Pulmonary Function Tests, Fluticasone Propionate, Azithromycin Dihydrate, and Montelukast Sodium in Managing Bronchiolitis Obliterans Syndrome in Pediatric Patients Who Have Undergone Donor Stem Cell Transplant

Inclusion Criteria

• Patients undergoing myeloablative allogenic hematopoietic stem cell transplant for any indication (both malignant and non-malignant) are eligible
• Eligible patients will have one or more of the following donor stem cell sources: * Bone marrow * Placental blood (umbilical cord blood) * Cytokine mobilized peripheral blood
• Eligible patients will have one of the following sources of donor stem cells: * Human leukocyte antigen (HLA) matched family member * Partially matched family member (mismatched for a single HLA locus at A, B, C or DR) * Fully HLA matched or partially mismatched unrelated marrow or peripheral blood stem cells (per institutional donor selection standards) * HLA matched or partially mismatched (at least 4/6 match at A, B, DR) cord blood
• Conditioning Regimen: Patients expecting to receive any type of myeloablative HSCT conditioning regimen are eligible
• Prior therapies: Patients undergoing stem cell transplant of any kind
• Required laboratory parameters: Patients able to adequately perform pulmonary function testing per American Thoracic Society/European Respiratory Society (ATS/ERS) guidelines, as determined by the enrolling investigator and trained respiratory therapists
• The patient and/or the patient's legally authorized guardian must acknowledge in writing that consent to become a study subject has been obtained in accordance with the institutional policies approved by the United States (U.S.) Department of Health and Human Services; informed consent must be signed prior to registration on study

Exclusion Criteria

• Subjects with a previous solid organ transplant
• Recurrent or progressive malignancy requiring anti-cancer therapy
• Subjects with evidence of underlying obstructive pulmonary disease prior to transplant (clinical history of asthma or baseline forced expiratory volume in 1 second [FEV1] < 80% predicted with FEV1/forced vital capacity [FVC] < 80%)
• Known history of allergy or intolerance to montelukast, zafirleukast, azithromycin, erythromycin, clarithromycin, prednisone, or sirolimus
• Chronic supplemental oxygen requirement or hypoxemia < 92% blood oxygen saturation (SpO2)
• Clinical asthma (variable and recurrent symptoms of airflow obstruction and airway hyper-responsiveness)
• Pregnancy or nursing: All females of childbearing age must have a negative serum or urine pregnancy test < 7 days before study drug administration
• Chronic treatment with any inhaled steroid for > 1 month in past three months
• Treatment with montelukast or zafirukast for > 1 month in past three months
• Treatment with systemic steroids for > 1 month in past three months
• Treatment with any Food and Drug Administration (FDA) non-approved study medication within the past four weeks; off label treatment with FDA approved medication is allowed
• Evidence of any viral, bacterial, or fungal infection involving the lung and not responding to appropriate treatment
• Inability to perform pulmonary function testing (PFT), as determined by the enrolling investigator or PFT lab
• Any condition that, in the opinion of the enrolling investigator, would interfere with the subject’s ability to comply with the study requirements