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Journal of Inborn Errors of Metabolism and Screening

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Original Article • J. inborn errors metab. screen. 4 • 2016 • https://doi.org/10.1177/2326409816669373 copy

An Adaptation of the Profile of Mood States for Use in Adults With Phenylketonuria

Authorship SCIMAGO INSTITUTIONS RANKINGS

Abstract

Adults with phenylketonuria (PKU) experience disturbances in mood. This study used qualitative and quantitative techniques to adapt the 65-item Profile of Mood States (POMS) for the assessment of key mood domains in adults with PKU. First, cognitive interviews on 58 POMS items (excluding 7 Friendliness domain items) among 15 adults and adolescents (age ≥16 years) with PKU were conducted to eliminate items poorly understood or considered irrelevant to PKU; 17 items were removed. Next, the remaining POMS items were quantitatively examined (Mokken scaling and Rasch analysis) in 115 adult patients with PKU. An additional 21 items were removed iteratively, resulting in the 20-item draft PKU-POMS. Finally, the psychometric properties of the draft PKU-POMS were examined. The instrument displayed strong psychometric properties (reliability, validity, and responsiveness) over 6 domains (Anxiety, Depression, Anger, Activity, Tiredness, and Confusion) and all items were well understood in the final cognitive interviews with 10 adults with PKU.

Keywords
phenylketonuria; profile of mood states; patient-reported outcome; psychometric; item reduction; cognitive interview

POMS Domain (Number of Items)	Items Removed
Vigor-activity (8)	Q56. Full of pep
	Q60. Carefree
	Q63. Vigorous
Confusion-bewilderment (7)	Q37. Muddled
	Q50. Bewildered
Tension-anxiety (9)	Q2. Tense
	Q10. Shaky
	Q16. On edge
	Q27. Restless
Fatigue-inertia (7)	Q11. Listless
	Q65. Bushed
Anger-hostility (12)	Q12. Peeved
	Q24. Spiteful
Depression-dejection (15)	Q9. Sorry for things done
	Q18. Blue
	Q23. Unworthy
	Q62. Guilty

		Day 1
Age, years
	n	115
	Mean (SD)	29.1 (8.8)
	Median (range)	28.0 (16—51)
Sex,n (%)
	Male	59 (51.3%)
	Female	56 (48.7%)
	Race, n (%)
American Indian or Alaska Native1 (0.9%)
	Black or African American	3 (2.6%)
	White or Caucasian	109 (94.8%)
	Other	2 (l.7%)
Plasma Phe level at day 1 (µmol/L)
	n	114
	Mean (SD)	1217 (389.0)
	Median (range)	1211 (510-2330)
ADHD RS-IV at day 1
	n	114
	Mean (SD)	9.9 (5.7)
	Median (range)	9.5 (0-26)

			Frequency, n (%)
Item	n	Mean (SD)	Not At All (Floor)	A Little	Moderately	Quite a Bit	Extremely (Ceiling)	Missing
Angry	15	1.1 (1.1)	40 (34.8%)	38 (33.0%)	27 (23.5%)	6 (5.2%)	4 (3.5%)
Worn out	15	2.1 (1.2)	11 (9.6%)	28 (24.3%)	29 (25.2%)	31 (27.0%)	16 (13.9%)
Unhappy	14	1.0 (1.0)	42 (36.5%)	47 (40.9%)	15 (13.0%)	7 (6.1%)	3 (2.6%)	1 (0.9%)
Lively	15	2.2 (l.0)	6 (5.2%)	21 (18.3%)	42 (36.5%)	33 (28.7%)	13 (11.3%)
Confused	15	1.0 (1.0)	47 (40.9%)	36 (31.3%)	22 (19.1%)	7 (6.1%)	3 (2.6%)
Sad	15	1.0 (1.1)	46 (40.0%)	39 (33.9%)	17(14.8%)	8 (7.0%)	5 (4.3%)
Active	15	2.4 (1.1)	4 (3.5%)	22 (19.1%)	33 (28.7%)	40 (34.8%)	16 (13.9%)
Grouchy	15	1.3 (1.2)	33 (28.7%)	40 (34.8%)	26 (22.6%)	8 (7.0%)	8 (7.0%)
Energetic	15	2.1 (1.1)	10 (8.7%)	23 (20.0%)	41 (35.7%)	28 (24.3%)	13 (11.3%)
Panicky	15	1.0 (1.2)	50 (43.5%)	33 (28.7%)	18 (15.7%)	8 (7.0%)	6 (5.2%)
Hopeless	15	0.5 (0.9)	80 (69.6%)	18 (15.7%)	13 (11.3%)	2 (1.7%)	2 (1.7%)
Relaxed	15	2.1 (1.1)	7 (6.1%)	28 (24.3%)	36 (31.3%)	34 (29.6%)	10 (8.7%)
Uneasy	15	1.2 (1.1)	37 (32.2%)	34 (29.6%)	29 (25.2%)	10 (8.7%)	5 (4.3%)
Unable to concentrate	15	1.5 (1.2)	28 (24.3%)	33 (28.7%)	26 (22.6%)	19 (16.5%)	9 (7.8%)
Fatigued	15	1.9 (1.2)	15 (13.0%)	37 (32.2%)	21 (18.3%)	3l (27.0%)	11 (9.6%)
Annoyed	15	1.8 (1.2)	17 (14.8%)	29 (25.2%)	36 (31.3%)	23 (20.0%)	10 (8.7%)
Discouraged	15	1.2 (1.2)	45 (39.1%)	33 (28.7%)	20(17.4%)	8 (7.0%)	9 (7.8%)
Resentful	15	0.5 (0.9)	75 (65.2%)	24 (20.9%)	12 (10.4%)	3 (2.6%)	1 (0.9%)
Nervous	15	1.6 (1.2)	26 (22.6%)	36 (31.3%)	23 (20.0%)	22 (19.1%)	8 (7.0%)
Lonely	14	0.9 (1.2)	62 (53.9%)	26 (22.6%)	12 (10.4%)	8 (7.0%)	6 (5.2%)	1 (0.9%)
Miserable	15	0.6 (1.1)	79 (68.7%)	18 (15.7%)	7(6.1%)	8 (7.0%)	3 (2.6%)
Cheerful	15	2.3 (1.0)	3 (2.6%)	20 (17.4%)	40 (34.8%)	39 (33.9%)	13 (11.3%)
Bitter	15	0.5 (0.8)	69 (60.0%)	31 (27.0%)	13 (11.3%)	2 (1.7%)
Exhausted	15	1.8 (1.3)	19 (16.5%)	33 (28.7%)	27 (23.5%)	22 (19.1%)	14 (12.2%)
Anxious	15	1.8 (1.2)	18 (15.7%)	33 (28.7%)	28 (24.3%)	23 (20.0%)	13 (11.3%)
Ready to fight	15	0.6 (1.0)	77 (67.0%)	20 (17.4%)	10(8.7%)	6 (5.2%)	2 (1.7%)
Gloomy	15	0.8 (1.1)	63 (54.8%)	30 (26.1%)	10(8.7%)	8 (7.0%)	4 (3.5%)
Desperate	15	0.3 (0.8)	91 (79.1%)	13 (11.3%)	8 (7.0%)	2 (1.7%)	1 (0.9%)
Sluggish	15	1.5 (1.3)	33 (28.7%)	30 (26.1%)	24 (20.9%)	20 (17.4%)	8 (7.0%)
Rebellious	15	0.6 (0.9)	69 (60.0%)	31 (27.0%)	8 (7.0%)	6 (5.2%)	1 (0.9%)
Helpless	15	0.4 (0.8)	86 (74.8%)	17 (14.8%)	7(6.1%)	4 (3.5%)	1 (0.9%)
Weary	15	0.8 (0.8)	50 (43.5%)	43 (37.4%)	20(17.4%)	l (0.9%)	1 (0.9%)
Alert	14	2.4 (1.0)	3 (2.6%)	19 (16.5%)	37 (32.2%)	42 (36.5%)	13 (11.3%)	1 (0.9%)
Deceived	14	0.5 (0.9)	83 (72.2%)	15 (13.0%)	9 (7.8%)	5 (4.3%)	2 (1.7%)	1 (0.9%)
Furious	14	0.7 (1.2)	77 (67.0%)	16 (13.9%)	11 (9.6%)	2 (1.7%)	8 (7.0%)	1 (0.9%)
Efficient	13	2.1 (1.1)	9 (7.8%)	23 (20.0%)	35 (30.4%)	38 (33.0%)	8 (7.0%)	2 (1.7%)
Bad-tempered	14	0.9 (1.1)	58 (50.4%)	27 (23.5%)	15 (13.0%)	l0 (8.7%)	4 (3.5%)	1 (0.9%)
Worthless	13	0.5 (0.9)	84 (73.0%)	12 (10.4%)	11 (9.6%)	6 (5.2%)		2 (1.7%)
Forgetful	14	1.6 (1.3)	24 (20.9%)	35 (30.4%)	30 (26.1%)	12 (10.4%)	13 (11.3%)	1 (0.9%)
Terrified	14	0.4 (0.7)	80 (69.6%)	24 (20.9%)	7(6.1%)	3 (2.6%)		1 (0.9%)
Uncertain about things	14	1.7 (1.3)	25 (21.7%)	25 (21.7%)	30 (26.1%)	22 (19.1%)	12 (10.4%)	1 (0.9%)

PKU-POMS Domain, Item		Standardized Coefficient or Model Fits (RMSEA, CFI, WRMR)^a a Model fit thresholds for acceptable fit included CFI =0.90, RMSEA =0.10, and WRMR =0.05.
Vigor-activity		0.14, 0.97, 0.53
	Lively	0.72
	Active	0.72
	Energetic	0.82
	Cheerful	0.57
	Alert	0.35
Tension-anxiety		0.17, 0.96, 0.66
	Panicky	0.79
	Relaxed	-0.35
	Uneasy	0.69
	Nervous	0.82
	Anxious	0.77
Confusion-bewilderment		0.00, 1.00, 0.26
	Confused	0.62
	Unable to concentrate	0.80
	Efficient	-0.28
	Forgetful	0.87
	Uncertain about things	0.59
Fatigue-inertia		0.09, 1.00, 0.39
	Worn out	0.84
	Fatigued	0.81
	Exhausted	0.90
	Sluggish	0.73
	Weary	0.30
Anger-hostility		0.12, 0.95,0.86
	Angry	0.70
	Grouchy	0.71
	Annoyed	0.79
	Resentful	0.70
	Bitter	0.70
	Ready to fight	0.76
	Rebellious	0.62
	Deceived	0.78
	Furious	0.84
	Bad-tempered	0.80
Depression-dejection		0.08, 0.99, 0.68
	Unhappy	0.78
	Sad	0.86
	Hopeless	0.85
	Discouraged	0.86
	Lonely	0.84
	Miserable	0.85
	Gloomy	0.85
	Desperate	0.77
	Helpless	0.75
	Worthless	0.93
	Terrified	0.56

		Initial MSA	Final MSA (Pre-Rasch Analysis)	Final MSA (Post-Rasch Analysis)
PKU-POMS Domain, Item		H Scaling Coefficient^b b Low scalability defined as H coefficient <0.40.	H Scaling Coefficient^b b Low scalability defined as H coefficient <0.40.	H Scaling Coefficient^b b Low scalability defined as H coefficient <0.40.
Tension-anxiety		Fit of item to domain	Fit of item to domain	Fit of item to domain
	Full-scale H	0.46 (0.06)	0.57 (0.06)	0.57 (0.06)
	Panicky	0.53 (0.06)	0.56 (0.07)	0.56 (0.07)
	Relaxed	0.28^c c Items removed after iteration 1 of MSA. (0.08)	-^c c Items removed after iteration 1 of MSA.	-^c c Items removed after iteration 1 of MSA.
	Uneasy	0.47 (0.07)	0.51 (0.07)	0.51 (0.07)
	Nervous	0.52 (0.06)	0.60 (0.06)	0.60 (0.06)
	Anxious	0.49 (0.06)	0.60 (0.06)	0.59 (0.06)
Vigor-activity
	Full-scale H	0.39 (0.06)	0.55 (0.07)	0.54 (0.07)
	Lively	0.43 (0.07)	0.49 (0.09)	0.48 (0.09)
	Active	0.41 (0.06)	0.55 (0.07)	0.54 (0.07)
	Energetic	0.47 (0.06)	0.61 (0.06)	0.60 (0.06)
	Cheerful	0.38 (0.07)	-^d d Items removed after iterations 2 and 3 of MSA.	-^d d Items removed after iterations 2 and 3 of MSA.
	Alert	0.24^c c Items removed after iteration 1 of MSA. (0.08)	-^c c Items removed after iteration 1 of MSA.	-^c c Items removed after iteration 1 of MSA.
Confusion-bewilderment
	Full-scale H	0.39 (0.06)	0.55 (0.07)	0.55 (0.07)
	Confused	0.40 (0.07)	0.49 (0.1)	0.49 (0.10)
	Unable to concentrate	0.47 (0.06)	0.58 (0.07)	0.58 (0.07)
	Efficient	0.21^c c Items removed after iteration 1 of MSA. (0.08)	-^c c Items removed after iteration 1 of MSA.	-^c c Items removed after iteration 1 of MSA.
	Forgetful	0.50 (0.05)	0.59 (0.07)	0.59 (0.06)
	Uncertain about things	0.37^c c Items removed after iteration 1 of MSA. (0.07)	-^c c Items removed after iteration 1 of MSA.	-^c c Items removed after iteration 1 of MSA.
Fatigue-inertia
	Full-scale H	0.53 (0.05)	0.65 (0.05)	0.67 (0.05)
	Worn out	0.59 (0.05)	0.69 (0.05)	0.71 (0.05)
	Fatigued	0.57 (0.05)	0.63 (0.06)	-^e e Items removed through Rasch analysis.
	Exhausted	0.62 (0.04)	0.69 (0.05)	0.68 (0.06)
	Sluggish	0.53 (0.06)	0.58 (0.06)	0.63 (0.06)
	Weary	0.23^c c Items removed after iteration 1 of MSA. (0.09)	-^c c Items removed after iteration 1 of MSA.	-^c c Items removed after iteration 1 of MSA.
Anger-hostility
	Full-scale H	0.47 (0.05)	0.53 (0.05)	0.612(0.06)
	Angry	0.46 (0.07)	0.51 (0.07)	0.63 (0.06)
	Grouchy	0.47 (0.06)	0.49 (0.07)	0.58 (0.08)
	Annoyed	0.55 (0.06)	0.57 (0.06)	0.63 (0.06)
	Resentful	0.41 (0.07)	-^d d Items removed after iterations 2 and 3 of MSA.	-^d d Items removed after iterations 2 and 3 of MSA.
	Bitter	0.43 (0.08)	-^d d Items removed after iterations 2 and 3 of MSA.	-^d d Items removed after iterations 2 and 3 of MSA.
	Ready to fight	0.47 (0.06)	0.49 (0.07)	-^e e Items removed through Rasch analysis.
	Rebellious	0.39^c c Items removed after iteration 1 of MSA. (0.08)	-^c c Items removed after iteration 1 of MSA.	-^c c Items removed after iteration 1 of MSA.
	Deceived	0.47 (0.07)	0.47 (0.07)	-^e e Items removed through Rasch analysis.
	Furious	0.51 (0.06)	0.56 (0.07)	-^e e Items removed through Rasch analysis.
	Bad-tempered	0.53 (0.05)	0.58 (0.05)	-^e e Items removed through Rasch analysis.
Depression-dejection
	Full-scale H	0.59 (0.05)	0.63 (0.05)	0.68 (0.06)
	Unhappy	0.58 (0.07)	0.61 (0.07)	0.66 (0.07)
	Sad	0.65 (0.05)	0.67 (0.05)	0.72 (0.06)
	Hopeless	0.62 (0.06)	0.65 (0.06)	-^e e Items removed through Rasch analysis.
	Discouraged	0.65 (0.06)	0.68 (0.06)	0.66 (0.06)
	Lonely	0.61 (0.06)	0.65 (0.06)	0.68 (0.06)
	Miserable	0.60 (0.07)	0.63 (0.07)	-^e e Items removed through Rasch analysis.
	Gloomy	0.63 (0.06)	0.64 (0.06)	-^e e Items removed through Rasch analysis.
	Desperate	0.50 (0.09)	0.51 (0.09)	-^e e Items removed through Rasch analysis.
	Helpless	0.52 (0.08)	0.51 (0.09)	-^e e Items removed through Rasch analysis.
	Worthless	0.65 (0.06)	0.67 (0.06)	-^e e Items removed through Rasch analysis.
	Terrified	0.32^c c Items removed after iteration 1 of MSA. > (0.07)	-^c c Items removed after iteration 1 of MSA.	-^c c Items removed after iteration 1 of MSA.

		Category Threshold Parameters
PKU-POMS Domain, Item		a1	a2	a3	a4	Fit Residual	Item Fit χ²	χ², P Value
A. Initial Rasch Model Parameters Item Fit of 31 Items Retained After Mokken Scaling Analysis^a a Items examined in Rasch analyses were those retained as acceptable items from Mokken scaling. ,^b b Separate Rasch analyses were conducted within each of the 6 domains.
Vigor-activity
	Lively	-2.45	-0.82	0.91	2.37	1.09	0.51	.77
	Active	-2.34	-0.30	0.79	2.95	0.63	1.92	.38
	Energetic	-2.62	1.05	0.74	1.82	-0.54	1.84	.40
Tension-anxiety
	Panicky	-0.73	0.24	1.03	1.46	-0.03	0.73	.70
	Uneasy	-1.27	-0.34	1.18	1.56	1.20	0.94	.62
	Nervous	-1.99	-0.36	-0.11	1.80	-0.12	2.41	.30
	Anxious	-2.51	-0.80	-0.20	1.01	-0.34	0.56	.76
Confusion-bewilderment
	Confused	-0.88	0.11	1.33	2.12	1.42	0.88	.64
	Unable to concentrate	-1.85	-0.51	0.20	1.26	-0.30	1.97	.37
	Forgetful^c c Indicates disordered thresholds.	-2.23	-0.61	0.73	0.33	-0.42	1.70	.43
Fatigue-inertia
	Worn out	-3.25	-0.85	-0.013	1.88	-0.28	2.38	.31
	Fatigued^c c Indicates disordered thresholds.	-2.70	0.02	-0.069	2.39	0.55	0.51	.78
	Exhausted	-2.08	-0.40	0.57	1.96	-0.44	2.69	.26
	Sluggish	-0.89	-0.042	0.71	2.76	1.27	1.45	.48
Anger-hostility
	Angry^c c Indicates disordered thresholds.	-1.78	-0.47	1.32	0.92	0.257	1.77	.41
	Grouchy^c c Indicates disordered thresholds.	-2.13	-0.56	0.83	0.06	1.3	1.24	.54
	Annoyed	-3.70	-1.56	-0.23	0.70	0.095	1.13	.57
	Ready to fight	0.12	0.32	0.34	1.89	-0.676	1.53	.47
	Deceived^c c Indicates disordered thresholds.	0.53	0.18	0.54	1.31	-0.161	0.90	.64
	Furious^c c Indicates disordered thresholds.	0.24	-0.04	1.62 -	1.19	-0.628	2.34	.31
	Bad-tempered	-0.85	-0.12	0.17	1.56	-1.232	4.42	.11
Depression-dejection
	Unhappy	-3.28	-0.31	0.09	1.32	0.87	0.73	.69
	Sad	-2.84	-0.80	0.03	0.70	-0.37	2.55	.28
	Hopeless^c c Indicates disordered thresholds.	-0.73	-0.68	2.16	1.03	-0.46	0.83	.66
	Discouraged^c c Indicates disordered thresholds.	-2.88	1.24	-0.11 -	0.31	-0.08	0.34	.84
	Lonely	-1.80	-0.53	-0.51	0.46	0.24	0.41	.81
	Miserable^c c Indicates disordered thresholds.	-0.79	-0.06	-0.53	1.29	-0.02	1.49	.48
	Gloomy^c c Indicates disordered thresholds.	-1.84	-0.20	-0.32	0.98	-0.13	1.75	.42
	Desperate^c c Indicates disordered thresholds.	0.04	-0.22	1.57	1.24	0.83	0.82	.66
	Helpless	-0.41	0.14	0.55	1.47	0.80	0.88	.65
	Worthless^c c Indicates disordered thresholds. -0.22 -0.72			0.66	7.59	-1.33	3.80	.15
B. Final Rasch Model Parameters and Item Fit of 20 Items inDraft PKU-POMS^b b Separate Rasch analyses were conducted within each of the 6 domains. ,^d d Items examined in Rasch analyses were a reduced set from those retained from both Mokken scaling and the first Rasch analysis.
Activity
	Lively	-2.45	-0.82	0.91	2.37	1.09	0.51	.77
	Active	-2.34	-0.30	0.79	2.95	0.63	1.92	.38
	Energetic	-2.62	1.05	0.74	1.82	-0.54	1.84	.40
Anxiety
	Panicky	-0.73	0.24	1.03	1.46	-0.03	0.73	.69
	Uneasy	-1.27	-0.34	1.18	1.56	1.20	0.94	.62
	Nervous	-1.99	-0.36	-0.11	1.80	-0.128	2.42	.30
	Anxious	-2.51	-0.80	-0.17	1.01	-0.34	0.56	.76
Confusion
	Confused	-0.76	0.24	1.548774	2.19	1.36	1.17	.56
	Unable to concentrate	-1.71	-0.38	0.365705	1.45	-0.41	1.98	.37
	Forgetful	-2.10	-0.45	0.336567	－^e e The response option for “extremely” was collapsed with “quite a bit” in final model.	-0.08	2.70	.26
Tiredness
	Worn out	-3.41	-0.92	-0.14	2.00	-0.11	3.13	.21
	Exhausted	-2.09	-0.47	0.42	2.08	0.32	0.89	.64
	Sluggish	-1.04	-0.19	0.75	3.00	1.07	0.55	.76
Anger
	Angry	-1.40	-0.05	2.00	3.19	0.05	1.52	.47
	Grouchy	-1.85	-0.04	0.86	－^e e The response option for “extremely” was collapsed with “quite a bit” in final model.	1.40	0.24	.89
	Annoyed	-2.9	1.25	0.12	1.68	-0.08	0.30	.86
Depression
	Unhappy	-2.53	0.14	0.86	2.76	0.74	1.10	.58
	Sad	-2.15	-0.22	0.54	2.04	-0.36	2.56	.28
	Discouraged	-2.05	-0.66	0.32	0.45	0.18	0.46	.79
	Lonely	-1.02	-0.14	0.02	1.65	0.19	1.00	.61

	Convergent Validity^a a Convergent validity assessed using values at day 1.						Responsiveness^b b Responsiveness assessed using change scores from day 1 to EOS.
PKU-POMS Domain	ADHD RS-IV			Plasma Phe			ADHD RS-IV			Plasma Phe
PKU-POMS Domain	N	Pearson’s r		N	Pearson’s r		N	Pearson’s r		N	Pearson’s r
Activity	114	-0.04		114	-0.06		65	-0.40^c c P < .05.		52	-0.25
Anxiety	114	0.33^d d P < .001.		114	0.09		65	0.43^d d P < .001.		52	0.34^c c P < .05.
Confusion	114	0.60^e e P < .0001.		114	0.09		65	0.52^e e P < .0001.		52	0.47^d d P < .001.
Tiredness	114	0.28^c c P < .05.		114	0.17		65	0.34^c c P < .05.		52	0.15
Anger	114	0.31^d d P < .001.		114	0.08		65	0.32^c c P < .05.		52	0.26
Depression	114	0.15		114	0.00		65	0.21		52	0.32^c c P < .05.

Latin American Society Inborn Errors and Neonatal Screening (SLEIMPN); Instituto Genética para Todos (IGPT) Rua Ramiro Barcelos, 2350, CEP: 90035-903, Porto Alegre, RS - Brasil, Tel.: 55-51-3359-6338, Fax: 55-51-3359-8010 - Porto Alegre - RS - Brazil
E-mail: rgiugliani@hcpa.edu.br

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