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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