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Testing and interventions prior to curative therapy for sickle cell disease

Testing and interventions prior to curative therapy for sickle cell disease
Condition Evaluation
Psychosocial preparedness
  • Evaluations by psychologist, social worker, and/or specialized nurse
Financial preparedness
  • Social worker evaluation and financial counseling
Fertility preservation
  • Fertility clinic referral
Organ function
  • Echocardiography (TRJV, EF, diastolic function)
  • PFTs
  • Kidney function (eGFR, proteinuria)
  • Brain MRI and cognitive assessment; MRA for those with pre-existing neurologic disease
  • Gonadal function (females: AMH, LH, FSH, estradiol; males: testosterone)
Infectious diseases screening
  • TB, endemic microorganisms depending on location
Iron balance
  • Iron studies and liver and heart MRI for individuals who have received more than 20 transfusions or have ferritin above the reference range
Alloimmunization
  • Early consultation with transfusion specialist, evaluation and storage of rare type RBC units if needed
Refer to UpToDate for details of this testing and implications for pursuing curative therapy.
AMH: Anti-Müllerian hormone; EF: ejection fraction; eGFR: estimated glomerular filtration rate; FSH: follicle stimulating hormone; LH: luteinizing hormone; MRA: magnetic resonance angiography; MRI: magnetic resonance imaging; PFTs: pulmonary function tests; RBC: red blood cell; SCD: sickle cell disease; TB: tuberculosis; TRJV: tricuspid regurgitant jet velocity.
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