1. Inherited Bleeding Disorder Comprehensive
Care Centres / Standards of Care
Impact on Quality of Care and Health
Outcomes
David Page
National Executive Director
Canadian Hemophilia Society
CORD Rare Disease Day Conference
March 5, 2015

2. Inherited bleeding disorders
Bleeding disorder
Number registered in
centres*
Incidence
Hemophilia A 3094 1 in 10,000
Hemophilia B 869 1 in 40,000
Von Willebrand disease 4184 1 in 1000?
Rare factor
deficiencies (I, II, V, VII,
X, XI, XIII)
1517
1 in 100,000 to 1 in
5,000,000
Platelet function
disorders
954 Very low
* Data from 2013-14 CHS centre resource assessment

3. Inherited bleeding disorders
Severity of
bleeding
symptoms
Hemophilia A
and B
von Willebrand
disease
Rare factor
deficiencies
Platelet
function
disorders
Joints ✓✓✓
Muscles ✓✓ ✓
Bruising ✓ ✓ ✓✓ ✓
Vital organs ✓✓ ✓✓✓
Mucosal ✓ ✓✓✓ ✓ ✓✓✓
Menorrhagia ✓ ✓✓✓ ✓ ✓✓
Post-trauma ✓ ✓ ✓✓✓ ✓
Surgical ✓ ✓ ✓✓ ✓
Lacerations ✓✓ ✓ ✓✓

5. Comprehensive care centres
5 pediatric centres
5 adult centres
15 combined ped-adult centres

6. Comprehensive care
Hemophilia Care
“Medical care for hemophilia is specialized. A person
with hemophilia must receive care from healthcare
workers who have expert knowledge of the bleeding
disorder. The wide-ranging needs of people with
hemophilia and their families are best met through
Hemophilia Treatment Centres rather than by
individual doctors.”
- World Federation of Hemophilia: Organizing a
National Programme for Comprehensive Hemophilia
Care.

7. Comprehensive care
Hemophilia Care
“Medical care for hemophilia is specialized. A person
with hemophilia must receive care from healthcare
workers who have expert knowledge of the bleeding
disorder. The wide-ranging needs of people with
hemophilia and their families are best met through
Hemophilia Treatment Centres rather than by
individual doctors.”
- World Federation of Hemophilia: Organizing a
National Programme for Comprehensive Hemophilia
Care.

8. Comprehensive care principles*
• Improved quality of life
• Collaboration among HTCs and networks needs to be
encouraged
• Bleeding disorders are associated with a number of
complications… and so care needs to be comprehensive.
• Evaluation of clinical outcomes is essential.
• Standards of care are needed and should be audited.
• Accountability for utilization of coagulation therapy is
necessary
• Regional differences within the province or region must be
acknowledged in the provision of care.
* CANADIAN COMPREHENSIVE CARE STANDARDS FOR HEMOPHILIA AND OTHER
INHERITED BLEEDING DISORDERS, First Edition, June 2007

9. Health outcomes
“A two-year study of 40 children in Montreal indicates
that home care treatment of bleeding episodes,
supported by a comprehensive care centre, reduces
hospitalizations by 85%, arrests the development of
severe hemorrhages and reduces costs by 85%.”

10. Health outcomes
“A two-year study of 40 children in Montreal indicates
that home care treatment of bleeding episodes,
supported by a comprehensive care centre, reduces
hospitalizations by 85%, arrests the development of
severe hemorrhages and reduces costs by 85%.”
(Dr. Hanna Strawczynski, MD of Canada, October
1972)

11. Health outcomes
“Patients who used both home therapy and received
care in HTCs* had the highest probability of avoiding
hospitalization for bleeding complications during the
follow-up period.”
J. M. SOUCIE et al, Haemophilia (2001), 7, 198±206
“Those persons who had received care in an HTC had
a 40 percent decreased risk of morbidity and
mortality.”
J.M. Soucie et al, BLOOD, 15 July 2000 • Volume 96, Number 2
*HTC – Hemophilia Treatment Centre

12. Comprehensive care
1. Provincial designation
2. National patient registries
3. Self/family administration of therapeutics
4. Patient, family and association involvement in care
5. Education of patients, families and health care
providers
6. Standards of care and portability
7. Decentralization through outreach
(Adopted by Network of Rare Blood Disorder
Organizations, 2006 conference)

13. Comprehensive care
8. Inter-disciplinary care
9. Defined core services delivered by a
comprehensive care team
10. Program evaluation and accreditation
11. National collaboration
12. Post-marketing surveillance
13. Collaborative research
14. Flexibility in organization
(Adopted by Network of Rare Blood Disorder
Organizations, 2006 conference)

14. Hemophilia/IBD standards of care
Adopted in June 2007 after interdisciplinary study
by…
Association of Hemophilia Clinic Directors of Canada
Canadian Association of Nurses in Hemophilia Care
Canadian Physiotherapists in Hemophilia Care
Canadian Social Workers in Hemophilia Care
Canadian Hemophilia Society (patient organization)

15. Standards of care document
• Principles of care
• Populations served
• Definition of core team members (MD, RN, PT, SW,
admin/data)
• Definition of extended team members (e.g. OB-
GYN)
• Services provided
• Responsibilities of an HTC
• Standards
• Scope of care & key indicators
• e.g Establish and maintain a full complement of core team
members
• Quality measures & key indicators
• e.g. Participate in data collection
• Therapeutic services & key indicators
• E.g. Have sufficient number of assessment clinics

16. Assessing standards of care
2007: Standards adopted
2009: Self-assessment of standards by 22 of 24 HTCs
2009: Standards found to be realistic, useful,
acceptability judged high, and appropriate to be used
for external accreditation (audit)
2010: External audit process developed based on
similar process used by HTC networks in U.K.
and Ireland
2011-2012: Audit process delayed by hospital patient
privacy issues
2013: CHS developed own “centre assessment”
process based on work done by its Quebec
Chapter

17. Centre assessments
In three parts:
1.In-person interviews with all core team members in
24/25 centres across Canada (approximately 150
people) to assess their capacity to respect standards
of care
2.Data collection on numbers of patients and FTEs of
core team members
3.In-depth, anonymous patient satisfaction survey
sent to a random sample of patients (400 responses)

18. Centre assessments: deliverables
20 individual centre assessments (plus a collective
assessment for 4 Quebec centres) with…
• HTC strengths
• HTC weaknesses
• Assessments of human resource needs
• Assessments of physical resource needs
• Patient observations and appreciation
• Recommendations to HTCs, hospitals and
Ministries of Health

19. Centre assessments: key observations
• Most centres have insufficient human resources to
respect standards
• Many centres have insufficient physical resources
(space and time) to properly conduct clinics
• Core teams are not complete in some centres (e.g.
no dedicated physiotherapist or social worker)
• Many centres do not have adequate resources to
collect and act on health outcome and factor
utilization information
• Patients are overwhelmingly appreciative of the
services and care they receive!

20. Centre assessments: deliverables
A national report (to be published in June 2015)
including…
•Common strengths
•Common weaknesses
•Recommendations that apply across Canada
•Data:
• Ratios of FTEs in each centre by discipline per
100 patients with hemophilia A and B
• Comparison of the cost of coagulation
therapies to the cost of delivery of care
(staffing, procedures, hospital admissions, lab
work…)

21. Centre assessments: advocacy
• Delivery of final centre assessment reports to
centre staff and provincial chapters of CHS (✔)
• Meetings with local chapters and centre staff to
develop local advocacy plans
• Meetings with local and provincial health officials,
where needed
• Meeting with Provincial / Territorial Blood Liaison
Committee

22. The concept of comprehensive care for inherited
bleeding disorders, based on standards of care, is
accepted and widely adopted in developed and
developing countries around the world …
… and is waiting to be widely copied by other rare
disorders!