Details - CureSickleCell

Study ID	1002
Name	Treatment of Pulmonary Hypertension and Sickle Cell Disease With Sildenafil Therapy
Contact Email	biolincc@imsweb.com
Acronym	walk-PHaSST
Period	2007 – 2009
Design	Clinical Trial
Study PI	Mark T. Gladwin
Institution	Multiple institutes
URL	https://biolincc.nhlbi.nih.gov/studies/walk_phasst/
CTGovID	https://clinicaltrials.gov/ct2/show/NCT00492531
Funding Agency	NHLBI
Grant Number	HHSN268200617182C

Overview	The Walk-PHaSST study explored whether sildenafil, a medication used to lessen symptoms of pulmonary hypertension, is also effective and safe for those with sickle cell disease who develop pulmonary hypertension.
Focus Areas	1. 6-Minute Walk 2. Sickle Cell Anemia 3. Sildenafil/Viagra 4. Tricuspid Regurgitant Velocity 5. Sickle Cell Disease 6. Pulmonary Hypertension
Outcomes	1. Change in Exercise Capacity as Assessed by 6 Minute Walk. [ Time Frame: Baseline to week 16/Imputed last visit. ] 2. Change From Baseline in Pulmonary Hypertension at Week 16 as Assessed by Tricuspid Regurgitant Jet Velocity [ Time Frame: 16 weeks ] 3. Borg Dyspnea Score [ Time Frame: baseline to 16 weeks ] 4. Brain Natriuretic Peptide(BNP)Levels. [ Time Frame: 16 weeks ]

Data Location	BioLINCC, BioData Catalyst
Biospecimen Available	N/A
Data Consent	Data sharing via BioLINCC; Consent restricts use of data to research related to sickle cell disease.
Biospecimen Consent	NA
Contact Consent	NA
Genomic Data	No

Age Range	12-70
No. Participants	720
Inclusion Criteria	1. Screening- Males or females, greater than or equal to 12 years of age and less than or equal to 70 years of age. 2. Screening - Diagnosis of sickle cell disease (including, but not limited to SS, SC, SD, or S-beta zero thalassemia). 3. Screening - Provision of informed consent and, where applicable, assent. 4. Participants with mild to severe pulmonary hypertension, defined as Doppler echocardiography peak tricuspid regurgitation velocity (TRV) of = 2.7 m/s, and a six minute walk distance of 150-500 meters, were further eligible for the interventional trial.
Exclusion Criteria	1. Current pregnancy or lactation. 2. Any one of the following medical conditions:Stroke within the last six weeks.Diagnosis of pulmonary embolism within the last three months.History of retinal detachment or retinal hemorrhage in the last 6 months.Non-arteritic anterior ischemic optic neuropathy (NAION) in one or both eyes.History of sustained priapism requiring medical or surgical treatment, unless currently impotent or on transfusion program within the last two years.Any unstable (chronic or acute) condition that in the opinion of the investigator will prevent completion of the study. 3. Subjects taking nitrate-based vasodilators (including, but not limited to nicorandil [available in the UK only]), prostacyclin (inhaled, subcutaneous or intravenous) or endothelin antagonists. Subjects taking calcium channel blockers will be allowed to participate if they are on a stable dose for greater than or equal to 3 months. 4. Left ventricular ejection fraction (LVEF) less than 40 percent or clinically significant ischemic, valvular or constrictive heart disease: LVEF less than 40 percent or SF less than 22 percent. 5. Subjects in other research studies with investigational drugs (with the exception of hydroxyurea) unless the other trial has been approved by the walk-PHaSST Executive Committee for co-participation. 6. Acute or chronic impairment (other than dyspnea), limiting the ability to comply with study requirements (in particular with 6MWT), e.g., angina pectoris, intermittent claudication, symptomatic hip osteonecrosis. 7. Tonsillectomies for sleep apnea within 3 months prior to randomization. Active therapy for pulmonary hypertension, including prostacyclin analog, endothelin-1 antagonist, or PDE-5 inhibitor. 8. Protease inhibitor therapy for HIV treatment Subjects taking potent CYP3A4 inhibitor therapy (e.g., itraconazole, ritonavir, ketoconazole) Subjects who are anticoagulated and have proliferative retinopathy (unless they have had ophthalmologist recommended intervention (e.g., phototherapy) or have been cleared by the ophthalmologist to participate in the study. 9. Subjects with systolic blood pressure greater than or equal to 140 mmHg OR diastolic blood pressure greater than or equal to 90 mmHg.

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Clinical status (302)

Behavior (12)

Concept	Variables
Substance Use	ALC ALCNUM AM COC DRGOTH DRUG HEMAR HER SMK SMKPKS SMKPKS SMKYST

Blood (10)

Concept	Variables
Acute Anemic Episode
Aplastic Crisis	B_APL
Bleeding
Other Blood Problem	B_ANEM B_HEMO B_OTH B_UNSU BLOOD
Sickle Cell Disease	SCD_DIAG SCD_TYPE SCGENO SCGENSP

Cancer (2)

Concept	Variables
Cancer	O_CAN O_CANSP

Cardiovascular (14)

Concept	Variables
Aneurysm	N_ANE
Cardiac Failure	H_FAIL
Cardiovascular Problem
Heart Problem	EV_RHF H_ARH H_ATK H_CMY H_ENL H_HVP H_OTH H_OTSP H_UNSU HEART S2_AHS S2_EVP

Digestive (17)

Concept	Variables
Gastrointestinal Problem	L_CHCY L_CHLS L_GALL
Hepatitis
Liver Problem	L_CIRR L_FAIL L_FIB L_HEPA L_HEPB L_HEPC L_HEPU L_IC L_OTH L_OTSP L_SEQ L_TH L_UNSU LIVER

Endocrine (1)

Concept	Variables
Diabetes Mellitus	O_DIAB
Endocrine Problem

Family MedHx (6)

Concept	Variables
Family MedHx	FALIVE FCAUSE MALIVE MCAUSE MDIS MDIS

General Health (0)

Genitourinary (16)

Concept	Variables
Genitourinary Problem	K_ED K_HEM K_OTSP K_PROT K_PYL K_UNSU KIDNEY
Kidney Problem	K_ARF K_CR K_CRF
Priapism	K_PRI PRIA PRIAEX PRIANUM
Renal Dialysis History	RRT RRT_SP

Immune (9)

Concept	Variables
Fever
HIV Infection	PIT RESULT
Immune Problem	O_SLE
Infection	I_BACT I_OTH I_SEPO I_SEPP I_UNSU INFECT
Vaccination

Lymphatic (8)

Concept	Variables
Sarcoidosis
Spleen Problem	L_PAN S_HYPER S_INF S_OTH S_SEQ S_SPMG S_UNSU SPLEEN

Medical Event (60)

Concept	Variables
Adverse event	AETEXT EV_NO NARRATE NARRPR NARRSP OUTCOME RELSCD RELSP S1_ABDO S1_CHST S1_FNT S1_OTH S1_SP S1_SPTM S2_ENLV S2_OTH S2_SP S2_SWFA SERIOUS SEVRTY SP_SP STARTDA STARTMO STARTYR
Death	AUTOP CAUSE DEATHDA DEATHMO DEATHYR
Disability Status
Hospitalization
Medical Event
Serious Adverse Event	COND FACT1 FACT2 FACT3 FACT3SP FACT4 FACT5 FACT5SP FACT6 FACT6SP HISTNA HISTPR HISTSP HONGO HSTPDA HSTPMO HSTPYR HSTRTDA HSTRTMO HSTRTYR LDATEDA LDATEMO LDATEYR RANGE RESULT SAE1 SAE2 SAE3 SAE4J SAEE SAES

Medical History (5)

Concept	Variables
Medical History	O_AMOF O_OTH O_OTSP O_UNSU OTHDIS

Mental Health (13)

Concept	Variables
Anxiety
Depression	N_DEP
Emotional Health	SFQ5A SFQ5B SFQ5C SFQ9A SFQ9B SFQ9C SFQ9D SFQ9E SFQ9F SFQ9G SFQ9H SFQ9I
Mental status
Psychiatric Disorder

Musculoskeletal (12)

Concept	Variables
Musculoskeletal Problem	M_HIP M_OSP M_OTH M_OTSP M_SHL M_ULC M_UNSU MBJ
Osteomyelitis	M_OMY
Osteonecrosis	M_AVH M_AVH
Rheumatic Diseases	O_RA

Neurodevelopmental (6)

Concept	Variables
Behavior
Cognition	N_MEM NEUR1 NEURCM
Developmental
Motor	NEUR2 NEUR3 NEUR4
Sensation

Neurologic (12)

Concept	Variables
Meningitis	I_MEN
Neurologic Event	N_NPTHY N_OTH N_UNSU NEURO
Seizure	N_SZR
Stroke	N_SCI N_STR N_STRH NEUR5 STROKE
Transient Ischemic Attack	N_TIA

Nutritional and Metabolic (2)

Concept	Variables
Nutritional and Metabolic	O_IRON O_VITD

Ocular (3)

Concept	Variables
Ocular Problem	VISDES VISION
Sickle Retinopathy	O_RET

Oral (0)

Pain (20)

Concept	Variables
Abdominal Pain
Headache	HEAD HEADEX HEADNUM N_HDC N_HDM
Management	ANLG ANLGAE CH_TR1 CH_TR2 CH_TR3 CH_TR4 CH_TR5 CH_TR6 CH_TRO CH_TRS EVT2 EVT6 NUMSCP PAIN PAINEX

Reproductive (11)

Concept	Variables
Birth Outcome
Gestational Age
Menstruation	AGEMEN1 AGEMEN2 CYCLE LMPMO LMPYR NOTAPP ONSET REGULAR STATUS
Pregnancy	BIRTHS PREG
Reproductive Behavior

Respiratory (28)

Concept	Variables
Acute Chest Syndrome	ADJ_NAR EV_ACS EVT1
Asthma	P_ASTH
Lung Disease	CLASS EV_PH H_HYP LUNG P_CLD P_PE P_PH PHDA PHMO PHNA PHYR RELPH
Other Respiratory Problem	BRGPOST P_COPD P_CRPD P_OTH P_PNEU P_UNSU S1_CGH S1_SHBR S2_ALS S2_CGH S2_ICR S2_TCHY

Skin (0)

Sleep (1)

Concept	Variables
Sleep Problem	P_OSA

Surgery History (34)

Concept	Variables
Cardiovascular
Digestive	CHL CHL_C CHL_YR
Genitourinary	PEN PEN_C PEN_YR
Lymphatic	SPL SPL_C SPL_YR
Musculoskeletal	HCP HCP_C HCP_YR HR HR_C HR_YR
Ocular	LPE LPE_C LPE_YR VIT VIT_C VIT_YR
Respiratory	TON TON_C TON_YR
Surgery History	IPL IPL_C IPL_YR PROC PROC_C PROC_YR RPL RPL_C RPL_YR SUR_OTH

COVID-19 (0)

Diagnosis (0)

Impact (0)

Treatment (0)

Vaccination (0)

Demographics (29)

Concept	Variables
Address
Age
Ethnic Group	ETHNIC
Gender	GENDER
Language
National Origin	COUNTRY CYUNK SWHERE
Nuclear Family	CAUSE FULLSIB FULLUNK HALFSIB HALFUNK LIVING RELATE SCD SCT
Race	OTH_SP RACE1 RACE2 RACE2A RACE2B RACE2C RACE2D RACE2E RACE2F RACE3 RACE4 RACE5 RACE6 RACE7 RACE8

Social Determinants of Health (SDoH) (0)

Socioeconomic Status (0)

Environmental Exposure (0)

Exposure (0)

Health Care (15)

Acute Care Utilization (13)

Concept	Variables
Facility Type	LOC
Provider Type
Quality of Care
Visit	EVENT EVT10 EVT10SP EVT11 EVT3 EVT4 EVT5 EVT7 EVT8 EVT9 EVTOT EVTOTSP

Health Services (0)

Healthcare utilization (2)

Concept	Variables
Country of Care	CAREYRS OUTCARE
Facility Type
Quality of Care

Hospitalization (0)

SCD Management (0)

Imaging (71)

Angiogram (0)

Catheterization (0)

CT Scan (0)

Echocardiogram (20)

Concept	Variables
Echocardiogram	DGTS-TYPE4 S3_ECH S3_ECHR AR DIABP DRUGDA DRUGHH DRUGMM DRUGMO DRUGYR ERAP LVEF LVFUN MR NODRUG OTHSIG SYSBP TR TRJV TRJVND

Electrocardiogram (ECG) (3)

Concept	Variables
Electrocardiogram (ECG)	DGTS-TYPE6 S3_ECG S3_ECGR

Electrodiagnosis (0)

General Imaging (1)

Concept	Variables
General Imaging	DIAGTST

MRI/MRA (2)

Concept	Variables
Brain	DGTS-TYPE1 DGTS-TYPE2
Heart
Hip
Liver

Nuclear Medicine Imaging (0)

PET scan (0)

Transcranial Doppler (TCD) (2)

Concept	Variables
Brain	N_TCD DGTS-TYPE3

Ultrasound (0)

X-Ray (43)

Concept	Variables
Abdomen
Biliary Tract
Chest	S3_XCE S3_XCON S3_XDA S3_XFIND S3_XINF S3_XMO S3_XPE S3_XRAY S3_XYR ABN1 ABN10 ABN1LIL ABN1LSL ABN1RIL ABN1RML ABN1RSL ABN2 ABN3 ABN4 ABN5 ABN5LIL ABN5LSL ABN5RIL ABN5RML ABN5RSL ABN6 ABN6LIL ABN6LSL ABN6RIL ABN6RML ABN6RSL ABN7 ABN7LIL ABN7LSL ABN7RIL ABN7RML ABN7RSL ABN8 ABN9 CODE NOCODE OTH_SP RESULT
Extremities
Gallbladder
Liver
Skull

Parent/Caretaker (33)

Demographics (33)

Concept	Variables
Demographics	FETHNIC FOTH_SP FRACE1 FRACE2 FRACE2A FRACE2C FRACE2D FRACE2E FRACE2F FRACE3 FRACE4 FRACE5 FRACE6 FRACE7 FRACE8 FRACEZB FWHERE METHNIC MOTH_SP MRACE1 MRACE2 MRACE2A MRACE2B MRACE2D MRACE2E MRACE2F MRACE3 MRACE4 MRACE5 MRACE6 MRACE7 MRACE8 MWHERE
Socioeconomic Status

Psychosocial (0)

Quality of Life (0)

Quality of Life (228)

Functional Status (114)

Concept	Variables
Behavior
Cognitive
Mental Health	EMOFC1 EMOFC1 EMOFC2 EMOFC2 EMOFC3 EMOFC3 EMOFC4 EMOFC5 EMOFC5 EMOFP1 EMOFP1 EMOFP2 EMOFP2 EMOFP3 EMOFP3 EMOFP5 EMOFP5 SFQ4B
Physical	PHYFC1 PHYFC1 PHYFC2 PHYFC2 PHYFC3 PHYFC3 PHYFC4 PHYFC4 PHYFC5 PHYFC5 PHYFC6 PHYFC6 PHYFC7 PHYFC7 PHYFC8 PHYFC8 PHYFP1 PHYFP1 PHYFP2 PHYFP2 PHYFP3 PHYFP3 PHYFP4 PHYFP4 PHYFP5 PHYFP5 PHYFP6 PHYFP6 PHYFP7 PHYFP7 PHYFP8 PHYFP8 SFQ1 SFQ11A SFQ11B SFQ11C SFQ11D SFQ2 SFQ3A SFQ3B SFQ3C SFQ3D SFQ3E SFQ3F SFQ3G SFQ3H SFQ3I SFQ3J SFQ4A SFQ4C SFQ4D
SCD Management
Sleep/Fatigue	EMOFC4 EMOFP4 EMOFP4
Social	SCHFC1 SCHFC1 SCHFC2 SCHFC2 SCHFC3 SCHFC3 SCHFC4 SCHFC4 SCHFC5 SCHFC5 SCHFP1 SCHFP1 SCHFP2 SCHFP2 SCHFP3 SCHFP3 SCHFP4 SCHFP4 SCHFP5 SCHFP5 SFQ10 SFQ6 SOCFC1 SOCFC1 SOCFC2 SOCFC2 SOCFC3 SOCFC3 SOCFC4 SOCFC4 SOCFC5 SOCFC5 SOCFP1 SOCFP1 SOCFP2 SOCFP2 SOCFP3 SOCFP3 SOCFP4 SOCFP4 SOCFP5 SOCFP5

General Health (0)

Pain (114)

Concept	Variables
General Pain	AC_LOC1 AC_LOC2 AC_LOC3 AC_LOC4 AC_LOC5 AC_LOC6 AC_LOC7 AC_LOCO AC_LOCS AC_QUAL AC_RATE AC_TR1 AC_TR2 AC_TR3 AC_TR4 AC_TR5 AC_TR6 AC_TR6 AC_TRS CH_LOC1 CH_LOC2 CH_LOC3 CH_LOC4 CH_LOC5 CH_LOC6 CH_LOC7 CH_LOCO CH_LOCS CH_QUAL CH_RATE CHRON FOOTL FOOTM FOOTR IQ2LLEGM Q1 Q2ABDOC Q2ABDOL Q2ABDOM Q2ABDOR Q2BUTTL Q2BUTTM Q2BUTTR Q2CHSTC Q2CHSTL Q2CHSTM Q2CHSTR Q2ELBL Q2ELBM Q2ELBR Q2GEN Q2GENM Q2HANDL Q2HANDM Q2HANDR Q2HEADC Q2HEADL Q2HEADM Q2HEADR Q2KNEEL Q2KNEEM Q2KNEER Q2LARML Q2LARMM Q2LARMR Q2LBCKC Q2LBCKL Q2LBCKM Q2LBCKR Q2LLEGL Q2LLEGR Q2NECK Q2NECKM Q2SHLDL Q2SHLDM Q2SHLDR Q2UARML Q2UARMM Q2UARMR Q2UBCKC Q2UBCKL Q2UBCKM Q2UBCKR Q2ULEGL Q2ULEGM Q2ULEGR Q3 Q4 Q5 Q6 Q7 Q8 Q9A Q9B Q9C Q9D Q9E Q9F Q9G SFQ7 SFQ8
Pain Attack	PNC_MEX PNC_MMI PNC_MMO PNC_MSE PNC_WEX PNC_WMI PNC_WMO PNC_WSE PNC_YEX PNC_YMI PNC_YMO PNC_YSE
Skeletal and Joint Pain	M_DAC

Tests (360)

Biopsy (0)

Blood (117)

Concept	Variables
Blood Differential Test
Blood Gases	AOPOST AOPRE FRPOST FRPRE OSPOST OSPRE S3_ABG S3_BNP S3_CA02 S3_PA02 S3_PDEC S3_SA02 S3_SP02
Blood Smear
Blood Typing
Coagulation Test	APPT APPTSG INR INRSG
Complete Blood Count	B_LPT B_LWBC ANC ANCSG ANCUNIT ARC ARCSG ARCUNIT HCT HCTSG HCTUNIT HGB HGBSG HGBUNIT MCHC MCHCSG MCHUNIT MCV MCVSG MCVUNIT NEUT NEUTSG NEUTUNIT PLAT PLATSG PLATUNIT RBC RBCSG RBCUNIT RET RETSG RETUNIT WBC WBCSG WBCUNIT
Comprehensive Metabolic Panel (CMP)	ALB ALBSG ALBUNIT ALK ALKSG ALKUNIT ALT ALTSG ALTUNIT AST ASTSG ASTUNIT BUN BUNSG BUNUNIT CAL CALSG CALUNIT CHL CHLSG CHLUNIT CO2 CO2SG CO2UNIT CRE CRESG CRUNIT MAG MAGSG MAGUNIT PHOS PHOSSG POT POTSG POTUNIT PUNIT S3_AST SOD SODSG SODUNIT TBIL TBILSG TBUNIT TPROT TPROTSG TPUNIT
Enzyme	LDH LDHSG LDHUNIT S3_CE S3_CPH S3_CTP S3_LCD S3_MYG
General	BIOPDA BIOPMO BIOPYR
Hemoglobin Typing	HEMA HEMA2 HEMC HEMF HEMOSP HEMOTH HEMS HIST
Iron

Cardiovascular (104)

Concept	Variables
Cardiovascular Test	ASMT1HH ASMT1MM ASMT1TM ASMT2HH ASMT2MM ASMT2TM ASMT3HH ASMT3MM ASMT3TM ASMT4HH ASMT4MM ASMT4TM ASMT5HH ASMT5MM ASMT5TM CODE DEVICE DIST DPAP1 DPAP2 DPAP3 DPAP4 DPAP5 DRGHH DRGMM DRGTM DSBP1 DSBP2 DSBP3 DSBP4 DSBP5 FRWALK HRATE1 HRATE2 HRATE3 HRATE4 HRATE5 INOHH INOMM INOTM LDSDDA LDSDHH LDSDMM LDSDMO LDSDYR LVEDP1 LVEDP2 LVEDP3 LVEDP4 LVEDP5 METHOD MPAP1 MPAP2 MPAP3 MPAP4 MPAP5 MPCWP1 MPCWP2 MPCWP3 MPCWP4 MPCWP5 MRAP1 MRAP2 MRAP3 MRAP4 MRAP5 MSP1 MSP2 MSP3 MSP4 MSP5 NOTSENT OUTPUT1 OUTPUT2 OUTPUT3 OUTPUT4 OUTPUT5 SAS1 SAS2 SAS3 SAS4 SAS5 SIDE SPAP1 SPAP2 SPAP3 SPAP4 SPAP5 SSBP1 SSBP2 SSBP3 SSBP4 SSBP5 STARTHH STARTMM SUBSTOP SVOX1 SVOX2 SVOX3 SVOX4 SVOX5 TRJET USE02 VISIT

Genitourinary (28)

Concept	Variables
Genitourinary Test
Urine Test	ACDA ACMO ACYR ALB BLD BLDCODE BLDVAL COLLDA COLLMO COLLYR CREAT GLU GLUCODE GLUVAL MENSES MICROYN NEWAE OMICDES OMICYN OVERALL PH PRO PROCODE PROVAL RATIO RBC SPGRAV WBC

Immune (0)

Microbiology Culture (0)

Musculoskeletal (0)

Neurologic (0)

Ocular (0)

Other (0)

Physical Exam (66)

Concept	Variables
Abdomen	GASTCM GASTNR GASTOT HEP SPL
Anthropometrics	APPWT HEIGHT HEIGHT HTUNITS S2_WTG S3_WT WEIGHT WEIGHT WTUNITS
Cardiovascular	AIRO2 CARCM CARDES CARJD CARM CARM CAROTH CARP2 CARR CARRD CARS12 CARS3 CARS4 DIABP HRATE SYSBP O2 O2FLOW
Extremities	EXTCM EXTED EXTLUL EXTLULD EXTNC EXTNH EXTNN EXTPL EXTPLD EXTSL EXTSLD SKINHP
General	APP APPCM HEENTCM HEENTHP HEENTSI HEENTTH
Liver
Reproductive
Respiratory	PULBC PULBW PULCL PULCM RESP
Spleen
Vital signs	BSA DIAPOST DIAPRE HRPOST HRPRE S2_RHR S3_RESP S3_TEMP SYSPOST SYSPRE TEMP

Reproductive (6)

Concept	Variables
Pregnancy	OTH_SP TYPE REASON RESULT HCG HCGSYM

Respiratory (39)

Concept	Variables
Pulmonary Function Test	DGTS-TYPE5 A_25 A_25P A_DIV A_DIVP B_25 B_25P B_DIV B_DIVP B_FEV1 B_FEV1P B_FVC B_FVCP D_VA D_VAP DC_VAC DC_VACP DLCO DLCOC DLCOCP DLCOP ERV ERVP FEV1 FEV1P FRC FRCP FVC FVCP IC ICP RDIV RDIVP RV RVP TLC TLCP VA VAP
Respiratory Test

Therapeutics (269)

Medication (233)

Concept	Variables
Antibiotics	PEN
Asthma
Blood Medication History	ACG ERY ERYC FOL IRON IRONC IRONSP IRONTYP
Cardiovascular	ACE BETA CCB DIUR ERA PCY PDE PHT_OTH PHT_SP VASO
Compliance
Current Medication
Depression Medication History	ADP ADPSP
Hydroxyurea	HYD HYDC
Medication	STATUS ALT ALT_SP DOSE FREQ FREQ_SP IND INHAL MEDNAME MEDS NAME REAC1 REAC2 REAC3 REAC4 REAC5 REACOTH REACSP RELSDRG ROUTE RTE_SP S3_MD S3_MDIM S3_MDSP S3_MDWL S3_TH SAE STARTDA STARTMO STARTYR STOPDA STOPMO STOPYR TDOSE TONGO TREAT TREATNA TREATPR TREATSP TSTPDA TSTPMO TSTPYR TSTRTDA TSTRTMO TSTRTYR TUNITS UNIT_SP UNITS
Neurologic Medication History	ACV ACVSP
Pain	COD DEM DIL METH MOR NSAID NSAIDC NSAIDSP OPM OPMSP OXCD OXCT PERC TYL3 VIC
SCD Medication	BIOP BIOPRS OASA OASASP
Sildenafil	BOTNUM D1_AMHH D1_AMMM D1_AMNT D1_AMPT D1_DA D1_MDHH D1_MDMM D1_MDNT D1_MDPT D1_MO D1_PMHH D1_PMMM D1_PMNT D1_PMPT D1_YR D2_AMHH D2_AMMM D2_AMNT D2_AMPT D2_DA D2_MDHH D2_MDMM D2_MDNT D2_MDPT D2_MO D2_PMHH D2_PMMM D2_PMNT D2_PMPT D2_YR D3_AMHH D3_AMMM D3_AMNT D3_AMPT D3_DA D3_MDHH D3_MDMM D3_MDNT D3_MDPT D3_MO D3_PMHH D3_PMMM D3_PMNT D3_PMPT D3_YR D4_AMHH D4_AMMM D4_AMNT D4_AMPT D4_DA D4_MDHH D4_MDMM D4_MDNT D4_MDPT D4_MO D4_PMHH D4_PMMM D4_PMNT D4_PMPT D4_YR D5_AMHH D5_AMMM D5_AMNT D5_AMPT D5_DA D5_MDHH D5_MDMM D5_MDNT D5_MDPT D5_MO D5_PMHH D5_PMMM D5_PMNT D5_PMPT D5_YR D6_AMHH D6_AMMM D6_AMNT D6_AMPT D6_DA D6_MDHH D6_MDMM D6_MDNT D6_MDPT D6_MO D6_PMHH D6_PMMM D6_PMNT D6_PMPT D6_YR D7_AMHH D7_AMMM D7_AMNT D7_AMPT D7_DA D7_MDHH D7_MDMM D7_MDNT D7_MDPT D7_MO D7_PMHH D7_PMMM D7_PMNT D7_PMPT D7_YR DOSE OTH_SP REASON STARTDA STARTMO STARTYR STOPDA STOPMO STOPYR ACTION DBFU DOSE DRUGDA DRUGHH DRUGMM DRUGMO DRUGYR DSPDA DSPMO DSPYR DSTDA DSTMO DSTYR MDOSE MONGO MSTPDA MSTPMO MSTPYR MSTRTDA MSTRTMO MSTRTYR MUNITS ONGO RELATE SDYMED
Supplements

Surgery (0)

Transfusion (29)

Concept	Variables
Blood Transfusion	REAC1-5 REACOTH REACSP REAS_OT REAS_SP REAS1 REAS10 REAS11 REAS2 REAS3 REAS4 REAS5 REAS6 REAS7 REAS8 REAS9 S3_PRHG S3_TRAN S3_TYPE S3_UNITS TRANNA TRANTOT TYPE UNITS UNITS
Chronic Transfusion	THRPY THRPYDA THRPYMO THRPYYR
Transfusion Complications

Transplantation (0)

Treatment (7)

Concept	Variables
Discontinuation
Oxygen	OXY OXYC S3_O2 S3_O2HI S3_O2MX
Treatment	S3_OTH S3_OTHS

Study Publications

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Mental Health (0)

Neurodevelopment (0)

Neuropsychological (0)

Pain (68)

Concept	Variables
ASCQ-Me Pain Episode
ASCQ-Me Pain Impact
ASCQ-Me Stiffness Impact
Brief Pain Inventory (BPI)	FOOTL FOOTM FOOTR IQ2LLEGM Q1 Q2ABDOC Q2ABDOL Q2ABDOM Q2ABDOR Q2BUTTL Q2BUTTM Q2BUTTR Q2CHSTC Q2CHSTL Q2CHSTM Q2CHSTR Q2ELBL Q2ELBM Q2ELBR Q2GEN Q2GENM Q2HANDL Q2HANDM Q2HANDR Q2HEADC Q2HEADL Q2HEADM Q2HEADR Q2KNEEL Q2KNEEM Q2KNEER Q2LARML Q2LARMM Q2LARMR Q2LBCKC Q2LBCKL Q2LBCKM Q2LBCKR Q2LLEGL Q2LLEGR Q2NECK Q2NECKM Q2SHLDL Q2SHLDM Q2SHLDR Q2UARML Q2UARMM Q2UARMR Q2UBCKC Q2UBCKL Q2UBCKM Q2UBCKR Q2ULEGL Q2ULEGM Q2ULEGR Q3 Q4 Q5 Q6 Q7 Q8 Q9A Q9B Q9C Q9D Q9E Q9F Q9G
Chronic Pain
PROMIS Neuropathic Pain Quality
PROMIS Nociceptive Pain Quality
PROMIS Pain Behavior
PROMIS Pain Interference

Physical Function (26)

Concept	Variables
6-Minute Walk Test	AOPOST AOPRE BRGPOST CLASS DEVICE DIAPOST DIAPRE DIST DRUGDA DRUGHH DRUGMM DRUGMO DRUGYR FRPOST FRPRE FRWALK HRPOST HRPRE OSPOST OSPRE STARTHH STARTMM SUBSTOP SYSPOST SYSPRE USE02
PROMIS Physical Functioning
Tanner Stage

Psychosocial (0)

Quality of Care (0)

Quality of Life (127)

Concept	Variables
ASCQ-Me SCD Medical History Checklist
ASCQ-Me Sexual Health
Child Health Questionnaire (CHQ)
Ladder of Life
Pediatric Quality of Life Inventory (PedsQL) (ages 1-12ms)
Pediatric Quality of Life Inventory (PedsQL) (ages 13-24ms)
Pediatric Quality of Life Inventory (PedsQL) (ages 2-4ys)
Pediatric Quality of Life Inventory (PedsQL) (ages 5-7)
Pediatric Quality of Life Inventory Child Report (ages 13-18)	EMOFC1 EMOFC2 EMOFC3 EMOFC4 EMOFC5 PHYFC1 PHYFC2 PHYFC3 PHYFC4 PHYFC5 PHYFC6 PHYFC7 PHYFC8 SCHFC1 SCHFC2 SCHFC3 SCHFC4 SCHFC5 SOCFC1 SOCFC2 SOCFC3 SOCFC4
Pediatric Quality of Life Inventory Child Report (ages 8-12)	EMOFC1 EMOFC2 EMOFC3 EMOFC4 EMOFC5 PHYFC1 PHYFC2 PHYFC3 PHYFC4 PHYFC5 PHYFC6 PHYFC7 PHYFC8 SCHFC1 SCHFC2 SCHFC3 SCHFC4 SCHFC5 SOCFC1 SOCFC2 SOCFC3 SOCFC4 SOCFC5
Pediatric Quality of Life Inventory Parent Report for Child (ages 13-18)	EMOFP1 EMOFP2 EMOFP3 EMOFP4 EMOFP5 PHYFP1 PHYFP2 PHYFP3 PHYFP4 PHYFP5 PHYFP6 PHYFP7 PHYFP8 SCHFP1 SCHFP2 SCHFP3 SCHFP4 SCHFP5 SOCFP1 SOCFP2 SOCFP3 SOCFP4 SOCFP5
Pediatric Quality of Life Inventory Parent Report for Child (ages 8-12)	EMOFP1 EMOFP2 EMOFP3 EMOFP4 EMOFP5 PHYFP1 PHYFP2 PHYFP3 PHYFP4 PHYFP5 PHYFP6 PHYFP7 PHYFP8 SCHFP1 SCHFP2 SCHFP3 SCHFP4 SCHFP5 SOCFP1 SOCFP2 SOCFP3 SOCFP4 SOCFP5
PROMIS Global Health
PROMIS Global Physical Health
PROMIS Self-Efficacy for Managing Medications and Treatment
SF-36 Health Survey	SFQ1 SFQ10 SFQ11A SFQ11B SFQ11C SFQ11D SFQ2 SFQ3A SFQ3B SFQ3C SFQ3D SFQ3E SFQ3F SFQ3G SFQ3H SFQ3I SFQ3J SFQ4A SFQ4B SFQ4C SFQ4D SFQ5A SFQ5B SFQ5C SFQ6 SFQ7 SFQ8 SFQ9A SFQ9B SFQ9C SFQ9D SFQ9E SFQ9F SFQ9G SFQ9H SFQ9I

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Study Documentation Select All	1. Data Managment Plan 2. Manual_of_Operations 3. Protocol 4. Study_Summary
Forms Select All	1. Brief Pain Inventory 2. Demographics 3. SF-36 Health Survey 4. Symptoms Documentation 5. 6-Minute Walk Test 6. Adverse Events 7. Medical History, Part 1 8. Medical History, Part 2 9. Medical History, Part 3 10. Visit Record 11. Walk-PHaSST_Physical_Examination 12. Chemistry 13. Hematology 14. Hemoglobin Electrophoresis 15. Urinalysis 16. Pregnancy Test 17. Echocardiogram (Local) 18. Right Heart Catheterization 19. Pulmonary Function Studies 20. Chest X-Ray 21. Pediatric Quality of Life Inventory Parent Report for Teens (13 Yrs Old) 22. Pediatric Quality of Life Inventory Teen Report (13 Yrs Old) 23. Pediatric Quality of Life Inventory Parent Report for Child (12 Yrs Old) 24. Pediatric Quality of Life Inventory Child Report (12 Yrs Old) 25. Study Drug Diary 26. Study Completion/Early Termination 27. Study Drug Dosing 28. Study Drug Accountability Log 29. Concomitant Medications 30. Study Termination 31. Biomarker/Genotype Sample Collection 32. HIV Test

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