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Sickle Cell

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Managing Sickle Cell Disease in the ED

Section IconCommunication
Patient report of pain is the gold standard
There are no vital sign changes or lab values that confirm or rule-out a sickle cell pain crisis
Do not refer to patients with SCD as “sicklers,” as this is a derogatory term
Requests for specific pain medicines/doses are most commonly due to past experience, not drug-seeking behavior
  • Opioid use was stable from 2008-2013 in the SCD population, in contrast to the general US population
  • Deaths from opioid overdose was ≤ 10 per year in individuals with SCD from 1999-2013, representing only 0.77% of all deaths in this population, significantly lower than other non-cancer conditions including low back pain, fibromyalgia and migraine
Build trust by believing the patient is in pain
Patients may not be glad to see you – show them you are here to help
  • Negative ED experiences in the past may make them guarded or mistrustful
  • Pain can make anyone irritable, impatient, or upset
  • Empathetic nonverbal communication is essential (eye contact, facial expression, gestures)
  • Be patient when asking questions; it is often difficult to speak when in severe pain
Patients may bring a caregiver with them, as it may be difficult to understand treatment plans and ask questions when in severe pain.
References
  1. National Academies of Sciences, Engineering, and Medicine 2020. Addressing Sickle Cell Disease: A Strategic Plan and Blueprint for Action. Washington, DC: The National Academies Press. Learn More Here.
  2. Ballas SK, Kanter J, Agodoa I, et al. Opioid utilization patterns in United States individuals with sickle cell disease. Am J Hematol 2018;93(10):E345-47.
  3. Ruta NS, Ballas SK. The opioid epidemic and sickle cell disease: Guilt by association. Pain Med 2016;17(10):1793-98.
  4. Power-Hays A, McGann PT. When actions speak louder than words — Racism and sickle cell disease. N Engl J Med 2020 Nov 12;383:1902-3.
  5. Glassberg J. Improving emergency department-based care of sickle cell pain. Hematology Am Soc Hematol Educ Program. 2017 Dec 8; 2017(1): 412–417.
  6. Videos demonstrating excellent communication strategies to assess and manage pain, address high ED utilization and perception of drug addiction, and patient experiences of acute SCD pain can be found here
Section IconTriage
Sickle cell pain is usually severe and requires immediate treatment; evidence-based guidelines recommend administering pain medication within 60 minutes of arrival
Assign an emergent priority (e.g., ESI level 2) due to
  • severe pain that cannot be managed in the waiting room
  • high risk situation that needs rapid evaluation for other serious complications
Increase level of concern if pain is not similar to prior episodes or new symptoms are present
Patients with ≥ 3 admissions/year for sickle cell pain are at increased risk of death
Vital Signs/Neuro Status
Normal vital signs do not rule out pain crisis
Patient report of pain is gold standard
Hypoxia should be judged against patient’s baseline
Fever ≥ 100.4 degrees (≥ 38 degrees C) should prompt work-up for underlying cause, as sickle cell disease decreases splenic function
  • Children and adolescents with fever ≥ 101.5 degrees F (≥ 38.5 degrees C) should receive broad spectrum antibiotics ≤ 1 hour of arrival
Hypotension and tachycardia should raise concern for sepsis, dehydration and significant anemia due to aplastic anemia or splenic sequestration
Tachypnea should raise concern for acute chest syndrome, occult hypoxemia and pulmonary embolus
Altered mental status, severe or atypical headache, focal neurologic findings (such as vision changes), or new seizure should raise concern for stroke in both children and adults
Past Medical History
Ask about history of severe SCD complications (such as acute chest syndrome, pain crises, stroke), recent admissions and recent blood transfusions
Patients are born with SCD; onset and severity of symptoms vary
Initiation of Care Should Not Be Delayed Due to Space Constraints
Consider alternative spaces (such as asthma bay) if no treatment spaces available
Consider administering pain medications in triage (e.g., subcutaneous opioids for adults and intranasal fentanyl for children – see Treatment)
Utilize physician in waiting room, split flow model, etc. to expedite care when available
References
  1. U.S. Department of Health and Human Services. National Heart Lung, and Blood Institute. Evidence-Based Management of Sickle Cell Disease, Expert Panel Report, 2014 - View PDF.
  2. National Academies of Sciences, Engineering, and Medicine 2020. Addressing Sickle Cell Disease: A Strategic Plan and Blueprint for Action. Washington, DC: The National Academies Press. Learn more here.
  3. Elimariah H, Garrett ME, de Castro LM, et al. Factors associated with survival in a contemporary adult sickle cell cohort. Am J Hematol 2014;89(5):530-35.
  4. Videos demonstrating excellent communication strategies to assess and manage pain, address high ED utilization and perception of drug addiction, and patient experiences of acute SCD pain can be found here.
Section IconHistory
History – Part 1
From the patient
  • Where is pain most intense? Where does it hurt the most?
  • Is there anything unusual or concerning about the pain you are experiencing?
    • Does this feel like your typical pain crisis?
    • If the pain is different, how is it different – in location? Sensation? Intensity?
  • Have you had a fever in the last 24 hours?
From patient, caregiver, or chart (patient may be in too much pain to give complete history)
  • When did the pain start?
  • What medicines did you try at home? What dose did you take, and when? Did they help?
  • Do you have a pain plan in your chart (with preferred pain medicines and doses)? If not:
    • What pain medicine(s) work best for your pain in the ED? Do you know what dose works for you?
    • Are there any pain medicines that do not work, or you have had a bad experience with?
  • What other things will make you more comfortable?
    • Heat packs
    • Blankets
    • Distraction – such as talking on the phone or watching TV
TIP: Share the initial plan and rationale with the patient and caregiver (if present)
History – Part 2 (after Evaluation and initial orders placed)
From chart, patient or caregiver (patients may be in too much pain to give complete history)
  • Do you have pain most days? What level of pain is typical for you?
  • What do you take every day for pain?
  • If patient is followed at your institution:
    • I have your list of medicines here (review list); are there others that you take?
    • I see that you have had the following problems in the past (review list); are there others that I missed?
    • I found/did not find (list) surgeries in your chart [review list]; are there others that I missed?
  • If patient is not followed at your institution:
    • Do you have a primary hospital, clinic or doctor who treats you for sickle cell disease? Do you have other doctors that you see, such as primary care?
    • Do you take medicines every day, such as hydroxyurea? Are there other medicines you take only when needed (such as for pain)?
    • When was your last blood transfusion? Do you get regularly scheduled blood transfusions (~once/month)?
    • Do you have any SCD-related health issues?
      • History of acute chest syndrome, stroke, sepsis, kidney problems, blood clots?
      • Have you had surgery, such as removal of gall bladder or spleen?
    • What is your hemoglobin or hematocrit when you are well?
TIP: Regularly update the plan and rationale with the patient and caregiver (if present)
References
  1. U.S. Department of Health and Human Services. National Heart Lung, and Blood Institute. Evidence-Based Management of Sickle Cell Disease, Expert Panel Report, 2014. View PDF here.
  2. National Academies of Sciences, Engineering, and Medicine 2020. Addressing Sickle Cell Disease: A Strategic Plan and Blueprint for Action. Washington, DC: The National Academies Press. Learn more here.
  3. Videos demonstrating excellent communication strategies to assess and manage pain, address high ED utilization and perception of drug addiction, and patient experiences of acute SCD pain can be found here.
Section IconEvaluation
Vital Signs
Normal vital signs do not rule out pain crisis or correlate with severity of pain
Hypoxia should be judged against patient baseline; raises concern for acute chest, pneumonia, and pulmonary embolism
Tachypnea should raise concern for acute chest syndrome and pulmonary embolism
Fever should prompt rapid work-up for underlying cause since patients are functionally asplenic; also consider delayed hemolytic transfusion reaction if transfused in previous 4 weeks
Hypotension and tachycardia raise concern for sepsis, dehydration and significant anemia due to aplastic crisis or splenic sequestration
Physical Exam/Differential Diagnosis
NOTE: Severe SCD complications often develop during a pain crisis or mimic one. Contact hematology EMERGENTLY if you suspect one, such as acute stroke or acute chest syndrome with significant respiratory distress.
Location of pain
  • Localized bony pain that is different from previous pain crises
    • Osteomyelitis/septic arthritis
    • Priapism (sometimes report thigh pain)
    • Avascular necrosis
  • Calf pain could be DVT
  • Diffuse pain could be systemic infection: Check vital signs, look for rash or petechiae on skin (including palms/soles) and gums
Altered mental status, severe or atypical headache, focal neurologic findings (such as vision changes), or new seizure: Consider stroke in children and adults
Fatigue/jaundice/pallor: Consider aplastic anemia, sepsis or hemolysis due to delayed hemolytic transfusion reaction
Chest pain: Consider acute chest syndrome (leading cause of death in SCD)
Respiratory distress: Consider acute chest syndrome, pulmonary embolism and sepsis
Abdominal pain or distension: Assess for splenomegaly or hepatomegaly (splenic or hepatic sequestration), RUQ pain (acute cholecystitis), and consider priapism if low abdominal pain
Assess fluid status for dehydration
Laboratory Workup
Use order set for SCD if available
Get CBC with differential and reticulocyte count; consider comprehensive metabolic panel in adults if clinically indicated.
  • Hemoglobin and retic count should be interpreted relative to baseline values
    • Anemia + low retic = concern for aplastic anemia
    • Anemia + low platelet count + normal retic = concern for splenic sequestration
  • Anemia can progress extremely quickly due to low baseline hemoglobin and short half- life of RBCs due to clearance of abnormal cells.
Obtain blood culture in all children with fever, and adults with central venous access or history of sepsis or splenectomy
Imaging
CXR (2 view if able) for fever, chest pain, or respiratory symptoms/findings to assess for acute chest syndrome
Other Considerations
Patients should not be penalized for using coping mechanisms to manage pain, including naps, distraction techniques, discussions with family, or meditation
Patients with frequent visits to the ED are at increased risk of serious complications
  • Patients with ≥ 3 admissions/year for sickle cell pain are at increased risk of death
  • Some may also have significant mental health and/or psychosocial complications which can exacerbate their disease course. This should not be used to dismiss the severity of their pain. Consult social work or case management if available.
References
  1. U.S. Department of Health and Human Services. National Heart Lung, and Blood Institute. Evidence-Based Management of Sickle Cell Disease, Expert Panel Report, 2014. View PDF here.
  2. National Academies of Sciences, Engineering, and Medicine 2020. Addressing Sickle Cell Disease: A Strategic Plan and Blueprint for Action. Washington, DC: The National Academies Press. Learn more here.
  3. Glassberg J. Improving emergency department-based care of sickle cell pain. Hematology Am Soc Hematol Educ Program. 2017 Dec 8; 2017(1): 412–417. doi: 10.1182/asheducation-2017.1.412
  4. Elimariah H, Garrett ME, de Castro LM, et al. Factors associated with survival in a contemporary adult sickle cell cohort. Am J Hematol 2014;89(5):530-35.
Section IconTreatment
Treat Immediately
Sickle cell pain is usually severe and requires immediate treatment
Do not wait for lab results before starting pain medications; there are no lab values that confirm or rule-out a sickle cell pain crisis
Requests for pain medicines/doses are most commonly due to past experience, not drug-seeking behavior
  • Opioid use was stable from 2008-2013 among individuals with SCD, in contrast to the general US population
  • Deaths from opioids was ≤ 10 per year in individuals with SCD from 1999-2013 (representing only 0.77% of deaths in this population), significantly lower than other non-cancer conditions including low back pain, fibromyalgia and migraine
Evaluation and treatment should begin in alternative spaces if no treatment rooms available
Consider administering pain medicines while in waiting room/triage
Treatment Approach
Use a standardized pain plan or protocol to treat acute SCD pain
Check electronic health record for an existing patient-specific pain plan (individualized medications and doses); if none found, elicit patient preference based on previous experiences.
Treating acute moderate to severe pain
  • 1st Dose within 60 min of ED arrival
    • Consider intranasal fentanyl as 1st dose in children
    • Consider subcutaneous route for adults with difficult IV access
  • Reassess & re-dose every 30 min
    • until pain improves
  • Reassess 15-30 min after 1st dose
    • Can increase dose by 25% once if no change
Routes of medication administration
  • Use ultrasound-guided IV placement if available; gaining IV access is often challenging
  • Subcutaneous route can also be used to provide rapid analgesia
  • Avoid IM doses of opioids unless other routes attempted and failed
Sleep following opioids does not usually indicate pain control
  • Wake the patient, assess pain level and re-dose based on patient report
  • Drowsiness is a side effect of opioids, compounded by sleeplessness due to severe pain
Suggested Parenteral Opioid and NSAID Doses for Children (< 12 years or < 50 kg):
Scroll →
Pain Medication
Dose
Max Single Dose
Frequency

Intranasal fentanyl*

2 mcg/kg

100 mcg or 1 ml per nare

May repeat x1 after 10 minutes

Morphine IV**

0.1 mg/kg

10 mg

Repeat every 15-30 minutes until pain controlled

Hydromorphone IV

0.015 mg/kg

1.2 mg

Repeat every 15-30 minutes until pain controlled

Ketorolac IV**

0.5 mg/kg

15-30mg

Once

*Recommended as 1st pain medication in children
**Use with caution in patients with mild renal dysfunction
Suggested Parenteral Opioid and NSAID Doses for Adults and Adolescents (≥ 12 years or ≥ 50 kg)
Scroll →
Pain Medication
Dose
Max Single Dose
Frequency

Morphine IV*

0.1 mg/kg

10 mg

Repeat every 15-30 minutes until pain controlled

Hydromorphone IV

0.015 mg/kg

4 mg

Repeat every 15-30 minutes until pain controlled

Ketorolac IV*

0.5 mg/kg

15 mg

Once

*Use with caution in patients with mild renal dysfunction
Adjuncts for Pain
NSAIDs: Can be given in addition to opioids to manage acute pain and treat the inflammation associated with a sickle cell crisis
  • Exclusions include patients with significant renal dysfunction (up to 30% of adults with SCD) and full-dose anticoagulation; use caution in patients with peptic ulcer disease
IV fluids: No evidence that IV fluids are beneficial in euvolemic patients; may cause fluid overload and acute chest syndrome in certain patients
  • Consider IVF infusion rather than bolus and administer less than maintenance fluids if able to drink (e.g., 75% maintenance)
Non-pharmacologic interventions: Heat packs (no ice), blankets, distraction (using phone, watching TV)
Manage Opioid Side Effects
Pruritus:
  • Oral diphenhydramine 25 mg recommended. Avoid IV diphenhydramine push because it worsens sedation.
  • Low dose naloxone infusion (1 mcg/kg/hr) does not reverse effects of opioids; can be titrated up 0.5 mcg/kg/hr every 3-4 hours, maximum 4-5 mcg/kg/hr
  • IV diphenhydramine 25 mg if alternatives listed above do not control pruritus or are unavailable
Nausea/vomiting: Ondansetron is commonly used; use caution with prochlorperazine and metoclopramide due to sedating effects
Respiratory depression: Naloxone 0.4 mg/dose IV to alleviate sedation (not reverse analgesia)
Patient-Controlled Analgesia (PCA)
Consider for patients being admitted to prevent lapses in dosing
If EHR order set, pharmacy/hospital protocols, and/or SCD patient plans are available, use demand and basal dosing regimens; otherwise use demand dosing alone
Suggested Doses for Children (< 12 years or < 50 kg)
Scroll →
Medication
Demand Dose
1 Hour Limit

Morphine

0.015 - 0.02 mg/kg

0.12 mg/kg

Hydromorphone

0.003 mg/kg

0.03 mg/kg

Notes: Lockout period 6-10 minutes. Titrate as needed.
Suggested Doses for Adults and Adolescents (≥ 12 years or ≥ 50 kg)
Scroll →
Medication
Demand Dose
1 Hour Limit

Morphine

2 mg (0.02-0.03 mg/kg)

10 mg

Hydromorphone

0.4-1 mg (0.01-0.03 mg/kg)

4 mg

Notes: Lockout period 10 minutes. Titrate as needed. Order oral long-acting opioid if prescribed
Treatments to Use with Caution or Avoid
Steroids: Can trigger severe pain crises, but short courses can safely treat select conditions (e.g., asthma) when done in coordination with hematology/SCD provider
Ketamine: Guidelines found insufficient evidence for use outside of the inpatient setting
Blood transfusions: Do not give transfusions to manage sickle cell pain crisis unless directed by the patient’s hematologist or SCD provider
Oxygen: Do not use oxygen unless patient is hypoxic compared to their usual oxygen level
Special Populations
Pregnant patients with pain crisis require treatment with opioids; the benefits of treatment outweigh the harms to fetus. Offer acetaminophen as an option or adjunct for pain
For patients on methadone, long-acting opioids, or lidocaine or fentanyl patch: Continue home dose
For patients on methadone or buprenorphine: Look for individualized pain plan; if none found, consult hematologist/SCD provider for guidance on pain medication dosing
References
  1. Brandow AM, Carroll CP, Creary S, et al. American Society of Hematology 2020 guidelines for sickle cell disease: management of acute and chronic pain. Blood Advances. 2020;4(12):2656-2701.
  2. U.S. Department of Health and Human Services. National Heart Lung, and Blood Institute. Evidence-Based Management of Sickle Cell Disease, Expert Panel Report, 2014. View PDF here.
  3. National Academies of Sciences, Engineering, and Medicine 2020. Addressing Sickle Cell Disease: A Strategic Plan and Blueprint for Action. Washington, DC: The National Academies Press. Learn more here.
  4. Tanabe P, Hafner JW, Martinovich Z, et al. Adult emergency department patients with sickle cell pain crisis: Results from a quality improvement learning collaborative model to improve analgesic management. Acad Emerg Med 2012;19(4):430-38.
  5. He F, Jiang Y, Li L. The effect of naloxone treatment on opioid-induced side effects: A meta-analysis of randomized and controlled trials. Medicine 2016;95(37):e4729. doi: 10.1097/MD.0000000000004729.
  6. Ballas SK, Kanter J, Agodoa I, et al. Opioid utilization patterns in United States individuals with sickle cell disease. Am J Hematol 2018;93(10):E345-47.
  7. Ruta NS, Ballas SK. The opioid epidemic and sickle cell disease: Guilt by association. Pain Med 2016;17(10):1793-98.
  8. Power-Hays A, McGann PT. When actions speak louder than words — Racism and sickle cell disease. N Engl J Med 2020 Nov 12;383:1902-3.
  9. Glassberg J. Improving emergency department-based care of sickle cell pain. Hematology Am Soc Hematol Educ Program. 2017 Dec 8; 2017(1): 412–417.
Section IconDisposition
Admit/Observe
Consider admission if pain is not well-controlled; patients usually know if their pain has stabilized at a manageable level
Other complications found (e.g., infiltrate on CXR; suspect serious or systemic infection)
Consider admission for young (< 1 year) patients, or older patients with fever if they cannot seek care quickly or follow-up with SCD provider the next day
Consider alternative treatment settings for improving but not relieved pain, such as observation units, to allow for a longer duration to treat pain and spare a hospital admission
Discharge
If patient believes they can manage pain at home
Recommend close follow-up with hematology/SCD provider. Refer to a specialist if not already established
  • Recognize that there is not always ready access to a hematologist. Efforts should be made to establish a referral pathway to a local physician to manage these patients
Ensure adequate access to medications for management of pain as an outpatient
  • o Opioids:
    • Short-term (e.g., 3 days) opioid prescription as bridge to next outpatient visit
    • If concern for opioid misuse/overdose – contact outpatient provider to make plan
  • Non-opioids including acetaminophen, topical or oral NSAID (if not contraindicated): Continue for short-course (5-7 days total)
  • Non-pharmacologic means of pain control: Continue heat, hydration, distraction; avoid using ice
  • Bowel regimen to avoid opioid-induced constipation
Give clear return precautions, such as fever, difficulty breathing, chest pain, changes in mental status, uncontrolled pain
Some patients with frequent visits have unmet social or behavioral health needs including lack of insurance or transportation, unable to pay for prescriptions, unstable housing, etc. Make referrals to social work or case management as appropriate. Learn more here.
References
  1. Brandow AM, Carroll CP, Creary S, et al. American Society of Hematology 2020 guidelines for sickle cell disease: management of acute and chronic pain. Blood Advances. 2020;4(12):2656-2701.
  2. U.S. Department of Health and Human Services. National Heart Lung, and Blood Institute. Evidence-Based Management of Sickle Cell Disease, Expert Panel Report, 2014. View PDF here.
  3. Rushton S, Murray D, Talley C, et al. Implementation of an emergency department screening and care management referral process for patients with sickle cell disease." Prof Case Manag Sept/Oct 2019:24(5):240-248.
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Acknowledgments

CONTRIBUTORS
Caroline Freiermuth, MD, MS, FACEP (co-Chair) Patricia Kavanagh, MD (co-Chair) Lakiea Bailey, PhD Matoya A. Robinson, MS, MA Vicki Basalyga, PharmD, BCPS, BCPPS, ASHP Amanda Brandow, DO David Brousseau, MD, MS Aubri Carman, MD Patrick Carroll, MD Corrie Chumpitazi, MD, MS, FAAP, FACEP Emelia Frederick, MSN, RN, CEN Jon Mark Hirshon, MD, PhD, FACEP E. Donnell Ivy, MD Sophie Lanzkron, MD, MHS Logan Ramsey, MD JJ Strouse, MD, PhD Henry Young II, MD, FACEP

 

ACEP Staff
Loren Rives, MNA Riane Gay, MPA Mandie Mims, MLS Eileen Aguilar, MS Marla Payne

 

The tool was supported with an unrestricted educational grant provided by Novartis Pharmaceuticals.

 

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