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Sickle Cell Patient at HIgh Risk for Acute Chest Syndrome for Urgent C-Section
Abstract Number: F-65
Abstract Type: Case Report/Case Series
Pregnancies of mothers with sickle cell disease (SCD) pose significant fetal and maternal risk. Maternal mortality is as high as 1%, fetal mortality is as high as 20%. In this case we discuss a 29 year old patient at 36 weeks + 5 days gestation presenting with sickle cell crisis and possible acute chest syndrome.
The patient was a 29yo woman G2P0101 with a PMH of homozygous sickle cell disease who presented at 36 weeks +5 days to the labor and delivery floor. Her chief complaint was bilateral lower and upper extremity pain. She was found to be in sickle cell crisis and early labor. Vital signs and labs on admission were BP=105/65, HR=105, RR=24, T= 100, Hb/Hct 7/20, WBC 13. The patient had a history of severe sickle cell disease with multiple hospital admissions, multiple transfusions, MCA infarct, bilateral hip replacement, and recent admission for acute chest syndrome. Recent echocardiogram showed cardiomegaly. Obsterical history was significant for prior C-section. The decision was made that the patient should have a c-section. Supplemental O2 via NC was given. In preparation for surgery an epidural and A-line were placed. Hydration with warm crystalloid and transfusion of 4 units of PRBCs to Hb of 9.6 was given. C-section proceeded without complication. The epidural was left in place post-operatively. Early post-op the pateint developed high grade fever, with SOB. CXR showed bilateral pleural effusions and infiltrate. Acute chest syndrome was suspected. The patient was placed on broad spectrum antibiotics and sent to the ICU for close monitoring. On POD#2 the patient was transferred to regular maternity floor and on POD #7 discharged from the hospital.
Women with SCD have a significantly higher rate of pneumonia, systemic inflammatory response syndrome, preeclampsia, eclampsia, and genitourinary tract infections. All anesthesiologists taking care of sickle cell patients in labor especially in crisis should be aware of the possibility of developing Acute Chest Syndrome which is the most common cause of death in patients with SCD. Early epidural analgesia is essential for laboring sickle cell patients especially in crisis for control pain, to achieve peripheral vasodilatation and to decrease opioid requirements. Slow closely monitored hydration with preoperative transfusion should be employed. Dehydration can increase sickling and over hydration can cause pulmonary edema and predispose to the development of ACS. A hemoglobin of 10 is proven to decrease complication rates. Early post-op therapy in ICU setting is warranted. We advocate for post-op epidural PCEA in combination with IV medications PRN.
Villers MS, Jamison MG, De Castro LM, James AH. Morbidity associated with sickle cell disease in pregnancy. Am J Obstet Gynecol 2008; 199:125.e1.
Serjeant GR, Loy LL, Crowther M, et al. Outcome of pregnancy in homozygous sickle cell disease. Obstet Gynecol 2004; 103:1278.