Factors associated with Noninvasive ventilation compliance in patients with ALS/MND

Figures & data

Table 1 Addressing the issues leading to intolerance.

Problem	Intervention
Air Leaks Around the Mouth	Chin strap, Full Face Mask
Feeling of Claustrophobia	Nasal pillows, zolpidem
Feeling of Air Blowing on Face	Reduce the IPAP by 1cm
Patients “Fighting” the Machine	Change the rise and/or the % of time in IPAP
Stuffy Nose	Steroid inhaler, heated humidifier, saline nasal spray
Secretions	Anticholinergics, guaifenesin, improve hydration, nebulizer treatments

Table 2 Baseline demographics and disease characteristics for the entire population overall and by group.

	Total	Group 1	Group 2
Number of Subjects	73	47	26
Age (Years)	59.4 (35.8, 82.0)	56.3 (35.8, 82.0)	62.9 (40.6, 78.5)
Gender, N (%)
F	27 (37.0%)	17 (36.2%)	10 (38.5%)
M	46 (63.0%)	30 (63.8%)	16 (61.5%)
BMI	25.97 (18.83, 40.26)	25.97 (18.83, 38.77)	26.00 (20.66, 40.26)
Duration of Disease from Onset to Baseline		11.8 ± 13.0	11.0 ± 10.9
Site of Onset N (%)
Bulbar	15 (20.5%)	8 (17.0%)	7 (26.9%)
Extremity	58 (79.5%)	47 (83.0%)	19 (63.1%)
Sitting FVC (% expected)	82.0 (43.0, 96.0)	89.0 (74.0, 96.0)	61.0 (43.0, 76.0)
Household Income N (%)
<$30,0000	18 (25.0%)	10 (21.7%)	8 (30.8%)
$30–50,000	17 (23.6%)	10 (21.7%)	7 (26.9%)
>50,000	37 (51.4%)	26 (56.5%)	11 (42.3%)
Education
Elementary	3 (4.1%)	1(2%)	2 (7.7%)
High School	22 (30.1%)	12 (25.5%)	10 (38.5%)
Associates	17 (23.3%)	12 (25.5%)	5 (19.2%)
Bachelors	16 (21.9%)	10 (21.3%)	6 (23.1%)
Masters	9 (12.3%)	6 (12.8%)	3 (11.5%)
Doctorate	6 (8.2%)	6 (12.8%)	0
ALS-FRS-R	35.5 (15.0, 45.0)	37.0 (22.0, 45.0)	34.0 (15.0, 43.0)
ALS-FRS Respiratory subscore Mean (SD)	10.44 (1.37)	10.85 (1.21)	9.73 (1.37)
PSQI	7.5 (2.0, 19.0)	7.0 (2.0, 17.0)	9.0 (2.0, 19.0)
ESS	5.0 (0.0, 17.0)	4.50 (0.0, 16.0)	5.50 (1.0, 17.0)
Follow up (days)	333 (0.0, 371)	336 (0.0, 371)	251 (0, 347)
Number of Visits	7 (1, 10)	7 (1, 9)	5.5 (1.0, 10.0)

Table entries are median (range) or count (%); data was obtained on all subjects except where n is indicated.

Table 3 Compliance over time overall and by group.

	28 Days	56 Days	84 Days	140 Days	168 Days	196 Days	224 Days	252 Days
Total % (n)
Compliant	52.3% (23)	53.2% (25)	57.8% (26)	61% (25)	61% (25)	56.7% (21)	74% (20)	66.7% (12)
Non-Compliant	47.7% (21)	46.8% (22)	42.2% (19)	39% (16)	39% (16)	43.3% (16)	26% (7)	32.3% (6)
Group 1 % (n)
Compliant	53.6% (15)	53.6% (15)	57.1% (16)	56% (14)	53.8% (14)	54.2% (13)	68.4% (13)	61.5% (8)
Non-Compliant	46.4% (13)	46.4% (13)	42.9% (12)	44% (11)	46.2% (12)	45.8% (11)	31.6% (6)	38.5% (5)
Group 2 % (n)
Compliant	50% (8)	52.6% (10)	58.8% (10)	68.8% (11)	73.3% (11)	61.5% (8)	87.5% (7)	80% (4)
Non-complaint	50% (8)	47.4% (9)	41.2% (7)	31.2% (5)	26.7% (4)	38.5% (5)	12.5% (1)	20 % (1)

Table 4 Variables associated with long term compliance.

	Compliant (n = 24)	Noncompliant (n = 23)	p-value
Group, N (% compliant or non-compliant)			1.00
1	15 (62.5%)	15 (65.2%)
2	9 (37.5%)	8 (34.8%)
Age			0.16
Median (Range)	58.0 (47.1, 77.6)	54.3 (35.8, 82.0)
Gender, N (%)			0.14
F	7 (29.2%)	12 (52.2%)
M	17 (70.8%)	11 (47.8%)
Site of Onset, N (%)			1.00
Bulbar	5 (20.8%)	5 (21.7%)
Limb	19 (79.2%)	18 (78.3%)
Marital Status			0.048
Married	23 (95.8%)	17 (73.9%)
Other	1 (4.2%)	6 (26.1%)
TotYrEd, N			0.002
Median (Range)	16.0 (12.0, 20.0)	12.0 (8.0, 20.0)
HousInc, N (%)			0.001
$30,000–$5	4 (16.7%)	8 (36.4%)
<$30,000	2 (8.3%)	9 (40.9%)
>$50,000	18 (75.0%)	5 (22.7%)
Slope FVC,			0.59
Median (Range)/month	–1.9 (–7.2, 0.3)	–2.3 (–8.1, 2.3)
Slope MIP,			0.48
Median (Range)/month	–2.0 (–6.1, 2.3)	–0.97 (–8.4, 1.7)
Slope ALSFRS, N	22	20	0.83
Median (Range)/month	–1.2 (–2.5, −0.1)	–1.14 (–2.3, 0.1)
Slope FRS Resp10–12, N	22	20	0.34
Median (Range)/month	–0.5 (–0.9, 0.1)	–0.3 (–0.8, 0.0)
Slope PSQI Total N			0.036
Median (Range)/month	–0.2 (–1.01, 0.75)	0.1 (–0.7, 1.5)
Slope ESS Total,			0.84
Median (Range)/month	0.0 (–0.7, 1.5)	0.0 (–0.6, 2.1)

Table entries are median (range) or count (%); data was obtained on all subjects except where n is indicated.

The bolded values are the variables that were significant with a p value <0.05.

Table 5 Symptoms associated with NIV use (scale of 0–10) and perceived impact on QOL.

Symptom	Percentage of Subjects with Symptom				Symptom Severity Mean (SD) of Severity on scale 1–10
	Overall	NIV Compliant	NIV Noncompliant	P Compliant vs Noncompliant	Overall	NIV Compliant	NIV Noncompliant	P Compliant vs Noncompliant
Excessive Dryness nose, throat	68.4	74.1	63.1	p = 0.08	6.3 (3.3)	6.8 (3.0)	5.8 (3.5)	p = 0.06
Air leakage from mask	63.7	67.6	59.6	p = 0.16	4.5 (3.41)	4.3 (2.7)	4.7 (3.2)	p = 0.33
Frequent night arousals	59.6	55.6	63.3	p = 0.23	4.56 (3.2)	4.1 (3.2)	5.0 (3.2)	p = 0.06
Stuffed, congested blocked nose	58.6	58.7	58.3	p = 0.97	3.9 (2.8)	3.9 (2.6)	4.3 (3.0)	p = 0.40
Discomfort from mask	56.7	51.7	63.6	p = 0.07	4.4 (2.93)	4.23 (2.75)	4.57 (3.1)	p = 0.49
Marks or rash on the face	42.7	49.3	34.5**	p = 0.02	6.1 (3.42)	6.3 (3.45)	5.78 (3.4)	p = 0.47
Runny nose	40.9	60.4	36.7.8	p = 0.64	4.1 (3.4)	3.6 (3.2)	4.6 (3.5)	p = 0.14
Soreness of nose or throat	37.9	34.9	40.3	p = 0.36	4.2 (3.2)	4.5 (3.1)	4.0 (3.4)	p = 0.59
Sense of suffocation or claustrophobia	35.4	32.4	38.7	p = 0.25	3.5 (2.8)	2.6 (1.9)	4.3 (3.3)*	p = 0.004
Difficulty getting back to sleep	32.2	45.3	16.8	p = 0.58	3.8 (2.9)	3.2 (2.4)	4.5 (3.1)*	p = 0.03
Difficulty with pressure of air on face	27.9	21.1	34.7**	p = 0.02	3.7 (1.9)	3.0 (1.8)	4.3 (3.0)*	p = 0.03
Eye Irritation	20.9	24.5	16.8	p = 0.19	3.7 (2.8)	3.2 (2.6)	4.5 (2.9)	p = 0.09
Headaches	20.2	28.8	17.5	p = 0.31	2.8 (2.6)	2.6 (2.3)	2.9 (3.0)	p = 0.74
Difficulty for caregiver to help due to time/availability	16.9	18.3	13.6	p = 0.24	4.0 (2.9)	3.3 (1.8)	5.0 (3.8)*	p = 0.05
Complaints from your partner about noise	14.2	21.4	4.3**	p < 0.0001	3.2 (2.2)	3.4(2.3)	2.2 (1.6)	p = 0.14
Ear pain	11.5	17.5	4.2 **	p < .001	2.4 (1.4)	2.3 (1.3)	3.0 (1.6)	p = 0.40
Bloating	10.7	15.5	3.4**	p = 0.002	3.8 (2.7)	4.0 (2.9)	3.8 (2.7)	p = 0.24
Percent of Users Sensing Improvement in QOL and the Level of Impact on QOL
	Overall	NIV Compliant	NIV Noncompliant
% NIV users Feeling Improved QOL	73.8%	74%	72%
Impact (0–10)	5.6	6.0	5.0*

*Indicates significant difference in mean score of severity between compliant and noncompliant at p < 0.05.

**Indicates a significant difference in the percentage of compliant vs noncompliant subjects complaining of the symptom (chi square).