Health Related Quality of Life in Children with Thoracic Insufficiency Syndrome
b - Synthes Spine
c - Synthes Spine
Purpose: In contrast to children with adolescent idiopathic scoliosis, children with early onset scoliosis often have significant comorbidities and can rapidly develop thoracic insufficiency and pulmonary compromise which has a profound effect on their lives. The traditional surgical or non-surgical techniques to treat Thoracic Insufficiency Syndrome (TIS) are not able to stabilize or improve chest wall size or pulmonary function while allowing spine growth. To this end, Vertical Expandable Prosthetic Titanium Rib (VEPTR) was specifically designed to treat TIS by allowing growth of the thoracic cavity and control/correction of spine deformity. The purpose of this study is to compare quality of life (QOL) of children with TIS, prior to implantation of VEPTR, with previously published QOL of healthy children as well as to that of children with other diseases.
Method: As part of the original multicenter evaluation of the VEPTR, Child Health Questionnaire (CHQ) was collected preoperatively on forty five patients who were subsequently treated with expansion thoracoplasty using the VEPTR. The average age was 8.2±2.6 and parent form of the CHQ was filled out by the primary caretaker. Patients were divided in to three broad diagnostic categories; Rib Fusion (RF, N=15), Hypoplastic Thorax Syndromes (HT, N=17) and Progressive Spinal Deformity (PS, N=13).
Results: There were significant differences between the study patients and healthy children in Physical Functioning, Role/Social Limitations-Physical, General Health Perceptions, Parental Impact-Emotional, Parental Impact-Time, Family Activities, Physical Summary, and Psychosocial Summary (Table).
Discussion: Patients with thoracic insufficiency syndrome have significant and profound perturbations in QOL when compared with other children. These scores are among the lowest observed in pediatric populations. Current efforts are underway to better understand the clinical features that have the most profound effects on the life of these children. Finally, these data will serve as an important baseline on ongoing studies of these patients after expansion thoracoplasty.
|
RF Mean (SD)
|
HT Mean (SD)
|
PS Mean (SD)
|
Norms Mean (SD)
|
|
| Physical Functioning |
81.3
(22.7)* |
66.0
(30.1)** |
53.5
(37.0)** |
96.1 |
| Role/Social Limitations - Physicial |
97.8
(8.6) |
67.6
(37.0)* |
74.4
(40.6) |
93.6 |
| General Health Perceptions |
627
(20.1) |
45.8
(19.9)*** |
50.1
(18.9)** |
73.0 |
| Parental Impact - Emotional |
52.3
(29.9)** |
57.8
(27.0)** |
46.8
(29.8)** |
80.3 |
| Parental Impact - Time |
56.3
(23.6)*** |
54.7
(24.7)*** |
60.4
(20.8)** |
87.8 |
| Family Activities |
78.3
(19.7)* |
74.7
(24.7)* |
77.4
(28.5) |
89.7 |
| Physical Summary |
47.4
(8.3)* |
32.2
(20.1)** |
30.2
(14.7)** |
53.0 |
| Psychosocial Summary |
45.4
(5.9)** |
48.9
(6.1) |
47.1
(8.3) |
51.2 |
*p<.05.
**p<.01.
***p<.001