Sickle Cell Pain & Ketamine Use

Diagram Credit: S Rezaie

Adapted from Abdulla Alhmoudi's GR presentation May, 2016.

The management of sickle cell pain crises can be difficult due to our inability to rely on vital signs to lead our management, as well as the need for maintaining a broad differential in a patient with sickle cell disease. 

Sickle cell patients can present in both hemolysis (anemia, gallstones, nephropathy) and/or vaso-occlusive symptoms (acute chest, priapism, CVA, pain crisis).

Sickle cell pain crisis can be caused by a broad range of triggers, and vary broadly between patients, including heat/cold, exercise, dehydration, infection, menses and other causes. Many episodes are thought to be spontaneous. Vitals signs can frequently be normal, despite the patient suffering severe pain crisis. The NIH gold standard for sickle cell pain assessment is the patient's report of pain, and no combination of lab or clinical findings exist to determine if the patient is indeed in pain. Additionally, as the patient ages, the amount of pain between crises increases. 

Evaluation of the sickle cell patient: Maintaining a high suspicion of additional medical illnesses is important. A WBC >20,000 is not typical for sickle cell.. When evaluating reticulocyte counts, <3% is indicative of  an aplastic crisis, while >12% indicates rapid hemolysis. Additional labs to consider include LDH, biirubin and haptoglobin. 

Pain Management: Prompt administration and rapid reassessment. If pain is not relieved with >2 doses, hospitalization is recommended for analgesia. 

• Opioids are first line therapy with rapid reassessment within 20 minutes with titration to pain control: Morphine 0.1-0.15mk/kg max initial dose 10mg or Hydromorphone 0.02-0.05mg/kg, max initial dose 1.5mg
• Ketamine: dosage 0.3mg/kg given as a push or over 10 minutes
• Adjuncts: Hydration (risk of developing acute chest due to excess volume), Oxygen (for sats <93%), Ketorolac, 

Yawn, Barbara P., et al. "Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members." Jama 312.10 (2014): 1033-1048.

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Raphael, Jean L., and Suzette O. Oyeku. "Sickle cell disease pain management and the medical home." ASH Education Program Book 2013.1 (2013): 433-438.

Benjamin, Lennette J., Gwendolyn I. Swinson, and Ronald L. Nagel. "Sickle cell anemia day hospital: an approach for the management of uncomplicated painful crises." Blood 95.4 (2000): 1130-1136.

Okomo, Uduak, and Martin M. Meremikwu. "Fluid replacement therapy for acute episodes of pain in people with sickle cell disease." The Cochrane database of systematic reviews 3 (2015).

Bartolucci, Pablo, et al. "A randomized, controlled clinical trial of ketoprofen for sickle-cell disease vaso-occlusive crises in adults." Blood 114.18 (2009): 3742-3747.