Targeted Therapy of Bronchiolitis Obliterans Syndrome
Condition: Bronchiolitis Obliterans
MeSH terms: Bronchiolitis; Bronchiolitis Obliterans; Bronchiolitis Obliterans Syndrome
Intervention: fluticasone propionate; montelukast sodium; azithromycin
Intervention description: Given inhaled PO; Given PO; Given PO
Intervention MeSH terms: Fluticasone; Xhance; Azithromycin; Montelukast
Phase: Phase 2
Study type: Interventional
Primary purpose: Treatment
Intervention model: Single Group Assignment
Masking: None (Open Label)
Enrollment: 36.0
Sex: All
Minimum age: 6 Years
Maximum age: 99 Years
Healthy volunteers: No

Summary:

      This phase II trial studies how well giving fluticasone propionate, azithromycin, and
      montelukast sodium (FAM) together works in treating patients with bronchiolitis obliterans
      who previously underwent stem cell transplant. FAM may be an effective treatment for
      bronchiolitis obliterans
    

Eligibility criteria:

        Inclusion Criteria:

          -  Diagnosis of BOS after HCT within the 6 months before study enrollment; for this
             study, BOS is defined as:

               -  Forced expiratory volume in 1 second (FEV1) < 75% of the predicted normal and
                  FEV1 to slow or inspiratory vital capacity ratio (FEV1/SVC or FEV1/IVC) =< 0.7,
                  both measured before and after administration of bronchodilator OR

               -  Pathologic diagnosis of BOS demonstrated by lung biopsy

          -  The baseline absolute FEV1 must be >= 10% lower than the pre-transplant absolute FEV1
             as defined by the pre-transplant FEV1 minus the baseline FEV1, both measured before
             administration of a bronchodilator

          -  Participant (or parent/guardian) has the ability to understand and willingness to sign
             a written consent document

        Exclusion Criteria:

          -  Recurrent or progressive malignancy requiring anticancer treatment

          -  Known history of allergy to or intolerance of montelukast, zafirlukast, azithromycin,
             erythromycin, or clarithromycin

          -  Pregnancy or nursing; all females of childbearing potential must have a negative serum
             or urine pregnancy test < 7 days before study drug administration

          -  Transaminases > 5 X upper limit of normal (ULN)

          -  Total bilirubin > 3 X ULN

          -  Chronic treatment with any inhaled steroid for > 1 month in the past three months

          -  Treatment with montelukast or zafirlukast for > 1 month during the past three months

          -  Treatment with prednisone at > 1.2 mg/kg/day (or equivalent steroid)

          -  Treatment with rifampin or phenobarbital, aspirin at doses > 325 mg/day, or ibuprofen
             at doses > 1200 mg/day

          -  Treatment with any Food and Drug Administration (FDA) non approved study medication
             within the past 4 weeks; off-label treatment with an FDA-approved medication is
             allowed

          -  Chronic oxygen therapy

          -  Evidence of any viral, bacterial or fungal infection involving the lung and not
             responding to appropriate treatment

          -  Clinical asthma (variable and recurring symptoms of airflow obstruction and bronchial
             hyper-responsiveness)

          -  Any condition that, in the opinion of the enrolling investigator, would interfere with
             the subject's ability to comply with the study requirements

          -  Uncontrolled substance abuse or psychiatric disorder

          -  Inability to perform pulmonary function tests (PFT) reliably, as determined by the
             enrolling investigator or PFT lab

          -  Life expectancy < 6 months at the time of enrollment as judged by the enrolling
             investigator

          -  Baseline post-bronchodilator FEV1 < 20% of predicted normal before or after albuterol