What Brugada Syndrome patients should do to help prevent a cardiac event

A British organization, Cardiac Risk in the Young, was established in 1995 to raise awareness of conditions that can lead to young sudden cardiac death. In the video found on this page, posted earlier this year, a consulting physician does an excellent job explaining Brugada Syndrome. Near the end, he discusses some things that patients should be aware of and avoid, in order to lessen the probability of a cardiac event.

He mentions the family of tricyclic antidepressants as being dangerous to the Brugada Syndrome patient and also warns patients to monitor their body temperature, as fevers are associated with increased probability of having a cardiac event if one has Brugada Syndrome.

Most patients with Brugada Syndrome know that it's primarily a passive condition, with no symptoms. Other than the inconvenience of having and maintaining an ICD, the typical healthy patient leads a normal life. It's easy to forgot how serious this condition is!

So, the wise patient keeps up with the literature on the syndrome. Since Brugada Syndrome is so rare, progress seems to move at a snail's pace. But every once in a while, an article, video, interview or event is discovered to help patients learn more about it, and this helps them manage their own lives and feel more in control of their own health and futures.

 

Brugada Syndrome and show business?

Last evening on the television show, House, starring Hugh Laurie, Brugada Syndrome was suggested as a possible explanation for the collapse of this week's diagnostically challenging patient.  Once in a while there are references to Brugada Syndrome in movies or television.

Wes Craven

Wes Craven first came up with a basic idea for the movie Nightmare On Elm Street after reading about the mysterious deaths of some young, healthy Cambodian refugees who had come to America to escape the reign of Pol Pot. It's been well documented that this phenomenon, now known as Brugada Syndrome, is seen more frequently among the Asian population and statistically strikes young males in every population more often than females. I've never seen the movie but have seen Brugada Syndrome mentioned as one of Craven's influences often enough to believe this little bit of trivia is probably true.



If you'd like to see a movie starring Brugada Syndrome, this modeling of the presence of Brugada Syndrome, from Elizabeth Cherry, of Cornell University's College of Veterinary Medicine, is mesmerizing. But this video that models a Brugada Syndrome event through stimulation of the heart muscle from the left is hauntingly beautiful even as it represents a potentially dreadful outcome.

In With The New, Out With The Old

My husband had his Medtronic ICD removed last week, and a new St. Jude ICD called Fortify was implanted in its place.


Unfortunately just prior to surgery, the surgeon discovered my husband was in atrial fibrillation.  What was hoped to be a routine procedure turned into more. Now he has to be on blood thinners for six weeks, go through a tranesophageal echocardiogram to check for clots, be treated for the a-fib (a shock was mentioned) and then go back under to have the new ICD and his old lead tested so the surgeon can sign off on it.


The surgeon couldn't test the unit upon implantation, because he has no idea how long my husband has been in a-fib or what the clot situation is.

My husband's primary concern before the surgery was the lead - it had been recalled a few years ago but many cardiologists, including his, agreed it should stay in place. But since a new device was being installed, the surgeon was going to closely inspect the old lead and replace it if necessary.


There was another surprise - my husband's heart rate dropped to 30 when they disconnected the old ICD. He'll follow up with his regular cardiologist on all this next week.

In What Circumstances Should An ICD Be Removed?

My husband and son had their Medtronic ICDs implanted in the fall of 2004. In the meantime, both models had the leads recalled but on the recommendation of their health care providers, they opted not to have them replaced.

Now my husband's ICD battery is running low. His cardiologist tells him that he has to have the unit replaced within a few months. He doesn't want it replaced. He wants it removed, once and for all.

The ICD has never misfired. That's not the problem. It's twofold. First of all, my husband was diagnosed with Brugada Syndrome within a week of our son's diagnosis. While our son's diagnosis was strong based on many factors, my husband's was not. Brugada Syndrome is statistically more likely to strike males between the ages of 25 and 45. My husband is 60. And his VF was not as easily induced as our son's was during the EP study. Finally, I'm reading now that the earlier studies that determined a relationship between EP study results and the likelihood of future cardiac sudden death has been challenged through later studies. So perhaps if this had happened to my family in 2011 rather than 2004, he would not have been implanted with an ICD.

In addition, he just absolutely hates it. He complains constantly about the fact that he cannot sleep through the night because of the ICD. He awakens many mornings with great soreness at the side, because he's a lifelong left-side sleeper and he can't seem to break himself of the habit of rolling over onto that side during the night, only to be awakened by discomfort and fall fitfully back to sleep on his right side.

When he explained this to his cardiologist, he was basically told that he had no choice. The ICD had to be replaced. Period.

I guess I'm not exactly sure what to think. Is it truly that rare to remove the ICD? What happens to the leads if the ICD is removed? It probably doesn't make sense to remove them, since by this time I'm sure they've been embedded into the surrounding tissue by now.

One Mother's Story About Brugada Syndrome

There is a temptation to focus on the technical advances that help protect individuals with Brugada Syndrome from experiencing sudden cardiac death. New types of ICDs, studies about quinine or quinidine treatments as an alternative to ICD implantation in certain cases and so on.

What we can't forget is that Brugada Syndrome is a real disease with terrifyingly real consequences for people who are struck down before they are diagnosed. Since Brugada Syndrome was identified in recent years, the medical community is not completely up to speed on the condition, within the United States and throughout the world.

Consider the heartbreaking story told by this mum in the United Kingdom, and her years-long struggle to confirm the diagnosis of Brugada Syndrome for her young son. From the National Genetics Education and Development Centre.

Twiddler Syndrome - No Fooling

I'm serious. Such a condition exists and it has to do with ICDs. Wait for it...Twiddler Syndrome is caused by the patient twiddling with his or her newly implanted ICD to the point where the leads dislodge, rendering the ICD ineffective.

You can read more about Twiddler Syndrome and other conditions that might be encountered by an Emergency Technician here in Emergency Medicine.

Xray photo from article.

Medtronic introduces Protecta line of ICDs in United States

Found this posting on medgadget blog, describing a new ICD by Medtronic. Introduced in Europe in summer of 2010 it is now cleared for use in the United States.

The Protecta line of ICDs should allow patients who fear inappropriate shocks to breathe a sigh of relief. "Findings from the Virtual ICD study, based on a statistical model, shows that 98 percent of patients with SmartShock Technology will be free of inappropriate shocks one year after implant and 92 percent will be free of inappropriate shocks five years after implant."

Might be worth a discussion with your health care team if your ICD, like my son's and my husband's, is near replacement time!

ICD Wearers: Do you realize how valuable you really are? Technically, I mean...

This article was posted on Facebook today.

It's entitled, "Oops...ICDs Don't Bounce" and is a rather unique look at the value of that little box of magic called an ICD.

(The doctor's blog is worth checking out, by the way!)

Are You Kidding Me? A Leadless Pacemaker by Medtronics?

I visited this great blog and poked around. If you have an ICD, check it out, and also read about this new gadget by Medtronics, information about it was posted on October 29, 2010.


Here is TED's update about this promising alternative to the current device and lead system.

Article about Brugada Syndrome For Nursing Students Works

Leslie Foran Lee and Nancy Felmlee, staff development specialists in clinical education and research at Virtua Health, Mt. Laurel, New Jersey, provide an excellent overview of Brugada Syndrome in this article, published this past spring in Nursing 2010, a peer reviewed journal.

While the article covers the history of Brugada Syndrome and the distinctive features of the disease, it also addresses the practical knowledge that hands-on medical professionals must know.

For example, after a thorough section on the key elements in recognizing Brugada Syndrome, they suggest how to slightly adjust the leads on an ECG reading of a patient with symptoms that may point to Brugada Syndrome, so as "to capture a complete right bundle-branch block or type 1 ECG pattern".

A section entitled, "On The Alert" reminds readers that, now knowing about Brugada Syndrome and its distinctive features, they should be diligent about taking patient histories, probing about symptoms, and helping ensure that Brugada patients get the appropriate care and follow up.

A reassuring read for the Brugada Syndrome patient, or the professional health care provider.

Students Create Informative Presentation on Brugada Syndrome

Check out this video, less than 7 minutes long, in which two high school students, Alburuj Rahman and Yared Selemon, do a nice job describing the basics of Brugada Syndrome. Watch this video - their "Mastery Project for Biomedical Engineering of the Bodies Program about a cardiovascular disease."

I especially appreciated their detailed explanation of the polarization phases of the heart beat, and what happens differently when Brugada Syndrome is involved.

Nice job!

Have you ever watched an ICD at work in a real life situation?

Interesting video on YouTube of a Belgian soccer player, with an ICD, experiencing sudden cardiac death on the field.

This cardiologist blogs about it, and posts a follow up video featuring an interview with the 20-year-old footballer, who is just fine now.

NEW STUDY QUESTIONS ICD IN ASYMPTOMATIC BRUGADA SYNDROME CASES

A report posted on February 3 of this year, involving 1029 Brudaga Syndrome patients followed for nearly three years, suggests that perhaps the 2005 guidelines for treating asymptomatic patients might need to be revised.

While maintaining a tentative tone, the study indicates that only 0.5% of asymptomatic patients (no history of sudden cardiac death episodes or syncope) experienced any cardiac events during a 31 month period. Currently certain conditions such as sudden cardiac death among immediate relatives, or inducibile ventricular tachyarrhythmias during E.P. studies warrant treatment with an implantable defibrillator(ICD).

Future studies are recommended.

DISCOVERED A NEW (TO ME) WEBSITE

Here is a website of interest to Brugada Syndrome patients and, more specifically, their physicians.

"BrugadaDrugs.org has been initiated by the University of Amsterdam Academic Medical Center, department of Cardiology, to aid physicians who treat patients with Brugada Syndrome."

Check it out. It is worth forwarding to your cardiologist.

Boston Scientific has informed doctors of a potential problem with their Cognis or Teligen defibrillators. Some patients (15) have been delivered inappropriate shocks - a very small number of the 8,000 who might be affected.

I read about this in the morning newspaper. Further investigation led me to an AP news release dated March 27.

There is no mention of this release on Boston Scientific's own website. They have a media page of information and news releases. Their press release the previous day, March 26, gave the schedule of major announcements at the March 28-31 58th Annual Scientific Session of the American College of Cardiology, and their press release the following day, March 29, was entitled SYNTAX Analysis Finds Treatment With TAXUS® Express2™ Stents More Cost Effective Than Bypass Surgery in Many Patients With Complex Coronary Artery Disease.

Their News Release page states that "Boston Scientific Corporation is not obligated to update the press releases and information contained in this section of the Site."

Boston Scientific purchased Guidant in 2006, and Cognis and Teligen are the first defibrillators launched under its own name.

Boston Scientific, one of the leaders in the medical device field, might think about the impact of allowing AP to share their news that will create anxiety, and saving their own website just for their good news.

If you don't manage your own reputation, others will manage it for you.

Brugada Syndrome and Prostate Cancer

This attention-getting title is partially explained in an abstract on a blog that posts about arrhythmias. Males with Brugada Syndrome are at higher risk for prostate cancer, even when other elements are factored in (age, smoking, etc).

As always, use caution when reading any online information about anything. In this particular abstract, I do see at least one familiar name, Dr. Charles Antzelevitch, who is Director of Research at the Masonic Medical Research Laboratory. So I would predict that this is probably a solid study.

If you are concerned, contact the authors or researchers that are listed following the article.

Online Article about Brugada Syndrome

I like to search the web for new or interesting information about Brugada Syndrome.

Here is a recent article that gives a good overview of the disease. It appeared in an online blog named maintained by the Faculty of Medicine at Odlar University located in Baku, Azerbaijan.

One symptom that I had not known before has to do with disrupted sleeping. The article mentions nightmares and thrashing during the night in some Brugada Syndrome patients. I know the cardiac event happens most frequently when a person is at rest, but wonder what, if any, relationship there is between the disrupted sleep and cardiac arrest from Brugada Syndrome. In other words, is the actual syndrome associated with the nightmares or thrashing, or are those symptoms independent of the disease?

Health Insurance



Dave (on the right; his dad is on the left) is about to graduate from college this spring. He has decided to take a year off and then attend graduate school to study mathematics. During that time, he'll have a job that he loves, working in a cafe near campus. He may even work full time, and be able to cover all living expenses plus the student loans that will come due in a few months.

What he will lose is health insurance. He's currently covered under our (his parents') plan, but as soon as he graduates, that will end his eligibility.

Dave can't really afford to be without health insurance. He needs to be under the continuing care of his cardiologist, and to continue to receive his meds.

We're looking into options that our state offers for the "gap" 20-somethings that fall into this category. Our daughter simply went without health insurance for 16 months, until she enrolled in graduate school and was eligible for their coverage.

Health insurance has become prohibitively expensive, and we already pay the full cost of our own health insurance and simply can't afford to pay for a new policy to cover him as well.

Medtronic recall - for those of you who have Medtronic ICDs, please read about the lead they are recalling. Check with your doctor if you believe you may have the lead that is causing concern. Medtronic, as usual, is forthcoming about the recall and being proactive with information.

Of course this is something to investigate, but to put it into some perspective,"If somebody is at risk of sudden death and they have an event, and they don't have a defibrillator, their mortality is 95%," Lindsay told Dow Jones Newswires. There is at least a 99% chance, meantime, that defibrillators will function properly when called upon to help such patients, he said, as quoted in CNN Money.com yesterday.

My son and husband are doing very well. I haven't updated the blog in a while because they have not had any problems or events, and I really haven't learned much new about Brugada Syndrome. Occasionally I read an interesting blog, usually written by a medical student. But I haven't seen new research published that points to anything of major significance.

I was very saddened to learn that a beloved member of the Brugada family, their sister Dolors, died suddenly at the age of 50 of sudden cardiac death. It is a cruel irony that the very condition these men are dedicating their careers to study, sudden cardiac death, should take one of their own. My deepest sympathy goes out to them, especially Ramon, whom I have the privilege of knowing.

Several months ago I attended a dinner dance in my town to raise money for the American Heart Association and awareness for Women's Heart Health. It was a beautiful event! The ballroom theme was elegantly black and red; the dress was formal or semi-formal. Small tables and chairs were arranged here and there, and a fabulous band played in the background as people mingled. The highlight of the evening was a fashion show, and local celebrities paraded down the runway in their dressiest red and black. From tuxedos to elaborate costumes, it was all there! Everyone in the fashion show had a personal, intimate connection to heart disease.

The event was organized by my good friend, Gwen Webber-McLeod, who lost a young beautiful sister to heart disease just a few years ago. A compelling and dynamic speaker, Gwen gave a riveting tribute to her sister.

I just want to share a photo of one of the fashion plates' heads (above, he kindly allowed his chrome to be painted with the evening's theme logo). His name is Jim, and he has an implanted defibrillator.

This November I hope that I can persuade my son and husband to stroll down the runway in their snazziest suits.