#422 LIVE! Inpatient Management of Sickle Cell Crisis, Acute Pain, RBC Transfusions & Acute Chest Syndrome

January 15, 2024 | By Matthew Watto, MD

Audio

Video

Transcripts available via YouTube

Learn practical tips for the inpatient management of patients with sickle cell disease and vaso-occlusive crisis from guest, Dr. Yoo Mee Shin (Emory). We cover acute pain management including adjuvant therapies, IV fluids, oxygen therapy, the management of acute chest syndrome, and when to perform simple vs exchange transfusions. Key takeaways include the importance of early triage and analgesia, addressing misconceptions about opioid use disorder in patients with sickle cell disease, and recognizing complications of vaso-occlusive crises.

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Show Segments

Timecodes for version without ads

• 00:00 Intro
• 01:27 Getting to know our guest
• 07:48 Case Presentation and Initial Management of Sickle Cell Disease (SCD)
• 13:46 Misconceptions about opioid use disorder and sickle cell
• 17:02 Management of Acute Pain in Sickle Cell Disease
• 25:46 Adjuvant therapy for pain
• 29:04 Transition to outpatient care
• 32:46 General admission orders for sickle cell
• 38:44 Management of Acute Chest Syndrome
• 42:01 Simple vs exchange transfusion
• 47:08 Transfusion in preoperative and pregnant patients
• 48:03 Transitions and follow-up care
• 49:35 Key takeaways and Outro

Inpatient Sickle Cell Disease Pearls

1. Administer analgesia within 60 minutes of arrival in ED for vaso-occlusive crisis and reassess every 30-60 minutes.
2. Manage acute pain crises with IV PCA using bolus doses. Do not use a basal rate, but continue any long-acting oral opioids taken at baseline.
3. Use multimodal pain management. Adjuvant therapy may include NSAIDs, acetaminophen, heating pads/blankets, and topical agents.
4. Coordinate close follow-up with the outpatient team and communicate any changes to opioid regimen
5. Patients with sickle cell disease (SCD) are not at a higher risk of opioid misuse or overdose compared to the general population.
6. Acute chest syndrome might require antimicrobials and blood transfusion (exchange or simple) in addition to the standard management of acute vaso-occlusive crisis.
7. Simple transfusion is reasonable when hemoglobin 2 g/dL below baseline.

Inpatient Sickle Cell – In-Depth Show Notes

Sickle Cell – Emergency Room Management

• Ask where the pain is located and if it feels like prior pain crises. Consider it a RED FLAG if pain deviates from their norm and look for complications (expert opinion).  
• Ask about a prior history of acute chest.
• Early triage and administration of analgesia within 60 minutes (coined Door to Analgesia by Paul Williams) is the standard for managing vaso-occlusive crises as per American Society of Hematology (ASH) guidelines (Brandow, 2020).
• Reassessment should be performed every 30 to 60 minutes (Brandow, 2020).
• Specialized sickle cell infusion centers (which provide rapid triage, analgesia, IV fluids, individualized pain management, and necessary lab work) can reduce hospitalization rates (Lanzkron, 2021).

Misconceptions about opioid use disorder

• A common misconception is that patients with sickle cell disease have a higher risk of opioid misuse or addiction, which is not supported by evidence (Ruta, 2016).
• It’s important to treat pain adequately and appropriately, avoiding under-treatment which could worsen symptoms and the misperception of “pseudo addiction”.
• Physicians should seek help from pain management or palliative care specialists if unsure about opioid dosing (expert opinion).

Inpatient Acute Pain Management

• Continue long-acting opioids if taken at home (expert opinion).
• Replace the patient’s home, short-acting oral opioids with IV therapy. Note: Acute pain will require a higher opioid equivalent dose than a patient’s home dose. 
• IV patient-controlled anesthesia (PCA) with bolus dosing without a continuous rate is recommended by Dr. Shin for safety. She often chooses a 15-minute lockout.
• The ASH guidelines do not recommend for or against continuous PCA dosing due to a lack of evidence (Brandow, 2020).
• Dr. Shin deploys multimodal pain management strategies including the use of oral NSAIDs (Brandow, 2020), heating pads/blankets, acetaminophen, and topical agents like lidocaine patches or topical NSAIDs (expert opinion).
• Exit strategy: Communicate the treatment plan to the patient. Set expectations that the PCA will be stopped and they will be transitioned back to their home regimen at the time of discharge (expert opinion).
• Home opioid doses might be adjusted at discharge, but only through coordination with the patient’s outpatient prescriber (expert opinion).

Basic Orders for Inpatient Sickle Cell Management

The following recommendations are supported by the Society for Hospital Medicine module on “Sickle Cell Disease: Triage and Initial Management” (Groningen, 2021) and are consistent with Dr. Shin’s recommendation.

• Bowel regimen for opioid-induced constipation 
• Venous thromboembolism (VTE) prophylaxis
• Maintain euvolemia. IV fluids can be used if needed (bolus or maintenance). Dr. Shin prefers hypotonic D5 ½ normal saline (expert opinion) for its theoretical benefit.
• Maintain oxygen sats above ≥95%.
• Continue hydroxyurea unless sepsis or major cytopenias; dose adjustment recommended for eGFR under 60 or on renal replacement therapy (per Lexicomp).
• Labs (complete blood count, comprehensive metabolic panel, lactate dehydrogenase, reticulocyte count) can be checked at baseline and then as needed for changes.
• Incentive spirometry and early ambulation for lung function and prevention of acute chest (McCavit, 2014 – ASH pocket guide). 

Acute Chest Syndrome

• Acute chest syndrome (ACS) is a complication of sickle cell disease that often presents like pneumonia. It can progress rapidly to respiratory failure or multi-organ failure.
• Diagnose ACS in patients with a new infiltrate on chest imaging plus any 2 of the following: pleuritic chest pain, hypoxemia, tachypnea, or fever (Kavanagh, 2022).
• VTE, fat embolism, and in situ sickling can precipitate ACS (Kavanagh, 2022).
• Reevaluate the level of care required for patients with ACS.
• Consult hematology.
• Antibiotics (pneumonia coverage) +/- antivirals (e.g. treat influenza) often necessary due to absent splenic function in patients with SCD (“ACS in SCD” UpToDate accessed 27 Dec 23).
• Blood transfusion (simple or exchange) might be needed, in addition to the standard management discussed above.

Blood transfusion in acute sickle cell crisis

• Transfusions carry the risk of transfusion reactions, iron overload, hyperviscosity, and formation of alloantibodies.
• Exchange transfusions require specialized centers with appropriate equipment and knowledgeable personnel (Stussi, 2019). 
• A common threshold for simple transfusion is symptomatic anemia with hemoglobin 2 grams or more below baseline (“RBC transfusion in SCD” UpToDate accessed 27 Dec 2023)
• Simple transfusion is reasonable for symptomatic anemia, blood loss, aplastic anemia, sequestration in liver or spleen, or mild acute chest syndrome (Groningen, 2021; Han, 2021)
• Exchange transfusions considered for multiorgan dysfunction, moderate-severe acute chest syndrome, stroke, priapism, surgery requiring general anesthesia, complicated pregnancy (Groningen, 2021; Han, 2021)

Transition from inpatient to outpatient

• Ensure patient has timely follow up appointments with hematology and/or primary care (expert opinion).
• Patients may need adjustments to their pain medications post-discharge, and clear communication with outpatient providers is essential for managing this transition (expert opinion).

Key takeaways

• Early and adequate pain management is key in sickle cell crises.
• Collaboration with specialists and clear communication with patients about their treatment plans and expectations are vital.
• Avoid misconceptions about opioid misuse in sickle cell disease.
• Focus on individualized patient care to improve outcomes.

Links

1. TMNT Mutant Mayhem (film)
2. Spider-Man: Across the Spider-verse (film)
3. ESPN app for sports scores

Goal

Listeners will improve inpatient management of sickle cell disease

Learning objectives

After listening to this episode listeners will…

1. Manage sickle cell pain crises in a hospitalized patient
2. Recognize acute chest syndrome and other acute complications from sickle cell disease
3. Recognize when to perform blood transfusions in a patient hospitalized with sickle cell crisis 

Disclosures

Dr. Shin reports no relevant financial disclosures. The Curbsiders report no relevant financial disclosures. 

Citation

Watto MF, Shin YM, Okamoto E, Williams PN. “#422 LIVE! Inpatient Management of Sickle Cell Disease, Acute Pain, RBC Transfusions & Acute Chest Syndrome”. The Curbsiders Internal Medicine Podcast. thecurbsiders.com/category/curbsiders-podcast Final publishing date January 15, 2024.