is two years or older
• “Stalling” behaviors at bedtime
–
–
–
–
Needs a drink or food
Multiple stories
Crying, clinging
Gets out of bed (“curtain calls”)
• Parent’s behavior contributes to problem
– Irregular or inappropriate schedules
– Inconsistent application of rules
– Secondary gain for child
70
Contributing Factors
• Circadian rhythms develop over the first few months of
life – infants have frequent awakenings and irregular
schedules at birth
• Homeostatic drive to sleep is blunted by frequent
napping
• Environmental factors such as warmth, soothing sounds
and vestibular stimulation promote sleepiness
• Learned associations serve as triggers for sleep onset
71
Behavioral Insomnia of Childhood:
Treatment Options
•
•
•
•
•
•
•
Extinction
Graduated extinction (“Ferberizing”)
Positive routines
Faded bedtime with response cost
Scheduled awakenings
Parent education
Medications (efficacy unproven in children)
– Prescription
– Over-the-counter
72