General
TALK AT THE BAPTIST CHURCH, PARWOOD ESTATE
PACE had the priviledge to be invited by David Clarke to talk to the Senior Organisation of the Baptist Church near Parkwood Estate in Cape Town on Saturday, 17 July. Prof Paul Brink was kind enough to give a talk titled: "Matters of the Heart"! Paul certainly gave a very informative talk starting with basics of how the heart functions. He gave special attention to PACE's goal of educating the public on Sudden Cardiac Arrest and explained defibrillation in detail. A great and informative afternoon was enjoyed by all. Thank you very much David for inviting us! CLICK HERE to view photo of the meeting.
ARVD (Arrhythmic Right Ventricular Dysplasia) Meeting 8 May 2010 Baltimore USA
I had the privilege of attending the 11th Annual Johns Hopkins Hospital ARVD Patient and Family meeting on the 8th of May in Baltimore. This very interesting meeting was attended by nearly 200 young and older ARVD patients and families.
Johns Hopkins Hospital in Baltimore is one of the world leaders on ARVD matters and Prof Hugh Calkins, the Director of Electrophysiology, runs a program exploring ARVD genetics, diagnostic criteria and improved treatment options.
For this annual meeting, initiated by Prof Hugh Calkins, the guest speaker was Prof Gaetano Thiene, Professor of Cardiovascular Pathology at the University of Padua, Italy.
The Agenda was as follow:
1. Crystal Tichnell - Co-ordinater and Genetic Counsellor: Johns Hopkins ARVD Program
The ARVD program was started in 1999 by the combined efforts of affected families and a team of cardiologists. The aim is to provide ARVD education, facilitate the management of ARVD patients and most important to provide new knowledge about ARVD.
2. Prof Hugh Calkins: ARVD overview
ARVD affects about 1 in every 5000 individuals in the general population.
Johns Hopkins focuses on:
1. Education. They have their Annual Conference, have printed a booklet called “Cardio what?” for kids and host a website www.arvd.com.
2. Patient Care: The ARVD program staff are available for consultations regarding diagnosis of ARVD. Screening, procedures and general testing is also done. Clinical genetic testing is also available for up to 7 ARVD genes. Patients from all over the world travel to Johns Hopkins for advice.
3. Research: Johns Hopkins has an ARVD registry with more than 1000 people enrolled. This is the basis of most of their studies. Their research projects include Genetic Investigations and a milestone was the discovery of the desmoglein-2 genetic mutation as a cause of some cases of ARVD. The Cardiac Desmosome is the structure that holds the heart muscle cells together – like the glue. They are also researching: living with an ICD, what causes ICD shocks, what happens at a cellular level in ARVD, improving diagnosing, ARVD progression and catheter ablation in ARVD. www.arvd.com
4. Prof Gaetano Thiene: ARVC/D Historical Perspective
Prof Thiene gave us a very interesting history on the development of ARVD diagnosis. In 1950 Dr. Osler first described ARVD and in 1961, the first case was identified. To date, huge strides have been made over the years in diagnosis. One focus area has been on “sport activities which increases the risk of sudden death because effort facilitates cardiac arrest in people bearing hidden cardiac defects," researcher Dr. Gaetano Thiene said. "Therefore, identification of concealed diseases should be mandatory in order to rule out affected patients from sport eligibility”. Screening of athletes in Italy is regulated by law and has proved effective in reducing the risk of sudden death in athletes.
5. Dr Boon Yew Tan: John Hopkins Genetic Research
Dr Boon Yew Tan updated us on the research done at Johns Hopkins. He stressed that genetic testing is very important and useful. He suggested that DNA banking is a good idea.
6. David Edwin PHD: Living with an ICD
Psychologist David Edwin has worked with many ICD patients. The first ICD was implanted in 1980 at Johns Hopkins. He explained that Psychologists and Psychiatrists have a crucial role to play in helping affected families cope as there are many aspects to consider.
Many people are affected by anxiousness, depression and demoralization. He reminded us that depression is treatable and that negativity is a symptom. We must also understand that individuals respond differently and that things that have happened to us, affect our behaviour. To avoid something may actually reinforce it like a certain action before a ICD discharge. He also emphasized that certain breathing techniques are a good distraction.
7. Prof Thiene: The role of the Pathologist
Being a Professor in pathology, Prof Thiene stressed the importance of investigating SCD and they have set out guidelines for autopsy investigation of SCD. He also mentioned that experimental pathology is important for finding solutions.
8. Prof Hugh Calkins: Recent developments in ARVD
Prof Calkins highlighted some recent developments: New diagnostic criteria for ARVD have been published by Marcus (2010), there is a new ECG approach and Desmosome mutations are receiving a lot of attention.
9. Patient experiences with ARVD
Before lunch, three patients told us about their ARVD experiences. Two ICD patients aged 16 and 43 and a heart transplant patient of 31, told their stories. This was most interesting and very encouraging to hear how successfully they have learnt to cope with their trauma.
10. Questions and answer session
After lunch a group discussion was held for the under 30’s while there was Q and A session with Prof Hugh Calkins and Prof Thiene in the main auditorium.
The discussions went on for hours and were very challenging and interesting. There were many common issues like: how to avoid a shock, how much exercise ,why do I get nightmares, at what age must I check my children, how often must I check them, someone commented on breathing techniques that help control arrhythmias, partners asked advice, genetics! And much more.
Prof Calkins suggested that acceptable exercise may be golf, archery, walking etc. In ARVD highly dynamic exercise must be avoided as it results in a right ventricle overload.
Prof Thiene stressed that removing anxiety, screening and medication were important issues that need to be dealt with.
Many of the patients and families belong to the yahoo-group: MMettera. They have some great posts with a lot of interesting information! Please go to the following address to join: http://health.groups.yahoo.com/group/mmettera/?prop=eupdate and another very useful site is www.arvd-arvc-info.com
The meeting in total created a feeling of great Camaradie and it almost felt as if one belonged to a great big caring family. Everyone was so friendly and positive and found great benefits from the meeting.
I left feeling thankful that much is being done to manage and find a cure for ARVD yet anxious that it all needs to happen faster. Ways need to be found to make living with an ICD easier and support needs to be easily accessible. A similar meeting in South Africa would definitely be of great value to everyone affected by ARVD.
Lusan Luscombe
CEO
PACE