Spinal Muscular Atrophy Type I
Conditions
Keywords
Spinal Muscular Atrophy Type I, Oral Motor Function, Motor Function, Quality of Life
Brief summary
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.
Interventions
OTHERNeuro-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.
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.
OTHERBehavioral 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.
OTHERPediatric 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.
Sponsors
Zeynep HOŞBAY
Study design
Observational model
OTHER
Time perspective
CROSS_SECTIONAL
Eligibility
Sex/Gender
ALL
Age
2 Years to 4 Years
Healthy volunteers
No
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
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Neuro-sensory Motor Developmental Assessment | Once, beginning of the study | 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. |
| Functional Oral Intake Scale | Once, beginning of the study | 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. |
| Behavioral Pediatrics Feeding Assessment Scale | Once, beginning of the study | 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. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Pediatric Quality of Life Inventory | Once, beginning of the study | 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. |
Countries
Turkey (Türkiye)
Outcome results
None listed