II. Epidemiology

  1. Affects almost all Sickle Cell Anemia patients
  2. May occur as early as age 6 months

III. Pathophysiology

  1. Deoxygenated Hemoglobin polymerizes in triple helix chains
  2. Results in Red Blood Cells assuming sickled shape
  3. Results in Occlusion of small vessels and capillaries

IV. Precipitating Factors

  1. Preceded by infection 25% of the time
    1. Human Parvovirus B19 is a common viral cause
  2. Cold Weather due to reflex vasospasm
  3. Dehydration in warm weather

V. Symptoms

  1. Child under age 18 years
  2. Recurrent painful crises
  3. Sudden onset pain in Abdomen, chest, back and extremities (esp. joints)
  4. Dactylitis (infants)

VI. Labs

  1. No lab abnormality defines VOC (clinical diagnosis)
  2. Profound Anemia
  3. Reticulocyte Count <1%

VII. Differential Diagnosis: Bone Pain

  1. Avascular Necrosis
    1. Avascular Necrosis of the Femoral Head
    2. Avascular Necrosis of the Humeral Head
  2. Osteomyelitis
    1. Infection of infarcted bone, especially long bones
    2. Salmonella is most common organism in Sickle Cell Anemia (S. Aureus represents <25% of cases)

VIII. Precautions

  1. Sickle Cell Crisis is not typically a drug seeking mission
    1. Patients who present with symptoms of Sickle Cell Crisis typically have crisis
    2. Vital Signs (Heart Rate, Blood Pressure) and labs do not differentiate a Sickle Cell Crisis
    3. Believe patients presenting with Sickle Cell Anemia and effectively treat their pain
    4. Rarely, patients without Sickle Cell Anemia present with factitous crisis
      1. However these patients may be identified from the medical record
      2. Review with prescribing providers and consider behavioral health referral
    5. Address Drug Seeking Behavior after the acute pain crisis (treat pain adequately during crisis)
      1. Consider VOC therapy plan (on file in ED or patient carries with them)
  2. Evaluate for other concurrent sickle cell complications
    1. Transient Red Cell Aplasia
    2. Cerebrovascular Accident in Sickle Cell Anemia
    3. Sickle Cell Hemolytic Crisis
    4. Acute Chest Syndrome
    5. Sepsis

IX. Management: Pain control for acute crisis

  1. Requires aggressive Opioid analgesia
    1. Avoid delays in management
      1. Triage patient urgently to a management bed within 30-60 minutes of presentation
      2. Start at outset by titrating to high dose Opioids in first 10 minutes of management
    2. Pain management is based on patient's reported level of pain
    3. Avoid allowing Analgesics to wear off prior to re-dosing
    4. Re-evaluate pain level and Sedation every 15-30 minutes
      1. Increase dose by 25% if inadequate pain control
      2. Monitor closely for respiratory depression
    5. Consider Patient Controlled Analgesia (PCA Pump)
  2. Avoid agents with adverse effects
    1. Avoid Meperidine (Demerol)
      1. Meperidine metabolite normeperidine is neurotoxic and associated with increased Seizure risk
      2. Meperidine is associated with euphoria and less effective Analgesic effect
      3. Morphine or Dilaudid are preferred over Meperidine (Demerol)
    2. Avoid NSAIDS
      1. High Incidence of occult renal dysfunction in Sickle Cell Anemia
      2. Serum Creatinine typically underestimates renal dysfunction in Sickle Cell Anemia
      3. In some cases may be used for mild to moderate pain if no contraindications
    3. Avoid parenteral Antihistamines
      1. Associated with euphora
      2. Pruritus may be less common with Dilaudid than Morphine
      3. Pruritus may be treated with Benadryl
  3. Avoid agents not shown to offer benefit
    1. Magnesium does not appear to reduce pain, reduce Opioid use or reduce lengths of ED stays
      1. Brousseau (2015) Blood 126(14): 1651-7 [PubMed]
    2. Antiplatelet agents (e.g. Prasugrel) does not appear to prevent vaso-occlusive crisis
      1. Heeney (2016) N Engl J Med 374(7): 625-35 [PubMed]

X. Management: Other Measures

  1. Intravenous Fluids
    1. Avoid Fluid Overload (risk of Atelectasis and Acute Chest Syndrome)
    2. Limit total fluid to <1.5x maintenance requirements
    3. On achieving euvolemia, consider maintenance with Hypotonic Saline (D5 1/2NS) which may enter the RBC
  2. Oxygen
    1. Only indicated if Oxygen Saturation <93%
    2. Oxygen may be associated with a higher risk of Bone Marrow suppression and secondary need for Blood Transfusions
  3. Incentive Spirometry
    1. Decreases Atelectasis risk and risk of Acute Chest Syndrome
  4. Adjunctive measures
    1. Local heat applied to painful area
  5. Disposition
    1. Admission criteria after 3 Opioid doses is likely to be too restrictive and result in over-admitting
    2. Consider longer emergency department course (up to 6 hours) for pain stabilization to determine if admission indicated

XI. Course

  1. Acute Crisis usually resolves spontaneously 7-10 days

XII. References

  1. Welsh and Welsh (2016) Crit Dec Emerg Med 30(11): 15-23
  2. Glassberg and Weingart in Herbert (2012) EM: Rap 12(8): 5-6
  3. Preboth (2000) Am Fam Physician 61 [PubMed]
  4. Yawn (2015) Am Fam Physician 92(12): 1069-76 [PubMed]

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Ontology: Hemoglobin SS disease with crisis (C0238425)

Definition (CSP) broad term used to describe several different acute conditions occurring with sickle cell disease, including aplastic crisis, hemolytic crisis, and vasoocclusive crisis.
Concepts Disease or Syndrome (T047)
ICD9 282.62
ICD10 D57.0 , D57.00
SnomedCT 417425009, 72279006, 191197002
English Sickle-cell anaemia with crisis, Sickle-cell anemia with crisis, Sickle cell anaemia with crisis, SIckle cell anaemia with crisis, sickle cell crisis (diagnosis), sickle cell crisis, anemia hemolytic sickle Hb-SS disease with crisis, anemia hemolytic sickle cell crisis, sickle cell-hemoglobin SS disease with crisis, sickle cell-hemoglobin SS disease with crisis (diagnosis), Sickle cell anaem with crisis, HB-S disease with mention of crisis, Hb-SS disease with crisis, Hb-SS disease w crisis, Sickle-cell disease NOS with crisis, Hb-SS disease with crisis, unspecified, sickle cell anemia crisis, cell crisis sickle, crisis disease hb-s mention, sickle-cell crisis, anemia cell crisis sickle, cells crisis sickle, Hb-S disease with mention of crisis, sickle cell anemia with crisis (diagnosis), Hemoglobin S disease with crisis (disorder), Sickle cell anemia with crisis (disorder), Hemoglobin S disease with crisis, Haemoglobin S disease with crisis, crisis; sickle-cell, disease (or disorder); sickle-cell, with crisis, sickle-cell; anemia, with crisis, sickle-cell; crisis, sickle-cell; disorder, with crisis, anemia; sickle-cell, with crisis, Sickle-cell crisis NOS, Sickle cell crisis, Sickle cell anemia with crisis, Haemoglobin SS disease with crisis, Hemoglobin SS disease with crisis, Hemoglobin SS disease with crisis (disorder)
Italian Anemia a cellule falciformi con crisi, Emoglobinopatia -S con menzione di crisi, Emoglobinopatia SS con crisi, Crisi falciforme
Dutch Hb-SS aandoening met crisis, HB-S aandoening met vermelding van crisis, sikkelcelanemiecrisis, aandoening; sikkelcel, met crisis, anemie; sikkelcel, met crisis, crisis; sikkelcel, sikkelcel; aandoening, met crisis, sikkelcel; anemie, met crisis, sikkelcel; crisis, Sikkelcelanemie met crisis, sikkelcelanemie met crisis
French Hémoglobine S avec mention de crise, Hémoglobine SS avec crise drépanocytaire, Drépanocytose avec crise, Crise drépanocytaire
German HB-SS-Krankheit mit Krise, Sichelzellerkrise, HB-S-Krankheit mit Erwaehnung einer Krise, Sichelzellenanaemie mit Krisen, Sichelzellanaemie mit Krise
Portuguese Anemia de células falciformes com crises, Doença Hb-SS com crises, Doença Hb-S com referência a crises, Crise de células falciformes, Anemia de células falciformes com crise
Spanish Crisis de anemia falciforme, Enfermedad de la Hb SS con crisis, Enfermedad de la Hb S con mención de crisis, Anemia drepanocítica con crisis, drepanocitosis con crisis, anemia de células falciformes con crisis (trastorno), enfermedad por hemoglobina S con crisis, drepanocitosis con crisis hemolítica (trastorno), anemia de células falciformes con crisis, Anemia de células falciformes con crisis, enfermedad por hemoglobina SS con crisis hemolítica (trastorno), enfermedad por hemoglobina SS con crisis hemolítica
Japanese ヘモグロビンS症クリーゼ, 鎌状赤血球貧血クリーゼ, ヘモグロビンSS疾患クリーゼ, 鎌状赤血球クリーゼ, レンジョウセッケッキュウヒンケツクリーゼ, ヘモグロビンSSシッカンクリーゼ, カマジョウセッケッキュウヒンケツクリーゼ, ヘモグロビンSショウクリーゼ, レンジョウセッケッキュウクリーゼ
Czech Krize u srpkovité anémie, Homozygotní forma srpkovité anémie s vasookluzivní krizí, Hemoglobinopatie S s uvedením krize, Srpkovitá anémie s krizí
Korean 발증을 동반한 낫적혈구 빈혈
Hungarian Hb-Ss betegség crisissel, Sarlóssejtes anaemia crisissel, Sarlós sejt crisis, Sarlós sejt anaemia crisissel, Hb-S betegség crisis említésével

Ontology: sickle cell pain crisis (C0546176)

Concepts Finding (T033)
English sickle cell pain crisis, cell crisis pain sickle, sickle cell crisis pain

Ontology: Hb-SS disease with vasoocclusive pain (C2873760)

Concepts Disease or Syndrome (T047)
ICD10 D57.0
English Hb-SS disease with vasoocclusive pain