Out of Sorts

This past week Miss K has been a bit out of sorts.  I know, I know if she were a perfectly healthy baby I would not worry in the slightest, I'd probably blame it on teeth or "just a stage" but with her PJRT I am bound to worry about EVERY little thing she does differently.

Miss K has been quite irritable.  She wants to be held almost 24/7, and wont even allow us to dare to leave the room without her no matter who is left in the room with her.  Now when she cries over something it very quickly escalates into frantic screaming and breath holding.  When she drops or looses a toy she's playing with she gets upset and almost inconsolable.  I could go on and on but I'd be here all day so I'll just stop there, you get the point she's constantly crying.

And she has been wanting to sleep more and more, to the point of just laying down where ever she is and just dozing off...very unusual for my sweet baby girl who insists on nursing and being cuddled and rocked in order to fall to sleep.  If she does make it to a normal sleep time she lasts maybe 5 minutes nursing before she's out like a light and I could drop her on her head in her crib and she wouldn't even notice, this coming from a baby who normally requires that I stand up ever so carefully without much noticeable movement and lay her down so gently she doesn't even feel the difference between her bed and my arms.  One morning this week she got up at her usual 8:30 time and nursed, but then fell asleep nursing and was out like a light again so I lay her back in her crib and left and she slept for another 45 minutes or so before she was up for the day, this is also not normal for her once she's up in the morning she's up for good until morning nap 2-3 hours later.

Getting her to sleep is the easy part, the only easy part in fact.  But once she's asleep I can't guarantee she'll stay that way for long, especially at night.  She has been waking at least twice in the night screaming frantically, Daddy or I have to dash into her room as quickly as our sleep befuddled brains will allow and pick her up before she gets too worked up and then we rock..and rock...and rock sometimes it takes 10 minutes and sometimes it takes 30 + minutes before she's out enough to be laid down again.  And naps are difficult as well.  She'll sleep maybe 30 minutes and wake up crying, she normally sleeps 2-3 hours each nap.  I know, this paragraph makes absolutely no sense after the last one about "wanting to sleep" right?  Go figure.  She is exhausted pretty much the whole time she is awake but doesn't sleep long when we put her down.

It has been a struggle getting her to eat her solid foods this week as well, a lot of gagging and choking on it, she acts as though it hurts to swallow or something.  I know you're probably thinking "well maybe you're feeding her something she just doesn't like", I can assure you this is not the case.  In trying to keep her happy I've only been offering her the foods she loves and gets excited about and she does get all excited about it at the first taste but then gags and chokes on the rest of it.

Last night topped everything though when out of the blue, seemingly for no reason at all, Miss K started screaming.  I was getting her stripped down for her bath and she got very upset.  Bath time is her most favorite time of the day...usually.  But last night she screamed through her bath, arching her back and kicking her feet and refusing to sit up at all.  Any time I tried to have her sit she'd arch her back and flip out, ending up laying on her back and screaming harder.  I struggled to get her bathed, lotioned and into her jammies as she flopped around like a fish on land and screamed at me.  It was so dramatic I was in tears myself and had started thinking we may end up taking her to the ER if we couldn't get her to calm down.  When she was ready for bed she finally quit screaming and just wanted to snuggle me, cuddling in closer than she ever has before.  I gave her some Tylenol and Gripe Water hoping one or the other, possibly both, would calm her for the night and help her sleep.  She woke up 2 hours later screaming and it took Daddy 30 minutes to get her back to sleep in her crib.  And then she was awake screaming at 6:45 this morning, 2 hours before she usually wakes up, it took me 10 minutes to get her to calm down enough to nurse.

My "mommy instincts" tell me something is wrong but I can't even begin to guess what that "something" is.  I'm SO relieved her next Cardiology appointment is only 5 days away.  I'm hoping they'll listen to me and check every possibility rather than give me the usual "ask your pediatrician" comment that I tend to get when I voice any concerns they feel may not be heart related.  There are so many possibilities with Miss K.  Her medications could be showing some side effects or she could be going in and out of SVT persistently without us knowing it, or it could be something completely un-heart related...I guess we'll find out soon, until then I'm going to hope she starts to act more normal and be more like her happy little self soon.


Breakthrough SVT...again...and again.

Now the tally is 4 episodes...that's right 4!  I didn't post about the last one because it seemed insignificant to me at the time, it was last week either Wednesday or Thursday, I can't remember which.  She was in and out of SVT so fast I didn't really have even a second to panic.  I had just checked her heart rate for the night and it was perfectly fine.  I gave her her evening Propanolol, she choked on it a bit, I checked her heart rate again, it was rapidly beating around 240 BPM or so, I blew in her face, checked her heart again, and she was back to a steady 107 or so.  It was that fast.  But now I'm adding it in our "Breakthrough Tally."

Last night she had her 4th episode in 2 weeks :o(.  That's more than she's had in such a short period of time since she was first released from the hospital.  I had worried about her all evening since about 5:30 when she fell asleep in my dad's arms at a family party, one second she was awake and giving everyone snuggles and the next second she was just out cold, no amount of jiggling and talking could wake her.  We put her in the car seat to head for home.  I checked her heart rate just in case, since it's not normal for her to fall asleep in anyone but Mommy's arms and especially not so easily or so soundly.  She was fine, beating slow and steady.  We got home around 7:30pm.  We let the kids relax and be happy outside for a bit then we started bedtime routine.  Miss K was a little restless through her feeding, etc. but I attributed it to a messy diaper.  After her bath I checked her heart rate, I was quite surprised to hear it racing I really didn't expect it.  I had to blow in her face 3 times before it slowed.  The whole time Daddy was saying "Are you sure she's in SVT?  There's just no way you're right.  Look at her, she's fine!  How can she be in SVT looking so good and smiling and giggling at us?"  Yup, that's Miss K for you!  She doesn't show signs she's in it, other than seeming to be a little more restless and wiggly than normal, which she was last night but like I said she also had a messy diaper and those drive her absolutely nuts.  We have no idea what put her in SVT, it was quite obviously a sporadic spike in her heart rate without a cause and like I mentioned in my last post about an episode those are the worse breakthrough episodes.

I'm still sure the missed Propanolol dose on Mother's Day was the cause of the first 2 episodes she had.  Now I'm also pretty sure she wouldn't have had so much of an issue without that dose if she was on a high enough dose.  I think she's due for another dosage adjustment.  She has her 10 month Cardiology check up a week from today so I'm not going to call them, the last time I called so close to an appointment they advised us to stick to our appointment date and they had her EP Cardiologist adjust the Propanolol dose over the phone.  We mine as well wait it out this time, I don't feel it's worth panicking over when we can get her out of the SVT at home on our own and when she's acting so normal when she's in it.

All these months we've stayed consistently hopeful that she would grow out of her PJRT.  We kept telling ourselves "she's only ___ months old it can still happen we have plenty of time" but now that she's 2 months shy of 1 year old we're not so confident.  Her EP Cardiologist warned us in the beginning that if she didn't out grow it by 1 year then she wasn't going to outgrow it.  She's proving to us that her little heart is going to be extremely stubborn and refuse to work correctly without medication and future surgery :o(.


2 Breakthrough Episodes in 2 Days?!?

Last night Miss K had another SVT Episode.  We have absolutely NO idea why, nothing happened to set it off :o(.  Sporadic episodes are WAY worse than episodes brought on by something.  It's not good when we can't explain why she's in SVT.

How did I know to check on her heart last night?  She was acting kind of out of sorts, still wiggly and very active and happy but a little clingy and acting restless.  I picked her up and put my ear to her chest and just knew.  We tried to count the beats with a stethoscope but it's nearly impossible to keep count with a wiggly baby.  Just like last time I'm guessing her heart was buzzing at about 20 beats per every 5 seconds, calculating to about 240 BPM.

It only took a second to get Miss K out of SVT this time, the first physical maneuver I tried was blowing in her face and it only took one time.  When we checked her heart again it was beating slow and steady again.

I'm torn, I don't know if I should call her EP Cardiologist or not.  Two episodes in two days isn't a good thing, especially with this last one being unexplainable.  I can't decide because we know exactly why she went into SVT earlier this week, and we did accidentally skip a Propanolol dose on Sunday so I feel it's mainly based on the missing dose.  I don't know if it takes a little while for the body to get back on track after missing a dose or if these episodes may be a sign that we need another medication adjustment...So do I call today and let the EP Cardiologist know?  Or do I wait and see if she has anymore unexplained SVT?

It's times like this that it sucks going through this for the first time ever, sometimes I just wish I had experience with it already and always knew exactly what to do and when/how to do it.


Breakthrough SVT Epsiode

I think I may need to make a chart and place it in the side bar of this blog to keep track of Miss K's SVT episodes.  I always hope we're done with them when it's been a bit since the last one, but apparently we're in this for the long haul :o(.

Miss K had another SVT episode last night.  This one was brought on by fright, seriously she got startled and scared badly enough to cause SVT :o(.  I'd like to blame her Daddy but I just can't, he didn't know any better, and I'm sure he learned a valuable lesson from it for the future.

Daddy likes to vaccum, yes that's right DADDY likes to vaccum lol.  Last night he decided to vaccum the carpets.  Apparently, and we hadn't noticed this until last night, he has never vaccumed with Miss K sitting in the same room ever before.  Somehow he has managed to do any vaccuming when Miss K is with me either out running errands or in her room nursing.  Daddy decided he better warn Miss K that the vaccum was starting so he WOULDN'T scare her, it wasn't a good idea.  She was sitting quite near to the vaccum when Daddy said in an urgent tone "(Miss K!)" just trying to get her attention because she was very involved in playing with a toy, then "(Miss K!) Daddy's going to vaccum K?  I'm turning it on K?" and then he turned the vaccum on...Miss K jumped bigger than we've ever seen her jump, she flailed her arms frantically and held her breath then she started screaming frantically like someone (or something) had caused her great pain.  I rushed over and picked her up to soothe her and she was instantly fine.  She is not afraid of the vaccum, usually.  I run the vaccum all the time with Miss K right there on the floor and she doesn't even notice most of the time, but that urgent tone of voice her Daddy used to catch her attention set the tone that what was going happen was going to be frightening.  I listened to her heart with my ear instead of getting a stethoscope and it sounded fine to me so I didn't go get the stethoscope out to double check.  I should have though.  We went through normal bedtime routine of feeding her cereal and getting the tub ready when I felt I should check her heart again, this was almost an hour after her scary vaccum encounter.  Miss K was in SVT, I couldn't count it though because she was so wiggly and active I was lucky to have 10 seconds at the most with the stethoscope on her little chest, if I had to make an educated guess I'd say her heart was beating at about 20 beats for every 5 seconds, that's about double her usual 10-12 beats in 5 seconds.  I did some math and as inaccurate as it is I worked some numbers and I'm figuring she was beating around 240 BPM.  I first tried tipping her upside down, this did not work so I tried blowing in her face, another difficult feat with a wiggly and active baby.  After 2 attempts at blowing in her face (and I was sure there was no way it had worked) we check her heart rate again to find it beating a nice slow and steady pace again.

I'm disappointed.  Miss K's Cardiologist said he'd only consider taking her off the Amiodarone on her birthday IF she made it from her 8 month check up until her 12 month check up completely SVT free :o(.  I was hoping to get to cancel her 10 month check up since the Cardiologist suggested we skip it if we felt she was doing great, but I feel if she's still having episodes she needs to visit him and make sure everything is still OK.

Watch for a new chart on the sidebar if I can figure out how to make one...


The Strange Life of Amiodarone

I think I really need to stop stalking the internet for information about SVT and medications...seriously, lately I'm finding more and more information that literally scares me to death.  My most recent finding is an article about Amiodarone.  I found it while trying to figure out what a skin rash caused by Amiodarone looks like.  As I've posted before Miss K has a chronic case of Eczema but a few weeks ago she had a breakout on her chin that turned into an infection.  After a visit to the Pediatrician I was not too concerned about it, especially after it cleared up within days with an antibiotic cream and Hydrocortizone.  This week she developed the same rash on her right elbow and it is now showing up on her left forearm and elbow as well.  I immediately started applying Hydrocortizone to the areas but it still has ended up looking like her chin did so I've started applying the antibiotic as well.  I'm not sure if I need to be concerned or not, I'm guessing it may be the sunscreen we are using that could be causing these rashes so I have stopped using it and switched to another and I hope it works.  Otherwise I am afraid she's starting to have a reaction to the Amiodarone, but in the back of my mind I can't help but think that the rash wouldn't go away with an antibiotic cream if that were the case, or would it????  So today I started researching again and found this article, there wasn't anything helpful about skin rashes but it was enough to terrify me about other side effects, seriously what am I giving my poor little girl?

Source:  http://heartdisease.about.com/library/weekly/mcurrent.htm

The strange life of amiodarone
By DrRich
Amiodarone is the most effective, and certainly the strangest, antiarrhythmic drug ever developed.  (Antiarrhythmic drugs are used to treat heart rhythm disturbances.  Click here for a quick review of heart rhythm disturbances.)  Anyone being treated with amiodarone should understand the idiosyncrasies - and the risks - associated with this highly effective drug.
The strange history of amiodarone in the U.S.
Amiodarone was developed in Belgium in the 1960s as a drug for treating angina, and was quickly released for marketing in most countries except the United States.  Doctors noticed that, in their patients placed on amiodarone, heart arrhythmias greatly diminished.  Clinicians quickly began using the drug to treat cardiac arrhythmias of all sorts.  Within a few years, word began filtering into the United States - amiodarone was a unique antiarrhythmic drug that was said to "always work, and had no side effects."  Both of these assertions, of course, proved false.
In the late 1970s, American doctors began obtaining amiodarone from Canada and Europe to use in their patients with life-threatening arrhythmias who did not respond to any other drugs.  The FDA sanctioned this activity on a limited basis, but the drug seemed so effective that literally hundreds of American electrophysiologists were soon obtaining the drug (one way or another) and by the mid 1980's tens of thousands of Americans were receiving the drug.  The Americans, as a group, studied the effects of amiodarone somewhat more rigorously than their overseas colleages, and here's what they found: Amiodarone was indeed far more effective at suppressing arrhythmias than any other drug they had ever seen, but it produces a bizarre series of side effects that doctors around the world seemed to have "missed."  By the mid 1980s, the FDA was essentially forced to release amiodarone for marketing in the U.S. - the foreign manufacturers of the drug threatened to cut off the American supply (having supplied free drug to thousands and thousands of Americans for more than 5 years,) and the American doctors impressed on the FDA what a medical disaster that would produce.  So, unlike any other drug in modern history, amiodarone became FDA approved without rigorous, FDA-approved randomized clinical trials.  The true breadth of amiodarone-induced side effects took more than a decade to uncover.
Why amiodarone is a strange drug
Amiodarone has several characteristics that make it unique. 
First, the drug takes weeks to achieve its maximum effectiveness.  This is because amiodarone is stored in most of the tissues of the body, and to "load" the body with the drug, all the tissues need to be saturated.  The typical "loading" regimen of amiodarone, therefore, is to use very large doses for a week or two, then taper the dosage over the next month or so.  It is not unusual to give patients 1200 or 1600 mg per day at first, and then maintain them on as little as 100 or 200 mg per day chronically. 
Second, amiodarone leaves the body very, very slowly.  It is not excreted (like most drugs) by the liver or the kidneys.  It is lost when amiodarone-containing human cells are lost - such as skin cells or cells from the GI tract, which are shed by the millions each day.  Thus, if it is decided that one needs to stop amiodarone, the drug remains in the body in measurable quantities for months and months.  The "half life" of the drug, in contrast to most other drugs, is measured in weeks instead of hours.
Third, because amiodarone is stored in many different kinds of tissues, it can produce side effects affecting many different organs.  Some of these side effects take months or years to develop, so it is never true that one can stop being vigilant. 
Fourth, amiodarone works through many different mechanisms, unlike most drugs.  It fits into two separate categories of antiarrhythmic drugs (Class I and Class III, for what it's worth); it acts as a beta blocker; it acts as a calcium blocker; it acts to dilate blood vessels; and it often acts to "block" the effect of thyroid horomone.
The strange side effects of amiodarone
One reason the side effects of amiodarone were not spotted for years was that they often take weeks or months to develop (unlike side effects from typical antiarrhythmic drugs, that usually appear within days).  Also, the kinds of side effects produced by amiodarone are not the kind that heart doctors typically expect with antiarrhythmic drugs. In any case, it took more than a decade for many European doctors to admit (even after the American medical literature became saturated with articles) that they had been missing some rather remarkable side effects.
Amiodarone commonly causes deposits to form on the cornea of the eyes - in fact, this occurs in virtually every one taking the drug.  These deposits often cause no visual disturbances, but not infrequently patients complain of "halo-vision," where looking at bright lights at night is like looking at the moon on a foggy evening.
Amiodarone can cause a very disfiguring blue-grey discoloration of the skin, generally in areas of sun exposure, and that gradually worsens over a period of years.  It is not clear that this "smurf syndrome" clears up when the drug is stopped.  In younger patients this side effect can be devastating.
Amiodarone often sensitizes the skin to sunlight, so that even trivial exposure can cause a fairly nasty sunburn.  People taking amiodarone must often cover the body completely when going out during the summer, especially in hot, humid climates.
Each amiodarone molecule contains four iodine atoms - so a typical dose of amiodarone provides far more iodine to the body than is needed.  It is thought to be the iodine that mediates the thyroid side effects of amiodarone.  The more common of these is hypothyroidism - low thyroid.  This, fortunately, is relatively easy to treat with thyroid medication.  But some patients develop hyperthyroidism - high thyroid - and this can be a real problem and a real challenge to treat. (They hyperthyroidism often does not respond to the "typical" treatment used for this problem.)
Amidoarone can cause liver toxicity, so liver enzymes need to be monitored periodically.
The most serious side effect of amiodarone is pulmonary toxicity - lung disease.  This comes in two flavors.  Amiodarone can produce an acute pulmonary syndrome that looks and acts just like typical pneumonia - sudden onset of cough and shortness of breath.  This condition usually improves rapidly once amiodarone is stopped.  It can occur within days of beginning amiodarone.  The second flavor is more insidious - it is a gradual, unnoticeable, "stiffening" of the lungs that both the doctor and patient can overlook until finally severe, probably irreversible lung damage is done.  This problem can occur years after the drug is begun, or as early as a few months after beginning amiodarone.
When should amiodarone be used?
If Satan were going to develop an antiarrhythmic drug, he would make one that suppressed arrhythmias very well (so people would want to use it,) had none of the typical side effects, but that had a host of atypical, relatively subtle, but ultimately dangerous side effects that doctors were likely to overlook.  He might not be satisfied with amiodarone, but would likely consider it a pretty good first approximation.
Amiodarone should be used for arrhythmias that are life-threatening or that are very disruptive to one's life, and for which there are no other reasonable therapies. Despite its drawbacks the drug has helped tens of thousands of patients, and has restored them to a nearly normal life.  When used appropriately, amiodarone can be a major benefit.
But because of the potential toxicity its use should be limited.  No doctor should blithely prescribe the drug, but should do so with reluctance, realizing that he/she is exposing the patient to long-term risks.  The doctor should, by prescribing the drug, be committing him/herself to being a long-term partner of the patient. He/she should carefully coach the patient on what problems to look for, and together they should be ever vigilant for the side effects of the drug. 


Missed Dose...

Today, for the first time ever, I skipped a Propanolol dose :o(.  I feel like a horrible Mommy, I know it's because it's Mother's Day and my mind was more on myself and what I wanted to happen than on what needed to happen along with my plans...yes I believe I've been a bit selfish today and I don't feel bad about it except that I totally forgot to give Miss K her Propanolol this afternoon because I was in a hurry to get out the door following through with one of my plans to make my day awesome.  I remembered at 6:30 tonight when we got home from a beautiful canyon drive and I sat down to nurse her, that's when I always remember to give her medications because they're sitting right by the nursing chair.  It's considered a skipped dose because you cannot give doses closer than 6 hours and even though she doesn't take it every 8 hours on the dot anymore she's still supposed to take it at the same times every day...she takes it at 8:00am, 2:00pm and 9:30pm daily, this means at 6:30pm this evening we were only 3 hours from her usual final dose of the day :o(.  Yup, I feel like a horrible Mommy.

Now I'm going to stress for the next couple days about Miss K having break through SVT episodes, and I know if she has any it'll be because of the missed dose today, then I'll feel even more horrible :o(.

Though I do have to say I really don't think she'll have any issues missing this dose, it's only 1 she should be fine.  I am going to hope and pray that she really is fine...