NHC Memo from NHC Metting 23 November 2017 in Copenhagen
Nordic Haemophilia Council Meeting - Memo
Location: Copenhagen Airport (T3), Clarion Hotel, meeting room Pegasus I+II
Date: 2017-11-23, 09.00- 12.00 (breakfast 9-9.30)
Participants: Elina Armstrong (Helsinki), Jan Astermark (Malmö), Fariba Baghaei (Göteborg), Eva Funding (Copenhagen), Marianne Hoffmann (Copenhagen), Pål Andre Holme (Oslo), Margareta Holmström (Stockholm), Vuokko Nummi (Helsinki), Karin Strandberg (Malmö), Ines Vaide (Tallinn), Eva Zetterberg (Malmö)
- Appointment of chairman for the meeting -> Fariba
- Appointment of secretary for the meeting -> Elina
A new member, Marianne Hoffmann, pediatric hematologist from Copenhagen was introduced and welcomed to the council.
- Report of the working groups
3a. Nordic Hemophilia Guidelines:
Document is very long, it was discussed that recommendations are summarized (bullet points) in the beginning or the end of the full text.
Text on will be placed on the website in a form that can be searched, possibly with links to references and other chapters (Jan will ask Camilla)
Surveys for example Plt questionnaire (see 3c.-d. below), Nordic statistics, genetic counseling, pre-conception methods could be placed on the website separate from the guideline text.
Laboratory chapter is ready (Karin):
A short chapter on global assays, thrombin generation will be added.
Various APTT reagents were discussed and suggested that a table of Nordic methods could be added, but recommendations regarding their use cannot be made.
Carriers of Hemophilia -chapter is ready (Anna Olsson):
Eva Z: Survey regarding fetal diagnosis, pre-conceptual techniques / counceling, genetic testing by late amniocentesis, gender determination from maternal blood etc. could be added to the web site rather than the guidelines text (see General discussion above).
Physiotherapy chapter (Elisabeth Brodin):
HEAD-US should be mentioned here. US is discussed in the prophylaxis chapter (link?)
Surgery chapter (Pål Andre):
Practices and recommendations have not changed much. EHL will be mentioned but no recommendations can be made.
Surgery in inhibitor patients –chapter (Eva Z): No issues to discuss this time
Inhibitor chapter (Jan and Fariba):
Risk factors in the pediatric prophylaxis chapter should be coordinated with this chapter (Eva F)
Factor type and risk (SIPPET dat) is not changing the current practices in the Nordics, recombinant factor products are exclusively used in all countries.
Regarding new treatments. Only emicizumab (Hemlibra) will be mentioned as it is FDA approved. Other therapies are still used only in clinical trials and can be mentioned without specifics or recommendations.
Prohylaxis doses were discussed. FEIBA 85 IU/kg -> 50-85 IU/kg, rFVIIa in small children. Indications; will add target joints, with significant bleeding tendency.
ITI; minor changes were made, hemophilia B. VWF containing products are mentioned. Switching as a non-risk factor could be added (in this chapter or prophylaxis chapter or both).
Prophylaxis chapter (Eva F, Susanna R, Pia P etc):
When to stop/taper – will be omitted -> will recommend to continue prophylaxis when possible. Recombinant factor products over plasma-derived.
EHL products are added. Several issues were discussed. What is the definition of EHL FVIII? Criterias for use / indications were discussed; medical vs convenience vs cost savings. FIX and FVIII EHL will be presented separately. PK is recommended, different tools like WAPPS etc. can be mentioned.
Comorbidities and aging chapter (Elina):
No significant new data in the literature. Pain chapter will be moved to the corresponding chapter (Elina to communicate with Lone).
Osteopenia / bone health part to include practical guide for evaluation and treatment recommendations, for example in a table.
Infectious disease issues are recommended to be handled by infectious disease, hepatologist etc. specialist.
Recent reference regarding antithrombotic therapy was added. Cardiovascular issues will be further updated regarding current practices regarding stents and other procedures. Elina will consult cardiology.
Renal disease /Hypertension – PA Holme 2016 publications to be added to references. PSA screening recommendation to be checked with an urologist. Biopsies should be avoided.
Recommendations to be added in the end: collaboration/consultatins and networking with other relevant specialists, primary care, home hospital care is emphasized.
Other chapters are to be submitted. Eva F volunteered to serve as a coordinating author to construct the revised chapters into a single document, which will be sent to all NHC members to review. The aim is to have final document ready on the website during the winter-spring 2018.
3b. Nordic Acquired Hemophilia Study and grant application (Eva Z):
- Protocol was sent to NHC members, comments have been taken to account
- Shire and Novonordisk have shown interest in sponsoring the study
- Contract is written with Shire, who will pay to NHC (NHC is a non-profit organization, no VAT)
- Invoice for small grants – taxable? Novo was asking on organization number
- Budget; total 1.036.00 SEK for 8 Nordic centers (120.000 SEK /each- or based on the amount of work (number of patients included) by each center)
- one center experience (Malmö); 40pt from the Swedish registry 2006-2016, prelim. results presented
- all centers involved -> possibility to gather data from 250 patients
- feasibility was discussed
- Contact persons from each center were identified, Eva Z will be in touch regarding feasibility and ethical permission practices. Skype meeting can be arranged. Dead-line for ethical application 1.2.18.
3c-d. Platelet function assessment in the Nordic countries (Riitta):
See the survey, https://www.webropolsurveys.com/S/B6450C7272F20329.par
Will be added to the website
3e.-f. Nordic aquired hemophilia study grant application:
-> next meeting
Next meeting will be held in Stockholm Thu 6th September morning in conjunction with the NordCoag2018
23 Nov 2017, Copenhagen
Fariba Baghaei, chairperson
Elina Armstrong, secretary