Chewing and Swallowing Training Program in Coffin-Lowry Syndrome

Sponsor

Hacettepe University (Other)

Overall Status

Completed

CT.gov ID

NCT04199546

Collaborator

(none)

Enrollment

Location

Arm

6.2

Actual Duration (Months)

0.2

Patients Per Site Per Month

Study Details

Study Description

Brief Summary

This study will report the chewing and swallowing disorders of a Coffin-Lowry Syndrome (CLS) patient, and effects of chewing and swallowing training on chewing and swallowing function.

Condition or Disease	Intervention/Treatment	Phase
Swallowing Disorder Chewing Problem	Other: Chewing and swallowing rehabilitation	N/A

Detailed Description

Patients with CLS suffer from spinal deformities, nonconvulsive status epilepticus, obstructive sleeep apnea syndrome, pneumonia, stimulus-induced myoclonus, cases of falls, mechanical ventilation, restrictive lung disease. It was also reported that patients with CLS had problems with eating and feeding functions. However there is no study investigating chewing and swallowing functions of this patient population. Therefore, the investigators have three main purposes:

To chewing and swallowing function in a boy with CLS diagnosis who was referred due to coughing during eating, long-lasting wheezing, sputum and inability to intake solid food
To investigate the effects of two months of chewing and swallowing training program
To present long term follow-up effects on chewing and swallowing function.

Study Design

Study Type:

Interventional

Actual Enrollment :

1 participants

Allocation:

N/A

Intervention Model:

Single Group Assignment

Masking:

None (Open Label)

Primary Purpose:

Treatment

Official Title:

Chewing and Swallowing Training Program in Coffin-Lowry Syndrome: A Case Report

Actual Study Start Date :

Dec 12, 2019

Actual Primary Completion Date :

Jun 19, 2020

Actual Study Completion Date :

Jun 19, 2020

Arms and Interventions

Arm	Intervention/Treatment
Other: A child with Coffin-Lowry Syndrome A boy with a known CLS diagnosis with a history of coughing during eating, long-lasting wheezing, sputum and inability to intake solid food will be included.	Other: Chewing and swallowing rehabilitation A home based chewing and swallowing training program (CST) will be applied by an experienced physical therapist. The Functional Chewing Training will be used to improve chewing function which has five steps including positioning the child and food, sensory stimulation, chewing training and adjustment of food consistency to improve chewing function. To support hyolaryngeal elevation, laryngeal mobilization will be performed. Hyolaryngeal mobilization will be performed by moving hyolaryngeal complex in right, left and up directions manually.

Arm

Intervention/Treatment

Other: A child with Coffin-Lowry Syndrome

A boy with a known CLS diagnosis with a history of coughing during eating, long-lasting wheezing, sputum and inability to intake solid food will be included.

Other: Chewing and swallowing rehabilitation

A home based chewing and swallowing training program (CST) will be applied by an experienced physical therapist. The Functional Chewing Training will be used to improve chewing function which has five steps including positioning the child and food, sensory stimulation, chewing training and adjustment of food consistency to improve chewing function. To support hyolaryngeal elevation, laryngeal mobilization will be performed. Hyolaryngeal mobilization will be performed by moving hyolaryngeal complex in right, left and up directions manually.

Outcome Measures

Primary Outcome Measures

Instrumental swallowing evaluation [6 months]
Videofluoroscopic Swallowing Evaluation (VFSE) will be performed. The swallowing physiology will be evaluated by using 3 ml of liquid and pudding barium during the VFSE. The Penetration and Aspiration Scale (PAS) will be used to determine penetration and aspiration severity, which is scored between 1 to 8. The score of PAS 1 means 'No aspiration', scores from 2 to 5 indicate 'Penetration', and scores from 6 to 8 indicate 'Aspiration'. High score indicates bad situation.

Secondary Outcome Measures

Swallowing screening [6 months]
The Pediatric Eating Assessment Tool (PEDI-EAT-10) instrument which has 10 questions to evaluate dysphagia symptom severity in children will be completed. Total scoring ranges from 0 to 40. Higher scores mean worse outcome.
Chewing evaluation [6 months]
Karaduman Chewing Performance Scale will be used to define chewing performance level. It is a 5 point scale between 0 to 4. Level 0 means 'Normal chewing function', and level 4 means 'No biting and chewing'. Higher scores mean worse chewing function.

Eligibility Criteria

Criteria

Ages Eligible for Study:

3 Years to 3 Years

Sexes Eligible for Study:

Male

Accepts Healthy Volunteers:

Inclusion Criteria:

Having a diagnosis of CLS
Having a history of coughing during eating, long-lasting wheezing, sputum
Inability to intake solid food.

Exclusion Criteria:

No history of coughing during eating, long-lasting wheezing, sputum

Contacts and Locations

Locations

	Site	City	State	Country	Postal Code
1	Hacettepe University	Ankara		Turkey

Sponsors and Collaborators

Hacettepe University

Investigators

Principal Investigator: AYŞE KÜBRA ŞAHAN, Hacettepe University

Study Documents (Full-Text)

None provided.

More Information

Publications

None provided.

Responsible Party:

Ayşe Kübra Şahan, Principal investigator, Hacettepe University

ClinicalTrials.gov Identifier:

NCT04199546

Other Study ID Numbers:

KA-94678

First Posted:

Dec 16, 2019

Last Update Posted:

Jul 1, 2020

Last Verified:

Jun 1, 2020

Studies a U.S. FDA-regulated Drug Product:

Studies a U.S. FDA-regulated Device Product:

Additional relevant MeSH terms:

Deglutition Disorders

Coffin-Lowry Syndrome

Study Results

No Results Posted as of Jul 1, 2020