The Effect of Spinal Orthosis on the Development of Scoliosis and Chest Deformity in Type I Spinal Muscular Atrophy

Philadelphia Children's Hospital Infant Test for Neuromuscular Disorders (CHOP-INTEND) will be used to evaluate motor functions. The CHOP INTEND is a functional scale that has been shown to reliably assess motor function in patients with infancy-onset Type I SMA and other neuromuscular disorders. The development of the scale is based on the natural history of motor function progression for infants with Type I SMA. The test contains 16 items that provide information on muscle strength and function, gravity eliminated, gravity assisted, and antigravity. Items are ranked from easy to difficult, with the least tolerated items being tested last.

• Scoring: rated from 0 (no response) to 4 (complete response). The maximum score is 64.
• Application time: It can be completed in 15-40 minutes.
• Equipment: mat, rattle, plush toy, toy phone.

Hammersmith Functional Motor Scale Extended Version (HFMSE) will be used to evaluate motor functions. The HFMSE test consists of 33 items in which movements such as sitting, rolling in the supine position, raising the head in the lying position, standing up, kneeling, standing and stepping, jumping, and climbing stairs are scored. Each item is evaluated on a scoring system with 2 points for "can do without support", 1 point for "can do with support" and 0 points for "can't".

The scores of all items are summed, the total score ranges from 0 to 66, and a higher score means better motor functions.

With the World Health Organization Motor Development Scale, 6 different gross motor levels will be evaluated as unsupported sitting, hand-knee crawling, assisted standing, assisted walking, standing alone and walking alone.

Chest deformity will be measured using a scoliometer (Basic Economics). The patient will be in the supine position with the head symmetrical. Scoliometer, 2nd-3rd in the upper part of the sternum. it will be located along the chest at the level of the rib (SATR upper) and below the sternum, where the corpus meets the xiphoid process of the sternum (SATR lower).

Pelvic tilt measurement will be made using a scoliometer (Basic Economics) placed at the posterior superior iliac spines in a modified sitting position with supported upper and lower extremities.

The Quebec Assistive Technology User Satisfaction Assessment Questionnaire (QUEST) will be used to assess spinal orthosis satisfaction. Since the patients are in childhood, the questionnaire will be filled in by their parents. Interviews will be conducted face to face. Responses are scored on a 5-point Likert scale for satisfaction (1=very dissatisfied and 5=very satisfied). The QUEST questionnaire has 3 points; these are device satisfaction, service satisfaction and total score. Satisfaction defined in the questionnaire is the individual's critical evaluation of the technological device. The individual's expectations, perception, attitude and personal characteristics affect this evaluation. The QUEST questionnaire contains 12 items questioning the satisfaction of the assistive device (8 items) and the service (4 items). At the end of the questionnaire, individuals/parents are asked to select and mark 3 of the 12 items as important.

Respiratory type, frequency and oxygen saturation will be examined. Respiratory type; It will be determined whether the patient has thoracic, abdominal, thoracoabdominal breathing or pursed-lip breathing. Respiratory frequency; It will be recorded as the number of respirations taken and exhaled per minute. Although the normal respiratory frequency is 12-20 respiratory rates/minute; <12 respiratory rate/minute is called bradypnea, >12 respiratory rate/minute is called tachypnea. Oxygen saturation measurement will be carried out by attaching the Contec brand CMS60D handheld pulse oximeter device, which is available at our university, to the children's toes before wearing the corset, while the corset is put on and 10 minutes after the corset is put on.