Trial record 2 of 4 for:    CENTRAL HYPOVENTILATION SYNDROME, CONGENITAL

Therapeutic Effect of Desogestrel on Ventilatory Control in Patients With Congenital Central Hypoventilation Syndrome

The recruitment status of this study is unknown because the information has not been verified recently.
Verified February 2012 by Children's Hospital Los Angeles.
Recruitment status was  Not yet recruiting
Sponsor:
Information provided by (Responsible Party):
Thomas Keens, MD, Children's Hospital Los Angeles
ClinicalTrials.gov Identifier:
NCT01500473
First received: December 22, 2011
Last updated: February 13, 2012
Last verified: February 2012
  Purpose

Background: Congenital Central Hypoventilation Syndrome (CCHS) is a rare disorder of automatic control of breathing. This disease can manifest as early as birth. Patients with this disease have a fundamental lack of central drive breathing. They do not mount any responses to hypoxia or hypercapnia during sleep or wakefulness. This places them at risk of injury or death whenever they are not consciously breathing. They require lifelong assisted ventilation while sleeping, and some while awake. Progesterone is a known respiratory stimulant in normal individuals, and it has been shown in one study of 2 patients that this drug may improve CO2 responsiveness in patients with CCHS. However, this observation requires confirmation.

Hypothesis: Exogenous progesterone (in oral contraception pills) will improve CO2 responsivity by hyperoxic hypercapnic ventilatory response testing, hypoxic responsivity using 5-breath nitrogen breathing, hyperoxic ventilatory response while breathing 100% oxygen, and improve spontaneous ventilation during sleep in CCHS females >15-years of age. The progesterone will also depress ventilatory response using a hyperoxia test.

Study Methodology: Baseline measures of CO2 and oxygen responsivity, and spontaneous ventilation during sleep, will be performed at baseline and after 3-weeks of taking a progesterone containing oral contraceptive agent. CO2 responsivity will be measured using a hyperoxic hypercapnic ventilatory response test. Hypoxic responsivity will be measured using a 5-breath 100% nitrogen breathing test. Hyperoxic responsivity will be measured by having subjects breathe 100% oxygen for 2-minutes. Subjects will perform an overnight polysomnogram to assess adequacy of gas exchange during spontaneous breathing while asleep. A progesterone containing oral contraception pill will then be given for 3-weeks, and the above measures repeated. Serum progesterone will be measured at baseline and at the time of study.


Condition Intervention
Congenital Central Hypoventilation Syndrome
Drug: Desogestrel

Study Type: Interventional
Study Design: Intervention Model: Single Group Assignment
Masking: Single Blind (Subject)
Primary Purpose: Treatment
Official Title: Therapeutic Effect of Desogestrel on Ventilatory Control in Patients With Congenital Central Hypoventilation Syndrome

Resource links provided by NLM:


Further study details as provided by Children's Hospital Los Angeles:

Primary Outcome Measures:
  • Minute ventilation [ Time Frame: 3 weeks ] [ Designated as safety issue: Yes ]

Secondary Outcome Measures:
  • Time to requirement of assisted ventilation [ Time Frame: 3 weeks ] [ Designated as safety issue: Yes ]

Estimated Enrollment: 8
Study Start Date: February 2012
Estimated Primary Completion Date: December 2012 (Final data collection date for primary outcome measure)
Arms Assigned Interventions
Experimental: Females with CCHS > 16 years old on desogetrel Drug: Desogestrel
Reclipsen oral contraceptive pill with 0.03mg ethinyl estradiol and 0.15mg desogestrel

  Eligibility

Ages Eligible for Study:   16 Years to 50 Years
Genders Eligible for Study:   Female
Accepts Healthy Volunteers:   No
Criteria

Inclusion Criteria:

  • diagnosed congenital central hypoventilation syndrome (CCHS)
  • female
  • greater than or equal to 16 years of age

Exclusion Criteria:

  • less than 16 years of age
  • male
  • pregnant
  • poor adherence to medications
  • inability to perform pulmonary maneuvers for tests
  • contraindications to oral contraceptives
  • pulmonary hypertension
  Contacts and Locations
Choosing to participate in a study is an important personal decision. Talk with your doctor and family members or friends about deciding to join a study. To learn more about this study, you or your doctor may contact the study research staff using the Contacts provided below. For general information, see Learn About Clinical Studies.

Please refer to this study by its ClinicalTrials.gov identifier: NCT01500473

Contacts
Contact: Thomas Keens, MD 323-361-2101
Contact: Douglas LI, MD 323-361-2101

Locations
United States, California
Children's Hospital Los Angeles
Los Angeles, California, United States, 90027
Children's Hospital Los Angeles Not yet recruiting
Los Angeles, California, United States, 90027
Contact: Thomas Keens, MD    323-361-2101      
Sponsors and Collaborators
Children's Hospital Los Angeles
  More Information

No publications provided

Responsible Party: Thomas Keens, MD, Senior Investigator, Children's Hospital Los Angeles
ClinicalTrials.gov Identifier: NCT01500473     History of Changes
Other Study ID Numbers: CCI-11-00057
Study First Received: December 22, 2011
Last Updated: February 13, 2012
Health Authority: United States: Institutional Review Board

Additional relevant MeSH terms:
Syndrome
Hypoventilation
Sleep Apnea, Central
Sleep Apnea Syndromes
Respiratory Insufficiency
Disease
Pathologic Processes
Respiration Disorders
Respiratory Tract Diseases
Signs and Symptoms, Respiratory
Signs and Symptoms
Apnea
Sleep Disorders, Intrinsic
Dyssomnias
Sleep Disorders
Nervous System Diseases
Desogestrel
Contraceptives, Oral, Synthetic
Contraceptives, Oral
Contraceptive Agents, Female
Contraceptive Agents
Reproductive Control Agents
Physiological Effects of Drugs
Pharmacologic Actions
Therapeutic Uses
Progestins
Hormones
Hormones, Hormone Substitutes, and Hormone Antagonists

ClinicalTrials.gov processed this record on September 15, 2014