WFH

2018

Poster presented at:

CBDR and MyCBDR: Advancing Hemophilia Nursing Practice in CanadaKay Decker, RN* and Claude Meilleur, RN†

*McMaster Children’s Hospital, Hamilton Health Sciences, Hamilton, ON and †CHU Sainte-Justine, Montreal, QC

In Canada, Hemophilia Treatment Centers (HTCs) provide care andservices to patients with bleeding disorders. The advancement ofroutine prophylaxis regimens allows patients to experience lessbleeds and fewer unscheduled visits to HTCs.1 To adapt to thesechanges in hemophilia management, comprehensive care teamsmust implement new strategies to accurately monitor bleedingepisodes, factor usage and adherence to treatment regimens.1-4 Thisrequires detailed record keeping and adequate patient education tohelp care teams manage patients and individualize factorreplacement treatment regimens.4-6 In 2015, Canada launched theCanadian Bleeding Disorder Registry (CBDR), along with MyCBDR, aninternet-based infusion tracking application that allows synchronouscommunication with CBDR at HTCs across Canada.7 Here, we presenttwo case studies that illustrate how CBDR/MyCBDR have improvedthe efficiency of our practice and can foster a rapid sharing ofinformation between patients and their care team.

Introduction

Technological advances have introduced several electronic-basedmethods for hemophilia tracking. In 2015, CBDR/MyCBDR waslaunched in Canada to provide the opportunity to improve patientadherence, education and engagement. These platforms have thecapability to assemble all patient data into one easily accessiblelocation and allow care teams to receive real-time updates ofinformation inputted by patients via computer or mobile device(Figure 1).7

Patient education is a fundamental component for successfulhemophilia self-management. Lack of hemophilia knowledge resultsin poor adherence and self-management.2,8 During one-on-one visitswith patients, nurses can use CBDR to help patients betterunderstand their hemophilia and how they can be active in itsmanagement. In addition, MyCBDR encourages users to take controlof their hemophilia management, and can be reinforced by careteams with one-on-one patient education.

Advancing Hemophilia Tracking

& Patient Education

Figure 1. Integration of CBDR and MyCBDR.7 These platforms allow efficient sharing of information between patients/caregivers and their care team. In addition, information on the CBDR database provides anonymous collection of patient data to support hemophilia research.

Database

Patient/Caregiver

HTC

Hemophilia Research

Case Studies

Health Care Team:• Performed medical assessment• Physiotherapist evaluation• Established trust by reviewing all treatment

options to select an approach best suited for Oscar• Evaluated inhibitor test = Positive (low titre)• Discussed value of using MyCBDR to monitor

bleeds, inhibitor levels and treatments to optimize therapy

Case Overview: • 04/1999: Severe Hemophilia A• 05/1999: Inhibitor results = 12 BU

Nurse:• Alert feature allowed nurse to receive notification of

hematoma via email (photo uploaded to MyCBDR)• Reviewed data inputted by Oscar into MyCBDR• Scheduled appointment with Oscar to discuss changing to

short-term treatment regimen• Educated Oscar on hemophilia management, his increased

risk of intracranial bleeding, importance of inhibitor testing and role for prophylaxis

• Performed inhibitor testing

• 2008/2013: Intracranial bleed • 06/2015: Enrolled in MyCBDR

• 06/2016: Inhibitor results = Negative• 06/2017: Large hematoma in left thigh

538

6

1

1

Total

Planned

Treatment

Nov15

Dec15

Jan16

Feb16

Mar16

Apr16

May16

Jun16

Jul16

Aug16

Sep16

Oct16

Nov16

Dec16

Jan17

Feb17

Mar17

Apr17

May17

Jun17

Jul17

Aug17

Sep17

Oct17

Nov17

Dec17

Jan17

Feb17

18 19Age

Product Usage

Helixate FS

Xyntha

FEIBA NF

Key Changes

Inhibitor Testing

New Treatment Plan

Weight Change

Interaction History

Coagulation

Pharmacokinetic Study

Hemophilia Clinic Visit

MyCBDR Treatment

Case 1: Oscar* | Age 20

Health Care team:• Encouraged David to switch from a paper diary to

MyCBDR to better evaluate bleed patterns/infusion regimes (dose/time/frequency)

• A medical assessment was performed by the physician, nurse and physiotherapist

• Performed inhibitor testing• PK study was performed (WAPPS-Hemo, McMaster

University) to evaluate FVIII half-life and optimize David’s treatment schedule

• Data from CBDR/MyCBDR and WAPPS-Hemo were evaluated

– WAPPS-Hemo confirmed a short half-life of 6 hours

*Some case information has been altered to protect patient identity. PK = pharmacokinetic.

• Using visuals from CBDR/MyCBDR and WAPPS-Hemo, health care team educated David on his bleeding patterns and explained rationale for his individualized treatment interval

• Provided an individualized treatment recommendation– Prophylaxis was adjusted to every 36 hours

Case Overview: • Severe Hemophilia A• Past history of inhibitors

• Using paper diary to record treatments

• Breakthrough bleeds despite prophylaxis every 48 hours• Prefers not to treat daily

50 10 15 20 25 30 35 40 450.0

0.5

1.0

1.5

2.0

Pla

sma

Fact

or

Co

nce

ntr

atio

n (

IU/m

L)

Time (hr)

0.02 0.010.95

Estimate

Parameter Conservative Balanced Optimistic

Time to 0.05 IU/ml (hr) 26.00 28.25 30.50

Time to 0.02 IU/ml (hr) 35.50 38.50 41.75

Time to 0.01 IU/ml (hr) 45.00 49.00 53.00

Half-life 6.00 6.50 7.00

Pharmacokinetic Estimate

Convert days to hours Plasma Factor Concentration Estimate (IU/mL)

Time (Days) Conservative Balanced Optimistic

1 0.000 0.068 0.000

2 0.000 0.005 0.000

2000

2500

1500

1000

500

001 02 03 04 05 06 07 08 09 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31

MyCBDR Product Treatment Over Time

Routine Prophylaxis Bleed

IU

Measured

Estimated

Case 2: David* | Age 41

1. Paper diaries may be the most appropriate choice for some patients, however MyCBDR provides care teams/patients with more complete information and easy to interpret graphs

2. By reviewing data from platforms such as CBDR/MyCBDR and WAPPS-Hemo, care teams can:– Obtain a more comprehensive overview of patients’

profile, make more informed treatment decisions and individualize treatment regimens

– Use patients’ own data to educate and empower them to better understand their bleed patterns and be active in the management of their hemophilia care

Case Key Teaching Points

1. MyCBDR supports efficient information sharing between patient and HTC – Alert feature allowed care team to intervene quickly to

adjust treatment plan– Although care team proactively contacted Oscar, patients

should always call their HTC if a major bleed occurs

2. Established line of trust and transparency between patient and HTC is essential – Important for care team to support patient’s treatment

choice

3. CBDR/MyCBDR allows HTC to assess treatment effectiveness and adjust as needed in real-time – graphs of bleed occurrences in context of inhibitor levels/treatments can help patients understand the rationale behind recommended treatment changes

Case 2: David

Summary

Case 1: Oscar 1. Build a trusting relationship with the patient:► Allows patients to be transparent with care teams and confidently

accept treatment recommendations

2. Remain nonjudgmental:► Care teams and caregivers need to remain open and

nonjudgmental – failure to do so could encourage dishonesty among patients

3. Start patient education early:► Beginning patient education in adolescents is fundamental in

promoting treatment adherence and hemophilia self-management

4. Use visuals:► During one-on-one visits, CBDR/MyCBDR provides visual tools to

help patients understand their bleed patterns and treatment efficacy

5. Individualize treatment regimens:► Review possible treatment options and engage patients in

choosing a regimen that is realistic for them to follow

References:1. Sholapur N et al. Haemophilia. 2010;19:289-295; 2. Peyvandi F et al. The Lancet. 2016;388:187-97; 3. Khair K. Orthopaedic Nursing. 2010;29:193-200; 4. Walker I et al. Haemophilia. 2004;10:698-704; 5. Srivastava A et al. Haemophilia. 2013;19:1-47; 6. Canadian Bleeding Disorders Registry – CBDR. Available from: www.hemophilia.ca; 7. Carcao and Iorio. Semin Thromb Hemost. 2015;41:864-71; 8. Saxena K. J Blood Med. 2013;4:49-56.

Acknowledgements:

Editorial and publication support provided by Shire Canada.

T-P-19

T-P-14Kay Decker DOI: 10.3252/pso.eu.WFH2018.2018

Databases and registries