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Patient Care Process for the Management of Sickle Cell Disease

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  • Patient characteristic including age, race, sickle cell disease type (see Table 102-1)

  • Patient medical history including frequency of pain episodes and hospitalizations due to acute or chronic complication of sickle cell disease (Table 102-2)

  • Clinical signs and symptoms (see Table 102-1)

  • Current medications

  • Immunization history (see Table 102-4)

  • Objective data (see Table 102-2)

    • BP, heart rate (HR), height, weight, and BMI

    • Labs (e.g., serum electrolytes, Scr, BUN, CBC, reticulocyte count, urinalysis)

    • Other diagnostic tests when indicated (e.g., cultures, parvovirus titers, chest x-ray)


  • Risk of infection or sepsis in febrile patients

  • Presence of acute complications such as vasoocclusive pain episodes, aplastic crisis, splenic sequestration, acute chest syndrome (see Table 102-2)

  • Presence of chronic complications associated with sickle cell disease

  • Health maintenance and preventive care (see Table 102-4)

  • Pain control for both acute episodes and chronic pain management


  • Goals of treatment

  • Hydroxyurea therapy regimen including dose, route, frequency and monitoring (see Tables 102-5, Figure 102-6)

  • Preventive care such as immunizations, penicillin prophylaxis, and stroke prevention

  • Pain management for acute and chronic pain

  • Patient education (e.g. purpose of treatment, side effects)

  • Referrals to other providers when appropriate (e.g., physician, dietician, psychological support programs)


  • Provide patient education regarding all elements of treatment plan

  • Use motivational interviewing and coaching strategies to maximize adherence

  • Schedule follow-up

Follow-up: Monitor and Evaluate

  • Presence of adverse effects

  • Occurrence of acute and chronic complications associated with sickle cell disease

  • Evaluate growth and development as well quality of life

  • Patient adherence to treatment plan using multiple sources of information

*Collaborate with patient, caregivers, and other health professionals


For the chapter in the Wells Handbook, please go to Chapter 34. Sickle Cell Disease.



  • Image not available. Sickle cell disease is an inherited disorder caused by a defect in the gene for β-globin, a component of hemoglobin, and is called a qualitative hemoglobinopathy. Patients can have one defective gene (sickle cell trait) or two defective genes (sickle cell disease).

  • Image not available. Although sickle cell disease usually occurs in persons of African ancestry, other ethnic groups can be affected. Multiple mutation variants are responsible for differences in clinical manifestations.

  • Image not available. Sickle cell disease involves multiple organ systems. Usual clinical signs and symptoms include anemia, pain, splenomegaly, and pulmonary symptoms. Sickle cell disease is identified through routine newborn screening programs available in all 50 states. Early diagnosis allows early preventive and comprehensive care.

  • Image not available. Patients with sickle cell disease are at risk for infection. Prophylaxis against pneumococcal infection reduces death during childhood in children with sickle cell anemia or hemoglobin SS.

  • Image not available. Hydroxyurea decreases the incidence of painful episodes, but patients treated with hydroxyurea should be carefully monitored.

  • Image not available. Neurologic complications caused by vasoocclusion can lead to stroke. Screening with transcranial ...

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