SCD Cardiovascular, Pulmonary, and Renal Scope

Element Considered	Final Measures in Toolkit
N-terminal-pro-brain natriuretic peptide (NT-proBNP)	N-terminal-pro-brain natriuretic peptide (NT-proBNP)
Cardiovascular Complications
Right Heart Catheterization (RHC)	Right Heart Catheterization (RHC)
Iron overload	Serum or Plasma Ferritin
Hemolytic Component	Reticulocyte Count
	Haptoglobin Level
	Bilirubin Level
	Lactate Dehydrogenase Level
	Red Blood Cell Aspartate Aminotransferase Level
Brachial Artery Relaxivity
Short Axis Function
Pulmonary Complications
Asthma/airways disease	Lung Function - Diffusion Capacity
	Lung Function - Lung Volume
Acute chest syndrome	Chest X-ray
Sleep Disordered Breathing
Other Complications
Temperature	Body Temperature
Liver iron

SCD Neurology, Quality of Life, and Health Services Scope

Element Considered	Final Measures in Toolkit
Neurologic Complications
Silent Stroke	Stroke Impact/Outcome
Overt Stroke	Functionality after Stroke
Transcranial Doppler (TCD)	Stroke Risk in Children with Sickle Cell Disease
Diffusion Tensor Imaging (DTI)
Angiography
Non-stroke neurologic complications
Cognitive complications Dementia, Visual Memory, Working Memory, Executive Function, Attention	Adaptive Behavior/Adaptive Function
	Intelligence Scale
Transfusion
Acute and Chronic Pain
Interference
Use of Substances of Addiction Marijuana use
Transfusion
Quality of Life and Patient-Reported Outcomes
Functional measures of well-being Fatigue, pain, pain interference, depression, anxiety, mobility	Quality of Life in Sickle Cell Disease
	Self-efficacy in Sickle Cell Disease
Pain Treatment, symptoms, communication, worry (pediatric)
Activities of Daily Living Physical, social, emotional, school functioning
Health Services
Quality of Care - Hospitalization, re-hospitalization - TCD, treatments, medications	Quality of Care
Preventive care (flu shots, hydroxyurea)
Acute Care
Quality indicators for pediatric sickle cell disease

SCD Curative Therapies Scope

Element Considered	Final Measures in Toolkit
Indications for Transplant
Pretransplant Evalution	Prothrombin Time
	Left Ventricular Global Longitudinal Strain by Speckle Tracking Echocardiography
	Determination of Liver Iron Concentration by R2*
	Determination of Liver Iron Concentration by Ferriscan
	Determination of Liver Fibrosis by Magnetic Resonance Elastography (MRE) (Supplemental Information)
	Recurrent Priapism
	Psychosocial Assessment of Candidates for Transplantation
	Fibrosis Staging of Liver Biopsy (Supplemental Information)
	Determination of Liver Fibrosis by Transient Elastography (Supplemental Information)
	Myocardia Iron Estimation Using T2* MRI (Supplemental Information)
Description of transplant conditioning regimen	Sickle Cell Disease Transplant Conditioning Regimen
Transplant stem cell source	Sickle Cell Disease Stem Cell Source and Cell Manipulation
Effect of transplant on donor derived erythropoiesis
Endpoints for curative therapy (complications)	White Cell Chimerism and Graft Failure
	Graft versus Host Disease
Pretransfusion evaluation
Endpoints for Transfusion	Pre-transfusion Antibody Testing
	Transfusion Reaction Reporting
	Thyroid Stimulating Hormone (hTSH) in Serum
Endpoints for transplant (complications)	Determination of Liver Iron Concentration by R2*
	Determination of Liver Iron Concentration by Ferriscan
	Determination of Liver Fibrosis by Magnetic Resonance Elastography (MRE) (Supplemental Information)
	Fibrosis Staging of Liver Biopsy (Supplemental Information)
	Determination of Liver Fibrosis by Transient Elastography (Supplemental Information)
	Myocardia Iron Estimation Using T2* MRI (Supplemental Information)

SCD Pain Scope

Element Considered	Final Measures in Toolkit
Intensity	Pain Intensity
Location	Pain Location
Pain Quality	Pain Quality Descriptors
Anxiety: Pain-related, state, trait, disorder
Catastrophizing	Pain Catastrophizing
Coping	Pain Coping
Emotional
Psychosocial
Self-efficacy
Social
Care utilization
Frequency
Pain Burden	Pain Burden
Physical Interference/Impact	Pain Interference
	Physical Mobility
Medication use	Treatment Response: Medication Use
Patient reported treatment, satisfaction, or effectiveness	Patient Reported Impression of Change

SCD Psychosocial and Social Determinants of Health Scope

Element Considered	Final Measures in Toolkit
Transition Readiness	Sickle Cell Disease Transition Readiness Assessment
	Sickle Cell Disease Transition Readiness Assessment (SI)
Response to Stress	Sickle Cell Disease Responses to Stress - Youth
	Sickle Cell Disease Responses to Stress - Parent Report
Self-Management	Sickle Cell Disease Self-Management - Adolescent Skills Checklist
	Sickle Cell Disease Self-Management - Caregiver Skills Checklist
Stigma	Sickle Cell Disease Health-Related Stigma - Child
	Sickle Cell Disease Health-Related External Stigma - Adolescent and Adult
	Sickle Cell Disease Health-Related Internal Stigma - Adolescent and Adult
Trust in Medical Care and Research	Trust in the Medical Professional - Adult
	Trust in Medical Researchers - 12-item
	Trust in Medical Researchers - 4-item
Grief	Inventory of Complicated Grief
	Reactions to Loss Scale
Resilience	Resilience
Spiritual Coping	Religious Behaviors and Congregational Support
Attitude Toward Illness	Attitude Toward Illness - Child (SI)
Self-Efficacy	General Self-Efficacy - Child (SI)
	General Self-Efficacy - Adolescent (SI)
	General Self-Efficacy - Child Parent Report (SI)
	General Self-Efficacy - Adult (SI)
Illness Identity	Illness Identity Questionnaire (SI)
Medication Adherence	Medication Adherence (SI)
Optimism	Optimism (SI)
Self-Perception	Self-Perception Profile - Child (SI)
	Self-Perception Profile - Adolescent (SI)
	Self-Perception Profile - Adult (SI)

SCD Genitourinary Scope

Element Considered	Final Measures in Toolkit
Puberty
Menstruation	Sickle Cell Disease Pain Associated with Menstruation
	Sickle Cell Disease Pain Associated with Menstruation - Quality of Life
Contraception	Female Contraception Use
Priapism	Priapism
Fertility	Fertility and Quality of Life
	Fertility History
Infertility
Testosterone Deficiency	Testosterone Level
Sexual Function	Sexual Function
Sexual, Romantic, and Partner Satisfaction
Exogenous hormone use	Hormone Therapy
Menopause	Perimenopausal Symptoms
Voiding Dysfunction	Overactive Bladder

SCD Pregnancy Scope

Element Considered	Final Measures in Toolkit
Pre-Implantation Genetic Diagnosis
Non-Invasive Prenatal Testing	Cell-free Fetal DNA Testing for Sickle Cell Disease (Supplemental Information)
Chorionic Villus Sampling (CVS)	Invasive Prenatal Testing Guidelines (Supplemental Information)
Amniocentesis	Invasive Prenatal Testing Guidelines (Supplemental Information)
Acute Chest Syndrome	Acute Chest Syndrome During Pregnancy
	Acute Chest Syndrome - General Populations (Supplemental Information)
Transfusion	Prophylactic Transfusion during Pregnancy
	Transfusion Indications
	Transfusion Reactions
Hydroxyurea	Hydroxyurea Use during Pregnancy
	Hydroxyurea Use Adherence - Prospective Studies
Thromboprophylaxis	Thromboprophylaxis
	Thrombosis - Catheter-related
	Thrombosis - Venous
Multidisciplinary Care for SCD	Multidisciplinary Care for Sickle Cell Disease Pregnancies (Supplemental Information)