Pulmonary Langerhans Cell Histiocytosis
Conditions
Keywords
PLCH, Air travel, pneumothorax, pleurodesis
Brief summary
The aim of this study is to conduct survey-based assessments for the safety of air travel in patients with pulmonary Langerhans cell histiocytosis (PLCH). The study will enroll patients through the clinic network at Rare Lung Disease Consortium (RLDC) and through the Histiocytosis Association website. Patients will have access to the questionnaire via REDCap (an online data management system) and each patient will be provided with a link to complete the survey. The investigators plan on enrolling approximately 200 patients with PLCH for the purpose of this study. Secondary aims of this study include further characterization of the clinical aspects of disease and to establish a contact registry for these patients, in order to facilitate future studies.
Detailed description
The study will enroll participants through the clinic network at the Rare Lung Disease Consortium (RLDC) and through the Histiocytosis Association websites. Participants will have access to the questionnaire via REDCap (an online data management system) and each participant will be provided a link to complete the survey. In case of lack of internet access or inability to complete the online questionnaire, a paper survey will be mailed out with a pre-paid envelope to return the survey. Primary Study Objective(s): To define the risk of pneumothorax associated with air travel in patients with PLCH. Secondary Study Objective(s): To establish a contact registry for patients with PLCH to facilitate future trials To assess the rate of pneumothorax recurrence in patients with PLCH To assess the efficacy of pleurodesis in preventing recurrent pneumothoraces among patients with PLCH
Interventions
None listed
Sponsors
Rare Diseases Clinical Research Network
National Heart, Lung, and Blood Institute (NHLBI)
University of Cincinnati
Study design
Observational model
CASE_ONLY
Time perspective
CROSS_SECTIONAL
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
Inclusion criteria
* Adult, age 18 or older * English literate * Signed, dated informed consent; either given electronically or via paper form * Confirmed diagnosis of PLCH based either on characteristic radiologic findings, or histopathological confirmation
Exclusion criteria
* Inability, or refusal, to sign informed consent.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Number of Participants With PLCH Who Experienced Spontaneous Pneumothorax During or Within 24 Hours Following Air Travel | During air travel or within 24 hours following air travel | The investigators will measure the number of pneumothoraces that occur either during air travel, or within 24 hours of air travel among patients with pulmonary Langerhans cell histiocytosis. The incidence of air travel related pneumothorax will be calculated by dividing the number of pneumothoraces to the total number of flights undertaken by the entire cohort. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Number of Patients With Pulmonary Langerhans Cell Histiocytosis Who Experienced a Spontaneous Pneumothorax | 2 years and 6 months | The investigators will catalogue the number of spontaneous pneumothoraces experienced by patients with pulmonary Langerhans cell histiocytosis. |
| Number of Patients With Pulmonary Langerhans Cell Histiocytosis Who Experienced Recurrent Pneumothorax | 2 years and 6 months | The investigators will measure the number of participants who experience a recurrent spontaneous pneumothorax. |
Countries
United States
Participant flow
Participants by arm
| Arm | Count |
|---|---|
| Cohort Self-reported PLCH participants who completed an online survey. | 94 |
| Total | 94 |
Baseline characteristics
| Characteristic | Cohort |
|---|---|
| Age, Continuous | 47 years |
| Race (NIH/OMB) American Indian or Alaska Native | 1 Participants |
| Race (NIH/OMB) Asian | 0 Participants |
| Race (NIH/OMB) Black or African American | 2 Participants |
| Race (NIH/OMB) More than one race | 0 Participants |
| Race (NIH/OMB) Native Hawaiian or Other Pacific Islander | 0 Participants |
| Race (NIH/OMB) Unknown or Not Reported | 1 Participants |
| Race (NIH/OMB) White | 90 Participants |
| Region of Enrollment United States | 94 participants |
| Sex: Female, Male Female | 78 Participants |
| Sex: Female, Male Male | 16 Participants |
Adverse events
| Event type | EG000 affected / at risk |
|---|---|
| deaths Total, all-cause mortality | 0 / 0 |
| other Total, other adverse events | 0 / 0 |
| serious Total, serious adverse events | 0 / 0 |
Outcome results
Primary
Number of Participants With PLCH Who Experienced Spontaneous Pneumothorax During or Within 24 Hours Following Air Travel
The investigators will measure the number of pneumothoraces that occur either during air travel, or within 24 hours of air travel among patients with pulmonary Langerhans cell histiocytosis. The incidence of air travel related pneumothorax will be calculated by dividing the number of pneumothoraces to the total number of flights undertaken by the entire cohort.
Time frame: During air travel or within 24 hours following air travel
| Arm | Measure | Value (COUNT_OF_PARTICIPANTS) |
|---|---|---|
| Cohort | Number of Participants With PLCH Who Experienced Spontaneous Pneumothorax During or Within 24 Hours Following Air Travel | 2 Participants |
Secondary
Number of Patients With Pulmonary Langerhans Cell Histiocytosis Who Experienced a Spontaneous Pneumothorax
The investigators will catalogue the number of spontaneous pneumothoraces experienced by patients with pulmonary Langerhans cell histiocytosis.
Time frame: 2 years and 6 months
| Arm | Measure | Value (COUNT_OF_PARTICIPANTS) |
|---|---|---|
| Cohort | Number of Patients With Pulmonary Langerhans Cell Histiocytosis Who Experienced a Spontaneous Pneumothorax | 22 Participants |
Secondary
Number of Patients With Pulmonary Langerhans Cell Histiocytosis Who Experienced Recurrent Pneumothorax
The investigators will measure the number of participants who experience a recurrent spontaneous pneumothorax.
Time frame: 2 years and 6 months
| Arm | Measure | Value (COUNT_OF_PARTICIPANTS) |
|---|---|---|
| Cohort | Number of Patients With Pulmonary Langerhans Cell Histiocytosis Who Experienced Recurrent Pneumothorax | 14 Participants |