Pediatric emergency medicine trisk 2786 2786

glands, it produces highly concentrated sweat, which impairs electrolyteconcentrating abilities causing excessive electrolyte loss and dehydration.
While patients with CF may visit EDs for common complaints and conditions,
having CF will impact even common respiratory ailments. Emergency
presentations for CF patients can be grouped as pulmonary, systemic, or GI
abnormalities. This section will discuss the pulmonary presentations. Some of
these are single events, while others are recurrent. Knowledge of successful
treatment for previous episodes will inform management.

Goals of Treatment
Treatment goals include identifying the cause of acutely worsening respiratory
distress. Patients may have worsening of underlying disease due to acute viral
illness, acute bacterial infection, bacterial colonization and subacute exacerbation,
worsening gastroesophageal reflux disease (GERD) symptoms, pneumothorax,
hemoptysis, or ABPA. Appreciation of baseline status is necessary, and
communication with the patient’s primary CF team should occur in a timely
manner.
The possibility of renal dysfunction and impact on bowel motility must be
considered when selecting medications. CF patients may not have normal renal
function after years of aminoglycoside exposure and other nephrotoxic therapies.
Renally excreted analgesics therefore should not be used long term without
assessment of renal function. Furthermore, narcotic analgesics can cause
decreased GI motility. This may, in turn, lead to constipation and bowel
obstruction.

Clinical Considerations
Clinical Recognition
It is rare for patients with CF to present to the ED without a known diagnosis.
Newborn screening and family history often lead to early diagnosis. For those
diagnosed later, recurrent pulmonary infections, or radiographic findings
including cystic changes or bronchiectasis may prompt evaluation leading to
diagnosis. In some cases, nonpulmonary complaints associated with CF may lead

to the diagnosis. Most patients presenting to the ED will have established
diagnoses and receive coordinated care through a CF center. The ED visit,
therefore, most commonly focuses on identifying and treating complications from
CF.
Triage