Inpatient / hematology and oncology

Sickle Cell Disease and Vaso-Occlusive Crisis (VOC)

Last Updated: 12/27/2022

# Sickle Cell Disease ***
# Vaso-Occlusive Crisis
# *** c/f Acute Chest

-- Chart Check: pain plan, outpatient hematologist, prior regimens when admitted, prior complications,
-- Admission Criteria: unable to control pain with home regimen; requiring IV medications; c/f acute chest or other complication
-- HPI Intake: location of pain, triggers, home pain regimen, evidence of stroke
-- Can't Miss: acute chest, PE, ACS, stroke
-- Admission Orders: CBC, BMP, LFTs, cogs, T+S, hemolyis labs (retic, LDH, haptoglobin), infectious workup if c/f underlying trigger, CXR to assess for acute chest
-- Initial Treatment to Consider: pain management ASAP with PCA if need be, antibiotics if c/f acute chest, and consult heme to discuss pheresis if c/f hyperviscosity syndrome

-- History:  *** prior VOC, pain plans, prior complications (stroke, acute chest, asplenia, AVN)
-- Clinical: *** chest pain, AMS, bony pain c/f AVN, priapism
-- Exam: *** localized pain, mental status, focal neuro deficits, LUQ pain (splenic sequestration),
-- Data: *** Hgb, CXR, hemolysis labs (retic, LDH, haptoglobin)
-- Etiology/DDx: Triggers: dehydration, infection, acidosis, stress, menses, alcohol

The patient's HPI is notable for ***. Exam showed ***. Labwork and data were notable for ***. Taken together, the patient's presentation is most concerning for ***, with a differential including ***.

-- f/u LDH, haptoglobin, retic count
-- Monitoring: Trend daily CBCs, transfuse Hgb <7
-- Consult heme for pheresis if c/f stroke, splenic sequestration, acute chest, hyperviscosity

-- Pain: *** IV dilaudid (if no known prior 0.02-0.05mg/kg to max 1.5mg), oxycodone, toradol, tylenol; PCA if not adequately treating
-- O2: currently *** NC, continuous pulse ox with goal >95%; wean as able
-- Volume: continue maintenance fluids *** cc/hr (usually at least 150-200cc/hr)
-- Abx: *** (Ceftriaxone and azithro if c/f acute chest)
-- transfuse for Hgb >7
-- Bowel regimen while on opioid analgesia
-- Continue home *** (hydroxyurea, folic acid)
-- Incentive Spirometry

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If You Remember Nothing Else

When patients present with a vaso-occlusive crisis, their pain should be treated early and adequately. This includes switching to a PCA if the regumen is not meeting the patient's analegesia needs. Do not miss acute chest, stroke, splenic sequestration, and involve the hematology consultants if there is concern for hyperviscosity requiring pheresis. Avoid stigmatizing language in documentation and in person when treating patients with sickle cell disease.

Clinical Pearls

  • Sickle Cell Anemia most commonly is caused by the change of A to T in both alleles of the beta-globin gene (Glu6Val) which causes a missense mutation; a change in one allele causes sickle cell trait
  • Homozygous (HbSS) is the most common variant; the HgbS gene is carried by 8% of AA population
  • Sickle cell disease is usually diagnosed in outpatient setting usually, as there are now neonatal screening programs with Hb electrophoresis and HPLC to test for HbS
  • Pathophysiology - RBC with HbS polymerizes when deoxygenated and takes on sickle shape which impacts the cell membrane causing it to be more adherent to vascular endothelium and cause vaso-occlusive events
  • Most patients with sickle cell have a life expectancy 40-60 years - the most common cause of death is infection (33-48%) and is most often respiratory; also stroke, splenic sequestration, PE, ESRD
  • Transfusions help by diluting HbS, and increasing SpO2 to reduce sickling, however should be used for indications (stroke, organ failure, acute chest) and not just for acute pain episode to avoid iron overload over lifetime
  • Patients can have acute chest syndrome without a pain crisis - it’s 50/50
  • Patients who autoinfarct spleen will be susceptible to encapsulated organisms
  • It is Important to use neutral and non-stigmatizing language in the EHR and among colleagues when discussing patients with sickle cell disease - avoid using terminology like “sickler”, “frequent flyer”, “drug-seeking”, etc.
  • All patients with sickle cell should be vaccinated against Neisseria Meningitidis, HiB, Strep pneumo, HBV, flu, COVID
  • Outpatient meds include hydroxyurea, folate/multivitamin, L-glutamine, voxelotor, crizanilizumab
  • Currenty, BMT is the only cure, but gene therapy options are being developed
  • Dactylitis is most common symptom in children and adolescents
  • Heart failure can develop in patients with sickle cell disease from chronic severe anemia
  • Parvovirus B19 can cause aplastic crisis - usually causes fifth disease in children
  • Staph is the more common cause of osteo (and in the overall population), but salmonella is a characteristic cause of osteo unique to the sickle cell population
  • Sickle cell trait can have painless hematuria due to renal papillary necrosis 
  • Hydroxyurea increases fetal hemoglobin so the proportion of sickled hemoglobin is lower and polymerization decreases - reducing vaso-occlusive episodes

Trials and Literature

  • Sickle Cell Disease NEJM Review 2017
  • Incentive Spirometry decreases complications associated with acute chest in a small trial
  • HOPE Trial - 2019 - voxeletor increased hemoglobin and reduced hemolysis but did not lead to improvement in incidence of VOC or other clinical events at 24 weeks in patients with one or more VOC in the last year

Other Resources