Session Information
Session Type: ACR Poster Session C
Session Time: 9:00AM-11:00AM
Background/Purpose: Pediatric localized sclerodermaÕs (LS) effects on patient/family well-being are underexplored. Contributions of HRQoL domains and family dynamics to LSÕs impact on patients has not yet been examined. This study evaluates correlates of LS patient/parent global assessments of disease impact (Pt-GA; Par-GA).
Methods: Cross-sectional data from the National Registry for Childhood Onset Scleroderma (NRCOS) spanning 2015-2016 were used. Pt-GA/Par-GA were scored on a 100mm visual analogue scale. Clinical data (i.e. modified Localized Scleroderma Skin Index [mLoSSI], Localized Scleroderma Damage Index [LoSDI], physician global assessment of disease activity/damage [PGA-A; PGA-D]) were examined. Patients completed PedsQL-Generic Core Scales and Rheumatology Module (PedsQL-GC/-RM). Parents completed PedsQL-Family Impact Module (PedsQL-Fam). ChildrenÕs Dermatology Life Quality Index (CDLQI) assessed skin-specific HRQoL. Descriptive statistics, HRQoL score distributions, and SpearmanÕs correlations (p<0.05) were evaluated for Pt-GA, Par-GA, activity/damage, PedsQL-GC/-RM/-Fam (and individual domains), and CDLQI.
Results: Table 1 summarizes clinical/HRQoL data. Pt-GA/Par-GA were correlated (0.67, p<0.001), with Par-GA greater than Pt-GA by paired samples Wilcoxon (p=0.007). Pt-GA/Par-GA did not correlate with mLoSSI, LoSDI, PGA-A, or PGA-D. Pt-GA/Par-GA correlated comparably with CDLQI (0.44, p =0.001). Pt-GA correlated with PedsQL-GC/-Fam (-0.35, p=0.012; -0.45, p=0.001). Par-GA correlated with PedsQL-Fam (-0.49, p<0.001), not PedsQL-GC. Pt-GA correlated with PedsQL-GC psychosocial health summary score (-0.37, p=0.008), and social/school domains(-0.41, p=0.003; -0.37, p=0.007). Pt-GA correlated with PedsQL-RM worry (-0.35; p=0.011) and treatment domains (-0.28, p=0.047). Par-GA correlated with PedsQL-GC social domain (-0.32, p=0.023) and weakly with PedsQL-RM worry domain (-0.28, p=0.047). Par-GA correlated with all PedsQL-Fam domains (-0.36 to -0.51, p<0.009), as did Pt-GA for all but the cognitive domain (-0.32 to -0.52, p<0.023). CDLQI items most often scored > 0 were about teasing (25%), embarrassment (23%), treatment burden (21%), and itch/pain (17%). Table 2 lists PedsQL items showing most frequent HRQoL impact.
| Table 1: Patient Characteristics (n = 52) | |||||
| Demographics |
|
|
|||
| Female, n (%) |
35 |
67 |
|||
| Caucasian, n (%) |
46 |
89 |
|||
| Age at study visit, median, IQR |
12.8 |
10.4-15.4 |
|||
| Age at onset, median, IQR |
7.2 |
3.8-9.6 |
|||
| Time from onset to diagnosis, median, IQR |
1.1 |
0.5-2.4 |
|||
| LS Subtype, n (%) |
|
||||
| Linear Trunk/Limb |
14 |
27 |
|||
| Linear Face/Scalp |
17 |
33 |
|||
| Generalized Morphea |
6 |
11 |
|||
| Circumscribed Morphea, Superficial |
4 |
8 |
|||
| Circumscribed Morphea, Deep |
2 |
4 |
|||
| Eosinophilic Fasciitis |
1 |
2 |
|||
| Pansclerotic Morphea |
1 |
2 |
|||
| Mixed Subtype |
7 |
13 |
|||
| On Systemic Immunosuppressive Therapy, n (%) |
37 |
88 |
|||
| Clinical measures (median, IQR) |
|
||||
| Cutaneous Activity/Damage |
|
||||
| mLoSSI |
0 |
0-1 |
|||
| LoSDI |
6 |
3.3-18.5 |
|||
| Physician Global Assessments |
|
||||
| PGA-A |
0 |
0-0 |
|||
| PGA-D |
32.5 |
22.3-39.8 |
|||
| Patient/Parent-Reported Measures (median, IQR) |
|
||||
| Patient/Parent Global Assessments |
|
||||
| Pt-GA |
4 |
0-12 |
|||
| Par-GA |
10 |
0-23 |
|||
| PedsQL-GC Total Score |
93.3 |
83.5-97.9 |
|||
| PedsQL-RM – Pain |
100 |
81.3-100 |
|||
| PedsQL-RM – Daily Activities |
100 |
100-100 |
|||
| PedsQL-RM – Treatment |
89.3 |
70.2-100 |
|||
| PedsQL-RM – Worry |
91.7 |
83.3-100 |
|||
| PedsQL-RM – Communication |
100 |
83.3-100 |
|||
| PedsQL-Fam Total Score |
90 |
71.4-96.2 |
|||
| CDLQI |
1 |
0-2 |
|||
| Table 2: PedsQL-GC/-RM/-Fam Items Most Frequently Scored < 75 (i.e. Sometimes/Often/Almost Always) | |||
| Scale/Item | Domain |
n |
% |
| PedsQL-GC |
|
||
| I have low energy | Physical Functioning |
13 |
25 |
| I miss work or school to go to the doctor or hospital | School Functioning |
24 |
46 |
| PedsQL-RM |
|
||
| I ache or hurt in my joints and/or muscles | Pain and Hurt |
14 |
27 |
| My medicines make me feel sick | Treatment |
13 |
25 |
| It is hard to be responsible for my medicines or physical therapy | Treatment |
15 |
29 |
| I get scared when I have to have blood tests | Treatment |
12 |
23 |
| I worry about my illness | Worry |
12 |
23 |
| It is hard for me to explain my illness to other people | Communication |
14 |
27 |
| PedsQL-Fam |
|
||
| I feel tired during the day | Physical Functioning |
14 |
27 |
| I feel tired when I wake up in the morning | Physical Functioning |
15 |
29 |
| I get headaches | Physical Functioning |
11 |
21 |
| I feel anxious | Emotional Functioning |
24 |
46 |
| I feel sad | Emotional Functioning |
19 |
37 |
| I feel frustrated | Emotional Functioning |
18 |
35 |
| It is hard for me to keep my attention on things | Cognitive Functioning |
12 |
23 |
| I feel that others do not understand my family’s situation | Communication |
13 |
25 |
| I worry about whether or not my child’s medical treatments are working | Worry |
32 |
62 |
| I worry about the side effects of my child’s medications/medical treatments | Worry |
38 |
73 |
| I worry about how others will react to my child’s condition | Worry |
21 |
27 |
| I worry about how my child’s illness is affecting other family members | Worry |
13 |
25 |
| I worry about my child’s future | Worry |
32 |
62 |
| Stress or tension between family members | Family Relationships |
11 |
21 |
Conclusion: Pt-/Par-GA correlate with measures of HRQoL and family impact, but not physician-scored disease activity/damage. Psychosocial function, child/parent worries, treatment burden, and family impact should be routinely measured as they may more fully explain LSÕs impact than cutaneous manifestations.
To cite this abstract in AMA style:
Ardalan K, Schollaert-Fitch K, Zigler CK, Zidek MA, Torok KS. Patient and Parent Global Assessments of Disease Impact in Pediatric Localized Scleroderma: Correlates of Patient Reported Health-Related Quality of Life and Parent Reported Family Impact Domains [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/patient-and-parent-global-assessments-of-disease-impact-in-pediatric-localized-scleroderma-correlates-of-patient-reported-health-related-quality-of-life-and-parent-reported-family-impact-domains/. Accessed .« Back to 2016 ACR/ARHP Annual Meeting
ACR Meeting Abstracts - https://acrabstracts.org/abstract/patient-and-parent-global-assessments-of-disease-impact-in-pediatric-localized-scleroderma-correlates-of-patient-reported-health-related-quality-of-life-and-parent-reported-family-impact-domains/