Page 6059: due to a production error, the numbering and the alignment of the lettered items in Table 3 are incorrect. In the online version, the numbering and lettering in this table are incorrect. The corrected Table 3 is shown below.

Table 3.

Adjudication survey.

Pain

  1. Number of severe pain events and/or hospitalizations for acute VOP events per year for the last 2 years? ______

  2. Prescribed hydroxyurea therapy? Yes ___ No ___

    • Baseline labs prior to starting hydroxyurea:

      • Hemoglobin

      • Hemoglobin F

      • MCV

      • Platelets

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

      • Hemoglobin

      • Hemoglobin 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: ________

  3. Prescribed other therapies to decrease VOEs?

    • 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: _____

  4. Has the possibility of pain associated with the following been evaluated?

    • Menstrual cycle: Yes ___ No___ NA___

    • Traumatic event or stress (e.g., divorce, trauma, 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 on-going? ______

  5. History of asthma? Yes___ No___

    • Did the first episode of ACS occur before 4 years of age? Yes___ No___

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

    • Referral to an asthma specialist? Yes___ No___

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

 
Priapism (Only report episodes happening at least 4 hours) 

  1. Number of priapism events per week/month for the last 12 months managed at home? _____

  2. Number of hospitalizations for priapism events per year for last 2 years? ____

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

 
Pain

  1. Number of severe pain events and/or hospitalizations for acute VOP events per year for the last 2 years? ______

  2. Prescribed hydroxyurea therapy? Yes ___ No ___

    • Baseline labs prior to starting hydroxyurea:

      • Hemoglobin

      • Hemoglobin F

      • MCV

      • Platelets

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

      • Hemoglobin

      • Hemoglobin 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: ________

  3. Prescribed other therapies to decrease VOEs?

    • 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: _____

  4. Has the possibility of pain associated with the following been evaluated?

    • Menstrual cycle: Yes ___ No___ NA___

    • Traumatic event or stress (e.g., divorce, trauma, 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 on-going? ______

  5. History of asthma? Yes___ No___

    • Did the first episode of ACS occur before 4 years of age? Yes___ No___

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

    • Referral to an asthma specialist? Yes___ No___

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

 
Priapism (Only report episodes happening at least 4 hours) 

  1. Number of priapism events per week/month for the last 12 months managed at home? _____

  2. Number of hospitalizations for priapism events per year for last 2 years? ____

  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; MCV, mean corpuscular volume; NA, not applicable; VOE, vaso occlusive event.

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