Supportive Care of the Patient Suspected to have Immune Mediated chILD
While disease-specific therapeutic regimens have been discussed throughout, the importance of generalized supportive care measures for all diagnoses, cannot be overstated. With underlying inflammation and increased metabolic demands, patients can experience faltering growth requiring consultation with nutrition and dietary support. Exercise, physical therapy, and pulmonary rehabilitation can improve functional capacity in these patients. Social work and nursing support is critical to assist with care coordination —navigating transportation, education, insurance, durable medical equipment, and the psychosocial and financial implications of these conditions, which can present a significant burden to families [171].
Counseling patients on avoidance of infectious exposures, smoking, vaping, and environmental pollutants is essential. Having a low threshold for evaluating for and treating infections is recommended. Patients should be encouraged to receive all appropriate vaccinations, keeping in mind that for patients with IEI or on immunosuppressive medications, live vaccines are often contraindicated. Depending on the underlying disorder or immunosuppressive treatment, prophylactic antimicrobials and intravenous or subcutaneous immunoglobulin treatment may be considered. In the setting of significant ILD and increased susceptibility, we recommend a goal IgG trough target of 800-1000 mg/dL. Higher dose (1-2g/kg) immunoglobulin can also serve an immune-modulatory purpose in patients with immune dysregulation and autoimmune disease.
In patients with advanced lung disease including ILD, consideration of lung transplantation may be necessary, particularly if the patient is at risk of death from pulmonary complications in the next 1-2 years, has poor quality of life despite optimal medical therapy, and has a reasonable chance of surviving transplantation. With ILD associated with connective tissue and immune mediated disorders, the risk for immune mediated lung disease recurrence in the transplanted lung is an important consideration, with a paucity of data to guide these decisions for most of these disorders. In some IEIs, allogeneic SCT may be curative, but this is more successful if the genetic defect is limited to the hematopoietic compartment and carries the risk for transplant related morbidity and mortality [22]. In advanced ILD cases, consultation with pediatric palliative and advanced care and ethics teams may be helpful for the medical team and families.