The inherited hemoglobin disorders stem from abnormal Hb structure (eg, sickle cell disease [SCD] or abnormal Hb production (eg, the thalassemias). Anemia occurs when destruction of red blood cells outstrips production. Most patients are aware of their dyshemoglobinemia status.
The most common complaints in SCD (homozygous (HbSS) or sickle trait (heterozygus [HbAS]) patients are pain, localized or generalized, weakness, or infectious complaints. Physical examination findings commonly include pale complexion, venous stasis changes, jaundice, hepatosplenomegaly, anemic cardiac flow murmurs, cardiomegaly, and high output CHF.
Painful vaso-oclusive crisis in the musculoskeletal system or diffusely in the abdomen are the most common presentation to the ED. Crisis occur when sickled RBCs mechanically obstruct blood flow, causing ischemia, organ damage, and infarcts. Crisis-causing stresses include fever or infection (especially encapsulated organisms Haemophilus influenza or Pneumococcus), cold exposure or high altitude, dehydration or overexertion, medication noncompliance, or drug use.
Acute symptomatic anemia results from splenic sequestration or bone marrow failure (aplastic crisis) and presents with weakness, dyspnea, CHF, or shock.
Life or limb-threatening events seen in SCD patients include acute chest syndrome (vaso-occlusive pulmonary insult), stroke, renal infarct, mesenteric infarcts, sepsis, osteomyelitis, pneumonia, or priapism.
Diagnosis and Differential
The degree of illness guides the evaluation of an acute crisis. Although workups should be individualized, the more common protean complaints (pain, weakness, fever) must include a search for a cryptic inciting event. See Table 136-1.
Table 136-1 Guidelines for the Assessment and Management of Acute Vaso-Occlusive Crisis |Favorite Table|Download (.pdf)
Table 136-1 Guidelines for the Assessment and Management of Acute Vaso-Occlusive Crisis
Duration and location of pain
History of fever
History of focal swelling or redness
Precipitating factors for acute episode
Medications taken for pain relief
Assess degree of pain
Inspect sites of pain, looking for swelling, warmth, redness
General: respiratory distress, pallor, hydration, jaundice, rash
Vital signs: especially temperature, pulse oximetry
Respiratory: chest wall, lung sounds
Heart: cardiomegaly and systolic murmur common with chronic anemia
Abdomen: tenderness, organomegaly
If moderate to severe pain, focal pathology is present, or pain is atypical for acute episode
Complete blood count, leukocyte differential, reticulocyte count, urinalysis
Chest radiograph, if signs of lower respiratory tract pathology
Blood cultures and additional blood tests: as indicated by clinical condition
Bed rest, provide warmth, and a calm, relaxing atmosphere
Distractions where appropriate—television, music, etc.
Oral fluids: typically about 3 L per day
IV fluids to correct dehydration or if reluctant to drink or vomiting is present
Oxygen: not routinely required, unless hypoxemia is present
Encourage deep breathing, incentive spirometry
Use analgesics appropriate to degree of pain
Acetaminophen for mild pain
NSAID for mild to moderate pain (avoid if renal insufficiency is present)
Opioids for moderate to severe pain, typical initial doses include: