RECRUITING

Neurocognition in Congenital Central Hypoventilation Syndrome (CCHS)

Study Overview

This clinical trial focuses on testing the efficacy of different digital interventions to promote re-engagement in cancer-related long-term follow-up care for adolescent and young adult (AYA) survivors of childhood cancer.

Description

Congenital central hypoventilation syndrome (CCHS) is a rare disorder of autonomic and respiratory regulation that frequently alters oxygen delivery to the brain. In CCHS, neurocognitive function has been of great concern because of the potential for repeated hypoxemia and hypercarbia in activities of daily living in addition to hypoventilation with related hypoxemia and hypercarbia during sleep. As the world's leading referral center for CCHS, the Center for Autonomic Medicine in Pediatrics (CAMP) is engaged in ongoing research to identify factors that impact neurocognitive performance in patients with CCHS in order to optimize clinical management and improve long term neurocognitive outcomes. The purpose of this IRB-approved research study is to implement the NIH Toolbox as a standard measurement of cognitive health in patients with CCHS. Further, the study aims to determine how intrinsic and extrinsic disease factors such as age at diagnosis, PHOX2B mutation type and genotype, and nature of past and present artificial respiratory intervention affect the NIH Toolbox Cognitive scores of individuals with CCHS. Eligible participants will complete a 45-minute NIH Toolbox assessment and parents (or adult participants) will complete an associated, 15-minute Research Electronic Data Capture (REDCap) questionnaire.

Official Title

Neurocognitive Outcome as a Metric for Evaluating Therapeutic Intervention and Treatment Mechanisms in Congenital Central Hypoventilation Syndrome (CCHS): A Multi-Site Study Using The NIH Toolbox

Quick Facts

Study Start:2016-01
Study Completion:2035-12
Study Type:Not specified
Phase:Not Applicable
Enrollment:Not specified
Status:RECRUITING

Study ID

NCT03568669

Participation Criteria

Researchers look for people who fit a certain description, called eligibility criteria. Some examples of these criteria are a person's general health condition or prior treatments.

Ages Eligible for Study:3 Years to 85 Years
Sexes Eligible for Study:ALL
Accepts Healthy Volunteers:No
Standard Ages:CHILD, ADULT, OLDER_ADULT
Inclusion CriteriaExclusion Criteria
  1. * PHOX2B mutation-confirmed CCHS diagnosis
  2. * Speaks and reads English as a primary language
  1. * Unsuspected or unconfirmed CCHS
  2. * Does not speak or read English as a primary language

Contacts and Locations

Study Contact

Casey Rand, BS
CONTACT
312-227-3300
crand@luriechildrens.org
Erin S Lonergan, BS
CONTACT
CAMP@LurieChildrens.org

Study Locations (Sites)

Children's Hospital Los Angeles
Los Angeles, California, 90027
United States
Ann & Robert H. Lurie Children's Hospital of Chicago
Chicago, Illinois, 60611
United States
Children's Hospital of Philadelphia
Philadelphia, Pennsylvania, 19104
United States
Seattle Children's Hospital
Seattle, Washington, 98105
United States

Collaborators and Investigators

Sponsor: Debra Weese-Mayer

Study Record Dates

These dates track the progress of study record and summary results submissions to ClinicalTrials.gov. Study records and reported results are reviewed by the National Library of Medicine (NLM) to make sure they meet specific quality control standards before being posted on the public website.

Study Registration Dates

Study Start Date2016-01
Study Completion Date2035-12

Study Record Updates

Study Start Date2016-01
Study Completion Date2035-12

Terms related to this study

Keywords Provided by Researchers

  • NIH Toolbox
  • Toolbox
  • Congenital Central Hypoventilation Syndrome
  • CCHS
  • Autonomic Nervous System Dysregulation
  • PHOX2B

Additional Relevant MeSH Terms

  • Congenital Central Hypoventilation Syndrome
  • Congenital Central Hypoventilation
  • CCHS
  • CCHS With Hirschsprung Disease
  • CCHS With Neural Crest Tumor
  • CCHS With Neuroblastoma