Re: pacemaking, etc. _Joanne

[Guest guest]

Anne,

I can see why you are worried. I worry about the same things you do. Caden has had pretty bad bradycardia. 50 being the lowest. This went on for over a month once. His heart would beat so slow and I'd be screaming BEAT by the time the next beat came! We used a portable hand held device called an Event Recorder. You place it to the chest when you see there are symptoms or know there is an abnormal rate. Everyone agreed that his heart was too low and constantly in a sinus arrhythmia! and just said, I don't think it'll stop! Back then, I was so inexperienced with the heart issues, actually still am not exactly that knowledgeable on the subject. Caden also wore a holter monitor which showed the exact same thing. Then, his heart looked really well but maybe a slightly enalrged LV but not sure if it was at this point or not.. However, it LOOKED to good to sound like it did, so noone worried over it! Thank God, that it got better. Oh, another thing, he could run and become completely exhausted in just a bit with his hrt rate only reaching maybe 75 at the highest. I was scared to death.

Just recently he done the holter and he still has a sinus arrhythmia and hrt rate ranged from 63 to 211. If you ask me 211 is too high. I know he's more active outdoors than he is inside. What is going to when outside or at school, playing? He had some PACS ( premature atrial contractions) and a lot of sinus arrhythmias. His average tachycardia was over 13hrs. But his day time average was not as high as Carsen's. It ways 107 but that's considering the sleeping heart rates as well. I think they should break it down to awake hrt rate average and sleeping heart rate average. Instead it's all grouped together.

I haven't seen Caden's heart 'act up' like that or about 2.5-3 yrs. Still, that doesn't keep me from checking him multiple times during the night. Caden's LV and aortic root is enlarged and I think that's it..

My little trouble maker Carsen has a few more complications. His heart is getting thick (hypertrophy) in the LV. The LV is also enlarged. He has just recently developed a aortic insufficiency. He has been on propranolol ( beta blocker ) since 20mths. He's now 22 mths. I just threw away the first big empty bottle and stared at it forever before I did. I can't believe he's taken SO much! Still, he's tachycardic and very worrisome. His average is still 130 on 36mg of the propranolol. If he misses a dose at bed time (which we've only done a handful of times) his heart rate is 140-160 by morning!

Every once in a while, it will get down to 90 asleep. It went to 87 once on his holter and that was a sinus arrhythmia. He also had the PACS. He was tachycardic for 24:41:36 hours during the holter. His EKG actually looks much worse than his echo.

I know of a couple of times, that his heart rate has not responded when active or upset. Last time it was only around 113 when screaming with what we presumed was belly pain but still not sure what happened that night. I would have expected it to be at lest 160. I think the propranolol blocks adrenaline somewhat, so I'm not sure if the heart not responding is a direct effect from that or not.

I need to sit down and write out my questions this weekend because we see cardiology Monday and I want some answers. She says his heart is going to wear out before the rest of him does if we don't get the rate down. Kind of blunt but I appreciate her honesty. Have either of your boys used propranolol before? Was there a reason for going with the labetaol that Zach is on? Does Zach say it makes him feel funny or anything? I don't think I see any side effects from the propranolol but then again Carsen is only almost 2 and cant' tell me how he feels.

If you learn anything new at all about this, please pass it on. It's one of my highest concerns right now but try not to stress too much over it.

Krystena sMom to:Caden 6.9 yrs MADD/GA2 variantCarsen 22 mths MADD/GA2 varianthttp://www2.caringbridge.org/ky/cadencarsen/ Warren 10 yrs Unaffected

"Hope is the companion of power and the mother of success, For those of us who hope strongest, have within us the gift of miracles." ---S. Bremer

pacemaking, etc. _Joanne

Joanne:

what was the situation that led to a pacemaker for . This has been discussed as a potential intervention for Z - not now but perhaps if things get worse. His HR fluctuates between 65 and 130 at rest. With activity it does not get any higher and in fact often just stays in the 90's - this of course is not perfect that he has no response. There are days when he is very exhausted and those days his HR will jump 30 beats just from laying down to a standing position - without even walking. I am sure this is all just signs of his dysautonomia worsening. That his HR can get that high at rest when he is on a beta blocker continuous infusion (labetalol) is odd in and of itself.

I was just shocked that can maintain a heart rate of 12, 20, etc like you described and not need intervention. if I understand correctly her pace maker is not working so does she self correct or what do you do to get her heart rate to go back up. The night Sam died his heart rate dropped - something he had not done since infancy. I have always set our monitor limits very conservatively - 60 for the low HR. So as soon as it alarmed - for a rate of 58 - I actually called the paramedics. I have never called 911 and a HR of 50 is not bad but when I looked at him I just knew something was very wrong. Things went downhill and he had passed away 1 hour and 30 min later. It was too fast. I guess that is why the HRs you mentioned seem so alarming to me - and yet it sounds like the MDs are not sure if it is real? is that what the issue is?

I wonder if a pacemaker would have helped Sam. of course we had no idea he would need one - he just never never never had bradycardia. But now i think about Z....I don't think there are good indications of needing to be paced but his variability is beginning to worry me. He has had 48 hour holters done and they show "exaggerated sinus arrhythmia" . I used to find comfort in the sinus rhythm but not sure I do now given Sam had sinus rhythm right before he progressed into asystole. What is this other monitor you talk about....it is worn for 30 days?

We need to have a more thorough way to assess Z's cardiac physiology and the annual echoes don't seem to be the way to go. Sam needed 4 cardiac drips - his normal cvp was between 15 and 18 (way too high) and yet his echo showed a "perfect heart". Then why could he not even walk without developing a gallop and why did he need all those meds in order for his heart to compensate? Everyone agreed he had heart failure and it needed to be treated but no one seems to be able to say what was wrong....there must be a better way to assess hearts than this.

AnnePlease contact mito-owner with any problems or questions.

[Guest guest]

original working diagnosis is/was Congenital Central

Hypoventilation Syndrome (CCHS). CCHS is a disorder of the central

nervous system in which the autonomic control of breathing and heart

rate and rhythm is absent or significantly impaired. A CCHS child's

respiratory and cardiac responses to low blood oxygen saturation

(hypoxia) or to CO2 retention (hypercapnia) are typically sluggish

during awake hours and absent (to varying degrees) during sleep and

serious illness or stress. All of her sleep studies at CHW initially

showed multiple prolonged episodes of heart rates under 30 with drops

in O2 sat into the 30, 40 ans 50's. With vigorous stimulation to

arouse her, the rates would come back up and stay up until she went

into quiet sleep again. With well over 3000 such events, Cardiology

did NOT want to intervene. The electrophysiologist felt (and I

quote), " Kids like this just aren't worth the risk. " Our Intensivist

fought for several months and eventually succeeded in convincing

Cardiology that for this child to survive she would need to be cardiac

paced. Note: Approximately 20% of the children with CCHS have cardiac

pacers for the same reason. 's first pacemaker was placed when

she was 3 years old. Her generator was replaced when she was 10.

Starting the weekend of Halloween, our homecare nurses and I started

to respond to bardycardia alarms during the night, something we have

not had to do since the pacer generator was replaced. Simultaneous to

this, started to complain that she was feeling her heart stop

beating and/or a shock feeling with certain positions. Since then we

have done 96 hours of Holter monitoring, had her cr monitor memory

downloaded and have now done 3 MONTHS of event recording. Our

Intensivist read the monitor download and firmly believes the events

are real and the pacer wires are not functioning correctly. The Peds

Cardiologist, though, keeps insisting it has to be artifact (even

though the tracings look flat line for several seconds). The event

recording strips match the monitor download. IS symptomatic.

When her heart rate is that low, she absolutely does NOT respond. The

days after the " events " she complains of a headache, asks for extra

time on her vent or extra oxygen and, often, falls asleep in the

afternoon for 2-3 hours (not her normal). Peds Cardiology keeps

saying if there was a problem, she would pace and she's NOT pacing.

Our Intensivist and I keep insisting that she will only pace if the

generator receives the signals that there is a problem.

Out of sheer frustration, and with the blessing of our Intensivist, I

set up a consultation appointment with the Peds Cardiology group at

Cleveland Clinic several weeks ago. openly told both

cardiologists that saw her (the first one called in an

electrophysiologist) that she feels like she is going to die because

her heart " keeps not beating " . These cardiologists reviewed the event

recordings and the monitor download and interrogated her pacemaker.

The upper wire is frayed and not functional at all. The lower wire is

questionable. They reprogrammed the pacer and they are hoping that

they have it regulated so the pacer will sense the drops and pace. We

are still getting some alarms, but not as many. If they continue,

they will try a different evaluation approach with a plan to replace

the system sometime soon. They have concerns about surgical risk

given 's ventilatory status and her bleeding/coagulation

issues, but they are committed to doing the replacement if the drops

continue. In this situation, they have to remove and replace the

faulty wires which is difficult and labor intensive without any of the

added medical issues.

The other cardiac issues we are experiencing in both lies and

have to do with tachycardia and questionable failure. Their

echos are read as normal or unchanged, yet they have the

signs/symptoms/physical evaluation findings of patients with mild

failure, per Drs. Whiteman and Cohen. So they do NOT has this

" official " diagnosis, but are being treated, medically as such.

Fortunately, out Intensivists are willing to treat symptomatically. I

am so frustrated with PEds Cardiology at our managing institution, I

could spit fire. We had a Cardiologist that the girls and I trusted

fully who was very knowledgeable of mito. He passed away quite

unexpectedly several years ago and the level of Cardiac care since

then has been sorely lacking. The managing docs get a consult note

that says the entire exam, listing all of the normal areas evaluated,

but in actuality, the only thing actually done is clinic is placement

of a stethoscope in one area of the chest for less than 5 seconds.

The docs at CCF actually both did a comprehensive exam! The other

constantly apparent problems are intermittent tachycardia and air

hunger. They come on with minimal activity and last quite a while.

We have been treating both with rest and supplemental oxygen. On one

of the Holters, had a resting heart rate of 160-180, lies

had hours of heart rates over 140. The Intensivists are concerned and

said they will monitor it. The Peds Cardiologist says, it is

completely normal, that he is sure the girls were playing actively

(Yeah, right. Not my girls!). With 4 minutes of minimal to moderate

activity their lactate levels are over 90...on multiple, repeat

studies. This is almost a bigger concern to our Intensivists, Dr.

Whiteman and Dr. Cohen. The problem is, no one has any idea how to

treat it. The Intensivist are wondering if the high lactates are

contributing to the tachycardia, air hunger, headaches and increasing

fatigue.

Unfortunately, I have NO answers, only similar concerns. If it

wasn't for the help of dedicated, very bright Intensivists, my girls

cardiac issues would not be addressed. In the interim, we evaluate

them frequently, give all of their prescribed medications (regular and

PRN) and document, document, document. When the monitor sounds for

bradycardia (rates below 40) we stimulate until we get the

situation turned around. This has been the cause of multiple ER and

clinic visits. I just hope and pray that either the system will start

to work better then it has (it is somewhat better since CCF

reprogrammed it) and if the alarms continue that will respond

to stimulation until the docs figure out how to repair or replace

the system. I know I am not alone. A few of the other CCHS

children's parents have had similar situations. In each case, the

system was ultimately replaced but it took months of documentation and

verbal complaints from the affected children.

Anne: Do you know of any other children with a Plasminogen Activator

Inhibitor Deficiency? If so, how is it being treated? I'm not sure

which is more disconcerting, the cardiac issues or the active bleeding.

I wish there were easy answers to aome of this, but I haven't

discovered them. I just feel fortunate to have a case managing

physician who cares and follows through when other drop the ball or

don't seem to care.

[Guest guest]

Krystena:

Zach was tried on atenalol at some point but that was last year and I

think it was quite brief. We switched to labetalol because his heart

failure was not improving with the atenalol. Between labetalol and

Lasix he seems to compensate adequately if he is not too active.

Activity raises his Blood pressure and that in turn seems to lead to

a heart gallop (literally sounds like a horse is galloping),

shortness of breath and a dropping sodium level. All of those are

signs of decompensated heart failure.

the labetalol has been good although I am not sure it would be

adequate without the lasix. The good thing about labetalol is that

it is made in an intravenous form. Zach has recently switched to a

continuous IV infusion of labetalol that runs 24 hrs/day. It is good

because it eliminates the peaks and valleys that he had when he was

getting it 4x/day.

Sam's heart failure needed more help. His was treated with 2

diuretics: lasix and chlorothiazide (diurel), continuous infusions of

labetalol and milrinone and then 2 other infusions to keep his BP

down because the highs always led to worsening heart function. It was

only with all of those things that we finally eliminated the heart

rates of 150-180 and persistent gallop that plagued him for months

before that. He still had periods of heart decompensation and his

heart was very very fragile but the drugs made a big difference and I

believe they added years to his life.

I hope your appt goes well.

Anne J.