Beruflich Dokumente
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Published by:
Malaysian Health Technology Assessment Section (MaHTAS)
Medical Development Division, Ministry of Health Malaysia
Level 4, Block E1, Precinct 1
Federal Government Administrative Centre 62590
Putrajaya, Malaysia
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The copyright owner of this publication is MaHTAS. Content may be
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context.
ISBN: 978-967-2173-64-9
STATEMENT OF INTENT
TABLE OF CONTENTS
1. INTRODUCTION 1
2. CLINICAL PRESENTATION 2
3. INVESTIGATIONS 4
3.1 Laboratory Tests 4
3.2 Genetic Tests 5
5. TREATMENT 10
5.1 Pharmacological Treatment 10
5.1.1 Factor Replacement Therapy 10
5.1.2 Adjunct Therapies 14
5.1.3 Analgesia 16
5.2 Non-pharmacological Treatment 18
5.2.1 Rehabilitation of Musculoskeletal System 18
5.2.2 Protection, Rest, Ice, Compression and Elevation 20
5.2.3 Joint Protection 21
5.2.4 Sports/physical Activity 21
5.2.5 Post-operative Care 21
5.2.6 Weight Management 22
TABLE OF CONTENTS
8. INHIBITORS 33
8.1 Treatment of Acute Bleeding 34
8.2 Prophylaxis Therapy 34
8.3 Eradication of Inhibitors 35
9. HOME THERAPY 37
13. MONITORING 48
13.1 Inhibitors 48
13.2 Bleeding Frequency 48
13.3 Joint Health 49
13.4 Radiological Measures 49
REFERENCES 53
Appendix 1 Example of Search Strategy 59
Appendix 2 Clinical Questions 60
Appendix 3 Guidelines on Sample Collection and 62
Transportation
Management of Haemophilia
TABLE OF CONTENTS
List of Abbreviations 79
Acknowledgement 81
Disclosure Statement 81
Source of Funding 81
Management of Haemophilia
LEVELS OF EVIDENCE
Level Study design
FORMULATION OF RECOMMENDATION
i
Management of Haemophilia
KEY RECOMMENDATIONS
Investigations
Pharmacological Treatment
Non-pharmacological Treatment
Inhibitors
ii
Management of Haemophilia
Home therapy
Special Situations
Dental Care
Monitoring
iii
Management of Haemophilia
GUIDELINES DEVELOPMENT
The members of the DG for this CPG were from the Ministry of Health
(MoH). There was active involvement of a multidisciplinary Review
Committee (RC) during the process of the CPG development.
The literature used in these guidelines were graded using the US/
Canadian Preventive Services Task Force Level of Evidence (2001),
iv
Management of Haemophilia
v
Management of Haemophilia
OBJECTIVES
CLINICAL QUESTIONS
Refer to Appendix 2.
TARGET POPULATION
Inclusion Criteria
• All patients with congenital haemophilia A and B
Exclusion Criteria
• Patients with:
a. Acquired haemophilia
b. Other congenital bleeding disorders
TARGET GROUP/USERS
HEALTHCARE SETTINGS
vi
Management of Haemophilia
DEVELOPMENT GROUP
Chairperson
vii
Management of Haemophilia
REVIEW COMMITTEE
Chairperson
Associate Professor Dr. Azlan Husin Dr. Lily Wong Lee Lee
Consultant Physician & Clinical Senior Consultant Haematologist
Haematologist Hospital Universiti Sains Hospital Queen Elizabeth, Sabah
Malaysia, Kelantan
Dr. Carol Lim Kar Koong Professor Dr. Noraini @ Nun Nahar Yunus
Head of Department & Consultant Professor in Paediatric Dentistry
Obstetrician & Gynaecologist (Maternal Lincoln University College
Fetal Medicine) Petaling Jaya, Selangor
Hospital Sultan Ahmad Shah, Pahang
viii
Management of Haemophilia
ix
Management of Haemophilia
XY XX
XY XY XX XX
XY XX
XY XY XX XX
x
Management of Haemophilia
Counselling Counselling
*Send samples in sodium citrate tubes of index case and both parents if testing
is done in Pusat Darah Negara and in ethylenediaminetetraacetic acid tubes
of index case and mother if testing is done in IMR. Refer to Appendix 3 on
Guidelines on Sample Collection and Transportation.
xi
ALGORITHM 3. PHYSIOTHERAPY MANAGEMENT
Physiotherapy Management
- PRICE
xii
Acute Subacute Chronic - All PWH should do regular
(within 72 hours): (>3 days) (>6 months): - Isometric exercise sports/physical activities.
PRICE - Mobilisation
P - Protection - Hydrotherapy - Hydrotherapy • Active exercise - Refer to Appendix 4 for
(splint) - Mobilising - Mobilising exercise • Bed mobility the type of sport/physical
R - Rest exercise - Strengthening • Ambulation activities in relation to the
I - Ice - Strengthening exercise risk of bleeding.
C - Compression exercise - Mechanical exercise
E - Elevation - Mechanical - Weight-bearing
exercise exercise
• All these activities are preferably carried out within 24-hour of factor infusion.
• Sports and physical fitness are important to maintain good muscle tone to protect the joints from the haemophilic-induced injuries, and
these activities contribute to improvement in quality of life.
Management of Haemophilia
Age group Scale
Management of Haemophilia
1 - 3 years and adult patient unable Face, Legs, Activity, Cry, Consolability
to communicate
Pain verbally
severity is categorised (FLACC)
in Table 8 and Scale
its management is described
following it.
>3 - 7 years Visual Analogue Scale (VAS)
>7 years and adults Numeric Rating Scale (NRS)
Table 8. Category of pain
17