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Protocol - Counts of Acute Bleeds in Hemophilia

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Description:

This protocol includes a bleeding log from the American Thrombosis and Hemostasis Network (ATHN) and consensus definitions from the International Societies for Thrombosis and Hemostasis (ISTH) network. The bleeding log includes six self-administered questions that capture date and location of bleed, symptoms, treatment, and response to treatment. ISTH definitions cover response to treatment (e.g., excellent, good, moderate, none) as well as classification of new bleeds, joint bleeds, and target joints. This protocol also includes guidance from the Hemophilia Inhibitors Working Group for calculating annualized bleeding rate (ABR).

Specific Instructions:

The Hemophilia Inhibitor Research Working Group (WG) recommends that the calculation of annualized bleeding rate (ABR) be based on the number of bleeds instead of the number of infusions or the amount of prescribed therapeutics. Additionally, when reporting ABR, investigators should specify the methodological details, including the measures used (e.g., bleed logs, infusion logs, dispensing logs); monitoring methods (e.g., patient recall, electronic recording, administrative databases); and the time frame of data collection.

Protocol:

Bleeding Log

1. Date of bleed event

2. Location of bleed event

3. Which of the following symptoms did you have? (All that apply)

  [ ] Unusual feeling in the joint

  [ ] Warmth over the skin

  [ ] Pain

  [ ] Swelling

  [ ] Stiffness

  [ ] Loss of range of motion

  [ ] None of the above

4. Did you treat your bleed with RICE?

  [ ] Yes

  [ ] No

  [ ] Unknown

5. Did you treat your bleed with factor?

  [ ] Yes

  [ ] No

   5a. If yes, did you take more factor than your normal prescribed dose?

     [ ] Yes

     [ ] No

     [ ] Unknown

6. How would you describe your response* to factor treatment?

  [ ] Excellent

  [ ] Good

  [ ] Moderate

  [ ] None

RICE stands for: Rest, Ice, Compression, Elevation.

* International Society on Thrombosis and Hemostasis definition for response to treatment:

  • Excellent - Complete pain relief within 8 hours and/or complete resolution of signs of bleeding after the initial injection and not requiring any further replacement therapy for relief of persistent symptoms and signs in the same joint within 72 hours.
  • Good - Significant pain relief and/or improvement in signs of bleeding within approximately 8 hours after a single injection but requiring more than one dose of replacement therapy within 72 hours for complete resolution.
  • Moderate - Modest pain relief and/or improvement in signs of bleeding within approximately 8 hours after the initial injection and requiring more than one injection within 72 hours but without complete resolution.
  • None - No or minimal improvement, or condition worsens, within approximately 8 hours after the initial injection.

International Society on Thrombosis and Hemostasis Definitions

New Bleed:

  • After an initial moderate to excellent response to treatment, a new bleed is defined as a bleed occurring > 72 hours after stopping treatment for the original bleed for which treatment was initiated.

Joint Bleed:

  • An unusual sensation "aura" in the joint, in combination with any of the following:
    • increasing swelling or warmth of the skin over the joint;
    • increasing pain; or
    • progressive loss of range of motion or difficulty in using the limb as compared with baseline.
  • In infants and young children, reluctance to use the limb alone may be indicative of a joint/muscle bleed.

Target Joint:

  • Three or more spontaneous bleeds into a single joint within a consecutive 6-month period.
  • Where there have been < 2 bleeds into the joint within a consecutive 12-month period, the joint is no longer considered a target joint.

Annualized Bleeding Rate (ABR)

The Hemophilia Inhibitors Working Group recommends that the calculation of annualized bleeding rate (ABR) be based on the number of bleeds instead of number of infusions or amount of prescribed therapeutics. Additionally, when reporting annualized bleeding rate, investigators should specify the methodological details including the measures used (e.g., bleed logs, infusion logs, dispensing logs); monitoring methods (e.g., patient recall, electronic recording, administrative databases); and the time frame of data collection.

Protocol Name from Source:

N/A; see source.

Availability:

Publicly available

Personnel and Training Required

None

Equipment Needs
None
Requirements
Requirement CategoryRequired
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

Life Stage:

Infant, Toddler, Child, Adolescent, Adult, Senior, Pregnancy

Participants:

Any age

Selection Rationale

The Hemophilia Inhibitors Working Group selected the American Thrombosis and Hemostasis Network (ATHN) Bleeding Log and the consensus definitions from the International Society of Thrombosis and Hemostasis (ISTH) as the best standardized methodology for capturing and classifying bleeding events and response to treatment.

Language

English

Standards
StandardNameIDSource
Common Data Elements (CDE) Hemophilia Bleeding Log Assessment Text 6706549 CDE Browser
Derived Variables

None

Process and Review

Not applicable.

Source

Source for Bleeding Log:

American Thrombosis and Hemostasis Network (ATHN). (2015, October 30). ATHNdataset Core Data Elements, Version 2.0. Bleed/Infusion Data: Bleed Events. 2018 American Thrombosis and Hemostasis Network. All rights reserved.

Source for International Society of Thrombosis and Hemostasis definitions:

Blanchette, V. S., Key, N. S., Ljung, L. R., Manco-Johnson, M. J., van den Berg, H. M., & Srivastava, A.; Subcommittee on Factor VIII, Factor IX, and Rare Coagulation Disorders of the Scientific and Standardization Committee of the International Society on Thrombosis and Hemostasis. (2014). Definitions in hemophilia: Communication from the SSC of the ISTH. Journal of Thrombosis and Haemostasis, 12(11), 1935-1939.

General References

Berntorp, E., Astermark, J., Bjrkman, S., Blanchette, V. S., Fischer, K., Giangrande, P. L., Gringeri, A., Ljung, R. C., Manco-Johnson, M. J., Morfini, M., Kilcoyne, R. F., Petrini, P., Rodriguez-Merchan, E. C., Schramm, W., Shapiro, A., van den Berg, H. M., & Hart, C. (2003). Consensus perspectives on prophylactic therapy for haemophilia: Summary statement. Haemophilia, 9(Suppl. 1), 1-4.

Oldenburg, J., Mahlangu, J. N., Kim, B., Schmitt, C., Callaghan, M. U., Young, G., Santagostino, E., Kruse-Jarres, R., Negrier, C., Kessler, C., Valente, N., Asikanius, E., Levy, G. G., Windyga, J., & Shima, M. (2017). Emicizumab prophylaxis in hemophilia A with inhibitors. New England Journal of Medicine, 377(9), 809-818.

Protocol ID:

910101

Variables:
Export Variables
Variable NameVariable IDVariable DescriptionVersiondbGaP Mapping
PX910101_Acute_Bleeding_Date_Bleed_Event PX910101010000 Date of bleed event N/A
PX910101_Acute_Bleeding_Describe_Response_Factor_Treatment PX910101060000 How would you describe your response* to more
factor treatment? show less
N/A
PX910101_Acute_Bleeding_Location_Bleed_Event PX910101020000 Location of bleed event N/A
PX910101_Acute_Bleeding_Symptoms_Did_Have PX910101030000 Which of the following symptoms did you have? N/A
PX910101_Acute_Bleeding_Take_More_Factor_Than_Prescribed PX910101050100 If yes, did you take more factor than your more
normal prescribed dose? show less
N/A
PX910101_Acute_Bleeding_Treat_Bleed_Factor PX910101050000 Did you treat your bleed with factor? N/A
PX910101_Acute_Bleeding_Treat_Bleed_RICE PX910101040000 Did you treat your bleed with RICE? N/A
Hemophilia Inhibitor Research
Measure Name:

Counts of Acute Bleeds in Hemophilia

Release Date:

May 7, 2019

Definition

Bleeds in hemophilia are not typically the results of massive trauma (e.g., gunshot, stabbing) but instead are most often internal bleeding events into joints, organs, mucosa, or soft tissue and involve little or no known trauma.

Purpose

This measure can be used to document the occurrence, treatment, response to treatment, and classification of a bleeding event. This measure also includes guidance for calculating annualized bleeding rate (ABR).

Keywords

New bleed, target joint, joint bleed, annualized bleeding rate, ABR