Assessing narcolepsy with cataplexy in children and adolescents: development of a cataplexy diary and the ESS-CHAD

Figures & data

Figure 1 Provisional version of the ESS-CHAD.

Abbreviation: ESS-CHAD, Epworth Sleepiness Scale for Children and Adolescents.

Table 1 Demographic characteristics of the pediatric patients who participated in cognitive debriefing interviews (n=29)

Variable	Value
Sex, n (%)
Male	15 (51.7)
Female	14 (48.3)
Age, years, mean (SD)	11.6 (3.5)
Age group, n (%)
7–9 years	13 (44.8)
10–17 years	16 (55.2)
Race, n (%)
Black/African American	19 (65.6)
White	8 (27.6)
Others	2 (6.9)

Table 2 Main concepts of cataplexy endorsed by ≥2 participants in any interview group

Concept	Participants endorsing concept, n (%)
	Children, 7–9 years (n=13)	Adolescents, 10–17 years (n=16)	Parents/caregivers (n=29)
Manifestations
Weakness, lower limb (eg, falling down, leaning over)	11 (84.6)	10 (62.5)	23 (79.3)
Weakness, body (overall sensation)	6 (46.2)	9 (56.3)	11 (37.9)
Weakness, upper limb (eg, arms flailing/dropping to side)	4 (30.8)	5 (31.3)	13 (44.8)
Weakness, neck (eg, head slumping)	–	5 (31.3)	12 (41.4)
Weakness, face (eg, facial tick, eyes rolling, jaw dropping, tongue sticking out)	1 (7.7)	2 (12.5)	10 (34.5)
Pain (eg, headache, leg hurting) after an attack	2 (15.4)	3 (18.8)	3 (10.3)
Dizziness	–	3 (18.8)	–
Triggers
Laughter	7 (53.8)	11 (68.8)	16 (55.2)
Angry	–	4 (25.0)	8 (27.6)
Excited	3 (23.1)	1 (6.3)	7 (24.1)
Scared	2 (15.4)	2 (12.5)	5 (17.2)
Upset	–	3 (18.8)	4 (13.8)
At school	2 (15.4)	2 (12.5)	2 (6.9)
At home	2 (15.4)	2 (12.5)	–
Nervous	–	2 (12.5)	2 (6.9)
Crying	–	–	3 (10.3)
Happy	2 (15.4)	–	1 (3.4)
Situations
Walking/running	1 (7.7)	6 (37.5)	2 (6.9)
In the morning	–	2 (12.5)	2 (6.9)
In the evening	–	1 (6.3)	2 (6.9)
While playing	–	–	3 (10.3)
When tired	1 (7.7)	–	2 (6.9)

Table 3 Main concepts of symptoms and settings of narcolepsy endorsed by ≥2 participants in any interview group

Concept	Participants endorsing concept, n (%)
	Children, 7–9 years (n=13)	Adolescents, 10–17 years (n=16)	Parents/caregivers (n=29)
Symptoms
Excessive sleeping	10 (76.9)	12 (75.0)	29 (100)
Tiredness/sleepiness	11 (84.6)	11 (68.8)	22 (75.9)
Difficulty waking up	5 (38.5)	9 (56.3)	17 (58.6)
Vivid dreams/hallucinations/sleep paralysis	7 (53.8)	4 (25.0)	14 (48.3)
Difficulty falling/staying asleep at night	5 (38.5)	4 (25.0)	15 (51.7)
Eyes closing/drooping	2 (15.4)	6 (37.5)	8 (27.6)
Sleepwalking/talking while asleep	2 (15.4)	1 (6.3)	10 (34.5)
Low energy/weakness	–	4 (25.0)	8 (27.6)
Difficulty paying attention/zoning out	–	2 (12.5)	3 (10.3)
Eating while asleep/in the middle of the night	1 (7.7)	–	3 (10.3)
Dizziness	1 (7.7)	2 (12.5)	–
Settings
In a car	10 (76.9)	14 (87.5)	27 (93.1)
At school	10 (76.9)	14 (87.5)	20 (69.0)
When watching TV/Internet/videos/movies	7 (53.8)	12 (75.0)	21 (72.4)
When inactive	1 (7.7)	6 (37.5)	16 (55.2)
At home	2 (15.4)	8 (50.0)	12 (41.4)
When sitting down	2 (15.4)	6 (37.5)	11 (37.9)
While doing homework	4 (30.8)	6 (37.5)	8 (27.6)
When reading a book	1 (7.7)	5 (31.3)	11 (37.9)
When eating	2 (15.4)	–	10 (34.5)
During a conversation	1 (7.7)	2 (12.5)	8 (27.6)
At other’s home	1 (7.7)	3 (18.8)	3 (10.3)
When waking up	3 (23.1)	1 (6.3)	3 (10.3)
When playing sports/training	1 (7.7)	3 (18.8)	2 (6.9)
When playing video games	1 (7.7)	2 (12.5)	3 (10.3)
After eating/feeling full	2 (15.4)	2 (12.5)	2 (6.9)
Playing with friends	–	–	5 (17.2)
Showering/bathing	1 (7.7)	1 (6.3)	3 (10.3)
Taking a test	–	3 (18.8)	2 (6.9)
In public transportation	1 (7.7)	1 (6.3)	2 (6.9)
Lying down	–	3 (18.8)	1 (3.4)
After H1N1 vaccination	–	–	2 (6.9)
Church	–	–	2 (6.9)
After a long day	–	2 (12.5)	–
Watching a show	–	–	2 (6.9)

Figure 2 Final version of the electronic cataplexy daily diary.

Figure 3 Final general version (A) and an alternative (research) version (B) of ESS-CHAD.

Abbreviation: ESS-CHAD, Epworth Sleepiness Scale for Children and Adolescents.