By George Nassar, MD PGY-4 Inova Fairfax Hospital

14 yo F h/o HbSS presenting with 1.5 weeks of chest pain associated with non-bloody, occasionally bilious emesis. Chest pain (CP) is intermittent and was improving with oxycodone 5mg until today. Discharged from inpatient hospitalization for pain crisis just before this pain came on. Described as sharp and pleuritic, radiating to her back, and associated with dizziness, nausea, and intermittent, vague abdominal pain.

ROS: Negative for fever, cough, URI sx, joint pain, rash.

Social: Issues with heme/onc follow-up due to insurance with unclear hydroxyurea (HU) compliance

Exam:
-VS: HR 76, BP 112/59, Resp 24, SpO2 99 % on RA, Temp 97.3 °F (36.3 °C), Wt 54.7 kg
-Appearance: Well-developed. She is not ill-appearing or diaphoretic. In obvious discomfort and endorses 10/10 pain
-HEENT: NC/AT. Face symmetric. PERRLA. EOMI. +Scleral Icterus
-CV:  Normal rate and rhythm. 2+ pulses throughout. No murmurs or distant heart sounds
-Pulm: CTAB, breathing comfortably. No chest wall tenderness
-Abdomen: Soft. No tenderness to light palpation. Epigastric tenderness to deep palpation. No rebound or guarding. No CVA tenderness.
-MSK: Normal range of motion throughout. No joint tenderness or effusion. No obvious tenderness over any boney prominences.
-Neuro/Psych: Normal mood and affect. No FND’s. All cranial nerves intact.

Initial work-up:

-CXR no focal consolidations
-KUB: No acute abnormality. Normal bowel gas pattern.
-CBC: 11.76>8.3/24<45
-Retic 10.2. MCV 103. RDW 23
-CMP 140/4.2/107/22/10/0.
-LFT’s wnl; TBili 2.7 (stable compared to prior)
-Hb electrophoresis sent and pending
-HbA 16.7% ; HbF 10.8%; HbS 70%

Ketorolac and morphine x3 given and pain improved from 10/10 to 3-4/10. The case is discussed with her hematologist, who believes this may be due to HU non-adherence and will see her in the office tomorrow. Patient is counseled regarding HU adherence and discharged on famotidine/polyethylene glycol.

She returns to the ED 2 days later after seeing her hematologist with persistent epigastric pain radiating to her back without fever, as well as new RUQ pain and worsening scleral icterus.

Serum studies are repeated and notable for an increase in her total bilirubin to 12.5. US RUQ demonstrates 10mm diameter common bile duct (CBD), but no visible stone.