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.