Monday, September 26, 2016

Cystic Fibrosis

emDocs - June 24, 2016 - uthors: Tassin S and Long B
Edited by: Robertson J and Koyfman A
  • CF is not limited to Caucasians and over 33% of cases are missed by newborn screening programs.
  • Pulmonary exacerbations must be treated early and aggressively to slow the decline in lung function.
Treatment of pulmonary exacerbations:
Antipseudomonal antibiotics (high doses): Aminoglycoside + β-lactam.
Bronchodilator – either MDI or nebulized albuterol.
Nebulized 7% saline, 4mL
BiPAP if needed’.
Only use steroids if there are significant asthma-type symptoms (i.e. wheezing).

DIOS: (Distal Intestinal Obstruction Syndrome)
  • Diagnosed by classic history, mass in the RLQ, and localized fecal material on plain abdominal film.
  • Don’t miss appendicitis or intussusception. Get an ultrasound and/or CT scan if needed.
  • Correct fluids and electrolytes first.
  • If PO tolerant, may treat from above with PEG solution (e.g. GoLytely®) or Gastrografin diluted 1:4 in water or juice. Patients may require an NG tube in order to consume enough of the laxative.
  • If PO intolerant or with bilious vomiting (i.e. complete obstruction), decompress the stomach with a nasogastric tube. Call radiology for a Gastrografin enema.
  • Treat until bowel movements are clear and watery."