Sickle Cell Crisis in the Hospitalized Patient: Pain Protocols, Balanced Fluids, and Why You Must Review Hydroxyurea Now

In this episode of Hospital Medicine Unplugged, we tackle sickle cell disease (SCD)—manage pain, prevent complications, and optimize long-term care.

We start with the essentials: rapid pain management and early intervention. For vaso-occlusive crisis (VOC), opioids should be administered within 1 hour of presentation, with individualized dosing based on previous effective regimens. Monitor closely and adjust as needed to achieve adequate analgesia. Adjuncts like NSAIDs can be used when appropriate, and for opioid-refractory pain, consider ketamine infusions or regional anesthesia—but only in select cases, with specialist input.

Next, we talk fluids—balanced is the new standard. Lactated Ringer’s may reduce the risk of metabolic acidosis and microvascular sickling compared to normal saline, but practice patterns still favor saline. Keep fluids balanced, and watch for overload, especially in patients with renal or cardiac issues.

Complications? Keep a close eye on acute chest syndrome, infection, and stroke—critical threats in the hospital setting. Early recognition is key: treat infection with broad-spectrum antibiotics, perform incentive spirometry to reduce acute chest syndrome risk, and always screen for stroke, especially in patients with abnormal transcranial Doppler velocities.

For transfusion therapy, be strategic. Red blood cell transfusions are reserved for severe cases, such as acute chest syndrome or stroke, while simple transfusions are used to manage anemia. Exchange transfusion is the go-to for stroke or severe ACS, aiming to lower sickle hemoglobin and reduce viscosity.

We cover long-term management: hydroxyurea is the first-line therapy for preventing VOC and acute chest syndrome, and should be considered for all eligible patients. Crizanlizumab and L-glutamine are options for those who don’t respond well to hydroxyurea. Consider stem cell transplant in eligible patients for curative treatment, especially in children with matched sibling donors.

Before discharge, plan for secondary prevention. Ensure patients are on disease-modifying therapies, have received proper vaccinations, and are educated on avoiding triggers like dehydration and infection. Schedule follow-up appointments and reinforce adherence to medications.

Hospital-specific pearls: • Use admission to manage pain quickly and initiate fluid therapy—monitor for complications like ACS, infection, and stroke. • Ensure medication reconciliation, particularly with opioids and hydroxyurea—adjust regimens as needed. • Early involvement of nutrition and physiotherapy can help reduce the risk of long-term complications like avascular necrosis and pulmonary hypertension. • Offer alcohol counseling and support for substance use, which can worsen SCD outcomes.

We close with system moves: a bundle that (1) auto-calculates FIB-4 for VOC risk in the EHR, (2) triggers early pain management pathways, (3) sends fluid and transfusion orders based on clinical risk, (4) links to specialist consultations for complex cases like stroke, and (5) sets up post-discharge follow-up reminders to optimize care.

Pain control saves the day in SCD; preventing complications reduces morbidity; and long-term therapies keep patients out of the hospital—SCD care that’s actionable on the wards and sustainable after discharge.