Effects of a Disease Management Program in Individuals with Alpha-1 Antitrypsin Deficiency

Figures & data

Figure 1 Study flow diagram. The final analysis included 878 subjects who completed at least 22 monthly surveys and all health-related quality of life surveys.

Table 1 Baseline sociodemographic characteristics of the study cohort

Age, years	54.4 ± 9.6	Individual yearly income ($)
Age groups, years		< 20,000	24.8%
< 50	32.9%	20,000–40,000	30.3%
50–59	37.8%	40,000–60,000	20.9%
≥ 60	29.2%	60,000–80,000	11.9%
Gender, %		80,000–100,000	4.7%
Male	52.9%	> 100,000	7.4%
Female	47.1%
Ever used tobacco	82.3%	Actively working	32.3%
Tobacco pack-years	23.2 ± 14.5	Report disability, %	8.9%
Regular alcohol consumption
Never	28.7%	Report causing caregiver to miss workdays regularly, %	9.6%
< 1 drink/week	35.6%
> 1 drink/week	32.0%
Did not specify	3.6%	Report missing workdays regularly due to AATD, %	6.4%
Social status
Lives with partner only	55.7%
Lives alone	21.5%	Work impairment due to AATD (subjective scale 1–10)^†	2.63 ± 2.11, (2)
Lives with children only	3.5%
Lives with partner and children	14.8%
Did not answer	4.4%	Daily activities impairment due to AATD (subjective scale 1–10)^†	4.99 ± 2.58, (5)

*N = 878 subjects; results expressed as % of subjects or mean ± SD (median).

^†Scale range 1–10: 0: no impairment at all; 10: complete impairment.

AATD: alpha-1 antitrypsin deficiency.

Table 2 Baseline clinical characteristics of the study cohort

Clinical Features		Health-Related Quality of Life
BMI, %		SGRQ
< 20 kg/m^2	5.1%	Total	47.7 ± 17.5
20–24.9 kg/m^2	33.1%	Symptom	47.7 ± 22.8
25–29.9 kg/m^2	36.7%	Impact	36.8 ± 17.8
30–34.9 kg/m^2	15.3%	Activity	66.3 ± 21.7
> 34.9 kg/m^2	9.8%
FEV1 (L), mean ± SD^†	1.2 ± 0.7	SF-36
FEV1 (% predicted), mean ± SD^†	36.8 ± 16.9	Physical function	31.7 ± 10.1
FEV1/FVC (%), mean ± SD^†	42.8 ± 14.0	Role-physical	39.5 ± 11.2
GOLD stage, % ^†		General health	33.7 ± 11.8
Stage I	2.4%	Body pain	51.8 ± 11.2
Stage II	17.6%	Mental health	50.3 ± 11.0
Stage III	24.8%	Role-emotional	46.8 ± 11.9
Stage IV	55.1%	Social function	45.4 ± 12.3
Chronic bronchitis, N (%)	28.0%	Vitality	46.2 ± 11.5
Report co-morbidities ^‡		Physical Composite Summary	35.6 ± 9.8
none	34.4%	Mental Composite Summary	52.2 ± 11.6
1–3 co-morbidities	61%
≥4co-morbidities	4.5%

*N = 878; results expressed as % of subjects or mean ± SD.

^†N = 627 subjects with spirometry performed during study year 1.

^‡ Co-morbidities asked include: coronary artery disease, congestive heart failure, hypertension, diabetes mellitus, vascular problems (peripheral vascular disease and cerebrovascular accidents), peptic ulcer disease, connective tissue diseases, chronic renal problems and sinus allergies.

BMI: body mass index; GOLD: Global Initiative for COPD classification; FEV₁: forced expiratory volume in 1 second; FVC: forced vital capacity; SGRQ: St. George's Respiratory Questionnaire; SF-36: Short-Form 36 survey.

Table 3 Impact of ADMAP program on chronic COPD care.

	Pre-ADMAPP year	ADMAPP year	p-value
Maintenance Therapy
Short-acting bronchodilators	673 (76.7%)	673 (76.7%)	NS
Long-acting bronchodilators	561 (63.9%)	647 (73.7%)	< 0.001
Inhaled corticosteroids	599 (68.2%)	614 (69.9%)	NS
Theophylline	161 (18.3%)	144 (16.4%)	0.01
Antileukotrienes	157 (17.8%)	145 (16.5%)	NS
Systemic steroids	52 (5.9%)	38 (4.3%)	0.02
Long-Term Oxygen Therapy
N (%)	48.4%	53.4%	< 0.01
Average hours/day	15.6 ± 8.1	16.5 ± 7.9	< 0.01
% using > 16 h/day	52.1%	56.3%	< 0.01
Exercise practices during the follow-up year
Did not exercise regularly	456 (51.9%)	488 (55.6%)	NS
Exercised regularly (to the subject's limit)	399 (45.4%)	371 (42.3%)	NS
Started to exercise	23 (2.6%)	19 (2.2%)	NS

*N = 878; results expressed as % of subjects or mean ± SD.

NS = non-significant.

Table 4 Impact of ADMAP program on acute COPD care

	Pre-ADMAPP year	ADMAPP year	p-value
Management of acute exacerbations
Added nebulized bronchodilators (in addition to regular inhaled short-acting bronchodilator use)	37.1%	28.7%	<. 0001
Use of short course of systemic steroids	58.9%	64.5%	<. 0001
Took antibiotics	71.9%	70.2%	0.14
Behavior with acute exacerbations
Called primary care physician	17.5%	13.55%	< 0.001
Called pulmonary specialist	23.1%	22.0%	NS
Visited physician office	26.8%	30.6%	< 0.001
Went to an emergency room	5.4%	6.0%	NS
Self-treated exacerbation	28.7%	26.7%	NS

*N = 878; results expressed as % of subjects.

NS: non-significant.

Table 5 Frequency, severity and duration of exacerbations in the 12 months prior and after introduction of the ADMAP program (N = 878)

	Pre-ADMAPP year	ADMAPP year	p-value
Total Number of exacerbations	2125	1907
Exacerbations/subject/year
Median (Range)	2 (0–6)	2 (0–5)
Mean (SD)	2.42 ± 1.33	2.17 ± 1.32	< 0.001
By severity:
Mild (Mean, SD)	0.37 ± 0.67	0.29 ± 0.6	0.006
Moderate (Mean, SD)	1.78 ± 1.22	1.62 ± 1.2	< 0.001
Severe (Mean, SD)	0.27 ± 0.59	0.26 ± 0.57	NS
Duration of exacerbations, days (Mean, SD)
All	17.3 ± 11.0	16.6 ± 11.5	0.04
Time spent with exacerbations, N (%)
Never had	78 (8.88)	98 (11.16)	0.003
< 10% of days of the year	394 (44.87)	448 (51.03)
10–19% of days of the year	243 (27.68)	206 (23.46)
≥ 20% of days of the year	163 (18.56)	126 (14.35)

NS: non-significant.

Table 6 Impact of ADMAP program on healthcare utilization

	Pre-ADMAPP year	ADMAPP year	p-value
Regular physician visits
To primary care physician	3.2 ± 3.8	2.8 ± 3.6	< 0.01
To pulmonary specialist	3.1 ± 2.9	2.9 ± 2.8	NS
Unscheduled physician visits	1.96 ± 2.9	1.72 ± 2.7	0.03
Emergency room visits	0.39 ± 0.8	0.31 ± 0.8	0.02
Hospitalizations	0.22 ± 0.7	0.22 ± 0.6	NS

NS: Non-significant.

Figure 2 Saint George Respiratory Questionnaire variation during the study period according to age groups. The slopes of SGRQ changes for each of the study years are shown. The slopes were calculated by mixed model analysis using the baseline, 6th and 12th month measurement for year 1 and 12th, 18th and 24th month measurements for year 2 (intervention year). *: p < 0.05.

Figure 3 Proportion of subjects that improved SF-36 scores more than 10 points (1 standard deviation for the 1998 United States population) during the pre-ADMAPP and ADMAPP (intervention) year. *: p < 0.05. BP: Bodily Pain; MH: Mental Health; VT: Vitality; RE: Role impairment due to Emotional problems; SF: Social Function; RP: Role impairment due to Physical problems; GH: General Health; PF: Physical Functioning; PCS: Physical Component Summary; MCS: Mental Component Summary.

Figure 4 Participant self-satisfaction survey after the ADMAPP (intervention) year.