Table 3.

Adjudication survey

Pain
  1. No. of severe pain events and/or hospitalizations for acute VOP events per year for the last 2 y? ______

 
  1. Prescribed hydroxyurea therapy? Yes ___ No ___ a. Baseline laboratory tests before starting hydroxyurea:

    • Hb

    • Hb F

    • MCV

    • Platelets

    • If yes, current hydroxyurea dose and for how long at this dose: _____

    • Hb

    • Hb F

    • MCV

    • Platelets

    • Does the treating hematologist believe the patient has been adherent to their hydroxyurea therapy?

    • What is the local definition of maximum tolerated dose of hydroxyurea (MTD HU)?

    • Did the patient meet the local definition of MTD HU? Yes ____ No____

    • Specify any side effects: ________

 
  1. Prescribed other therapies to decrease VOEs? a. L-glutamine: Yes ___ No____

    • If yes, when started: ______

    • Maximum dose used: _____

    • Any side effects at MTD? Yes ____ No_____

    • If yes, provide details: _____

    • Crizanlizumab: Yes____ No____

    • If yes, when started: ____

    • Maximum dose used: ____

    • Any side effects of MTD? Yes___ No___

    • If yes, provide details: ____

    • Chronic blood transfusions: Yes___ No___

    • If yes, when started, length of time on transfusions: _____

 
  1. Has the possibility of pain associated with the following been evaluated? a. Menstrual cycle: Yes ___ No___ NA___

    • Traumatic event or stress (eg, divorce, trauma, and domestic violence) as precipitating pain been discussed with the:

    • Patient alone? Yes___ No___

    • Parent alone? Yes___ No___

    • AVN or spinal compression fracture? Yes___ No___ Not evaluated___

    • If yes to any of the above, have they been addressed or still ongoing? ______

 
  1. History of asthma? Yes___ No___ a. Did the first episode of ACS occur before 4 y of age? Yes___ No___

    • Evidence of optimal asthma medical care (eg, controller medication that matches asthma severity) _______

    • Referral to an asthma specialist? Yes___ No___

 
  1. Has the treating hematologist attending provided a statement that the patient has been prescribed disease-modifying therapy for SCD, asthma, or both and despite evidence of adherence continues to have acute VOEs? Yes___ No___

 
Priapism (Only report episodes happening at least 4 h) 
  1. No. of priapism events per week/month for the last 12 mo managed at home? _____

  2. No. of hospitalizations for priapism events per year for last 2 y? ____

  3. Length of each priapism episode documented in the medical record?

 
Pain
  1. No. of severe pain events and/or hospitalizations for acute VOP events per year for the last 2 y? ______

 
  1. Prescribed hydroxyurea therapy? Yes ___ No ___ a. Baseline laboratory tests before starting hydroxyurea:

    • Hb

    • Hb F

    • MCV

    • Platelets

    • If yes, current hydroxyurea dose and for how long at this dose: _____

    • Hb

    • Hb F

    • MCV

    • Platelets

    • Does the treating hematologist believe the patient has been adherent to their hydroxyurea therapy?

    • What is the local definition of maximum tolerated dose of hydroxyurea (MTD HU)?

    • Did the patient meet the local definition of MTD HU? Yes ____ No____

    • Specify any side effects: ________

 
  1. Prescribed other therapies to decrease VOEs? a. L-glutamine: Yes ___ No____

    • If yes, when started: ______

    • Maximum dose used: _____

    • Any side effects at MTD? Yes ____ No_____

    • If yes, provide details: _____

    • Crizanlizumab: Yes____ No____

    • If yes, when started: ____

    • Maximum dose used: ____

    • Any side effects of MTD? Yes___ No___

    • If yes, provide details: ____

    • Chronic blood transfusions: Yes___ No___

    • If yes, when started, length of time on transfusions: _____

 
  1. Has the possibility of pain associated with the following been evaluated? a. Menstrual cycle: Yes ___ No___ NA___

    • Traumatic event or stress (eg, divorce, trauma, and domestic violence) as precipitating pain been discussed with the:

    • Patient alone? Yes___ No___

    • Parent alone? Yes___ No___

    • AVN or spinal compression fracture? Yes___ No___ Not evaluated___

    • If yes to any of the above, have they been addressed or still ongoing? ______

 
  1. History of asthma? Yes___ No___ a. Did the first episode of ACS occur before 4 y of age? Yes___ No___

    • Evidence of optimal asthma medical care (eg, controller medication that matches asthma severity) _______

    • Referral to an asthma specialist? Yes___ No___

 
  1. Has the treating hematologist attending provided a statement that the patient has been prescribed disease-modifying therapy for SCD, asthma, or both and despite evidence of adherence continues to have acute VOEs? Yes___ No___

 
Priapism (Only report episodes happening at least 4 h) 
  1. No. of priapism events per week/month for the last 12 mo managed at home? _____

  2. No. of hospitalizations for priapism events per year for last 2 y? ____

  3. Length of each priapism episode documented in the medical record?

 

The survey was completed by enrolling institutions and reviewed by the adjudication committee.

AVN, avascular necrosis; Hb F, hemoglobin F; MCV, mean corpuscular volume; NA, not applicable; VOE, vaso occlusive event.

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