Impact of Motor and Oral Motor Function on Quality of Life in Children With SMA

Impact of Motor and Oral Motor Function on Quality of Life in Children With Type I Spinal Muscular Atrophy

The aim of this study is to investigate the impact of motor and oral motor functions on the quality of life in children aged 2-4 years diagnosed with Spinal Muscular Atrophy (SMA) Type I. In the study, the Neuro-Sensory-Motor Developmental Assessment (NSMDA) was used to evaluate motor functions, the Functional Oral Intake Scale (FOIS) to assess oral motor function, the Behavioral Pediatric Feeding Assessment Scale (BPFAS) to evaluate children's attitudes towards feeding, and the Pediatric Quality of Life Inventory (PedsQL) Neuromuscular Module to assess quality of life.

Study Overview

• Status: Completed
• Conditions: Spinal Muscular Atrophy Type I
• Intervention / Treatment: Other: Neuro-sensory Motor Developmental Assessment; Other: Functional Oral Intake Scale; Other: Behavioral Pediatrics Feeding Assessment Scale; Other: Pediatric Quality of Life Inventory

• Study Type: Observational
• Enrollment (Actual): 23

Contacts and Locations

Study Locations

• Turkey
  • Istanbul, Turkey, 34010
    • Biruni University

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: Child
• Accepts Healthy Volunteers: No

• Sampling Method: Probability Sample

Study Population

The study was completed with 23 children between the ages of 2-4 who were followed up with the diagnosis of Spinal Muscular Atrophy (SMA) Type 1 and whose parents volunteered to participate in the study.

Description

Inclusion Criteria:

• Diagnosed with Spinal Muscular Atrophy Type I
• Being between the ages of 2-4
• Families volunteering to participate in the study

Exclusion Criteria:

• Accompanied by another chronic disease other than SMA
• The mother or father has a cognitive problem that prevents them from expressing themselves
• The mother or father is illiterate

Study Plan

How is the study designed?

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Neuro-sensory Motor Developmental Assessment	The test used to assess children's development levels evaluates the child's development in six parameters: gross motor function, fine motor function, neurological status, infant movement patterns, postural development and sensory-motor function (tactile, proprioceptive, ocular and vestibular systems) on a scale of 1 to 5. High scores are interpreted as motor dysfunction.	Once, beginning of the study
Functional Oral Intake Scale	It is a scale consisting of a total of 7 levels and two sections, developed by Crary et al. to indicate the functional oral intake of patients with dysphagia. A higher score indicates a better nutritional level.	Once, beginning of the study
Behavioral Pediatrics Feeding Assessment Scale	The scale used to determine eating problems in children evaluates both the child's nutritional status and the parents' feelings about the child's nutritional status. It consists of 35 items in total and is scored from 1 to 5. High scores indicate problematic eating behaviors and habits.	Once, beginning of the study

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Pediatric Quality of Life Inventory	The PedsQL assesses health-related quality of life in children ages 2 to 18 years with chronic illnesses from both the child's and parents' perspectives. It consists of 25 items and 3 categories (About My Child's Neuromuscular Disease, Communication, About Our Family Resources). The Neuromuscular Module was used to assess the quality of life of the children in our study and was answered by the parents only. The scale is scored from 0 to 4, with higher scores indicating better quality of life.	Once, beginning of the study

Collaborators and Investigators

• Sponsor: Zeynep HOŞBAY

Study record dates

Study Major Dates

• Study Start (Actual): May 30, 2024
• Primary Completion (Actual): June 15, 2024
• Study Completion (Actual): August 30, 2024

Study Registration Dates

• First Submitted: March 4, 2025
• First Submitted That Met QC Criteria: March 4, 2025
• First Posted (Actual): March 25, 2025

Study Record Updates

• Last Update Posted (Actual): March 25, 2025
• Last Update Submitted That Met QC Criteria: March 4, 2025
• Last Verified: March 1, 2025

More Information

Terms related to this study

• Keywords: Quality of Life; Motor Function; Spinal Muscular Atrophy Type I; Oral Motor Function
• Additional Relevant MeSH Terms: Neurologic Manifestations; Central Nervous System Diseases; Nervous System Diseases; Neuromuscular Manifestations; Pathological Conditions, Anatomical; Neuromuscular Diseases; Genetic Diseases, Inborn; Neurodegenerative Diseases; Heredodegenerative Disorders, Nervous System; Spinal Cord Diseases; Motor Neuron Disease; Atrophy; Muscular Atrophy; Muscular Atrophy, Spinal; Spinal Muscular Atrophies of Childhood
• Other Study ID Numbers: Oral Motor Functions in SMA

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No