A very serious case was presented at Morning Report last week: a patient with sickle cell disease (SCD), very low hemoglobin and acute on chronic respiratory distress. What would you do?
With early screening and better treatment of patients with SCD, long term complications of the disease are becoming more prevalent. Pulmonary Hypertension (PH) -group 1, 2 and 5- and renal failure are late manifestations of SCD, often seen beyond the 2nd decade of life. It is strongly recommended to refer patients with SCD and proteinuria >300mg/24h to a nephrologist and initiate early RRT in the setting of acute kidney failure. Lack of RCTs limits the strength of screening recommendations for PH; however, based on observational studies, tricuspid regurgitant velocity of >2.5m/s on trans-thoracic echocardiography can be used to identify at risk patients. Both conditions have an independent effect on morbidity and premature mortality in patients with SCD. In the acute clinical setting, early recognition, aggressive blood pressure control, early RRT and transfusion are critical aspects of management.
- Sarah Kiani