Feeding and Swallowing Problems in Spinal Muscular atrophy A cross-sectional descriptive study to investigate feeding, mastication and swallowing disorders in Dutch patients with Spinal Muscular Atrophy type II en III.

Feeding and Swallowing Problems in Spinal Muscular atrophy A cross-sectional descriptive study to investigate feeding, mastication and swallowing disorders in Dutch patients with Spinal Muscular Atrophy type II en III. - Feeding and Swallowing Problems in SMA.

Status

Active, not recruiting

Phases

Unknown

Study type

Observational

Source

NL-OMON

Registry ID

NL-OMON46697

Enrollment

Registered

2018-03-28

Start date

2018-08-22

Completion date

Unknown

Last updated

2024-04-15

For informational purposes only — not medical advice. Sourced from public registries and may not reflect the latest updates. Terms

Conditions

Dysphagia Swallowing Disorders

Interventions

None listed

Eligibility

Age

2 Years to 99 Years

Inclusion criteria

Inclusion criteria: 1) a clinical diagnosis of SMA type II or III, and a genetically confirmed homozygous SMN1 deletion; 2) patients with complaints of feeding, mastication and/or swallowing problems; 3) given oral and written informed consent when *16 years old; 4) given oral and written informed consent by the parents or legal representative and the patient in case of patients aged *12 till

Exclusion criteria

Exclusion criteria: 1) Severe SMA, defined as clinical types 0 and I; 2) Inability to meet study visits; 3) Total tube feeding.

Design outcomes

Primary

Measure	Time frame
a. LINMA, filled in by the patients (same as used in the Prevalence study, METC protocol no. 09-307), amendment 11: Swallowing function in SMA) in terms of no problems, mastication problems, swallowing problems or mastication and swallowing problems by the patients. b. VFSS: Feeding, mastication and swallowing problems are investigated in the oral transit phase, the pharyngeal phase and upper oesophageal phase of swallowing, using VFSS, with outcome variables: Formation of bolus pureed food (yes/no); duration of oral transit phase in liquids (sec.); piecemeal deglutition (yes/no); laryngeal aspiration/penetration (8-point scale);post-swallow residue in the hypopharynx (score 1-6); reduced UES opening (yes/no) and premature closure UES (yes /no). Two independent raters will judge the video of the VFSS (from at least 10 patients of the total amount of VFSS, randomly chosen) and the data will used to assess interrater reliability. c. Ultrasound oral and facial muscles, outcome measures: echogenicity and thickness of the muscles (quantitative, comparisons with normal values, z-scores); Heckmatt -score (qualitative, score 1 = normal * score 4 = severely increased echogenicity with absent bone reflection); presence of fasciculations (yes / no) (Heckmatt, 1982). Two independent raters will judge the ultrasound images (both quantitative and qualitative) (from at least 10 patients of the total amount of measurements, randomly chosen) and data will compared to assess interrater reliability. d. Active maximum mouth opening (aMMO), outcome measures: distance between upper en lower teeth plus overbite in mm*s. e. Timed Water Swallowing test (TWST), outcome measures: ml/sec (comparisons with normal values)(J.G.Kalf, 2011; Nathadwarawala, 1992) f. Swallowing Volume Test (SVT), outcome measures: max ml water swallowed in one swallow (comparisons with normal values) (Ertekin C, 1996; J.G.Kalf, 2011). g. Test for Observation of Mastication and Swallowing	—

Measure

Time frame

a. LINMA, filled in by the patients (same as used in the Prevalence study, METC protocol no. 09-307), amendment 11: *Swallowing function in SMA*) in terms of no problems, mastication problems, swallowing problems or mastication and swallowing problems by the patients. b. VFSS: Feeding, mastication and swallowing problems are investigated in the oral transit phase, the pharyngeal phase and upper oesophageal phase of swallowing, using VFSS, with outcome variables: Formation of bolus pureed food (yes/no); duration of oral transit phase in liquids (sec.); piecemeal deglutition (yes/no); laryngeal aspiration/penetration (8-point scale);post-swallow residue in the hypopharynx (score 1-6); reduced UES opening (yes/no) and premature closure UES (yes /no). Two independent raters will judge the video of the VFSS (from at least 10 patients of the total amount of VFSS, randomly chosen) and the data will used to assess interrater reliability. c. Ultrasound oral and facial muscles, outcome measures: echogenicity and thickness of the muscles (quantitative, comparisons with normal values, z-scores); Heckmatt -score (qualitative, score 1 = normal * score 4 = severely increased echogenicity with absent bone reflection); presence of fasciculations (yes / no) (Heckmatt, 1982). Two independent raters will judge the ultrasound images (both quantitative and qualitative) (from at least 10 patients of the total amount of measurements, randomly chosen) and data will compared to assess interrater reliability. d. Active maximum mouth opening (aMMO), outcome measures: distance between upper en lower teeth plus overbite in mm*s. e. Timed Water Swallowing test (TWST), outcome measures: ml/sec (comparisons with normal values)(J.G.Kalf, 2011; Nathadwarawala, 1992) f. Swallowing Volume Test (SVT), outcome measures: max ml water swallowed in one swallow (comparisons with normal values) (Ertekin C, 1996; J.G.Kalf, 2011). g. Test for Observation of Mastication and Swallowing

—

Secondary

Measure	Time frame
not apllicable.	—

Countries

Netherlands

Outcome results