looking for info on CPVT and ICD

This is a forum for parents and kids with ICDs. Discuss issues unique to parents and kids, or just hang out with some newfound friends!

Moderator: Momof2HeartKids

zmom
Posts: 120
Joined: January 11th, 2010, 6:32 pm
Location: Ohio, USA

looking for info on CPVT and ICD

Post by zmom » January 14th, 2010, 9:28 am

Just joined this site yesterday and I'm hoping I'm post properly. I believe I posted an intro on the main forum?!? but think this is the forum I should be at. If I need to repost my intro for this forum, would someone let me know how to do that?!?

A short overview... my son has unconfirmed CPVT. Genetic testing at the Mayo Clinic is still ongoing. In 2007 at the age of 13, he suffered cardiac arrest and was implanted with ICD. We're researching CPVT since his cardiologist said "if it walks like a duck, quacks like a duck then it must be a duck". All his symptons indicate CPVT and it is possible to have CPVT and not test positive. Therefore, his cardiologist diagnosed his condition as CPVT.

Is anyone familiar with this condition?
Janet "ZMom"
Mother of 16 yr old son with
St Jude Medical V168 ICD and
Ventricular Tachycardia (VT) with CPVT
Zack's motto:
"Making mistakes simply means you are learning faster"

jdm111
Posts: 8
Joined: January 10th, 2010, 9:01 pm

Re: looking for info on CPVT and ICD

Post by jdm111 » January 14th, 2010, 8:17 pm

Hi zmom,

My son is 6 and also is suspected with CPVT (one doctor said classic case of R2Y2 mutation). He currently is having the genetic test processed at PGxHealth. We are going to have an ICD implanted soon, probably through U of M Mott....I would assume your pediatric cardiologist is an electrophysiologist? (specializes in rhythm disorders)...if no, I would suggest you find one....what your son potentially (probably) has is a very serious, very rare condition (estimated 1 in 10,000)....

What Rx do they have him on? Garrett is on 10mg of Propranolol 3 times daily...that isn't enough though, and they can't increase the dose, hence the ICD......

Information is tough because it is very rare, hence the need for the specialist

I am new to all of this in Oct 2009 but I am educating myself as much as possible

You may know just as much as I do, but I would be happy to talk if you like. email = smk0771@yahoo.com. If you want to email me your number I will call you when I can.

zmom
Posts: 120
Joined: January 11th, 2010, 6:32 pm
Location: Ohio, USA

Re: looking for info on CPVT and ICD

Post by zmom » January 15th, 2010, 1:26 pm

Yes, I'll be in touch but here's a copy of my intro. My son has a St Jude ICD since 2007. He was 13 years old when he had a cardiac arrest at school. When he was 8 years old he had a fainting episode during gym class and again during recess. After numerous tests he was diagnosised with "syncope". It wasn't until age 10 that he was diagnosised with VT. This came about after a fainting episode during swim class. We finally found a cardiologist to perform a stress test to appease his mother. The stress test indicated ventricular tachycardia and he started taking Atenolol (beta blocker). Future testing by a holter monitor continued to show PVCs. The doctor suggested genetic testing at the Mayo Clinic for CPVT (catecholamine polymorphic ventricular tachycardia), which we were told could take several years to get the results. In October 2007, we took Zack to an EP doctor to see if ablation would fix his VT. He was put on a calcium channel blocker 5 days later he suffered cardiac arrest. His science teacher performed CPR until the school's nurse arrived with an AED. When the ambulance arrived for transport he aspirated his lunch into his lungs. At the hospital he struggled to survive since he developed aspirated pneumonia along with his heart condition. He became a "miracle" and once his condition stabilized the EP doctor did a study and found that epinephrine caused his heart to go into VF. He implanted an ICD/pacemaker and prescribed 100mg Toprol XL. He was diagnosised with CPVT even though the results from Mayo Clinic had not come back yet. His cardiologist wanted to perform a stress for follow-up but Zack developed stenosis (scar tissue in his trachea from the ventilator tube) which closed his trachea 75%. He had numerous dilatation surgeries to widen the opening until in 2008 it finally stabilized. Two stress tests continue to show PVCs and couplets. He now takes Toprol XL 100 at 7 am and noon but still has PVCs and couplets when his heart rate reaches 105-107. Activities are restricted and he has had a hard time dealing with it especially when peers don't understand his condition. He looks strong and healthy on the outside other kids just don't understand. Need advice on adjusting!
Janet "ZMom"
Mother of 16 yr old son with
St Jude Medical V168 ICD and
Ventricular Tachycardia (VT) with CPVT
Zack's motto:
"Making mistakes simply means you are learning faster"

User avatar
Momof2HeartKids
Forum Moderator
Posts: 1062
Joined: October 27th, 2005, 11:12 am
Location: CT

Re: looking for info on CPVT and ICD

Post by Momof2HeartKids » January 20th, 2010, 5:53 pm

Hi Zmom - Wheew! Thank God for the quick response at your sons school! Zack is a miracle and its stories like yours that make me continue to work hard to educate the public!I just got back from a 4 day weekend event in which we reaised money for various organizations one of which is our efforts to get AEDs put in all schools.

I have a few suggestions for you in terms of reaching out for support during this difficult period of adjustment. First - here! - you came to the best place for support on living with an ICD... although there arent many of us parents of children with ICD we are a growing group and the adults on here can really relate and really "get it". The other is Parent Heart Watch, this is an organization I belong to as well that is comprised of parents who have lost a child to cardiac arrest and parents or suvivors. You can meet lots of people there who have similar stories to share. The other is SADS, Sudden Arrythmia Death Syndrome Foundation. They are a great resource for CPVT and other SADS conditions and if they cant answer your questions they are great at pointing you in the right direction to someone who can.

The short version of our story... I have an ICD for Brugada, Long QT and I have Sick Sinus Syndrome. My son has Brugada and Long QT, he had his ICD placed when he was 5 (removed last year due to staph infection, have to wait for it to be placed again until he is older) and my daughter has Brugada, had a 'plumbing issue' and successfully had her PVCs and VT ablated at 12, and had her ICD placed at 15. She has an ICD for the Brugada because the VT that was ablated is different from that of Brugada.

Welcome to the group!! wavhi Please take my private email cyntodb@comcast.net if you ever want to chat. I will keep you and your son in my prayers!


howdywelcome
Wishing You a Zap Free Day,
Cyndie


(Brugada Syndrome, Long QT Syndrome, Sick Sinus Syndrome, ICD & 2 children one with Brugada, ICD and one with Brugada, Long QT and removed ICD due to staph)

Nyone
Posts: 21
Joined: October 28th, 2005, 6:43 pm
Location: Bothell, WA

Re: looking for info on CPVT and ICD

Post by Nyone » January 26th, 2010, 3:57 pm

Hi jdm111 and zmom,
I had been diagnosed with long qt syndrome for 4 years but doubted the diagnosis and finally saw Michael Ackerman at the Mayo clinic for a 3rd opinion. Coincidentally (or more likely by a higher power) 2 days before my appointment, the Mayo got a hit on my blood I had sent in 2 years before for a new mutation in the Ryanodine receptor area. Because it is a new mutation, they can't be 100% positive that I have CPVT, but because of my episodes of syncope and ventricular fibrillation, it's a good guess that I do have it. Unfortunately, my stress tests do not show much of a problem and I have never had an episode with exertion, so it's still confusing to me. I have a 13 year old daughter that also tested positive with the same mutation at the Mayo, but has never had any problems with her yearly stress tests (since age 8) and never any syncopal episodes. Both she and I are on nadolol. I have an ICD implanted and we carry around an AED whereever my daughter participates in sports.
If you're unsure about the diagnosis of your kids, I highly recommend Dr Ackerman (who is a pediatric EP) at the Mayo.
Hope all goes well for your. I constantly wrestle with the fear of something happening to my kids and smothering their childhood with restrictions, but I'm fortunate they/she is not symptomatic.

zmom
Posts: 120
Joined: January 11th, 2010, 6:32 pm
Location: Ohio, USA

Re: looking for info on CPVT and ICD

Post by zmom » January 26th, 2010, 9:26 pm

Dr. Ackerman seems to be the doctor. Our cardiologist considers him the "pope" on CPVT. We never had an appointment with him, but Zack's blood work was sent for genetic testing in January, 2007. To date, it's been negative, though they said they plan to continue testing and doing research. I've spoken with Dr. Ackerman's office several times. They seem helpful but tell us that CPVT is hard to define.

Do the doctors feel that your stress tests are managed with medicine? Has an EP study shown signs of CPVT?

We have a daughter turning 13 on Feb 1st. They don't seem to want to do a genetic test on her unless her brother's testing was positive. She did have a stress test in 2008 but shows no signs or problems. Also, they don't want to do an EP study on her unless a stress test shows symptoms.

We're still praying for answers and results. We'd feel better knowing CPVT is confirmed throught the genetic testing. Our families have no history of sudden unexplained death, so we're wondering where it's coming from and if our daughter has it.

Thanks for letting us know about your family, and we'll be adding you to our prayers.
Janet "ZMom"
Mother of 16 yr old son with
St Jude Medical V168 ICD and
Ventricular Tachycardia (VT) with CPVT
Zack's motto:
"Making mistakes simply means you are learning faster"

jdm111
Posts: 8
Joined: January 10th, 2010, 9:01 pm

Re: looking for info on CPVT and ICD

Post by jdm111 » January 31st, 2010, 12:37 pm

Hi All,

Garrett had his ICD placed on January 21st. Unfortunately on the 1 week follow up, they found fluid accumulating around his heart. Turns out the lead had poked a hole in the pericardial sac due to its placement. They ultimately had to put a brand new lead in, which was done on the 28th. Coming home today. Seems to be doing well.

Oh, and it is official. Positive mutation on the R2Y2 gene

Nyone
Posts: 21
Joined: October 28th, 2005, 6:43 pm
Location: Bothell, WA

Re: looking for info on CPVT and ICD

Post by Nyone » January 31st, 2010, 3:12 pm

Wow jdm, I'm glad they caught the perforation early. Mine perforated about 3 months after implant (which had been unusual because like Garrett, this usually happens withing the first 6 weeks. They are making leads so thin that it's happening more often later and I think St Jude changed their design of the lead to try to prevent this (Riata lead)), and I ended up in cardiac tamponade, a life-threatening situation. I

Zmom, even when my heart rate would go >150s, which is usually when people start having PVCs with CPVT, it wouldn't show up on a stress test, so no, it wasn't the beta blockers blunting my stress test results. And I don't think they use EP studies to diagnose CPVT. My EP study just found a benign AVNRT (reentrant tachycardia) that wasn't necessarily related to my CPVT problems. They did ablate it so it wouldn't set off my ICD.

zmom
Posts: 120
Joined: January 11th, 2010, 6:32 pm
Location: Ohio, USA

Re: looking for info on CPVT and ICD

Post by zmom » February 1st, 2010, 8:13 am

jdm111, posting.php?mode=reply&f=2&t=5890&sid=3 ... 5a1306c#It was good to hear from you, thanks for the update. posting.php?mode=reply&f=2&t=5890&sid=3 ... 5a1306c#So sorry to hear about the complications and hope that Garrett will soon be up and running. Thanks to kids like Garrett and Zack, I believe the doctors' awareness of CPVT will soon lead to better treatment. Since my son's diagnosis in 2007, the advancement in treatment has come a long way. So far as long as Zack takes his medicine he's been shock free. It did take some time to tweek the right dosage. Zack's triggers are exercise (of course) but stress and emotion (any activity that produces adrenaline) can cause accelerated heart rate. He also needs to be extremely careful in taking over the counter medicine. I also check with his doctor first before giving him new medicine (even what other drs prescribe ie orthodonist wanted Zack to take something that our family doctor said no way to). In the meantime, take things slow and easy. We'll be thinking of you and keep us posted.
Janet "ZMom"
Mother of 16 yr old son with
St Jude Medical V168 ICD and
Ventricular Tachycardia (VT) with CPVT
Zack's motto:
"Making mistakes simply means you are learning faster"

zmom
Posts: 120
Joined: January 11th, 2010, 6:32 pm
Location: Ohio, USA

Re: looking for info on CPVT and ICD

Post by zmom » February 1st, 2010, 8:19 am

Just looked at my above post. Smilies didn't work. Anyone know what I did wrong?!?

For the record jdm111.. one was "A Big Hug, Just For You"
Janet "ZMom"
Mother of 16 yr old son with
St Jude Medical V168 ICD and
Ventricular Tachycardia (VT) with CPVT
Zack's motto:
"Making mistakes simply means you are learning faster"

User avatar
freckles1880
Moderator
Posts: 8664
Joined: April 18th, 2009, 7:19 pm
Location: Broomfield, Colorado

Re: looking for info on CPVT and ICD

Post by freckles1880 » February 1st, 2010, 9:37 am

zmom wrote:Just looked at my above post. Smilies didn't work. Anyone know what I did wrong?!?

For the record jdm111.. one was "A Big Hug, Just For You"
While you are in the "post a reply" mode go the left where you see the faces and just click on one. It shows by its "wavhi" name as you wright but as the icon after you submit the post.
Bob

Medtronic-Visia AF implanted 7-8-2016 stayed with the with 6947 Sprint Quattro Secure lead. Original ICD implant 2-4-2009. ICD turned off 10-6-17 as stage 4 lung cancer taking over.
Major heart attack, carcinogenic shock and quad bypass 10-13-08 post myocardial infarction, old inferior MI complicated by shock and CHF, combined, Atherosclerosis, abdominal aortic Aneurysm, Seroma 7 cm, left leg. Stent in the left main vein 10-7-2014

My "Wardens" are my bride of 54+ years and my daughters.

zmom
Posts: 120
Joined: January 11th, 2010, 6:32 pm
Location: Ohio, USA

Re: looking for info on CPVT and ICD

Post by zmom » February 1st, 2010, 11:27 am

:balloons:
Thank you freckles1880. My problem is solved. I was right clicking to copy into the text. Glad you straighten me out. dancee
Janet "ZMom"
Mother of 16 yr old son with
St Jude Medical V168 ICD and
Ventricular Tachycardia (VT) with CPVT
Zack's motto:
"Making mistakes simply means you are learning faster"

joshmyboy
Posts: 2
Joined: July 3rd, 2009, 1:36 pm
Location: Hockessin DE

Re: looking for info on CPVT and ICD

Post by joshmyboy » March 3rd, 2010, 11:31 am

I hope I am posting to the right place; so confused on this site. I have a 13 year old son, Josh, with what is assumed to be CPVT. We are lucky, to date, he is symptom free. So, at this point he does not have an ICD. If he does become symptomatic, one will be implanted. Hence why I am looking for what "moms" have to say about ICD's and Beta blockers in their kids. We found the condition by accident after we were referred to cardo. doc due to chest pain. At first, this chest pain was not a driving force to my ped. doc. because we were testing Josh for Epstein bar due to multiple viral symptoms and other complaints like being tired all the time, having chest pain while he was on the ice, and feeling short of breath (he was a hockey player on the ice 5 days a week, it top condition). When the doctors office called me and told me the test was negative I again questioned the chest pain. Only then did I get a referral (required by medical insurance) to see the cardo. doc. We are about 15 months into this diagnosis and have gone through multiple stress tests, ekg's, echo's and genetic testing, which came back negative for ryr2 gene. He is currently on 30mg daily of nadolol (beta blocker). He is not allowed to participate in any competitive sports and has restrictions when it comes to anything sports and even gym class. To me, This medication makes him tired and he has lost all his get up and go. It got up and went! His grades in school have gone down hill as well. The EP doctor thinks this is because he is depressed not the medication. Hence, he will be starting mental therapy this month. He has friends, laughs, is a manager for his school wrestling team, and has not withdrawn from the family. His new sport is " on line Gaming" in which he talks to school friends, old hockey friends as well as meet new friends. I take him once a week to a personal trainer who works with him while monitoring his heart rate. He loves this and we have doctor approval as well. Has anyone noticed a change in their child's grades and "get up and go" as a result of their beta blockers? I feel so lucky to have found Josh's condition before anything happened and I thank G-d everyday he is with us. Look forward to hearing from you. Beth

zmom
Posts: 120
Joined: January 11th, 2010, 6:32 pm
Location: Ohio, USA

Re: looking for info on CPVT and ICD

Post by zmom » March 3rd, 2010, 12:21 pm

Beth,
Know where you are coming from. We're dealing with the same conditions. Zack just wants to have a "normal" teenage life. Unfortunately, teenage life seems to center around sports and when you are restricted from participating it becomes hard to deal with. It's especially hard to join in with the active kids. Zack (age 15) has also started online gaming, though we do set limits on it. His doctor finally gave the ok to participate in golf. We started lessons last year and was able to join the golf team as a freshman. Of course, at our school golf is not considered a major sport (ie like basketball and soccer), but it has given Zack some comfort. It's required in high school to complete gym class. So far we're fortunate to have a gym teacher who allows Zack to participate on a revised schedule (no running, only walking, no stenuous activities, no activities that will damage his ICD). He's been allowed modified stretching (ie class has to do 50 sit ups in so many minutes, Zack can participate at a slower pace with rest in between). This has been an improvement to Zack's emotional state, but, of course, there are still peers who do not understand...that's the hardest part. Hope counseling will help your son because it seems each new stage of growing up brings new challenges. Has your son seen an electrolphysicologist doctor or had an EP study done? Hope to share more information, just get in touch with us wavhi
Janet "ZMom"
Mother of 16 yr old son with
St Jude Medical V168 ICD and
Ventricular Tachycardia (VT) with CPVT
Zack's motto:
"Making mistakes simply means you are learning faster"

*karenb*
Senior Member
Posts: 589
Joined: November 14th, 2005, 4:24 pm
Location: Warwickshire, UK

Re: looking for info on CPVT and ICD

Post by *karenb* » March 6th, 2010, 10:15 am

Betablockers are notorious for fatigue and a sense of exhaustion. It is not at all surprising that Josh feels so tired. The other factor is that often depression can manifest in other forms. My colleagues and students would never believe how depressed I have been while working. Some people are very good at hiding their true feelings - particularly the boys and men! I could not tolerate Nadolol - the treatment of choice for CPVT, which is one reason for my having an ICD. I hope things improve for Josh, you and your family.

Karen
CPVT
First ICD 2004.
Current ICD implanted 09/06

Post Reply