Case-Based Review

Sickle Cell Disease


 

References

CASE CONTINUED

The patient is discharged on the day after the procedure, with instructions to continue his hydroxyurea.

• Should the patient resume hydroxyurea therapy?

Hydroxyurea is hepatically cleared and thus it should be held until his liver function tests normalize.106

CASE CONTINUED

Two months later, the patient presents to the emergency department with abdominal pain that moves to his left leg. A CBC is obtained, showing a hemoglobin of 11.8 g/dL and a platelet count of 144,000/µL. He is given 2 doses of morphine 6 mg intravenously, and reports that his leg pain is now a 4/10. He is discharged home with a prescription for hydrocodone/acetaminophen.

• Is the emergency department evaluation sufficient?

This patient remains at high risk for splenic sequestration,93 with a hemoglobin 2 g lower than it was 2 months ago and platelets less than half. This decline could be consistent with early splenic sequestration.20 Additionally, he had elevated liver function tests on a recent admission, as well as rising creatinine, without evidence of resolution. It is not appropriate to discharge him without checking a chemistry and liver panel, and abdominal imaging should be considered. The best plan would be to admit him for observation, given his risk for splenic sequestration, and consult surgery for an elective splenectomy if he has a second episode of splenic sequestration 2 months after the first.100 His abdominal pain that migrates to his left leg could be due to his massive splenomegaly compressing his left kidney, as noted on imaging during his recent admission for splenic sequestration

CASE CONTINUED

An hour after discharge from the emergency department, EMS is called to his home for intractable pain. He is found lying on the floor, and reports excruciating left leg pain. He is brought to the closest hospital, a community hospital that he has not visited previously. There, he is admitted for hydration and pain control and placed on hydromorphone 2 mg every 4 hours as needed for pain. His hemoglobin is 10.8 g/dL, and platelets are 121,000/µL. A chemistry panel is remarkable for a creatinine level of 1.5 mg/dL and a potassium level of 3.2 mEq/L. Liver function tests are not obtained. After 3 doses of hydromorphone, he falls asleep. He is not in a monitored bed, and intravenous fluids, while ordered, are not started. At 6:30 AM the day after admission, he cannot be aroused on a routine vital sign check; he has an SpO2 of 60%, a blood pressure of 80/60 mm Hg, and heart rate of 148 beats/min. A rapid response is called, and naloxone is administered along with oxygen by face mask and several fluid boluses. His systolic blood pressure increases to 100 mm Hg from a low of 70 mm Hg. His SpO2 increases to 92%, and he is arousable and alert, although he reports 10/10 leg pain. His abdomen is noted to be distended and tender.

• What may have contributed to his clinical condition?

The patient is opioid tolerant and has received equivalent doses of opioids in the past without excess sedation. He may have liver dysfunction making him unable to metabolize opioids effectively. His hemoglobin and platelets continue to decline, raising concern for splenic sequestration versus sepsis. Failure to place him on a monitor allowed his hypoxia to continue for an unknown amount of time, placing him at high risk for developing ACS. Lack of intravenous hydration while he was too sedated to drink likely exacerbated his sickling.

Pages

Recommended Reading

Study links iron deficiency anemia and hearing loss
MDedge Hematology and Oncology
Drug can improve upon chelation therapy in thalassemia major
MDedge Hematology and Oncology
Drug granted fast track designation for PNH
MDedge Hematology and Oncology
Agios stops developing drug for PK deficiency
MDedge Hematology and Oncology
Predicting the risk of CKD in sickle cell anemia
MDedge Hematology and Oncology
Fanconi anemia linked to cancer gene
MDedge Hematology and Oncology
HU trial to prevent stroke in SCA feasible in Nigeria
MDedge Hematology and Oncology
MDS patients with mutated IDH2 benefit from enasidenib
MDedge Hematology and Oncology
Predicting therapy-related myeloid neoplasms
MDedge Hematology and Oncology
Another treatment on the horizon for SCD
MDedge Hematology and Oncology