HAE SA Update
It is a while since we last communicated but just to update everyone, we had a very successful inaugural meeting in Cape Town in October, attended by Tony Castaldo (CEO)from the USA and Henrik Balle-Boosyen from Europe ,both from HAEi. In addition Professors Jonny Peter (UCT Lung Institute), Mike Levin(Red Cross War Memorial Hospital) and Professor Emeritus Paul Potter (who many patients know from his dealings with HAE over the past 27 years or so at the Allergy Clinic at Groote Schuur Hospital) addressed the meeting. Patricia Karani who is the HAEi Sub-Saharan Representative came from Kenya .
31 patients from all over the country attended this get-together over lunch together with all the above. It was most informative and the very first time most patients had ever met anyone outside their families with HAE. Altogether now we have over 70 patients on record and we know there are still more that have not yet been formally diagnosed.
There are many more HAE medications being developed and tested in addition to those presently available, although they have not yet been registered in South Africa. It is possible however to access these on a named patient basis and all patients are advised to investigate this on a personal level.
The one point that has to be emphasised is that ultimately each patient needs to take responsibility for themselves and their own treatment plan. They need to have in place, a normal day to day management plan as well as an acute emergency plan and this plan should be written down on their AFSA patient card, which can be downloaded here
Everyone should also be registered with Medic alert and wear a medic alert bracelet at all times. There are some really funky styles available now
And don’t miss our AFSA approved write on Medibands!
Out of the 31 attendees at the meeting 5 had family members who had had fatal encounters with HAE. That should no longer ever happen. With increased awareness and personal responsibility everyone has the chance to live a fufilling and normal life.
- We also trust that all our patients have joined the HAEi community at www.haei.org or if they have not done so yet they do so soon. It is very uplifting to know that there are actually others out there living a most fulfilling life, even with HAE. All the management personnel worldwide have HAE themselves.
The magazine is out now too. Please feel free to download it – and distribute it to anyone you think could make good use of it:
2.We have listed all our patients on the international database HAEiConnect which is a cloud based program which will assist with the collation and analysis of data.
3.In order to improve the quality of treatment we need to create a list of medical personnel who are familiar with HAE treatment, either because they already have HAE patients or wish to develop an interest in the condition.
Jane Rushton and Susan Beauchamp will be requesting details from you so that we can keep these medical practitioners updated when necessary, on current treatment for example. We would prefer cell numbers so please get hold of them now if you can so that you have them.
4.We have attached a format for a card size international HAE card which downloads with power point and is editable for your personal use.
It contains references to medications that are not available in this country but obviously are elsewhere in the world but not in Africa. You could use it though in conjunction with your AFSA, card locally.
Please note that it also contains the new ICD-10 code for HAE
D84.1 is the international criteria for HAE and will serve as a reliable reference in emergency situations.
- We have created a South African site which is very basic at the moment but which we intend to develop in the future.
An international expert panel has reviewed the existing evidence and developed 20 recommendations that were discussed, finalized and consented during the guideline consensus conference in 2016 in Vienna, Austria. The final version of this update and revision of the guideline incorporates the contributions of a board of expert reviewers and the endorsing societies. The goal of the guideline update and revision is to provide clinicians and their patients with guidance that will assist them in making rational decisions in the management of HAE with deficient C1-inhibitor (type 1) and HAE with dysfunctional C1-inhibitor (type 2).
It would be a good idea for you to keep a copy of the following page in your medical file
Berinert would be used instead of CI-INH but has to be imported on a named patient basis. In SA Fresh Frozen Plasma is used in the absence of available medication.