High Yield Tips for Congenital Conditions that Adult Hospitalists Should Know-Slides

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This presentation offers practical guidance for adult hospitalists caring for adults with congenital conditions, emphasizing four key areas: congenital physical disabilities, cystic fibrosis (CF), sickle cell disease (SCD), and type I diabetes mellitus (DM).<br /><br />Congenital physical disabilities such as cerebral palsy (CP) and spina bifida require attention to airway clearance, neurogenic bowel and bladder management, tone control, and pain assessment. Maintaining patients’ home regimens, including medications and physical therapies, is crucial. Special considerations include managing intrathecal baclofen pumps and recognizing baclofen withdrawal as an emergency. Collaboration with specialties like urology, PM&R, and colorectal surgery enhances care.<br /><br />In CF, rising adult prevalence stems from improved survival and early diagnosis. Pulmonary exacerbations (PEx) are common causes for admission and are treated with intensified airway clearance (e.g., vest therapy, nebulized hypertonic saline, and dornase alfa) and dual anti-pseudomonal IV antibiotics guided by prior cultures. Continuation of CFTR modulators (like elexacaftor/tezacaftor/ivacaftor) is recommended during hospitalization. Monitoring and managing extra-pulmonary complications such as CF-related diabetes and GI issues is essential.<br /><br />Sickle cell disease hospitalizations often involve vaso-occlusive crises causing severe pain. Management highlights rapid initiation of opioids tailored to patient history, hydration to maintain euvolemia, and continuation of hydroxyurea. Inpatient complications requiring urgent attention include acute chest syndrome, priapism, and stroke, each with specific interventions like transfusion or urology consults. Emerging therapies like gene editing and monoclonal antibodies offer future promise.<br /><br />For type I diabetes mellitus, diabetic ketoacidosis (DKA) remains the leading admission cause. Initial care focuses on hydration, insulin infusion, electrolyte monitoring, and addressing triggers like infection. Insulin pumps and continuous glucose monitors are increasingly common and may be continued inpatient if the patient can self-manage. Importantly, basal insulin must never be stopped.<br /><br />Overall, the presentation stresses individualized, patient-aligned care, thorough medication reconciliation, interdisciplinary collaboration, and ongoing communication with outpatient providers to optimize outcomes for this growing adult congenital condition population.

Keywords

adult hospitalists

congenital physical disabilities

cystic fibrosis

sickle cell disease

type I diabetes mellitus

intrathecal baclofen pump

pulmonary exacerbations

vaso-occlusive crises

diabetic ketoacidosis

interdisciplinary collaboration