Protocol Details
Bronchodilator Effects of Nebulized versus Inhaled Albuterol in Subjects with Lymphangioleiomyomatosis
This study is currently recruiting participants.
Summary
Number |
13-H-0051 |
Sponsoring Institute |
National Heart, Lung and Blood Institute (NHLBI) |
Recruitment Detail |
Type: Participants currently recruited/enrolled
Gender: Female
Min Age: 18 Years
Max Age: 100 Years |
Referral Letter Required |
No |
Population Exclusion(s) |
Male;
Children;
Pregnant Women |
Keywords |
Albuterol;
Bronchodilator;
Nebulizer;
Metered Dose Inhaler |
Recruitment Keyword(s) |
None |
Condition(s) |
Lymphangioleiomyomatosis |
Investigational Drug(s) |
None |
Investigational Device(s) |
None |
Intervention(s) |
Drug: albuterol inhaler
Drug: albuterol nebulizer
Procedure/Surgery: PFT
|
Supporting Site |
National Heart, Lung and Blood Institute |
Background:
- Lymphangioleiomyomatosis (LAM) is a rare type of lung disease that occurs almost exclusively in women. In LAM, muscle tissue grows in the lungs and starts to block the flow of air. It is a progressive disease, and in severe cases may require a lung transplant. One possible treatment to improve breathing in people with LAM is inhaled albuterol. Albuterol can be given in a metered dose inhaler (MDI) or with a nebulizer. Researchers want to compare these methods to see which method best improves lung function in women with LAM.
Objectives:
- To see whether a nebulizer or MDI can better improve lung function in women with LAM.
Eligibility:
- Women at least 18 years of age who have impaired lung function because of LAM.
Design:
- Participants will be screened with a physical exam and medical history. No lab tests will be needed for this study.
- Participants will have a 3-day overnight stay at the National Institutes of Health. Those who are using long-acting inhalers will have to stop taking these drugs 1 week before the study.
- Participants will receive either the nebulizer or two or four puffs of the inhaler. Four puffs of albuterol is a higher dose than is normally prescribed, and is being tested on this study.
- Participants will have each treatment around the same time of day on each of the 3 days. Before and after taking the albuterol, participants will have lung function tests.
Eligibility
INCLUSION CRITERIA:
-Diagnosis of LAM either by tissue biopsy, evidence of lung and other organ involvement (renal angiomyolipomas, chylous effusions, lymphangioleiomyomas), high serum levels of vascular endothelial growth factor D (VGEF-D)(1) or a diagnosis of TSC associated with cystic lung lesions.
-Age 18 years or over
-Evidence of airflow obstruction: FEV1/VC ratio < fifth percentile of predicted normal and an FEV(1) <80% predicted of the normal values.
EXCLUSION CRITERIA:
Subjects will be excluded from the study if they meet one or more of the following criteria:
-History of hypersensitivity to albuterol or any of its components.
-Moderate or large pleural effusions (chest x-ray and or CT scan procedure completed under Protocol 95-H-0186)
-History of seizures other than during infancy
-Inability to withhold bronchodilators for 24 hours
-Cognitive Impairment
-Age less than 18 years
-Male sex
-Status-post lung or kidney transplantation
-Pregnant or breast feeding (women of childbearing potential will undergo a blood or urine pregnancy test under Protocol 95-H-0186).
-Treatment with monoaminoxidase inhibitors, tricyclic antidepressants or Beta-adrenergic receptor antagonists or long acting anticholinergic bronchodilators who are unable to be discontinued for at least seven days before enrollment.
-Patients with URI, uncontrolled hyperthyroidism or severe gastro-esophageal reflux. Major systemic diseases (i.e., malignancy; myocardial infarction or unstable angina; type 1 diabetes, severe hypertension; liver cirrhosis).
Citations:
McCormack FX. Lymphangioleiomyomatosis: a clinical update. Chest. 2008 Feb;133(2):507-16. doi: 10.1378/chest.07-0898. Review.
Ryu JH, Moss J, Beck GJ, Lee JC, Brown KK, Chapman JT, Finlay GA, Olson EJ, Ruoss SJ, Maurer JR, Raffin TA, Peavy HH, McCarthy K, Taveira-Dasilva A, McCormack FX, Avila NA, Decastro RM, Jacobs SS, Stylianou M, Fanburg BL; NHLBI LAM Registry Group. The NHLBI lymphangioleiomyomatosis registry: characteristics of 230 patients at enrollment. Am J Respir Crit Care Med. 2006 Jan 1;173(1):105-11. Epub 2005 Oct 6..
Costello LC, Hartman TE, Ryu JH. High frequency of pulmonary lymphangioleiomyomatosis in women with tuberous sclerosis complex. Mayo Clin Proc. 2000 Jun;75(6):591-4.
Contacts:
Clinical Trials Number:
NCT01799538