Friday, August 30, 2013

Surgery moved up


I mentioned in the last few posts Muriel's new full-time pulse rate "Event Monitor".   This device, which looks oddly like an ancient cell phone, tracks her heart rate continuously and sends alarms to a monitoring company if her rate falls below a set threshold.  The concern is that her average heart rate has fallen steadily for the last three months or so, and she is currently at risk for heart failure.

I also mentioned we recently had a Catheter Lab performed, to see if she is physically ready for the final plumbing surgery (the Fontan Procedure).  We have been told all along to expect the need for that surgery between 18 and 24 months, and that a drop in blood oxygen levels would be the trigger.  That has not happened.

We had the lab performed to see if she would be ready *early* for the Fontan, so we could install the pacemaker at the same time, and only open her chest one more time.

The results of the Catheter lab were positive - she is ready, and could actually have the Fontan at any time.  We went ahead and scheduled it for 9/26/13 - roughly 4 weeks from now.

Then her alarm started going off.  A lot.

This led to a handful of conversations with medical staff, and I made case to move her surgery up.  The basic idea is management of risk.  If the risk that she will have a monitor event that will cause us to have to drop everything and fly off to the hospital for immediate surgery is higher than the risk of performing the surgery sooner than 9/26/13, why wait?  The former risk is apparently reasonably high right now.  The latter risk is almost nil - there is no expectation that she will significantly grow in size in the few weeks difference.

The result is a new schedule date for Monday, 9/9/13.  We have an appointment with her new Cardiologist (Dr. Van Burgen, who happens to be the head of the Pediatric Surgical Heart Unit and very familiar with our case) next Thursday 9/5/13.  We are attempting to get her bloodwork drawn that same day, and arranging for a spot at the Ronald McDonald house next to the hospital for Sunday night 9/8/13.

We met with Dr. Ilbawi, her chief surgeon, this past Friday afternoon.  He is an amazing man with a talent for easing our anxiety, at least temporarily!  He went over the whole set of procedures again - her previous as well as upcoming.  Mostly we had heard all of it before, but its been a year.  The refresher was well received.  Her particular situation is rather unique, and there are some aspects of her condition that suggest she will have an easier time recovering from the operation than other children receiving the Fontan.  That would simply continue her trend of amazing everyone with her stamina and strength.

Its a bit unnerving to be at this point.  She looks and seems fine, but I can't help but be worried.  I haven't talked much in these blogs about my own state of mind, or Megan's, but stress levels right now are very high.  I'm having a lot of trouble concentrating on much else - kind of a constant anxiety.  Thanks to all who are wishing Muriel well and supporting us in this time.  I'm confident she is in good hands and all will be well.

Thursday, August 22, 2013

Next surgery dates

This isn't finalized yet, but Megan spoke with the nurse that is helping us schedule things, and it looks like the next surgery will be on Thursday, September 26th.  We will make it final tomorrow and I'll make another post.  The good news is the doctors in conference, given the results of the catheter lab and the monitoring that has been going on, DID NOT think she needed to come in immediately for the pacemaker.  They DID think that she is ready for the Fontan Procedure.  So this surgery will combine the two tasks - she will get the pacemaker and the Fontan at the same time, saving her from opening her chest twice.

This one will be more involved, and will take longer to recover from.  We will be in the hospital for up to ten days, and we have been warned that she will be miserable for another two weeks after getting home.  They do seem to over-warn us, though, and I will hang on to the idea that she will amaze everyone, as she often has, and bounce right back quickly.

More to come soon...

Muriel back to her old self

Super baby wins again.  I guess I should stop calling her baby - she is most certainly toddler stage now, walking, hitting her sisters, and driving too fast on the highway.  But I digress.

Back home as of late yesterday afternoon and other than the band-aid over her small puncture hole in her thigh and the open-to-air puncture hole in her neck, you would never know that 24 hours ago she was in a hospital bed.  I'll add some pictures to this post later (they are on mommy's phone) that show how well she handled her X-Ray, Ultrasound, and EKG yesterday morning.  She sure was happy to get out of that bed.  Makes us wonder how on earth she will handle being in bed for ten days after the next surgery.

All-in-all the results were good.  We should hear today or tomorrow about scheduling the next (hopefully final) surgery.  Will be sure to update everyone here as soon as I know.

j

Tuesday, August 20, 2013

Catheter Lab


Waiting to go into surgery

We left the house this morning at 8AM to make the rush hour drive to Hope Childrens' Hospital for Muriel's scheduled catheter lab.  Arrived just before 9AM and checked in, then sat in the waiting room.  A more pressing case was before ours and we didn't know how long we might have to wait.  Muriel had been up horribly crabby the night before and I had been up late working, so there was little sleep between the three of us, all dragging.

Around 10AM we were brought back to the Peds unit for vitals and prep talks with the Anesthesiologist and Dr. Patel, who would be performing the lab.  Poor Muriel hadn't eaten anything since 8PM the night before, and wasn't allowed to drink anything either (had a sip of water at 7AM and that was it).  She really wasn't in the best of moods.  Dr. Patel described that he would go into the vein in her neck and the artery in her thigh to reach her heart.  They would be taking pictures, injecting dye and taking more pictures, and measuring things like flow and the size of the pulmonary artery (this artery will be taking the blood flow from the lower half of the body, and must be deemed ready for that extra load).  He would also be looking for stray blood vessels that may have branched out from major vessels in and around the heart, which is apparently common in these cases, and would interfere with the coming Fontan procedure.  If found, he would actively block them with what he called a "coil".  We expected to go home the same afternoon unless active intervention was required, in which case we would spend the night.

Image from her lab showing the
"coil" closing off the discovered
extra blood vessel
At 11:15AM we took her to the door of the surgical area and let her be carried away.  She wasn't very happy about that either!  We were told the operation would take between 2 and 4 hours, and that someone would come to update us in the waiting area.  In the meantime, we had lunch.

At 12:30 the nurse came into the waiting room to say she was done!  Only an hour?  We weren't sure if that would mean good news or bad.  Dr. Patel came in shortly afterwards to explain that everything was fine, that she was fine and waking up, and that we would be able to see her soon.  He also explained (and showed pictures) that he *did* find a stray new blood vessel - two actually - and that he was able to close one off with a coil.  The other alluded him, and he said he would ask the surgeons to close it if they were able to see it during the Fontan Procedure, but that even if they missed it all would still be well, and she would simply have a slightly lower blood oxygen saturation than we would expect after the Fontan.  Given that she has done just fine with the quite low saturation levels between the Glenn and Fontan Procedures, this didn't seem like too much of a problem.  He mentioned he would be able to get it on her next Catheter lab, should one be needed in that case.

Chugging Pedialyte
Post-surgery
We eventually got to see her, and she was NOT a happy camper.  It took some time to calm her down, and she chugged several bottles of Pedialyte along the way.  They wanted to hold off on any food until we saw she was tolerating the water.  We stayed in the Peds unit for about an hour and a half before being transferred to the 2nd floor main hospital room.  She has mainly slept since then, on top of mommy in the recliner.  Various doctors and nurses have come in to check her vitals and set the monitors.  Fairly uneventful.
71bpm not bad... for a future marathon runner!
In recovery room

Eating dinner with mommy
Snacking with daddy!
She is now eating dinner with Mommy and in a better mood.  We will be here all night tonight.  In the morning there will be an EKG, an ultrasound, and an x-ray, and we should be able to leave before noon.  I'll send an update when we get home.














Cardiac Electrophysiology

Muriel met the latest addition to her Cardiac team on Friday.  Dr. Shetty met with us for over an hour and explained in great detail how the pacemaker works, what is it intended to do, and what we might expect maintenance-wise.  We got to see the device that will be implanted in Muriel's abdomen, just below her breastbone.  Its about the size of mini Altoid's tin, is hermetically sealed, and contains all the electronic goodness plus battery.

Muriel's condition, Heart Block, concerns signals passing from the upper (atrial) chambers to the lower (ventricle) chambers.  Her atrial rhythm is fine, and normal, if I understand correctly.  Somewhere around 110bpm.  Up until six months ago more than half of those beats were signaled to her one remaining ventricle, which would then beat as it should.  The others were simply lost, and her measured heart rate was low - typically in the 70-90bpm range.

The Holter Monitors that she has worn for her past two cardiologist appointments showed the average dropping, as well as the range.  Three months back her average was in the mid sixties, with a max reading in the nineties and minimum in the fifties.  That was concerning.  The last Holter, about a month ago, showed an average in the low fifties, with a max of only 64, and minimums in the forties.  That is what has prompted all this activity a few months earlier than expected.  Without the pacemaker she is at risk for total heart failure.

We still have a catheter lab scheduled for next Tuesday, which should let us know if it is too soon for her final plumbing operation (that would be very unfortunate).  In the meantime, and until the pacemaker surgery, we have been asked to outfit Muriel with an "Event Monitor", a cellular phone equipped device that will automatically send alerts to ECG monitoring technicians if her heart rate drops below a certain rate, or if we manually press a button to alert them.  Its kind of bulky and we are having challenges concealing it in her clothes without it falling out and dragging behind her!

Dr. Shetty is very nice and extremely knowledgeable, and we feel very comfortable in her care.  Fingers crossed we get the green light to do both operations at once, so this will be the last time we have to open her chest...

Pacemaker imminent

Megan, Muriel, and I saw Muriel's cardiologist Dr. Cuneo on Monday 8/12/13 for her quarterly checkup.
Since her Glenn operation just over a year ago we have had these checkups to keep track of her progress towards the final "plumbing" operation, the Fontan Procedure.  Along the way we discovered that Muriel is additionally suffering from Heart Block and will need to have a pacemaker installed - she will sport this bit of technology the rest of her life, or at least until she gets a replacement heart.  Given dad's faith in the accelerating pace of technology, perhaps outpatient surgery in her adult years!

The past two cardiologist appointments were prefaced by suiting up Muriel in a Holter Monitor for 24 hours, which records her heart rhythm (ECG) and stores it for review by the cardiology team.  A steadily declining 24 hour average heart rate indicated the pressing need for the pacemaker.  Now we need to determine if she is ready for the Fontan procedure, such that we can do both operations at once.  Ordinarily the Fontan would not be performed until at least 18 months, and she is just shy of 16 months now.  An exploratory catheter lab has been ordered, to be performed 8/20/13, which should answer that question (as well as evaluate her previous surgery's success and look for stray blood vessels that may have grown in the meantime that may need to be closed off).

We made an appointment to meet Dr. Shetty, a Cardiac Electrophysiologist, who will be managing her pacemaker installation and followup maintenance.  We meet her on Friday, 8/16/13.  She will play a role in the timing of the next surgery based on her evaluation of the Holter results and any further tests she may require.

A final note - we learned that Dr. Cuneo has accepted a position in Denver and will be leaving at the end of August, which is very sad for us.  Dr. Cuneo has taken the lead on Muriel's condition since several months before she was born, and we will miss her.  We don't know who will be taking over her regular cardiology appointments yet, but should be making that choice soon.

I haven't posted for a year, so here is a recent ob-pic of Muriel... pre-surgery :)