Nordic Hemophilia Guidelines
Authors
1
Hemophilia A and B
2
History of hemophilia in the Nordic countries
3
Organisation of hemophilia care
3.1
Multidisciplinary activities
3.2
Registries
3.3
Outcome analysis, QoL and health economy
4
Laboratory diagnosis
Recommendations
4.1
Pre-analytical aspects of hemophilia testing
4.1.1
For plasma-based coagulation assays the recommendation is:
4.2
Screening for hemophilia
4.3
Specific Factor VIII and IX assays
4.3.1
Differential diagnoses
4.3.2
Factor VIII:C assays
4.3.3
Factor IX:C assays
4.3.4
Antibodies against FVIII or FIX
4.3.5
Factor activity assays for monitoring treatment with EHL-products, emicizumab and gene therapy
4.4
Genetic diagnosis
5
Prophylaxis and on-demand treatment
Recommendations
5.1
Background
5.2
Assessment
5.2.1
Bleeding frequency
5.2.2
Quality of life
5.2.3
Physical score
5.2.4
Imaging technique scores
5.3
Pediatric issues
5.3.1
Choice of factor product
5.3.2
Starting prophylaxis
6
Adolescence
Recommendation
7
Inhibitors
7.1
Introduction
7.2
Bypassing agents for the treatment of bleeds
Recommendations
7.3
Prevention of bleeds
Recommendations
7.4
Immune tolerance induction (ITI) therapy
7.4.1
Recommendations
7.4.2
ITI and mild/moderate hemophilia
8
Surgery in hemophilia - practical guidelines
Recommendations
8.1
Preoperative planning
8.2
Substitution principles
8.2.1
Continuous infusion
8.2.2
Bolus injections
8.3
Major surgery including orthopedic surgery
8.3.1
Continuous infusion
8.4
Minor surgery
8.5
Specific surgery
8.5.1
Dental extraction
8.5.2
Circumcision
8.5.3
Liver biopsy
8.5.4
Tonsillectomy/adenotomy
8.5.5
Prostatectomy
8.5.6
Mild hemophilia
8.5.7
Tranexamic acid
8.5.8
Limitations
8.5.9
Adverse effects
8.6
Postoperative management
8.7
Orthopedic aspects
8.8
EHL products and surgery
8.9
Non-factor replacement therapy and surgery in non-inhibitor patients
9
Surgery in PWHs with inhibitors
Recommendations
9.1
aPCC and rFVIIa
9.2
Management of substitution therapy in the peri- and postoperative phase
9.3
aPCC - FEIBA®
9.4
rFVIIa - NovoSeven®
9.5
Emicizumab, Hemlibra®
9.6
Alternative treatments
9.6.1
Recombinant porcine FVIII
9.6.2
Bypassing agents and antifibrinolytics
9.6.3
Bypassing agents and thromboprophylaxis
10
Comorbidities in the ageing patients with hemophilia
Summary of recommendations
10.1
Introduction
10.2
Current status and recommendations / managing suggestions
10.2.1
Joint disease
10.2.2
Osteoporosis
10.2.3
Infection related issues / complications
10.2.4
Metabolic syndrome
10.2.5
Cardiovascular disease
10.2.6
Renal disease
10.2.7
Cancer
10.2.8
Conclusion
11
Treatment of pain
11.1
Analgetics
11.2
Orthopedic surgery and treatment by the orthopedist
11.2.1
Intraarticular corticosteroid injection in joints with hemophilia arthropathy
12
Physiotherapy
Recommendations
12.1
Introduction
12.2
Prevention
12.3
Assessment
12.4
Intervention (treatment/rehabilitation)
13
Hemophilia in women and girls and hemophilia carriers
13.1
Background
13.2
Bleeding symptoms
13.3
Prenatal diagnosis
13.4
Management of pregnancy and delivery
14
Hemophilia nurse role
Recommendations
15
Dental care
15.1
Hemostatic treatment
15.2
Anesthesia
References
Nordic Hemophilia Guidelines
Nordic Hemophilia Guidelines
NORDIC
HEMOPHILIA
COUNCIL
Published online
Last updated: June 2022
Next update: 2023
