Brugada Syndrome and children

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Brugada Syndrome and children

Postby markhop » Tue Nov 27, 2007 12:16 am

Hi, I'm a 29-year-old from the UK, I was diagnosed with Brugada Syndrome in 2002 and had an ICD implanted soon after.

I'm now about to become a father and am finding it difficult to get information from my doctors as to what to expect, so I'm hoping someone might be able to help me with a few questions.

Firstly, what are the procedures of diagnosis I can expect my child to go through? And how easy is it to reach a definitive diagnosis either way?

Also, does anyone know what the percentage chance of transmission of the disease are? I have heard conflicting things about this.

Basically, anyone who has Brugada Syndrome and has subsequently had children, I would very much like to hear of your experiences.

Many thanks,

Mark.
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Postby kitsunegari » Tue Nov 27, 2007 9:42 am

Mark, I was diagnosed and had an ICD fitted in 1998. I got married two years later and have had two children (girl 5 +boy 3).

I found that the procedures in diagnosing children were very vague.
Just after my daughter was born, I had a 6 month check up and asked about testing my little girl, I was verbally told (later followed up by a letter) that diagnosis in children was hard to determine and testing could be dangerous and that as the condition rarely affected children it would be best to wait until they reached their teens.

However, one of the first recorded Brugada cases involved a young child, so earlier this year, after reading posts on this site I contacted my children’s Dr, explained the situation and he referred us to our local hospital. The local hospital's cardiac unit checked the children’s blood pressure and gave them ECG’s. They found nothing but then referred us to the Bristol Royal Infirmary for a more thorough check. We met a Dr who specialised in heart conditions in children and he suggested we had the children tested with flecinide.
My children were given flecainide and the results showed no sign of Brugada. My children are now going to be tested every couple of years (I assume because flecinade is not 100% reliable).

I have never showed any signs of having Brugada. In 1998 my father suffered and survived a Brugada attack. My brother and I were tested with flecainide, he was ok but as I showed signs of having the condition I had an ICD fitted as a precautionary measure. I have been told there is a 50% chance that I could pass the gene onto my children.

My advice would be to keep asking your Dr’s what they will do when you child is born. If you want I could dig out the name of the heart specialist we saw in Bristol – he may be able to give you some advice on what to do to get your baby tested?
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Postby markhop » Tue Nov 27, 2007 4:53 pm

thanks a lot for your post. I've emailed Dr.Ramon Brugada and he says there may be a possibility of 'genetic testing' to try and establish a diagnosis. Do you know anything about this?

thanks,

Mark,
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Postby kitsunegari » Wed Nov 28, 2007 8:56 am

I brought this up at my last check up, they said it was a possibility but i got the impression they weren't keen. The best option would be to go private but obvioulsy that will cost.
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Postby Sam'sMom » Sat Jan 26, 2008 10:08 pm

I have a 15 month-old with Brugada and he was diagnosed at almost 11 months. There is a 12-lead ECG that is easily performed and when they move two of the leads up, it will show if a person has Brugada. That is how my son was officially diagnosed, but he also has had the genetic testing.

I assume you are a father by now? Our cardiologist said that the youngest baby ever diagnosed was two days old.
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Postby markhop » Tue Jan 29, 2008 8:40 pm

Thanks very much for posting your reply, Sam's mom. Yes, I am a Dad now, we had a girl on January 12th. An initial ECG was normal and cardiologists think that nothing should be done now until the next ECG in 6 months time. You seem to be saying that a certain kind of ECG should definitely be able to establish a diagnosis one way or the other, but from what I've been told, ECGs can never establish a negative diagnosis for sure. They say "Ajamalin" (I'm not sure what that is) testing won't be suitable until about 2 years and also they wouldn't put in an ICD until about 2 years old, if she does have Brugada. We are also being followed up by geneticists at Guy's Hospital. If you read this, could you answer a couple of questions:

- Is your 15-month old having an ICD fitted yet, if not, when do they think is the right age to do so?
- Did the genetic testing establish a definite diagnosis? Or was it just a pointer towards the right diagnosis? My partner and myself will have a consultation with a geneticist soon, but I'm not sure what I can expect going down the genetic route. I'm a bit confused about the various ways of trying to establish a firm diagnosis.

Thanks,
Mark.
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Postby Delonghi » Wed Jan 30, 2008 4:13 pm

Mark
Congratulations on the birth of your daughter, I hope you are enjoying your new born and not worrying about Brugada all the time. I was diagnosed in 2006 and had my ICD fitted shortly after. I have two children aged 3 and 8. I guess like you my overriding concern is for them. They get tested every year and have not shown any signs yet. However as you say the ECG alone is not conclusive and they are talking about doing the ajmaline test on my 8 year old next year. We are having genetic tests done through Dr Brugada and through our hospital in the Uk. If you would like to know more about our experience or if you think I can help you at all please feel free to email me at anndelany@tesco.net.
If its any reassuranve at all my Consultant is confident that reserach is making leaps and bounds of progress and it won't be long before we know so much more about this syndrome.
Regards
Ann
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Postby Sam'sMom » Fri Feb 01, 2008 1:30 pm

Mark, congratulations on the birth of your daughter! I hope you're getting some sleep.

Oh yes, you're right, the ECG pointed to a positive diagnoses. Sam had an ICD fitted when he was just 11 months old. It sits in his belly, since that is the only open spot for it. It has already saved his life four times - as recently as last week, when it shocked him three times in one day. He has a terrible time with fever, so anything above 102 and he goes into VTAC. One of the times it shocked him (on December 12), he had 103 degree F and his heart went to 353 bpm. You can learn more about him and our journey on our blog - klearlife.blogspot.com

The way that Sam's brugada revealed itself to us was through two febrile seizures when he was 10 months old. Apparently they couldn't get a pulse ox and took an ECG after the second febrile seizure, which showed he was in VTAC. I read some papers on Brugada Syndrome "masking" as febrile seizures. So, if your daughter ever has more than one febrile seizure, this might be an indication.
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Postby Sam'sMom » Fri Feb 01, 2008 1:32 pm

I forgot to add - when you look at our blog - check out 2007, September. That gives the most detailed version of how we discovered what happened.

Also, the genetic testing pointed to a definite diagnosis in Sam. They learned that he does not have the most common form of Brugada, but instead he has one they have never seen before.
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Postby Momof2HeartKids » Thu Feb 14, 2008 5:17 am

Mark,
Congratulations on your baby girl!!!! :D

To answer some of your questions, the consensus on the actual testing they will do for your daughter is that every doctor seems to do it differently. The norm is periodic ekgs but as you correctly stated a negative ekg does not mean a person dosent have it. Ajamalin testing is one of several medications, sodium channel blockers, they can use to see how you react to it as it will usually make a brugada patients arrythmias show. In our family those who tested positive genetically then had a procanamide challange done during eps studies to help determine their risk of having an event. Some doctors dont want to do this because it is invasive and is associated with its own risk.

Have you ever had genetic testing done? This is the easiest way to see if your daughter is carrying the gene, the problem is these tests are expensive and it can take time for them to find where the mutation lies. It is in the SCN5A gene but not always the same location, but once the defect is located it is much easier for subsequent testing in other family members.

As far as age to be fitted for an ICD, my son was a small 5 year old, my daughter was 15. My sons is in his abdomen, my daughters is fitted like an adults. Theproblem is these things werent made for little bodies so placement becomes difficult.
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Re: Brugada Syndrome and children

Postby c_ilai » Sun Mar 23, 2008 10:16 am

Hi All,
I am new to Brugada. I had a sudden death which discovered the syndrome.
I am also going to be a new father soon.
When and If the children are found with the syndrome, I understand it takes a while untill they can have an ICD.
My question is this, Are they receiving any other treatment? (Quinidine?)

Thanks,
Ilai
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Re: Brugada Syndrome and children

Postby kynetia » Tue Mar 25, 2008 1:03 am

hello everybody.
I will just answer some of the questions briefly, since I have posted my experience already in a different thread.

1. if a child has brugada but is too young for an ICD, does he get medication instead?
I was told that no medication is reliable and if a child - or even a baby - is highly at risk he/she will have an ICD implanted regardless age.

2. how to diagnose the syndrome for sure?
ECG's can give us a hint that there may be something wrong wit the heart's behavior. But ecg's of babies and young children do not seem to be reliable...
Invasive tests performed in association with specific drugs (like flecainide) are used to test the patient's heart, to evaluate "how risky" its condition is.
As far as I know, they are usually performed once the syndrome is more than "suspected": normally they do this kind of tests to adults whose ECG's always show the typical Brugada abnormalities, or to young patients ho have been experiencing fibrillations.
The "safe" way to determine if a patient has he brugada syndrome or not is the genetic study.
All it takes is a specimen of blood and a few months time, necessary to spot the mutation.
This kind of testing is performed 100% for free in Italy ... Dunno about different countries!

ciao
kynetia
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Re: Brugada Syndrome and children

Postby scottsmommyxo » Sun Mar 21, 2010 8:09 pm

my names samantha my son scott was diagnosed at 10 months old through an ekg & had an icd implanted right away. hes also on 2 heart medications one to keep his heart low (propranolol) and one for his sodium channels (quinidine) in the ep lab they could not induce a heart attack the first time. second time they could. hes been shocked over 275 times and has come to the point of them telling us they didnt know if he was going to make it. unlike most brugada children he dosnt get shocked once by his icd and its over. hes shocked multiple times over several hours. and according to pedro brugada hes the worst case they have ever seen or even heard of. all u need is an ekg to diagnose it and the sooner the better. hope that helps a little.
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Re: Brugada Syndrome and children

Postby TeesaTeesa » Mon Mar 22, 2010 1:46 am

Hi Samantha! We have all heard of you here. My son was also implanted with an ICD the day he turned 11 months old. He was shocked only 40-50 times immediately, but I know how scary that can be. Our cardiologist recently talked to us about your story and said he wrote your doctor through the special channels and told him our story too. I would love an update about what's happened. How is he tolerating the meds? Is he still going into arrhythmias?
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