Though the incidence of complications after a procedure in patients with sickle cell disease is low, the database review showed that acute chest syndrome is the most common complication. Other, more serious complications are rare. (Image source: Thinkstock)

Though the incidence of complications after a procedure in patients with sickle cell disease is low, the database review showed that acute chest syndrome is the most common complication. Other, more serious complications are rare. (Image source: Thinkstock)

There has not been a recent review of perioperative care of patients with sickle cell disease.  Dr. Paul G. Firth, Department of Anesthesia, Critical Care and Pain Medicine, Massachusetts General Hospital, Harvard Medical School, Boston, MA and colleagues used the Nationwide Inpatient Sample (NIS) database, maintained by the Agency for Healthcare Research and Quality to analyze patients younger than 18 years of age with sickle cell disease who underwent at least one procedure.  Their results are published this month in Anesthesia & Analgesia in the article “Surgical Procedures and Outcomes Among Children with Sickle Cell Disease.”

Of 313,385 discharges of children with sickle cell disease who underwent at least one procedure, 9948 underwent one of the 9 most common procedures: cholecystectomy, tonsillectomy/adenoidectomy, splenectomy, umbilical hernia repair, appendectomy, myringotomy, cesarean delivery, repair of inguinal or femoral hernia, or hip joint replacement.  Gender distribution was equal; most patients were African-American (88%), homozygous for Hb-S (85%), and were admitted electively (60%).  Most had Medicaid as their primary expected payer.  Blood transfusion during hospital stay was recorded mostly for patients undergoing appendectomy (45%) or cholecystectomy (37%).  The lowest incidence of blood transfusion was recorded for patients undergoing repair of inguinal or femoral hernia (28%) followed by cesarean delivery (29%).  Complications included acute chest syndrome (3%), postoperative fever (0.5%), and stroke (0.2%).  Splenic sequestration and in-hospital death were seen in < 0.2% of patients.  As expected, those with a complication stayed longer in the hospital.

Databases such as the NIS can provide much information about outcome for certain diseases, such as sickle cell, and procedures.  As we’ve indicated in previous posts, findings are dependent on accuracy of coding and the data cannot be verified.  It is also difficult to look at a timeline of what happened.  For example, how long after a procedure did a patient experience acute chest syndrome?  Was a transfusion administered before, during or after a procedure?  We are currently provided with a window into what goes on with sickle cell patients during a procedure.  The next step is to figure out how to predict who will experience complications and what we might do to decrease their incidence.