Protocol - Frequency of Sickle Cell Pain Episodes Per Year

Personnel and Training Required

None

Equipment Needs

None

Requirements

Requirement Category	Required
Major equipment	No
Specialized training	No
Specialized requirements for biospecimen collection	No
Average time of greater than 15 minutes in an unaffected individual	No

Mode of Administration

Self-administered questionnaire

Lifestage

Adult

Participants

Adults, ages 18 and older

Selection Rationale

These self-administered questions from the Cooperative Study of Sickle Cell Disease (CSSCD) are validated, well established and easy to implement and interpret.

Language

English

Standards

Standard	Name	ID	Source
Human Phenotype Ontology	Anemia	OMIM:603903	HPO
Human Phenotype Ontology	Sickle Cell	HP:0100749	HPO
caDSR Form	PhenX PX830101 - Frequency Of Sickle Cell Pain Episodes Per Year	6254586	caDSR Form

Derived Variables

None

Process and Review

The PhenX Sickle Cell Disease Research and Scientific Panel (SRSP) reviewed this protocol in May 2022. Guidance from the SRSP includes:

• Updated protocol

Protocol Name from Source

Cooperative Study of Sickle Cell Disease (CSSCD), Form 25 - Painful Episodes

Source

U.S. Department of Health and Human Services. National Institutes of Health. National Heart, Lung, and Blood Institute (NHLBI). Cooperative Study of Sickle Cell Disease (CSSCD). Form 25 - Painful Episodes. Questions 7a, 7b, and 7c.

General References

Platt, O.S., Brambilla, D.J., Rosse, W.F., Milner, P.F., Castro, O., Steinberg, M.H., & Klug, P.P. (1994). Mortality in sickle cell disease. Life expectancy and risk factors for early death. New England Journal of Medicine 330(23), 1639-1644.

Platt, O.S., Thorington, B.D., Brambilla, D.J., Milner, P.F., Rosse, W.F., Vichinsky, E., & Kinney, T.R. (1991). Pain in sickle cell disease. Rates and risk factors. New England Journal of Medicine 325(1), 11-16.

Wallen, G.R., Minniti, C.P., Krumlauf, M., Eckes, E., Allen, D., Oguhebe, A., Seamon, C., Darbari, D.S., Hildesheim, M., Yang, L., Schulden, J.D., Kato, G.J., & Taylor JG 6^th. (2014). Sleep disturbance, depression and pain in adults with sickle cell disease. BMC Psychiatry 14, 207.

Protocol ID

830101

Variables

Export Variables

Variable Name	Variable ID	Variable Description	dbGaP Mapping
PX830101_SCDPainSixMonths
	PX830101010100	In the last 6 months, how many times did you more visit an emergency room because of a sickle cell painful event? show less	N/A
PX830101_SCDPainSixMonths_City_Event1
	PX830101010400	In the past six months, what city was the more hospital (Event1) in? show less	N/A
PX830101_SCDPainSixMonths_City_Event10
	PX830101014000	In the past six months, what city was the more hospital (Event10) in? show less	N/A
PX830101_SCDPainSixMonths_City_Event2
	PX830101010800	In the past six months, what city was the more hospital (Event2) in? show less	N/A
PX830101_SCDPainSixMonths_City_Event3
	PX830101011200	In the past six months, what city was the more hospital (Event3) in? show less	N/A
PX830101_SCDPainSixMonths_City_Event4
	PX830101011600	In the past six months, what city was the more hospital (Event4) in? show less	N/A
PX830101_SCDPainSixMonths_City_Event5
	PX830101012000	In the past six months, what city was the more hospital (Event5) in? show less	N/A
PX830101_SCDPainSixMonths_City_Event6
	PX830101012400	In the past six months, what city was the more hospital (Event6) in? show less	N/A
PX830101_SCDPainSixMonths_City_Event7
	PX830101012800	In the past six months, what city was the more hospital (Event7) in? show less	N/A
PX830101_SCDPainSixMonths_City_Event8
	PX830101013200	In the past six months, what city was the more hospital (Event8) in? show less	N/A
PX830101_SCDPainSixMonths_City_Event9
	PX830101013600	In the past six months, what city was the more hospital (Event9) in? show less	N/A
PX830101_SCDPainSixMonths_Date_Event1
	PX830101010200	In the past six months, what was the date of more Event1? show less	N/A
PX830101_SCDPainSixMonths_Date_Event10
	PX830101013800	In the past six months, what was the date of more Event10? show less	N/A
PX830101_SCDPainSixMonths_Date_Event2
	PX830101010600	In the past six months, what was the date of more Event2? show less	N/A
PX830101_SCDPainSixMonths_Date_Event3
	PX830101011000	In the past six months, what was the date of more Event3? show less	N/A
PX830101_SCDPainSixMonths_Date_Event4
	PX830101011400	In the past six months, what was the date of more Event4? show less	N/A
PX830101_SCDPainSixMonths_Date_Event5
	PX830101011800	In the past six months, what was the date of more Event5? show less	N/A
PX830101_SCDPainSixMonths_Date_Event6
	PX830101012200	In the past six months, what was the date of more Event6? show less	N/A
PX830101_SCDPainSixMonths_Date_Event7
	PX830101012600	In the past six months, what was the date of more Event7? show less	N/A
PX830101_SCDPainSixMonths_Date_Event8
	PX830101013000	In the past six months, what was the date of more Event8? show less	N/A
PX830101_SCDPainSixMonths_Date_Event9
	PX830101013400	In the past six months, what was the date of more Event9? show less	N/A
PX830101_SCDPainSixMonths_Days
	PX830101030300	How many days were missed?	N/A
PX830101_SCDPainSixMonths_Hospital_Event1
	PX830101010300	In the past six months, which hospital did more you go for Event1? show less	N/A
PX830101_SCDPainSixMonths_Hospital_Event10
	PX830101013900	In the past six months, which hospital did more you go for Event10? show less	N/A
PX830101_SCDPainSixMonths_Hospital_Event2
	PX830101010700	In the past six months, which hospital did more you go for Event2? show less	N/A
PX830101_SCDPainSixMonths_Hospital_Event3
	PX830101011100	In the past six months, which hospital did more you go for Event3? show less	N/A
PX830101_SCDPainSixMonths_Hospital_Event4
	PX830101011500	In the past six months, which hospital did more you go for Event4? show less	N/A
PX830101_SCDPainSixMonths_Hospital_Event5
	PX830101011900	In the past six months, which hospital did more you go for Event5? show less	N/A
PX830101_SCDPainSixMonths_Hospital_Event6
	PX830101012300	In the past six months, which hospital did more you go for Event6? show less	N/A
PX830101_SCDPainSixMonths_Hospital_Event7
	PX830101012700	In the past six months, which hospital did more you go for Event7? show less	N/A
PX830101_SCDPainSixMonths_Hospital_Event8
	PX830101013100	In the past six months, which hospital did more you go for Event8? show less	N/A
PX830101_SCDPainSixMonths_Hospital_Event9
	PX830101013500	In the past six months, which hospital did more you go for Event9? show less	N/A
PX830101_SCDPainSixMonths_Number
	PX830101030200	How many episodes?	N/A
PX830101_SCDPainSixMonths_Severity
	PX830101030100	In the last 6 months, have you had a painful more episode severe enough to keep you from doing the usual daily activities, but without being seen by a doctor? show less	N/A
PX830101_SCDPainSixMonths_State_Event1
	PX830101010500	In the past six months, what state was the more hospital (Event1) in? show less	N/A
PX830101_SCDPainSixMonths_State_Event10
	PX830101014100	In the past six months, what state was the more hospital (Event10) in? show less	N/A
PX830101_SCDPainSixMonths_State_Event2
	PX830101010900	In the past six months, what state was the more hospital (Event2) in? show less	N/A
PX830101_SCDPainSixMonths_State_Event3
	PX830101011300	In the past six months, what state was the more hospital (Event3) in? show less	N/A
PX830101_SCDPainSixMonths_State_Event4
	PX830101011700	In the past six months, what state was the more hospital (Event4) in? show less	N/A
PX830101_SCDPainSixMonths_State_Event5
	PX830101012100	In the past six months, what state was the more hospital (Event5) in? show less	N/A
PX830101_SCDPainSixMonths_State_Event6
	PX830101012500	In the past six months, what state was the more hospital (Event6) in? show less	N/A
PX830101_SCDPainSixMonths_State_Event7
	PX830101012900	In the past six months, what state was the more hospital (Event7) in? show less	N/A
PX830101_SCDPainSixMonths_State_Event8
	PX830101013300	In the past six months, what state was the more hospital (Event8) in? show less	N/A
PX830101_SCDPainSixMonths_State_Event9
	PX830101013700	In the past six months, what state was the more hospital (Event9) in? show less	N/A
PX830101_SCDPainTwelveMonths
	PX830101020100	In the last 12 months, how many times did more you visit an emergency room because of a sickle cell painful event? show less	N/A
PX830101_SCDPainTwelveMonths_City_Event1
	PX830101020400	In the past twelve months, what city was the more hospital (Event1) in? show less	N/A
PX830101_SCDPainTwelveMonths_City_Event10
	PX830101024000	In the past twelve months, what city was the more hospital (Event10) in? show less	N/A
PX830101_SCDPainTwelveMonths_City_Event2
	PX830101020800	In the past twelve months, what city was the more hospital (Event2) in? show less	N/A
PX830101_SCDPainTwelveMonths_City_Event3
	PX830101021200	In the past twelve months, what city was the more hospital (Event3) in? show less	N/A
PX830101_SCDPainTwelveMonths_City_Event4
	PX830101021600	In the past twelve months, what city was the more hospital (Event4) in? show less	N/A
PX830101_SCDPainTwelveMonths_City_Event5
	PX830101022000	In the past twelve months, what city was the more hospital (Event5) in? show less	N/A
PX830101_SCDPainTwelveMonths_City_Event6
	PX830101022400	In the past twelve months, what city was the more hospital (Event6) in? show less	N/A
PX830101_SCDPainTwelveMonths_City_Event7
	PX830101022800	In the past twelve months, what city was the more hospital (Event7) in? show less	N/A
PX830101_SCDPainTwelveMonths_City_Event8
	PX830101023200	In the past twelve months, what city was the more hospital (Event8) in? show less	N/A
PX830101_SCDPainTwelveMonths_City_Event9
	PX830101023600	In the past twelve months, what city was the more hospital (Event9) in? show less	N/A
PX830101_SCDPainTwelveMonths_Date_Event1
	PX830101020200	In the past twelve months, what was the date more of Event1? show less	N/A
PX830101_SCDPainTwelveMonths_Date_Event10
	PX830101023800	In the past twelve months, what was the date more of Event10? show less	N/A
PX830101_SCDPainTwelveMonths_Date_Event2
	PX830101020600	In the past twelve months, what was the date more of Event2? show less	N/A
PX830101_SCDPainTwelveMonths_Date_Event3
	PX830101021000	In the past twelve months, what was the date more of Event3? show less	N/A
PX830101_SCDPainTwelveMonths_Date_Event4
	PX830101021400	In the past twelve months, what was the date more of Event4? show less	N/A
PX830101_SCDPainTwelveMonths_Date_Event5
	PX830101021800	In the past twelve months, what was the date more of Event5? show less	N/A
PX830101_SCDPainTwelveMonths_Date_Event6
	PX830101022200	In the past twelve months, what was the date more of Event6? show less	N/A
PX830101_SCDPainTwelveMonths_Date_Event7
	PX830101022600	In the past twelve months, what was the date more of Event7? show less	N/A
PX830101_SCDPainTwelveMonths_Date_Event8
	PX830101023000	In the past twelve months, what was the date more of Event8? show less	N/A
PX830101_SCDPainTwelveMonths_Date_Event9
	PX830101023400	In the past twelve months, what was the date more of Event9? show less	N/A
PX830101_SCDPainTwelveMonths_Hospital_Event1
	PX830101020300	In the past twelve months, which hospital more did you go for Event1? show less	N/A
PX830101_SCDPainTwelveMonths_Hospital_Event10
	PX830101023900	In the past twelve months, which hospital more did you go for Event10? show less	N/A
PX830101_SCDPainTwelveMonths_Hospital_Event2
	PX830101020700	In the past twelve months, which hospital more did you go for Event2? show less	N/A
PX830101_SCDPainTwelveMonths_Hospital_Event3
	PX830101021100	In the past twelve months, which hospital more did you go for Event3? show less	N/A
PX830101_SCDPainTwelveMonths_Hospital_Event4
	PX830101021500	In the past twelve months, which hospital more did you go for Event4? show less	N/A
PX830101_SCDPainTwelveMonths_Hospital_Event5
	PX830101021900	In the past twelve months, which hospital more did you go for Event5? show less	N/A
PX830101_SCDPainTwelveMonths_Hospital_Event6
	PX830101022300	In the past twelve months, which hospital more did you go for Event6? show less	N/A
PX830101_SCDPainTwelveMonths_Hospital_Event7
	PX830101022700	In the past twelve months, which hospital more did you go for Event7? show less	N/A
PX830101_SCDPainTwelveMonths_Hospital_Event8
	PX830101023100	In the past twelve months, which hospital more did you go for Event8? show less	N/A
PX830101_SCDPainTwelveMonths_Hospital_Event9
	PX830101023500	In the past twelve months, which hospital more did you go for Event9? show less	N/A
PX830101_SCDPainTwelveMonths_State_Event1
	PX830101020500	In the past twelve months, what state was more the hospital (Event1) in? show less	N/A
PX830101_SCDPainTwelveMonths_State_Event10
	PX830101024100	In the past twelve months, what state was more the hospital (Event10) in? show less	N/A
PX830101_SCDPainTwelveMonths_State_Event2
	PX830101020900	In the past twelve months, what state was more the hospital (Event2) in? show less	N/A
PX830101_SCDPainTwelveMonths_State_Event3
	PX830101021300	In the past twelve months, what state was more the hospital (Event3) in? show less	N/A
PX830101_SCDPainTwelveMonths_State_Event4
	PX830101021700	In the past twelve months, what state was more the hospital (Event4) in? show less	N/A
PX830101_SCDPainTwelveMonths_State_Event5
	PX830101022100	In the past twelve months, what state was more the hospital (Event5) in? show less	N/A
PX830101_SCDPainTwelveMonths_State_Event6
	PX830101022500	In the past twelve months, what state was more the hospital (Event6) in? show less	N/A
PX830101_SCDPainTwelveMonths_State_Event7
	PX830101022900	In the past twelve months, what state was more the hospital (Event7) in? show less	N/A
PX830101_SCDPainTwelveMonths_State_Event8
	PX830101023300	In the past twelve months, what state was more the hospital (Event8) in? show less	N/A
PX830101_SCDPainTwelveMonths_State_Event9
	PX830101023700	In the past twelve months, what state was more the hospital (Event9) in? show less	N/A

SCD Core: Tier 2

Measure Name

Frequency of Sickle Cell Pain Episodes Per Year

Release Date

October 30, 2023

Definition

A questionnaire to capture the frequency of acute pain episodes due to sickle cell disease (SCD).

Purpose

Frequency of acute sickle cell pain episodes is correlated with disease severity and early mortality.

Keywords

pain, sickle cell disease, SCD, frequency of sickle cell pain episodes, work absence, school absence

Measure Protocols

Protocol ID	Protocol Name
830101	Frequency of Sickle Cell Pain Episodes Per Year

Publications

Glassberg, J. A., et al. (2020) Publication of data collection forms from NHLBI funded sickle cell disease implementation consortium (SCDIC) registry. Orphanet J Rare Dis. 2020 July; 15(1): 178. doi: 10.1186/s13023-020-01457-x

Kanter, J., et al. (2020) Perceptions of US Adolescents and Adults With Sickle Cell Disease on Their Quality of Care. Jama Network Open. 2020 May; 3(5). doi: 10.1001/jamanetworkopen.2020.6016

Hankins, J. S., et al. (2018) Sickle Cell Clinical Research and Intervention Program (SCCRIP): A lifespan cohort study for sickle cell disease progression from the pediatric stage into adulthood. Pediatr Blood Cancer. 2018 September; 65(9): 27228. doi: 10.1002/pbc.27228